THE ACHD POPULATION
~1.4 M
Approximately 1.4 million adults now live with congenital heart disease in the United States — exceeding the number of children with CHD for the first time in history.[1]
What Is It
Adult congenital heart disease at end of life — an extraordinary surgical survival story reaching its genuine physiological limit. What the hospice team needs to understand on day one.
ACHD SPECIALIST GAP
~375:1
Patient-to-specialist ratio. Approximately 800 formally trained ACHD cardiologists serve 300,000 adults with moderate-to-complex CHD — a crisis-level workforce gap.[2]
SUDDEN CARDIAC DEATH
25–30%
Proportion of ACHD deaths attributable to sudden cardiac death — ventricular tachycardia, ventricular fibrillation, and sudden arrhythmic events in a population with surgical scar substrates.[3]
SURGICAL SURVIVAL ERA
85%+
More than 85% of individuals born with CHD now survive to adulthood — the surgical revolution transformed a uniformly pediatric disease into a predominantly adult disease.[1]
Congenital heart disease (CHD) is the most common birth defect, affecting approximately 0.8–1% of all live births — roughly 40,000 births per year in the United States. Historically, the majority of infants born with moderate-to-complex CHD did not survive to adulthood. The surgical revolution that began in the mid-twentieth century — the Blalock-Taussig shunt (1944), the first open-heart surgery with cardiopulmonary bypass (1953), the Mustard procedure for transposition of the great arteries (1964), the Fontan procedure for single-ventricle physiology (1971), and the arterial switch operation for TGA (1975) — transformed CHD from a disease of childhood death into a disease of adult survival.[1] These pioneering procedures were so novel that no data existed about how the repairs would age over decades, whether prosthetic patches and conduits would last, or whether ventricles operating under non-physiologic loads would eventually fail.[4]
The result is a new and rapidly growing clinical population: approximately 1.4 million adults with CHD in the United States, of whom roughly 300,000 have moderate-to-complex lesions requiring specialized ACHD care. This population grows at approximately 5% per year as surgical cohorts continue aging into adulthood.[2] These adults are now in their 30s, 40s, 50s, and 60s — living inside the long-term consequences of repairs that were designed to extend childhood survival rather than ensure lifetime function. The right ventricle that has been volume-overloaded for 40 years after tetralogy of Fallot repair is failing. The systemic right ventricle of the Mustard or Senning operation was never designed for systemic afterload and is progressively dilating. The Fontan circulation is producing hepatic cirrhosis, protein-losing enteropathy, and plastic bronchitis. The surgical scars are generating lethal ventricular tachycardia substrates.[3][5]
The provider gap is the defining crisis of ACHD care. There are approximately 800 formally trained ACHD cardiologists in the United States — a ratio of roughly 375 complex ACHD patients to every specialist. The majority of adults with complex CHD are managed by general adult cardiologists who may have limited knowledge of their specific anatomy and physiology.[2] This provider gap is directly and critically relevant to end-of-life care: the hospice team that encounters an ACHD patient must actively seek ACHD specialist input because the clinical decisions in ACHD are anatomy-specific, repair-specific, and physiology-specific — they cannot be generalized from standard cardiology frameworks.[6]
🧭 Clinical framing
Every clinical decision in ACHD requires specific knowledge of the specific lesion, the specific repair, and the specific physiology. The Fontan patient is not a standard heart failure patient. The Eisenmenger patient is not a standard pulmonary hypertension patient. The repaired tetralogy of Fallot patient is not a standard right heart failure patient. General cardiology frameworks do not apply here — ACHD frameworks do. The single most important act at hospice enrollment is contacting the ACHD specialist who knows this patient's anatomy.
"When you walk into an ACHD home, you walk into a story of extraordinary medical survival that has reached its genuine physiological limit. This person has outlived every prognosis ever given to them. The repair done at age 4 was the best surgery available then — it was not designed to last 45 years, because nobody knew if they'd need it to. Now they're 49 and the repair is failing in ways most adult cardiologists have never managed. Pick up the phone and call the ACHD specialist before you do anything else."
— Waldo, NP · Terminal2
How It's Diagnosed
ACHD diagnosis is established before hospice enrollment. This section helps you read the anatomy, understand the repair, and interpret the specific physiology that defines this patient's end-stage trajectory.
ACHD Anatomy and Repair History
The single most important clinical document in ACHD is the operative history. At enrollment, document:
- Primary cardiac diagnosis: The specific congenital lesion (e.g., tetralogy of Fallot, d-TGA, single ventricle, AVSD, Ebstein anomaly, truncus arteriosus)[1]
- Surgical and catheter-based interventions: All procedures with dates and institutions — include shunt type (Blalock-Taussig, Sano, Glenn), complete repair details, Fontan type (atriopulmonary, lateral tunnel, extracardiac conduit), Mustard/Senning vs. arterial switch, valve replacements (mechanical vs. bioprosthetic), conduit types (homograft, bovine jugular)[4]
- Current cardiac anatomy from imaging: Echocardiogram findings, cardiac MRI (gold standard for ACHD anatomy assessment and volumetric ventricular function), cardiac CT — document chamber sizes, valve function, residual shunts, conduit stenosis or regurgitation[7]
- Current cardiac physiology: Which ventricle is systemic? Which is pulmonary? Is there a Fontan circulation? What is the shunt direction and size? Is there residual cyanosis? What is the baseline SpO2?[2]
- Ventricular function: EF and dimensions for the systemic ventricle regardless of morphology — the systemic right ventricle (atrial switch TGA) has different normal values than the left ventricle; cardiac MRI-derived volumes are more reliable than echo for RV assessment[7]
- Arrhythmia history: Document all documented arrhythmias, ablation procedures, cardioversions, and current rhythm (sinus, paced, atrial flutter/fibrillation, junctional)[8]
- Device inventory: Pacemaker or ICD model, manufacturer, date of last interrogation, current programming (ATP settings, shock zones, lower rate), shock history — every shock the ICD has delivered and the arrhythmia that triggered it; leads and their position (epicardial vs. transvenous, abandoned leads)[9]
What to Look for in Hospice Records
- Functional classification (NYHA): Applies to ACHD as to other cardiac diseases; Class III–IV at enrollment is typical for hospice eligibility[2]
- 6-Minute Walk Test (6MWT): Distance and oxygen saturation at peak exercise; <300 m indicates severe functional limitation in ACHD[10]
- Cardiopulmonary exercise test (CPET) VO2 max: The most objective prognostic indicator in ACHD heart failure — VO2 max below 15 mL/kg/min indicates poor prognosis; below 12 mL/kg/min indicates very poor prognosis with median survival <2 years[10]
- BNP / NT-proBNP: Useful but thresholds differ in ACHD — levels are influenced by the specific anatomy; single-ventricle and Fontan patients may have lower BNP despite advanced failure due to non-compliant ventricle; serial trends are more informative than absolute values[11]
- Serum albumin: Critical in Fontan patients — hypoalbuminemia (<3.0 g/dL) raises concern for protein-losing enteropathy (PLE); PLE carries approximately 50% five-year mortality from diagnosis[12]
- Liver function panel: Fontan-associated liver disease (FALD) is nearly universal by 10 years post-Fontan — assess INR, bilirubin, platelets, and MELD score; hepatocellular carcinoma has been reported as early as age 20 in Fontan patients[13]
- SpO2 baseline: Eisenmenger patients have chronic cyanosis with baseline SpO2 typically 75–90% — document the patient's known baseline; do not reflexively prescribe oxygen for numbers that are "normal for them"[14]
- Complete blood count: Eisenmenger patients develop secondary erythrocytosis (Hgb 16–24 g/dL) as a compensatory response to chronic cyanosis; hematocrit and iron studies guide management[14]
💡 For families
💡 Para las familias
Your person's heart condition was diagnosed long before hospice — often at birth or in early childhood. The diagnostic workup is already complete. What the hospice team is doing now is reading the full story of your person's cardiac anatomy, their surgical history, and how their heart is functioning today. This helps us provide comfort care that is specific to their heart and their body. You do not need to worry about new testing or procedures — the focus now is entirely on comfort.
La condición cardíaca de su ser querido fue diagnosticada mucho antes del hospicio — frecuentemente al nacer o en la primera infancia. El enfoque ahora es enteramente en la comodidad.
Causes & Risk Factors
Why ACHD patients reach end stage — the specific physiology of each lesion pattern and why the repairs of childhood were never designed for a lifetime. Relevant for clinical understanding and family conversations.
End-Stage Physiology by Lesion
- Repaired tetralogy of Fallot — RV failure: The pulmonary regurgitation that is the inevitable consequence of the RVOT patch placed at initial TOF repair produces chronic volume overload of the right ventricle. The RV dilates over decades, becomes progressively dysfunctional, and the scar tissue at the RVOT patch provides the substrate for ventricular tachycardia. End stage: severe RV dilation and dysfunction, progressive functional decline, and sudden death risk from VT. Pulmonary valve replacement that could rescue RV function in earlier stages is not appropriate at end stage.[5][15]
- Atrial switch TGA — systemic RV failure: The systemic right ventricle of the Mustard or Senning repair was designed as the subpulmonary ventricle but has been performing systemic work since infancy. The systemic RV that was never designed for systemic afterload progressively dilates and fails. Sinus node dysfunction and junctional or atrial arrhythmias from the extensive atrial baffle surgery are the most common rhythm problems. Systemic RV failure is the primary end-stage physiology.[16]
- Fontan hemodynamic burden: The elevated central venous pressure that drives the Fontan circulation (typically 12–20 mmHg) is transmitted throughout the systemic venous system, producing hepatic congestion (FALD), mesenteric venous congestion (PLE), lymphatic congestion (plastic bronchitis, chylothorax), and progressive single ventricle failure — simultaneously. Fontan failure is not standard biventricular heart failure. It requires management that is completely distinct from ACE inhibitor, beta-blocker, and diuretic-based HF protocols.[17][12]
Eisenmenger & Shunt Reversal
- Irreversible pulmonary vascular disease: Eisenmenger syndrome develops when a large unrepaired left-to-right shunt (VSD, ASD, PDA, AVSD) produces progressive pulmonary vascular remodeling until pulmonary vascular resistance exceeds systemic resistance and the shunt reverses to right-to-left. Once established, the pulmonary vascular disease is irreversible — no surgical correction is possible.[14]
- Chronic cyanosis and compensatory erythrocytosis: The right-to-left shunt produces chronic systemic desaturation (SpO2 75–90%). The bone marrow responds with secondary erythrocytosis (Hgb 16–24 g/dL). Symptoms of hyperviscosity — headache, visual disturbance, paresthesias — occur when hematocrit exceeds 65% or with iron deficiency.[14]
- Hemoptysis risk: The hypertensive, dilated pulmonary arteries in Eisenmenger are prone to aneurysmal dilation and rupture. Hemoptysis is a known late complication and can be massive and fatal. Pre-position comfort medications as for any hemoptysis-risk diagnosis.[18]
- Paradoxically longer survival than other ACHD failure patterns: Eisenmenger patients may survive into their 40s–60s with preserved ventricular function despite chronic cyanosis. Median survival is approximately 50–55 years without transplant — longer than many repaired complex lesions, but with progressive functional decline and multiorgan consequences.[14][19]
❤️ For families: "Why is this happening now?"
The repairs that were done in childhood were remarkable achievements — they gave your person decades of life that would not have been possible a generation earlier. What is happening now is not a failure of those repairs. It is the reality that those repairs were designed to extend childhood survival, not to last a lifetime — because at the time they were performed, nobody knew whether the patient would need them to last a lifetime. The heart and the repairs have worked longer and harder than anyone predicted. What is happening now is the natural consequence of decades of extraordinary cardiac work, and it is not anyone's fault.
⚕ Clinician note: Genetic counseling
CHD recurrence risk in offspring ranges from 3–12% depending on the specific lesion and inheritance pattern — significantly higher than the 0.8–1% population background risk.[20] Bicuspid aortic valve and aortopathy have particularly strong heritable patterns. Even at hospice enrollment, referral for genetic counseling is appropriate if the patient has children or siblings of reproductive age. Identifying heritable CHD risk in surviving family members can save lives through early screening and surveillance. Document the genetic counseling referral in the care plan.
Treatments & Procedures
ACHD end-stage comfort management — anatomy-specific and physiology-specific clinical frameworks that diverge fundamentally from standard heart failure management.
ACHD end-stage management is defined by a single principle: every intervention must be matched to the specific anatomy and specific physiology of the specific lesion. The standard heart failure toolkit — ACE inhibitors, beta-blockers, ARNIs, loop diuretics at aggressive doses — was developed for biventricular systolic heart failure and validated in large RCTs of patients with structurally normal hearts. None of these RCTs included ACHD patients. Applying standard HF protocols to a Fontan patient, an Eisenmenger patient, or a patient with a systemic right ventricle is not evidence-based medicine — it is an extrapolation that may cause harm.[2][17] The clinical frameworks below are organized by lesion-specific physiology.
Fontan Failure Syndrome Management
This is NOT standard heart failure. Fontan-specific management diverges from standard HF at every level:
- ACEi / Beta-blockers: The cornerstone medications of systolic HF management have NOT been shown to improve outcomes in the Fontan circulation in any RCT. Their use should be guided by ACHD specialist input, not standard HF guidelines.[17][21]
- Sildenafil / PDE5 inhibitors: Reduce pulmonary vascular resistance in the Fontan circuit, reduce venous hypertension burden, and improve exercise capacity. Small RCTs and single-center series show modest improvements in 6MWT and hemodynamics. Continue if providing symptom benefit (improved exercise tolerance, reduced edema, reduced dyspnea).[22]
- Diuretics — extreme caution: The Fontan patient is preload-dependent — over-diuresis reduces already-reduced ventricular filling and can cause catastrophic reduction in cardiac output. Target: reduce symptomatic fluid overload (edema, effusions, ascites) without over-diuresis. Loop diuretic (furosemide) at the minimum effective dose for symptom control only.[17]
- Protein-losing enteropathy (PLE): The most devastating complication of Fontan failure. Albumin infusion (25% albumin 50–100 mL IV over 4–6 hours every 1–4 weeks) for symptomatic hypoalbuminemia and edema. Subcutaneous heparin (unfractionated heparin 5,000–10,000 units SC BID) has shown PLE reversal in case series — mechanism involves heparan sulfate restoration on intestinal epithelium.[23][24]
- Fontan-associated liver disease (FALD): Nearly universal by 10 years post-Fontan. Monitor with hepatic ultrasound, AFP, MELD score. HCC risk is real and occurs earlier than in other cirrhosis etiologies. Apply ESLD framework when MELD crosses 17.[13]
Eisenmenger Syndrome Management
PAH medications are comfort medications — NEVER STOP abruptly.
- PAH-specific therapy (sildenafil, bosentan, macitentan, prostacyclin analogs): These medications maintain functional status and exercise capacity in Eisenmenger. The BREATHE-5 trial demonstrated bosentan's efficacy in Eisenmenger-specific PAH.[25] Abrupt withdrawal produces rapid PAH decompensation with severe dyspnea, hemodynamic instability, and potentially rapid death. Continue all PAH medications at hospice enrollment. Document the never-stop instruction prominently.[14]
- Oxygen — nuanced, not automatic: Oxygen does not correct the right-to-left shunt physiology in Eisenmenger. Prescribe only when the patient reports specific symptomatic benefit. Document the symptom-benefit assessment. Do not prescribe reflexively for SpO2 values that are "normal for them."[2]
- Erythrocytosis management: Do NOT phlebotomize unless symptoms of hyperviscosity are present AND hematocrit exceeds 65% — therapeutic phlebotomy for asymptomatic erythrocytosis is harmful and causes iron deficiency that worsens symptoms. Maintain iron stores.[14]
- Hemoptysis protocol: Pre-position comfort medications (midazolam 5 mg IM/SL, dark towels, morphine for air hunger) as for any hemoptysis-risk diagnosis. Massive hemoptysis from pulmonary artery rupture is a recognized late event. Family education is essential.[18]
ICD Management in ACHD
- Deactivation conversation at enrollment: The ICD that has been protecting the ACHD patient from sudden arrhythmic death is simultaneously the device that may administer multiple painful shocks during the dying process if not deactivated. Have this conversation before any arrhythmia crisis — not during one.[9]
- Emotional weight in ACHD: The ICD in ACHD carries a specific emotional burden — this device may have already fired and saved the patient's life. Acknowledging the prior shock experience is essential: "The ICD worked before and that was real. The decision about deactivation now is about the dying process, which is different."[26]
- Magnet availability: Ensure a device magnet is available in the home for emergency ICD suspension if the patient experiences inappropriate shocks before device clinic deactivation can be arranged. Most ICDs will suspend tachycardia therapy when a magnet is placed over the generator.[9]
- Device clinic coordination: Contact the device clinic (EP lab) to arrange formal deactivation programming if the patient chooses to deactivate. Document the patient's decision in the advance directive. Pacemaker function (if the patient is pacemaker-dependent) can be preserved while disabling shock therapy.[9]
Repaired TOF / TGA End-Stage Management
- RV failure in repaired TOF: Progressive RV dilation and dysfunction from chronic pulmonary regurgitation. Diuretics for symptomatic fluid overload at conservative doses. Monitor for hepatic congestion from RV failure. No evidence that standard HF neurohormonal therapy (ACEi/BB) improves RV function in TOF.[5]
- Arrhythmia substrate management: Ventricular tachycardia from the RVOT scar is the primary sudden death mechanism in repaired TOF. QRS duration >180 ms on ECG is a validated predictor of sudden death risk. Atrial flutter/fibrillation is common and can cause rapid hemodynamic deterioration in compromised ventricles.[15][8]
- Amiodarone: The most commonly used antiarrhythmic in ACHD. If the patient is on amiodarone, continue for comfort-directed rhythm stability. Monitor thyroid and liver function (amiodarone-induced thyroid dysfunction and hepatotoxicity). Drug interaction profile is extensive — affects CYP3A4, CYP2C9, P-glycoprotein.[27]
- Systemic RV failure in atrial switch TGA: Management parallels severe systolic HF with cautious diuresis and ACHD specialist-guided neurohormonal modulation. Baffle leak or obstruction can cause acute decompensation — these are anatomy-specific emergencies requiring urgent ACHD specialist input.[16]
When Therapy Makes Sense
ACHD-specific criteria for continuing disease-directed and comfort-directed therapy at hospice enrollment. These are not standard HF criteria — they are anatomy-specific clinical acts.
In ACHD, "when therapy makes sense" is defined not by standard performance status thresholds or line-of-therapy counts, but by anatomy-specific clinical decisions that require ACHD specialist guidance. The medications that are continued at hospice enrollment in ACHD are comfort medications — PAH therapy in Eisenmenger, sildenafil in Fontan, albumin infusions for PLE — not disease-directed therapy in the traditional oncologic sense. The eight criteria below represent the clinical acts that should occur at or before enrollment for every ACHD hospice patient.[2][6]
- 01ACHD specialist consultation at enrollment: Contact the nearest ACHD center before or at the first hospice visit. The ACHD cardiologist who has knowledge of this patient's anatomy and repair history provides guidance that no general cardiologist or hospice clinician can replicate. Document the ACHD specialist name and direct contact in the care plan. Obtain operative reports and prior ACHD cardiology notes if not in the current chart.[6][2]
- 02ICD deactivation conversation at enrollment — before any arrhythmia crisis: Conduct the specific deactivation conversation, acknowledge the prior ICD shock experience if it occurred, and document the patient's decision in the advance directive. Arrange the deactivation procedure with the device clinic if the patient decides to deactivate. If the patient decides to keep the ICD active, document this decision and ensure the care team understands the implications during the dying process.[9][26]
- 03Continue PAH medications for Eisenmenger patients — never stop abruptly: Sildenafil, bosentan, macitentan, and prostacyclin analogs are comfort medications that maintain functional status. Abrupt withdrawal produces rapid PAH decompensation. Apply the same never-stop principle as in idiopathic PAH. Document the never-stop instruction as prominently as a medication allergy.[25][14]
- 04Minimum effective diuresis for Fontan patients — avoid over-diuresis: The Fontan patient who is volume-overloaded is simultaneously preload-dependent for cardiac output. Over-diuresis reduces already-reduced ventricular filling and can cause catastrophic hemodynamic deterioration. Diurese to relieve symptoms (edema, effusions, ascites) — not to a target weight or BNP value. Document the minimum effective furosemide dose.[17]
- 05PLE management with albumin infusions: For Fontan patients with symptomatic protein-losing enteropathy (albumin <3.0 g/dL with edema, ascites, or diarrhea), schedule regular albumin infusions (25% albumin 50–100 mL IV every 1–4 weeks). Consider subcutaneous heparin for PLE reversal if consistent with comfort goals. PLE-specific management provides direct symptom relief that standard HF management does not address.[23][24]
- 06Oxygen for Eisenmenger patients only with documented symptom benefit: Ask the patient directly: "Does oxygen make you feel better? When and how?" Oxygen does not correct the shunt physiology in Eisenmenger. If the patient reports no specific symptom benefit, oxygen adds equipment burden without comfort value. Document the specific symptom-benefit assessment at enrollment.[2]
- 07Hemoptysis comfort protocol for Eisenmenger patients: Pre-position comfort medications (midazolam 5 mg IM/SL, morphine for air hunger, dark towels, anxiolytic). Educate the family on the hemoptysis event plan. Massive hemoptysis from pulmonary artery aneurysm rupture is a recognized late event in Eisenmenger syndrome. Preparation prevents panic.[18]
- 08ACHD advance directive completed at enrollment: The ACHD-specific advance directive addresses: ICD deactivation decision, hospitalization for arrhythmia crisis, hospitalization for acute respiratory event, Fontan atrial flutter emergency cardioversion, hemoptysis management, code status, organ transplant listing status if relevant. This is more detailed than a standard advance directive because the ACHD patient faces anatomy-specific emergencies that require anatomy-specific decisions in advance.[26][28]
When It Doesn't
ACHD-specific clinical errors that cause harm when non-ACHD frameworks are applied to anatomy-specific problems. Knowing what NOT to do in ACHD is as important as knowing what to do.
The clinical errors that harm ACHD patients in the adult care system are not errors of omission — they are errors of inappropriate application. They occur when clinicians apply general cardiology frameworks to patients whose physiology does not follow general cardiology rules. ACHD patients managed at non-ACHD centers have significantly higher mortality and higher rates of preventable complications.[6] Every item below represents a documented pattern of harm that occurs when ACHD patients are managed without ACHD specialist guidance.[2]
- 01Applying standard HF management (ACEi, beta-blocker, ARNI) to the Fontan patient without ACHD specialist guidance: The Fontan circulation does not respond to standard HF medications in the same way as biventricular failure. ACE inhibitors that provide survival benefit in systolic LV failure have not been shown to improve outcomes in the Fontan circulation. Beta-blockers that reduce mortality in systolic HF may reduce cardiac output in the preload-dependent Fontan by lowering heart rate below the minimum needed to maintain cardiac output. Applying standard HF management blindly to a Fontan patient is a clinical error that requires ACHD specialist guidance before any HF medication change.[17][21]
- 02Removing PAH medications from Eisenmenger patients at hospice enrollment: The sildenafil, bosentan, macitentan, or prostacyclin that is maintaining functional status in the Eisenmenger patient is a comfort medication. Its abrupt withdrawal produces rapid PAH decompensation with severe dyspnea, hemodynamic instability, and potentially rapid death. The rationale that "they're end-stage anyway" is clinically incorrect — PAH medications provide direct, measurable symptom relief. Never stop PAH medications in Eisenmenger at hospice enrollment.[25][14]
- 03Prescribing supplemental oxygen automatically for cyanotic ACHD patients without symptom assessment: Oxygen prescribed reflexively for any patient with low SpO2 may add equipment burden without comfort benefit in the Eisenmenger patient whose cyanosis is hemodynamically compensated and for whom supplemental oxygen does not correct the right-to-left shunt physiology. Assess whether oxygen provides specific symptomatic comfort before prescribing. A baseline SpO2 of 82% may be normal for this patient — document their known baseline.[2]
- 04Defibrillating or performing CPR as the default response to an arrhythmia event in a comfort-directed ACHD patient: The advance directive that addresses the specific arrhythmia events common to the patient's ACHD lesion must be documented at enrollment and communicated to all team members. The ACHD patient with an active ICD whose advance directive specifies comfort-only management requires that the ICD be deactivated — not that the patient be defibrillated by EMS after the ICD fires during a natural dying process.[9][26]
- 05Over-diuresis in the preload-dependent Fontan patient: The Fontan circulation requires adequate preload to maintain passive pulmonary blood flow. Aggressive diuresis that would be appropriate in biventricular HF can cause acute hemodynamic collapse in the Fontan by reducing venous return below the threshold for pulmonary blood flow. Diurese to symptom relief only — never to a weight target derived from standard HF protocols.[17]
- 06Applying general cardiology frameworks to anatomy-specific ACHD problems: The systemic right ventricle is not a failing left ventricle. The Fontan circulation is not biventricular heart failure. Eisenmenger PAH is not idiopathic PAH. Atrial flutter in a Fontan patient produces hemodynamic compromise that atrial flutter in a structurally normal heart does not. Every clinical decision must be filtered through the specific anatomy, the specific repair, and the specific physiology of the individual patient.[2][6]
- 07Prescribing calcium channel blockers for PAH in Eisenmenger patients: Calcium channel blockers (nifedipine, diltiazem, amlodipine) are used for vasoreactivity-positive idiopathic PAH but are contraindicated in Eisenmenger syndrome. Systemic vasodilation from CCBs in Eisenmenger can worsen the right-to-left shunt, deepen cyanosis, and cause syncope or cardiovascular collapse. This is an ACHD-specific contraindication that general cardiology training may not emphasize.[14][2]
⚠️ Clinician warning
The catastrophic clinical errors in ACHD occur when specialists in adult medicine — cardiologists, intensivists, hospitalists, hospice physicians — apply their standard frameworks to a physiology that does not follow standard rules. Mylotte et al. (2014) demonstrated that complex ACHD patients managed at non-ACHD centers had significantly higher mortality. The ACHD specialist phone call at enrollment is the single most important act to prevent these anatomy-specific errors. If no ACHD specialist has been involved, find the nearest ACHD center and make the referral before applying any cardiac management changes.[6]
Out-of-the-Box Approaches
Evidence-graded approaches specific to ACHD hospice and palliative care. Grade A = guideline/RCT; B = multi-observational/meta-analysis; C = case series/limited clinical; D = expert opinion.
ACHD Specialist Consultation at Enrollment
Grade AProtocol: Contact ACHD center before or at first hospice visit; obtain operative reports; establish ongoing communication channel
The ACC/AHA 2018 ACHD guidelines (Stout et al. 2019) classify patients with complex ACHD as requiring regular care at ACHD-specialized centers — this includes all single-ventricle lesions (Fontan), TGA, Eisenmenger syndrome, and TOF with residual hemodynamic issues.[2] Mylotte et al. (2014) demonstrated that complex ACHD patients managed at non-ACHD centers had significantly higher mortality and higher rates of preventable complications.[6] The ACHD specialist phone call at enrollment prevents the anatomy-specific errors (standard HF medications to Fontan, stopped PAH medications in Eisenmenger, CCBs for Eisenmenger PAH) that cause measurable harm in this population. This is the single most important clinical act at hospice enrollment for any ACHD patient.
ICD Deactivation in ACHD
Grade AProtocol: Deactivation conversation at enrollment; document decision in advance directive; coordinate with device clinic for programming change
ICD deactivation at end of life is supported by ACC/AHA/HRS consensus statements and is an ethical and clinical obligation when the patient's goals shift to comfort.[9] In ACHD, the ICD conversation carries specific emotional weight: these devices have often already fired and saved the patient's life — the young adult with repaired TOF whose ICD terminated a sustained VT episode at age 28 is now being asked whether to deactivate the device that saved them. Acknowledging this history is essential to the conversation. The patient whose ICD fires repeatedly during a natural dying process because the deactivation conversation was never had is experiencing preventable suffering.[26] The ethical framework is clear: the patient has the right to refuse any therapy, including ICD therapy, at any time.
Subcutaneous Heparin for Fontan PLE
Grade BProtocol: Unfractionated heparin 5,000–10,000 units SC BID; monitor aPTT; reassess PLE markers (albumin, stool alpha-1-antitrypsin) at 4–8 weeks
Case series have demonstrated PLE reversal with subcutaneous heparin in Fontan patients. The proposed mechanism involves restoration of heparan sulfate proteoglycans on the intestinal epithelial surface — the glycoprotein layer that is disrupted by the elevated mesenteric venous pressure of the Fontan circulation.[23] Heparin therapy for PLE has shown improvement in serum albumin, reduction in diarrhea, and reduction in edema in published case series. The bleeding risk must be weighed against the PLE-related mortality (approximately 50% at 5 years from PLE diagnosis). Consistent with comfort goals when PLE symptoms (anasarca, severe diarrhea, malnutrition) dominate the clinical picture.[24]
Sildenafil for Fontan Hemodynamics
Grade BProtocol: Sildenafil 20 mg TID or tadalafil 20 mg daily; assess 6MWT and symptom response at 4–8 weeks
Small RCTs and single-center series demonstrate that PDE5 inhibitors reduce pulmonary vascular resistance in the Fontan circuit, reduce central venous pressure, and improve exercise capacity measured by 6MWT.[22] Goldberg et al. showed improvements in Fontan hemodynamics with sildenafil in a controlled study. In the hospice context, continue sildenafil/tadalafil if the patient reports symptom benefit (improved exercise tolerance, reduced edema, reduced dyspnea). Reassess for comfort benefit at each visit. Contraindication: concurrent nitrate use (severe hypotension). Drug interaction: grapefruit increases sildenafil levels via CYP3A4.[22]
Nebulized Heparin for Plastic Bronchitis in Fontan
Grade CProtocol: Nebulized unfractionated heparin 10,000 units in 3 mL NS via nebulizer BID–TID; mucolytic adjunct (nebulized tPA in severe cases per case reports)
Plastic bronchitis — the formation of rubbery bronchial casts from lymphatic fluid in the airways — is a rare but devastating complication of Fontan failure arising from lymphatic congestion and abnormal lymphatic drainage. Case reports and small case series describe symptom improvement with nebulized heparin, which is thought to disrupt fibrin formation in the bronchial casts.[29] The evidence base is limited to case reports and institutional experience, but the severity of plastic bronchitis (airway obstruction, respiratory failure) and the absence of alternative treatments make this a reasonable comfort-directed intervention. Bleeding risk from systemic absorption is minimal with nebulized administration.
Palliative Care Integration for ACHD
Grade BProtocol: Early palliative care referral at NYHA Class III; concurrent palliative-ACHD specialist co-management; advance care planning before NYHA Class IV
Harkness et al. conducted a systematic review demonstrating significant unmet palliative care needs in the ACHD population — including symptom burden, psychosocial distress, and inadequate advance care planning.[28] ACHD patients are significantly under-referred to palliative care compared to acquired heart disease patients. The Stout et al. 2019 ACC/AHA ACHD guidelines specifically recommend palliative care integration for patients with advanced ACHD.[2] The concurrent model — ACHD specialist and palliative care co-management — provides the anatomy-specific expertise alongside the symptom management and goals-of-care expertise that neither discipline provides alone.
Oxygen Titration Protocol for Eisenmenger
Grade BProtocol: Symptom-benefit assessment at enrollment; trial of 2–4 L/min NC for 1 week with patient-reported symptom diary; continue only if documented benefit
AHA/ACC ACHD guidelines acknowledge that supplemental oxygen in Eisenmenger does not correct the underlying shunt physiology and should be prescribed based on individual symptom benefit rather than SpO2 targets.[2] Some Eisenmenger patients report subjective improvement in dyspnea and exercise tolerance with supplemental oxygen, while others report no benefit and experience the oxygen equipment as burdensome. A structured symptom-benefit assessment — trial of oxygen with documentation of specific symptom response — prevents reflexive oxygen prescribing while ensuring patients who benefit receive it. The patient's own report is the primary outcome measure.
Fontan Liver Disease Screening with MELD Assessment
Grade BProtocol: Hepatic ultrasound + AFP at enrollment; calculate MELD score; apply ESLD framework when MELD ≥17; HCC surveillance per AASLD guidelines
Fontan-associated liver disease (FALD) is nearly universal by 10 years post-Fontan — the chronic hepatic venous congestion from elevated CVP produces hepatic fibrosis progressing to cirrhosis.[13] Asrani et al. documented hepatocellular carcinoma in Fontan patients occurring earlier (as young as age 20) than in other cirrhosis etiologies.[30] At hospice enrollment, MELD score assessment determines whether the Fontan patient's trajectory is dominated by cardiac failure, liver failure, or both. When MELD crosses 17, apply the ESLD palliative framework alongside the Fontan cardiac management. This dual-organ assessment prevents undertreatment of hepatic symptoms (pruritus, encephalopathy, variceal risk) in patients whose care is focused exclusively on the cardiac diagnosis.
Natural & Herbal Options
Evidence grading, dosing where supported, drug interaction flags, and explicit contraindications specific to ACHD physiology. Patients will use supplements — this section helps you have the right conversation.
"Patients are going to use supplements whether we ask or not. The conversation is: 'I want to know what you're taking — not to judge you, but because some of these interact with your heart pressure medications and blood thinners in ways that are dangerous and specific to your heart.' With ACHD patients, that conversation is five conversations at once — the anticoagulation, the sildenafil, the amiodarone, the arrhythmia risk, and the Fontan absorption. Ask. Write it down. Call the ACHD specialist before you say yes or no to any of it."
— Waldo, NP
⚠ ACHD-Specific Supplement Safety: Five Simultaneous Concerns
Adult congenital heart disease creates a supplement safety landscape defined by five simultaneous concerns specific to ACHD physiology:[1]
- (1) Complex anticoagulation management: Fontan patients on warfarin, Eisenmenger patients on anticoagulation, and ACHD patients with mechanical valves require anticoagulation that interacts with supplements affecting coagulation — vitamin K, omega-3 fatty acids, garlic, ginkgo, and ginger at high doses all alter coagulation parameters.[2]
- (2) PAH medication interactions: The sildenafil, bosentan, and prostacyclin analogs that maintain Eisenmenger functional status are metabolized through CYP3A4 — supplements that induce or inhibit this enzyme (St. John's Wort, grapefruit) alter drug levels with potentially catastrophic consequences.[3]
- (3) Fontan PLE malabsorption: Protein-losing enteropathy produces unpredictable oral supplement absorption. Supplements taken by a PLE patient may not reach therapeutic levels from malabsorption, or may produce erratic levels from the compromised intestinal barrier.[4]
- (4) Arrhythmia substrate sensitivity: The surgical scars and anatomical substrates for arrhythmia in ACHD patients create specific sensitivity to supplements with arrhythmogenic properties — sympathomimetics, high-dose caffeine, herbal stimulants, and high-dose magnesium in the context of digitalis use.[5]
- (5) Amiodarone drug interaction landscape: Amiodarone — the most common antiarrhythmic in ACHD — has one of the largest drug interaction profiles in pharmacology. Supplements affecting CYP3A4, CYP2C9, and P-glycoprotein — which amiodarone is both inhibitor and substrate of — require specific assessment before use.[6]
Guiding principle: Every supplement must be assessed against the ACHD-specific medication regimen with ACHD specialist input for any supplement that affects coagulation, cardiac rhythm, or the CYP450 enzymes that metabolize the critical PAH and antiarrhythmic medications.
| Herb / Supplement | Evidence Grade | Typical Dose | Potential Benefit | ⚠ Interactions / Contraindications |
|---|---|---|---|---|
| Coenzyme Q10 (CoQ10) | Grade B | 100–300 mg daily | Mitochondrial support for the failing myocardium; observational data show improved NYHA class and reduced hospitalizations in heart failure populations; may support the chronically stressed ACHD ventricle.[7] | Structurally similar to vitamin K — monitor INR closely in warfarin-anticoagulated Fontan and mechanical valve patients. May reduce warfarin efficacy. Requires ACHD specialist approval when on anticoagulation.[8] |
| Omega-3 Fatty Acids (EPA/DHA) | Grade B | 2–4 g daily | Anti-inflammatory; may reduce arrhythmia burden in some cardiac populations; modest triglyceride reduction; potential benefit for the systemic inflammatory state of Fontan failure.[9] | Antiplatelet effect at doses >3 g/day — additive bleeding risk with warfarin in Fontan and Eisenmenger patients. Monitor INR. In PLE patients, absorption is unpredictable — enteric-coated formulations may not release appropriately.[10] |
| Magnesium Glycinate | Grade C | 200–400 mg daily | Muscle cramp relief; may reduce atrial arrhythmia frequency; improves sleep quality; supports the chronically depleted magnesium in diuretic-treated ACHD patients.[11] | High-dose magnesium in the context of digitalis use can potentiate toxicity. In patients on amiodarone, magnesium levels affect QT interval — monitor serum levels. Glycinate form preferred for reduced GI side effects. Avoid in renal insufficiency.[12] |
| Vitamin D3 | Grade B | 1,000–4,000 IU daily | Deficiency is nearly universal in ACHD heart failure populations; correction associated with improved cardiac function biomarkers; supports bone health in the chronically deconditioned and steroid-exposed ACHD patient.[13] | Minimal drug interactions at standard doses. In PLE patients, absorption is impaired — may require higher doses or monitoring of 25-OH vitamin D levels. Hypercalcemia risk at doses >10,000 IU/day — avoid in patients with concurrent calcium supplementation without monitoring.[14] |
| L-Carnitine | Grade C | 1–2 g daily | Supports mitochondrial fatty acid oxidation in the energy-depleted ACHD myocardium; small studies show improved exercise tolerance in heart failure; may benefit the Fontan patient with hepatic dysfunction affecting carnitine metabolism.[15] | Generally well-tolerated. GI disturbance (nausea, diarrhea) at higher doses may exacerbate PLE symptoms. Rare reports of increased TMAO production — uncertain cardiovascular significance. Dose adjustment in renal insufficiency.[16] |
| Hawthorn (Crataegus) | Grade C | 300–900 mg standardized extract daily | Positive inotropic and vasodilatory effects; the SPICE trial in HF showed trend toward benefit; may provide modest symptom relief in the ACHD patient with systemic ventricular failure.[17] | Potentiates the effects of digoxin, beta-blockers, and antihypertensives. In ACHD patients on amiodarone, the additive negative chronotropic effect requires monitoring. Do not combine with PDE-5 inhibitors (sildenafil) without specialist guidance — additive vasodilation risk. ⚠ Contraindicated in combination with digoxin without monitoring.[18] |
| Melatonin | Grade B | 1–5 mg at bedtime | Sleep-wake cycle regulation; antioxidant properties; improves sleep quality in heart failure populations; may reduce anxiety and improve well-being in the ACHD patient with chronic sleep disruption from nocturnal dyspnea and arrhythmia-related insomnia.[19] | Minimal drug interactions at standard doses. Theoretical concern for additive sedation with benzodiazepines — clinically insignificant at 1–5 mg. May lower blood pressure slightly — monitor in patients with borderline hemodynamics. Well-tolerated in PLE patients.[20] |
| Iron Supplementation (for Eisenmenger) | Grade B | Ferrous sulfate 325 mg daily or IV iron as indicated | Iron deficiency is common in Eisenmenger syndrome (prevalence 30–50%) despite elevated hemoglobin from secondary erythrocytosis; iron deficiency worsens exercise intolerance and increases hyperviscosity symptoms; repletion improves functional capacity and quality of life.[21] | In Eisenmenger, iron repletion must be guided by ferritin and transferrin saturation — NOT hemoglobin, which is elevated from erythrocytosis. Avoid phlebotomy for erythrocytosis without confirmed hyperviscosity symptoms AND confirmed iron repletion. Oral iron poorly absorbed in patients with hepatic congestion or PLE — IV iron (ferric carboxymaltose) may be required. ⚠ Never phlebotomize an iron-deficient Eisenmenger patient.[22] |
🚫 Avoid in ACHD
- St. John's Wort (Hypericum perforatum): Potent CYP3A4 inducer — reduces plasma levels of sildenafil by up to 50% and bosentan significantly, directly undermining PAH therapy that maintains Eisenmenger functional status. Also reduces amiodarone levels, increasing arrhythmia breakthrough risk. Induces warfarin metabolism, destabilizing INR in anticoagulated Fontan and mechanical valve patients. Absolute contraindication in any ACHD patient on PAH therapy, amiodarone, or warfarin.[23]
- Ginkgo biloba: Inhibits platelet-activating factor — potentiates anticoagulant effect of warfarin with documented case reports of serious bleeding. Additionally, ginkgotoxin (4'-O-methylpyridoxine) has proconvulsant properties and ginkgo may lower seizure threshold, which is relevant in ACHD patients with cerebral abscesses or paradoxical emboli history. Reports of cardiac arrhythmia with high doses in susceptible individuals.[24]
- Ephedra / Ma huang: Sympathomimetic alkaloid — direct arrhythmogenic risk in the ACHD patient whose surgical scars and altered conduction provide the substrate for ventricular tachycardia. Increases heart rate and blood pressure, worsening pulmonary hypertension in Eisenmenger and increasing cardiac work in the failing systemic ventricle. FDA-banned for dietary supplements but available in some herbal preparations. Absolute contraindication.[25]
- High-dose garlic supplements (>4 g/day or concentrated extract): Inhibits platelet aggregation and enhances fibrinolytic activity — additive anticoagulant risk in Fontan and Eisenmenger patients on warfarin. Multiple case reports of perioperative bleeding attributed to garlic supplementation. Culinary garlic at normal dietary levels is not a concern — concentrated supplements are.[26]
- Grapefruit juice (regular consumption): Potent CYP3A4 inhibitor — increases sildenafil plasma levels by 23% (single glass) to >100% (regular consumption), increasing hypotension and flushing risk. Also increases amiodarone levels, increasing toxicity risk (pulmonary, thyroid, hepatic). In the ACHD patient on both sildenafil and amiodarone, the dual interaction is particularly dangerous. Counsel patients to avoid regular grapefruit consumption.[27]
- Licorice root (Glycyrrhiza glabra): Glycyrrhizinic acid inhibits 11β-hydroxysteroid dehydrogenase, producing pseudoaldosteronism — hypokalemia, sodium retention, and hypertension. Hypokalemia in the ACHD patient on amiodarone dramatically increases QT prolongation and torsades de pointes risk. Sodium retention worsens Fontan venous congestion and edema. Avoid in any ACHD patient on amiodarone or diuretics.[28]
Timeline Guide
The longest timeline in this series — from birth to hospice in the 40s, 50s, or 60s. A guide to the extraordinary medical journey, not a prediction.
The ACHD timeline is the longest and most medically eventful in this entire card series. From birth to hospice enrollment in the 40s, 50s, or 60s, the ACHD patient has undergone multiple surgical procedures, experienced multiple arrhythmia events, managed multiple hospitalizations, and lived every decade of their life with the knowledge that their heart is anatomically different from everyone else's. The surgical repair that was performed at age 3 was not a cure — it was an engineering solution designed with the best available knowledge of 1975 or 1985, applied to an anatomy that had never been repaired before, in a patient who was never expected to survive long enough to test how the repair would age over decades.[1]
The timeline below represents a composite ACHD trajectory — the specific lesion (tetralogy of Fallot, transposition of the great arteries, single ventricle/Fontan, Eisenmenger) determines the specific hemodynamic trajectory and the specific failure mode. Every ACHD patient's story is anatomy-specific. Use this guide to understand the arc of the journey, and consult the ACHD specialist for the lesion-specific prognosis.[2]
BIRTH–
CHILD
CHILD
Birth & Early Childhood — Diagnosis and Surgical Interventions
- Birth recognition: Cyanosis at birth recognized by the neonatologist; echocardiogram at 2 days of life revealing the cardiac anatomy — the moment the parents learn their child's heart is fundamentally different[29]
- First surgical interventions: The Blalock-Taussig shunt at 6 days; the first-stage palliation (Norwood) at 3 months; the Glenn bidirectional cavopulmonary shunt at 6 months; the complete repair at 18 months; the Fontan completion at age 3 — each surgery a pioneering act that extended life into the next stage[30]
- Parents become experts: Parents who learned cardiac anatomy from necessity — who can describe the Fontan circulation, who know the difference between a Glenn and a Norwood, who carry the operative reports in a folder to every appointment
- Childhood organized around the heart: Medical appointments, echocardiograms, cardiac catheterizations, and the careful management of activity; classmates who did not understand why certain activities were restricted; the scar on the chest that was both a badge of survival and a marker of difference
- First arrhythmia at ~14: The first arrhythmia episode requiring cardioversion — atrial flutter in the Fontan patient, ventricular tachycardia in the TOF repair — the first time the adolescent understands that the surgical fix of childhood has electrical consequences[5]
ADOL–
YNG AD
YNG AD
Adolescence & Young Adulthood — Transition and Identity Formation
- The transition gap (age 18–21): Transfer from the pediatric cardiac center to the adult system — the pediatric cardiologist who knew the anatomy perfectly, the new adult cardiologist who needed to learn the patient's anatomy from scratch; studies show up to 50% of ACHD patients are lost to follow-up during this transition, with documented increases in morbidity and mortality[31]
- Identity formation inside a different heart: The relationships shaped by the cardiac condition; the career choices influenced by functional capacity; the pregnancy counseling conversations; the life insurance applications that were refused; the dating conversations about scars and ICDs
- ICD implantation at ~26: After a sustained ventricular tachycardia episode — the device that will protect against sudden cardiac death for the next two decades, and whose deactivation will become the most emotionally charged advance directive discussion at end of life[32]
- First major Fontan hospitalization at ~31: Fontan-related atrial flutter requiring 3 days of IV amiodarone and electrical cardioversion; the progressive understanding that the surgical repair of childhood was not a permanent solution but a working hypothesis with a finite lifespan[33]
- Growing awareness: The progressive understanding that the repair was designed to extend childhood survival, not to last a lifetime — that no data existed about how these repairs would function at age 35, 45, or 55 because no one had survived long enough to generate that data
MID-
ADULT
ADULT
Middle Adulthood — Progressive Hemodynamic Decline
- PLE diagnosis at ~38: Protein-losing enteropathy — the most devastating complication of Fontan failure, with serum albumin dropping below 3.0 g/dL, producing peripheral edema, ascites, pleural effusions, and malabsorption; PLE carries approximately 50% 5-year mortality from diagnosis[4]
- Progressive RV failure in TOF: The right ventricle that has been volume-overloaded from pulmonary regurgitation for 30+ years progressively dilates and fails; the RVOT patch that saved life in infancy is now the substrate for ventricular tachycardia[34]
- Fontan liver disease progression: FALD (Fontan-associated liver disease) is nearly universal by 10 years post-Fontan — hepatic fibrosis progressing to cirrhosis; hepatocellular carcinoma reported as early as age 20 in Fontan patients; imaging surveillance becomes routine[35]
- Multiple re-interventions: Pulmonary valve replacement, conduit revisions, catheter-based interventions for collateral vessels, arrhythmia ablation procedures — each intervention providing months to years of improved function before the next decline
- Increasing functional decline: VO2 max declining from 20 to 15 to 12 mL/kg/min over years; 6-minute walk distance shortening; the progressive loss of activities that defined adult life — the job that becomes too physically demanding, the stairs that become impossible, the trips that stop
HOSP
TRANS
TRANS
Hospice Transition — End-Stage ACHD
- Functional class NYHA IV: Symptoms at rest; unable to carry out any physical activity without discomfort; VO2 max <12 mL/kg/min — the most objective prognostic marker confirming end-stage physiology[36]
- Refractory PLE in Fontan: Albumin infusions required every 1–4 weeks; anasarca despite maximum diuresis; pleural effusions requiring thoracentesis; the PLE that no longer responds to heparin therapy
- Fontan failure or Eisenmenger WHO III–IV: The single ventricle that can no longer drive the Fontan circulation; the Eisenmenger patient whose exercise tolerance has declined to household ambulation; recurrent life-threatening arrhythmias despite antiarrhythmic therapy[37]
- ICD deactivation decision: The conversation that must happen at enrollment — before any arrhythmia crisis: does the patient want the ICD to remain active during the dying process, or deactivated to allow natural death without shocks? Document the decision. Arrange deactivation if chosen.[38]
- Advance directive completion: ICD status, hospitalization preferences for arrhythmia, Fontan flutter emergency cardioversion, hemoptysis management, code status, organ transplant listing status — every decision specific to the ACHD anatomy and its specific failure modes
FINAL
HRS
HRS
Final Hours — The Specific ACHD Dying Process
- Arrhythmia risk if ICD not deactivated: The most urgent concern — if the ICD is still active, ventricular tachycardia or fibrillation during the dying process will trigger repeated shocks, causing pain and distress to the patient and trauma to the family witnessing it. Confirm ICD status. If active by patient choice, prepare the family for what they may see.[39]
- Cheyne-Stokes respiration: Periodic breathing with central apneas — common in advanced heart failure; the pattern of crescendo-decrescendo breathing followed by pauses may alarm families; explain in advance that this is the body's natural response, not suffering
- Progressive cyanosis in Eisenmenger: The baseline cyanosis deepens as cardiac output falls; oxygen saturation readings become unreliable and should not drive treatment decisions; the pulse oximeter may read 40–60% in the final hours — this number does not reflect the patient's comfort[40]
- Fontan-specific dying: Progressive volume overload with worsening edema and ascites; hepatic congestion producing jaundice; the low cardiac output state producing cool extremities and oliguria; morphine and midazolam for comfort management of dyspnea and agitation
- Family preparation: Prepare the family for the specific dying process of their person's anatomy — the Eisenmenger family needs to understand deepening cyanosis; the Fontan family needs to understand the swelling; every family needs to understand the ICD decision and what it means. The hospice team that prepares the family before the final hours prevents the crisis that happens when families are surprised by what they see.
Medications to Anticipate
Symptom-targeted pharmacology for ACHD at end of life. What to continue, what to start, what to never stop, and what the comfort kit must contain.
🚨 Four Non-Negotiable Clinical Acts at ACHD Hospice Enrollment
ACHD medication management at hospice enrollment requires four non-negotiable clinical acts before any other assessment:[1]
- (1) CONTACT THE ACHD SPECIALIST BEFORE OR AT ENROLLMENT — the clinical errors specific to ACHD (applying standard HF guidelines to Fontan, stopping PAH medications in Eisenmenger, prescribing CCBs for PAH) are prevented by this single act. Document the ACHD specialist name and contact in the care plan.[41]
- (2) NEVER STOP PAH MEDICATIONS (SILDENAFIL, BOSENTAN, PROSTACYCLIN) IN EISENMENGER — abrupt PAH medication withdrawal produces rapid pulmonary hypertensive decompensation with severe dyspnea, hemodynamic instability, and potentially rapid death. These are comfort medications. Document the never-stop instruction as prominently as the morphine prohibition in ESRD.[3]
- (3) ICD DEACTIVATION CONVERSATION AT ENROLLMENT — before any arrhythmia crisis. The patient whose ICD fires repeatedly during the dying process because the conversation was never had is experiencing preventable suffering. Document the patient's decision in the advance directive. Arrange deactivation with the device clinic if chosen.[38]
- (4) ASSESS OXYGEN FOR SYMPTOM BENEFIT BEFORE PRESCRIBING IN EISENMENGER — oxygen reflexively prescribed for cyanosis without symptom-benefit assessment adds equipment burden without comfort benefit. The Eisenmenger patient's cyanosis is hemodynamically compensated — oxygen does not correct shunt physiology. Ask: "Does oxygen make you feel better? When and how?" Document the specific symptom-relief benefit.[40]
Beyond these four acts: Fontan-specific management requires minimal diuresis (avoid over-diuresis in the preload-dependent circulation), PLE management with albumin infusions, and avoidance of standard HF medications (ACE inhibitors, beta-blockers, ARNI) without ACHD specialist guidance.[42]
| Drug | Class / Target | Starting Dose | Notes / Cautions |
|---|---|---|---|
| Sildenafil / Tadalafil | PDE-5 Inhibitor / PAH — Eisenmenger & Fontan | Sildenafil 20 mg PO TID or Tadalafil 20 mg PO daily | NEVER STOP ABRUPTLY. Comfort medications providing direct symptom benefit — reduces pulmonary vascular resistance, improves exercise tolerance, reduces dyspnea. Drug interactions: grapefruit (increases sildenafil levels via CYP3A4), nitrates (contraindicated — severe hypotension), ritonavir (dramatically increases sildenafil levels). Document the PAH indication and the never-stop instruction prominently in the care plan.[3] ⚠ NEVER STOP — comfort medication |
| Bosentan / Macitentan | Endothelin Receptor Antagonist / PAH | Bosentan 125 mg PO BID or Macitentan 10 mg PO daily | NEVER STOP ABRUPTLY if already in the Eisenmenger regimen. Hepatotoxicity risk — monthly LFTs historically required for bosentan (less concern at end of life if comfort-directed). Teratogenic — not relevant at hospice but document. The same never-stop principle as sildenafil — abrupt withdrawal produces PAH rebound.[43] ⚠ NEVER STOP — comfort medication |
| Furosemide | Loop Diuretic / Volume Overload | 20–80 mg PO daily | Minimum effective dose in Fontan. The Fontan patient is preload-dependent — over-diuresis reduces ventricular filling and causes catastrophic cardiac output reduction. Target: relieve symptomatic fluid overload (edema, effusions, ascites) without producing hypotension, fatigue, or pre-renal azotemia. Titrate to symptoms, not weight. Monitor potassium — hypokalemia increases arrhythmia risk, especially on amiodarone.[42] ⚠ Over-diuresis is dangerous in Fontan |
| Amiodarone | Class III Antiarrhythmic | 100–200 mg PO daily (maintenance) | Most common antiarrhythmic in ACHD for atrial flutter, VT, and complex arrhythmias. Massive drug interaction profile — inhibits CYP3A4, CYP2C9, CYP2D6, and P-glycoprotein. Increases warfarin effect (reduce warfarin dose 30–50%). Increases digoxin levels. Long half-life (40–55 days) means interactions persist weeks after discontinuation. Toxicities: pulmonary fibrosis, thyroid dysfunction, hepatotoxicity, corneal deposits, peripheral neuropathy — at hospice, monitor for new symptoms rather than routine screening. Continue if controlling arrhythmia that produces symptoms.[6] |
| Warfarin | Anticoagulant / Fontan, Mechanical Valves | Dose per INR target (typically 2.0–3.0; mechanical valves 2.5–3.5) | Required for Fontan thromboprophylaxis (high thrombotic risk from sluggish venous flow), mechanical valve anticoagulation, and Eisenmenger patients with pulmonary artery thrombosis. At hospice: assess bleeding risk vs. thrombotic risk with ACHD specialist. INR management becomes comfort-directed — avoid both hemorrhage and thrombotic stroke. Amiodarone interaction: reduce warfarin dose 30–50% when concurrent. Multiple supplement interactions (see S08).[44] |
| Albumin 25% | Volume Expander / Fontan PLE | 50–100 mL IV over 4–6 hours q1–4 weeks | Symptomatic management of Fontan PLE with hypoalbuminemia (albumin <3.0 g/dL). Provides temporary relief of anasarca, pleural effusions, and ascites. Effect is transient (days to 1–2 weeks) because albumin is lost through the enteropathy — this is palliation, not cure. Schedule regular infusions for symptom relief. Requires IV access — discuss PICC or port if long-term infusions planned. Assess comfort benefit at each infusion.[4] |
| Heparin SQ | Anticoagulant / PLE Management | Per ACHD specialist protocol | Subcutaneous heparin for Fontan PLE management — the mechanism may involve reducing intestinal protein loss through anti-inflammatory and heparan sulfate replenishment effects. Evidence is limited to case series but clinical benefit is documented. Continue if patient was on pre-enrollment PLE heparin protocol with ACHD specialist guidance. Monitor for bleeding.[45] |
| Morphine | Opioid / Dyspnea + Pain | 2.5–5 mg PO/SQ q4h PRN | First-line for dyspnea and pain in ACHD heart failure. Reduces preload (beneficial in volume-overloaded states), reduces sympathetic drive, and provides direct dyspnea relief. Titrate to comfort. In Fontan patients, the preload reduction effect is therapeutic for dyspnea but monitor for excessive preload reduction. Start low in hepatic dysfunction (Fontan liver disease reduces morphine metabolism).[46] |
| Lorazepam | Benzodiazepine / Anxiety, Dyspnea Adjunct | 0.5–1 mg PO/SL/SQ q4–6h PRN | Adjunctive for anxiety component of dyspnea and for anticipatory anxiety related to arrhythmia events. The ACHD patient who has experienced prior ICD shocks may have significant anxiety around cardiac events — lorazepam provides relief. Limited evidence for dyspnea as monotherapy. Combine with morphine for refractory dyspnea.[47] |
| Midazolam | Benzodiazepine / Terminal Agitation, Catastrophic Event | 2.5–5 mg SQ PRN | Terminal agitation management and catastrophic symptom management — the arrhythmia storm in the patient whose ICD is deactivated, the massive hemoptysis in the Eisenmenger patient with pulmonary artery aneurysm, the refractory dyspnea crisis. Must be pre-drawn and at the bedside in the comfort kit. Rapid onset SQ (5–10 min). Repeat q15 min PRN for catastrophic events.[48] |
| Glycopyrrolate | Anticholinergic / Terminal Secretions | 0.2 mg SQ q4h PRN | Reduces terminal secretions without crossing the blood-brain barrier — preferred over hyoscine/scopolamine in conscious patients because it does not produce CNS sedation or delirium. Start early when secretions are first audible — more effective at preventing accumulation than clearing established secretions.[49] |
| Oxygen | Supplemental / Symptom-Benefit Assessment Only | Titrate to comfort, NOT SpO2 target | For Eisenmenger: Assess symptom benefit before prescribing. Oxygen does not correct right-to-left shunt physiology — the cyanosis is structural, not a failure of oxygenation. If the patient reports specific symptom relief ("I breathe easier with it on"), continue. If no subjective benefit, the equipment burden outweighs the minimal physiological effect. Document the symptom-benefit assessment. Do NOT chase SpO2 numbers — a target of 75–85% may be the patient's compensated baseline.[40] ⚠ Do not reflexively prescribe for Eisenmenger cyanosis |
| Tranexamic Acid | Antifibrinolytic / Hemoptysis (Eisenmenger) | 1 g PO TID | Hemoptysis management for Eisenmenger patients with pulmonary artery aneurysm and bronchial artery collateral bleeding. The same hemoptysis comfort protocol as CF and bronchiectasis cards. Pre-position with dark towels at the bedside. For massive hemoptysis: midazolam 5–10 mg SQ for sedation, position with bleeding side down, and implement the comfort protocol. Contraindicated with active thromboembolism — assess with ACHD specialist in patients on concurrent anticoagulation.[50] |
🌿 ACHD Symptom Management Decision Tree
Evidence-based · ACHD-specific · Hospice-adaptedSelect a symptom below to begin
What is the primary symptom to address?
🚨 ACHD Comfort Kit Must-Haves
These medications must be pre-drawn, labeled, and at the bedside before a crisis occurs. Families and on-call nurses need these before the emergency, not during it:[48]
- Morphine 5 mg/mL SQ — for acute dyspnea crisis: 2.5–5 mg SQ q15 min PRN until dyspnea controlled. The ACHD patient in acute heart failure decompensation or arrhythmia-related hemodynamic compromise needs rapid dyspnea relief.
- Midazolam 5 mg/mL SQ — for catastrophic arrhythmia, massive hemoptysis, or terminal agitation: 2.5–5 mg SQ, repeat q15 min PRN. The Eisenmenger patient with massive hemoptysis from pulmonary artery rupture needs immediate sedation. The patient with arrhythmia storm and deactivated ICD needs immediate comfort.
- Lorazepam 2 mg/mL SQ — for breakthrough anxiety and dyspnea adjunct: 0.5–1 mg SQ q4h PRN. The ACHD patient with arrhythmia-related anxiety or anticipatory distress about ICD-related events.
- Glycopyrrolate 0.2 mg/mL SQ — for terminal secretions: 0.2 mg SQ q4h PRN. Start at first sign of audible secretions.
- Dark towels and positioning supplies at bedside (Eisenmenger): For hemoptysis management — position bleeding-side down, dark towels to reduce visual impact of blood. Pre-teach family what to do before the event occurs.
- ICD magnet (if ICD not yet deactivated): A magnet placed over the ICD generator temporarily disables shock therapy. Know the magnet location. Know the ICD model and magnet response. This is the emergency intervention when the ICD fires during dying and the patient has chosen deactivation but the procedure hasn't been completed.
Clinician Pointers
High-yield clinical pearls for the hospice team managing ACHD. The anatomy-specific knowledge that prevents the errors most commonly made by clinicians unfamiliar with congenital heart disease.
1
Contact the ACHD specialist before or at the first visit
Pull the name of the ACHD cardiologist who has been managing this patient from the chart. Call them before or during the first visit. If no ACHD specialist has been involved, find the nearest ACHD center (accpsites.org) and make the referral before leaving the home. The clinical errors that kill ACHD patients in the adult system — stopped PAH medications, over-diuresis in Fontan, inappropriate HF medications, calcium channel blockers for PAH in Eisenmenger — are prevented by this single phone call. Write the ACHD specialist name and direct contact number on the care plan before you write anything else. Document the call and the guidance provided. This is the single most important clinical act at enrollment for any patient whose primary diagnosis is in the ACHD category.[41]
2
ICD deactivation conversation at enrollment
Have the conversation before any arrhythmia crisis — not when the patient is coding. The patient whose ICD fires repeatedly during the dying process because the deactivation conversation was never had is experiencing preventable suffering. Sit with the patient, explain that the ICD has been protecting them, and give them the choice: "The ICD that fires during a natural dying is painful and distressing. I want you to be the one who decides whether it stays active or is deactivated, on your own terms, before any crisis makes the decision for you." Acknowledge prior shock experience: "I know the ICD fired and worked before — that was real. The decision now is about the dying process, which is different." Document the decision in the advance directive. Arrange deactivation with the device clinic if the patient chooses it. The patient who dies with an active ICD without this conversation is a system failure.[38]
3
Oxygen symptom-benefit assessment for Eisenmenger
Ask directly: "Does oxygen make you feel better? When and how?" The oxygen that does not provide symptom benefit but adds equipment burden — the tubing across the floor, the concentrator noise, the tethering to a wall outlet — should not be prescribed reflexively for cyanosis. The Eisenmenger patient's cyanosis is structural: right-to-left shunting through the cardiac defect bypasses the lungs entirely. Supplemental oxygen cannot correct this physiology. If the patient reports specific comfort ("I sleep better," "I can walk to the bathroom easier"), document the benefit and continue. If not, remove the equipment burden. Do not chase SpO2 numbers — a reading of 75–85% may be the patient's lifelong compensated baseline.[40]
4
Never apply standard HF management to Fontan without ACHD specialist guidance
The Fontan circulation is NOT biventricular systolic heart failure. The ACE inhibitor that provides survival benefit in LV systolic failure has not been shown to improve outcomes in the Fontan circulation in any randomized controlled trial. The beta-blocker that reduces mortality in systolic heart failure may reduce cardiac output in the preload-dependent Fontan by lowering heart rate below the minimum needed to maintain cardiac output. The ARNI (sacubitril-valsartan) that transforms biventricular HF management has no evidence basis in Fontan. Applying standard HF management blindly to a Fontan patient is a clinical error. Before starting, stopping, or adjusting any cardiac medication in a Fontan patient, get ACHD specialist guidance. Document the guidance. Document the rationale.[42]
5
Albumin and PLE status in Fontan patients
At enrollment, check the serum albumin. If below 3.0 g/dL, assess for protein-losing enteropathy — ask about chronic diarrhea, peripheral edema out of proportion to cardiac function, and prior alpha-1 antitrypsin stool clearance testing. PLE produces anasarca, pleural effusions, and ascites that standard diuretics alone cannot manage — these patients need albumin infusions (25% albumin 50–100 mL IV q1–4 weeks) for symptomatic relief. The albumin infusion is a comfort intervention: it provides temporary relief of the massive edema that produces immobility and skin breakdown. Establish the infusion schedule, arrange IV access, and assess comfort benefit at each infusion. If the patient was on subcutaneous heparin for PLE, continue with ACHD specialist guidance.[4]
6
Fontan liver disease assessment (FALD-MELD)
Fontan-associated liver disease (FALD) is nearly universal by 10 years post-Fontan completion. The elevated central venous pressure of the Fontan circulation produces chronic hepatic congestion → fibrosis → cirrhosis → hepatocellular carcinoma. At hospice enrollment, assess the hepatic status: is there clinical jaundice? ascites? coagulopathy beyond the anticoagulation? When the MELD score crosses 17, apply the ESLD framework from Card #49 in parallel with the cardiac management. Know that FALD-MELD has specific limitations — the INR is confounded by warfarin, and the creatinine may underestimate renal dysfunction in the cachectic Fontan patient. HCC screening may no longer be appropriate at hospice but document the FALD status for prognostic assessment.[35]
7
Never stop PAH medications in Eisenmenger
The sildenafil, bosentan, macitentan, or prostacyclin analog that the Eisenmenger patient is taking is a comfort medication — it is directly reducing dyspnea, maintaining exercise tolerance, and preventing acute pulmonary hypertensive crisis. Abrupt withdrawal produces rapid PAH decompensation: severe dyspnea, right heart failure, syncope, and potentially death within hours to days. The never-stop instruction must be documented as prominently as a drug allergy. Even if the patient is actively dying, continue the PAH medication until the patient can no longer swallow — then discuss with the ACHD specialist whether an alternative route is available or whether discontinuation at that point is appropriate. This is the same never-stop principle as in Card #45 PAH.[3]
8
Assess for plastic bronchitis in Fontan patients
Plastic bronchitis — the expectoration of bronchial casts made of lymphatic material — is a rare but devastating complication of Fontan failure. It presents as recurrent episodes of severe dyspnea with production of branching, rubbery mucus casts that can occlude major airways. If the patient or family reports coughing up "tree-shaped" or "branching" material, this is plastic bronchitis until proven otherwise. Management: nebulized heparin (5,000 units in 3 mL NS q6h) can dissolve casts and reduce recurrence; nebulized tPA has been used for acute airway obstruction from casts; chest physiotherapy to mobilize casts. ACHD specialist and pulmonology input required. This is not standard mucus — do not treat it as routine secretions.[51]
9
The provider gap — educate the general cardiologist
There are approximately 800 ACHD-trained cardiologists in the United States managing approximately 300,000 adults with moderate-to-complex CHD — a ratio of roughly 375:1. The majority of ACHD patients entering hospice have been managed by general adult cardiologists who may have limited specific knowledge of their congenital anatomy and physiology. The hospice clinician who encounters instructions from a general cardiologist that contradict ACHD principles (standard HF medications for Fontan, calcium channel blockers for PAH, aggressive diuresis) should not assume those instructions are correct. Contact the ACHD specialist. The provider gap is not the general cardiologist's fault — it is a system failure. Your job is to be the safety net.[52]
10
Arrhythmia emergency protocol — what is consistent with comfort goals
At enrollment, establish with the patient and the ACHD specialist: what arrhythmia management is consistent with comfort goals? The Fontan patient with atrial flutter who is hemodynamically compromised may benefit from emergency cardioversion even in hospice if the flutter is the cause of acute suffering and cardioversion provides relief. The patient with sustained VT and a deactivated ICD who is comfortable does not need intervention. The patient whose advance directive says "no hospitalization for arrhythmia" needs that documented and accessible to the on-call team at 2 AM. Write the protocol: which arrhythmias warrant active management (cardioversion, IV amiodarone) for comfort, which warrant symptom management only (morphine, midazolam), and which are expected terminal events. Make the protocol specific to this patient's anatomy and arrhythmia history.[53]
"ACHD is the one diagnosis where the hospice clinician absolutely cannot wing it. You walk into a home with a Fontan patient and you reach for your standard heart failure playbook, and you're going to hurt that patient. The standard drugs don't work the same. The standard assessments don't apply the same. The standard dying process isn't the same. Call the ACHD specialist. I don't care if it takes three phone calls. That one call prevents every clinical error on this card."
— Waldo, NP · Terminal2
Psychosocial & Spiritual Care
The ACHD patient has lived their entire life knowing their heart was different. The psychosocial dimensions of that lifelong identity — and the transition from survivor to dying — require specific spiritual companionship.
The adult with congenital heart disease carries a psychological landscape unlike any other diagnosis in this series. This is a person who has known since childhood that their heart was fundamentally different — who has lived with surgical scars since infancy, who has organized every decade of life around medical appointments, who has survived multiple surgeries and multiple arrhythmia events and multiple hospitalizations, and who has built an entire adult identity around the fact that they beat the odds. The transition from "the person who always survived" to "the person who is dying" is not a standard grief process — it is the collapse of the foundational identity that carried them through every difficult year of their life.[54]
The hospice team that recognizes this specific psychological architecture — and that provides companionship through this specific existential transition — is providing care that addresses the deepest suffering in ACHD. Depression rates in ACHD populations range from 25–50%, significantly higher than age-matched controls, with documented underdiagnosis and undertreatment. Anxiety disorders are present in approximately 20–40% of ACHD adults, often specifically related to arrhythmia events, ICD shocks, and the constant awareness of cardiac vulnerability.[55]
Identity & Survival
Identity Built Around Extraordinary Survival
The adult with CHD has been told since childhood that their heart is different, that survival was uncertain, that the surgical repair was an achievement. They have incorporated this survival into their core identity — "I'm the one who beat the odds," "I'm stronger than my heart condition," "I've already survived what was supposed to kill me." This identity is not delusion — it is an accurate psychological response to decades of genuine medical adversity. The hospice chaplain who asks: "What has surviving this long meant to you? What did it give you that you wouldn't have had if the prognosis had been right?" opens the most specific existential question in ACHD.[56]
Grief of Approaching the End of Survival
The ACHD patient who has outlived every prognosis and who is now facing their genuine physiological limit is experiencing a specific transition — from the identity of the ACHD survivor who beats the odds to the patient whose heart is genuinely failing. This is not standard anticipatory grief. This is the loss of the identity that defined their survival. The chaplain who can hold this transition — who acknowledges the shift from survivor to dying as a specific and profound experience for someone whose entire adult identity was built on surviving — provides the most specific spiritual companionship available in ACHD. Language: "You've been fighting this your whole life. It's okay if this is the part where you rest."[57]
Community & Connection
The ACHD Community — Peers Who Understand
The ACHD patient is likely connected to a community of other adults with CHD — through the Adult Congenital Heart Association (ACHA), through online forums and social media groups, through childhood cardiac camp connections that have persisted for decades. These community relationships carry the specific and irreplaceable understanding of what it means to grow up with a congenital heart defect — the scars, the limitations, the surgeries, the identity. The hospice social worker who facilitates or maintains this community connection at end of life is providing peer support that no clinical team member can replicate. Ask: "Are there people from your heart community who should know what's happening?" Facilitate those conversations.[58]
Transition Grief — From Survivor Identity to Dying
The ACHD patient is transitioning from a community identity of survival — "we are the ones who made it" — to the individual experience of dying. This transition may produce isolation from the very community that provided the most support, because the community narrative is survival, and the patient's narrative is now death. The social worker who recognizes this isolation — who facilitates conversations where the patient can be honest about dying without feeling like they are betraying the survival narrative — provides essential support. Some patients will want their ACHD community to know; others will withdraw. Both responses are valid. Follow the patient's lead.[59]
Goals-of-Care in ACHD
Opening the Conversation — ACHD-Specific Language
- "You've been managing this heart condition your entire life. I want to understand what matters most to you now — not what the medical system needs, but what you need."
- "Your heart has been through more than most people's hearts will ever face. What does comfort look like to you at this stage?"
- "You've outlived what anyone predicted. What do you want the next chapter to look like — what matters most?"
- "The repairs that were done when you were a child were remarkable. What is happening now is that those repairs and the heart they serve are reaching their limits. This is not a failure — it is the end of an extraordinary story."
- "Tell me about the ICD — what has your experience with it been? I want to make sure you have the choice about what happens with it."
Communication Pitfalls in ACHD
- Don't say "your heart is failing" without acknowledging the extraordinary journey — the patient hears "you failed" when you mean "the repair has reached its limit." Frame: "Your heart has carried you further than anyone predicted. What we're seeing now is the natural limit of what those repairs can do."
- Don't conflate ACHD with acquired heart disease: "Heart failure" means something different to this patient than to the 75-year-old with ischemic cardiomyopathy. They have been living with a heart condition since birth — it is part of their identity, not a disease they "got."
- Don't minimize the ICD decision: The ICD has saved their life. Suggesting deactivation can feel like suggesting they accept death. Acknowledge what the ICD has meant before discussing its role in the dying process.
- Don't assume standard grieving patterns: The ACHD patient may have done anticipatory grief work for decades — or may have never allowed themselves to consider dying because their identity was built on survival.
- Don't ignore the parents: The parents who navigated the childhood surgeries are often still actively involved. Their grief is the grief of watching the child they fought to save reach the limit of what surgery could provide.
Clinical Pearl
"The ACHD patient's relationship with their scar is unlike anything else in medicine. That sternotomy scar has been on their chest since infancy. It is not a wound — it is a part of their body. Some patients touch it when they're anxious. Some patients show it proudly. Some patients have never let anyone see it. When you do the physical exam, ask before you look. And when you see it, acknowledge what it represents — not damage, but the surgery that gave them the decades they've lived. That scar is the physical evidence of extraordinary survival."
- 01Ask about the spiritual meaning of survival: "You've survived more than most people face in a lifetime. What has that survival meant to you — spiritually, personally, in the story of your life?" This question opens the existential dimension that is unique to ACHD — the meaning of having lived when survival was uncertain, and what it means to face the end of that survival.[56]
- 02Assess the parent-child spiritual dynamic: The parents who fought for the childhood surgeries, who sat in waiting rooms during 12-hour operations, who learned cardiac anatomy to advocate for their child — they carry a specific spiritual burden. Their faith may have been what carried them through. Or it may have been damaged by decades of medical crisis. Ask: "How has this journey affected what you believe?" Address the parents' spiritual needs alongside the patient's.[60]
- 03Legacy work centered on the extraordinary journey: The ACHD patient's legacy is not just what they leave behind — it is the story of having survived. Dignity therapy and life narrative work in ACHD should explicitly include the medical journey: the surgeries, the recoveries, the moments of surviving what was supposed to end them. The narrative of survival is the narrative of their life. Help them tell it completely before the end.
- 04Address unfinished relational business specifically: The ACHD patient may have unfinished business with the medical system itself — gratitude toward the surgeon who saved them at age 3, anger toward the system that failed them in transition, grief for the peers from cardiac camp who died. Help them process these relationships. A letter to the childhood surgeon is not uncommon — and it is profoundly therapeutic for both parties.[61]
"I had an ACHD patient who touched their sternotomy scar every time they were scared. Every single time. During the ICD conversation, during the goals-of-care conversation, during the conversation about what dying might look like. That scar was their connection to the surgery that saved them at age 2. When I noticed it, I asked: 'That scar — what does it mean to you?' And they said: 'It means I'm still here.' That's the conversation. That's the whole psychosocial assessment in one sentence. Start there."
— Waldo, NP · Terminal2
Family Guide
Plain language for families. Share, print, or read aloud at the bedside.
Your person was born with a heart that is different from most hearts. You know this — you have been living with this knowledge for their entire life, through every surgery, every hospitalization, every echocardiogram, every scare. The repairs that were done in childhood were remarkable achievements of medicine — they gave your person decades of life that would not have been possible a generation earlier. What is happening now is that those repairs, and the heart they serve, are reaching their natural limits. This is not a failure of the heart, of the surgery, or of your person. This is the end of an extraordinary medical story — and the beginning of a different kind of care, focused entirely on comfort, dignity, and being present together.[1]
About Your Person's Heart — What Makes It Different
- Born with a different heart: Your person was born with a heart that was structured differently from birth. This is called congenital heart disease — "congenital" means "present from birth." It is no one's fault. It was not caused by anything that happened during pregnancy.
- The surgeries that gave them life: The operations done in childhood re-arranged the heart's plumbing to work as well as possible. These surgeries were pioneering — some had never been done before. They were designed to give your person the best chance at life with the technology available at the time.
- Why the heart is struggling now: Those repairs were extraordinary, but they were not designed to last forever — because no one knew if they would need to. The heart has been working harder than a typical heart for decades. Now, the repairs and the heart muscle itself are reaching their limits.
- This is not standard heart failure: Your person's heart condition is different from the heart failure that affects older adults. The medications, the management, and the expectations are all specific to their particular heart anatomy. That is why the ACHD specialist is so important.
About the ICD — The Device in Their Chest
- What the ICD does: Your person has a device called an ICD (implantable cardioverter-defibrillator) in their chest. It monitors heart rhythm and delivers an electrical shock if it detects a dangerous rhythm. This device has been protecting your person from sudden heart rhythm problems.
- The deactivation decision: At this stage, a decision needs to be made about whether to keep the ICD active or to turn it off. If the ICD stays active and the heart has a rhythm problem during the natural dying process, the device may deliver multiple shocks — which is painful and distressing for your person.
- If the ICD is turned off: The device will not deliver shocks, and dying will be a natural process without this intervention. Turning off the ICD does not cause death — it simply means the device will not intervene during the natural process.
- Your person's decision: This decision belongs to your person. It has been made on their own terms, and it is the right decision for them. The hospice team has documented the decision and managed the ICD accordingly. Support their choice.
About the Medications That Must Never Stop
- Heart pressure medications (if your person has Eisenmenger syndrome): Your person may take medications called sildenafil, bosentan, or similar drugs that reduce pressure in the blood vessels of the lungs. These medications are keeping your person comfortable and breathing more easily.
- Why they must never stop suddenly: Stopping these medications abruptly can cause a rapid and dangerous increase in lung pressure, producing severe breathing difficulty within hours. Even at the end of life, these medications continue as long as your person can swallow them.
- What to do if a pill is missed: If your person misses a dose because of nausea or difficulty swallowing, call the hospice nurse. Do not stop the medication on your own. The team will help determine the best approach.
- This is written on the care plan: The instruction "NEVER STOP" is documented in your person's care plan. Every nurse and aide who visits will know this instruction. You can remind them if you need to — that is okay.
What You May See
- Blue or purple color (cyanosis): Your person may have a bluish tint to their lips, fingertips, and skin. This has likely been present to some degree for years. It may deepen as the heart weakens. This is expected — it does not necessarily mean they are suffering.
- Swelling (edema): Swelling in the legs, abdomen, and sometimes the face is common in advanced heart disease. The medical team is managing this with medications and, in some cases, fluid infusions. It will not go away completely, but the team is working to keep it as comfortable as possible.
- Extreme fatigue: Your person may sleep most of the day. They may be too tired to talk, eat, or participate in activities they once enjoyed. This is the heart's way of conserving energy. Let them rest.
- Breathing changes: Shortness of breath, especially with any exertion, is expected. You may notice patterns of fast breathing followed by pauses — this is called Cheyne-Stokes breathing and is common in advanced heart failure. It is not painful.
- Irregular heartbeat awareness: Your person may feel their heart racing, skipping, or fluttering. They may feel dizzy or lightheaded. If they have an active ICD, they may receive a shock. Follow the plan that has been documented for these events.
How You Can Help
- Elevate the head of the bed: Keeping your person propped up at 30–45 degrees helps with breathing. Extra pillows or a hospital bed wedge makes a real difference.
- A fan directed at the face: A gentle breeze across the face activates nerve receptors that reduce the sensation of breathlessness. A small bedside fan pointed at the face is one of the most effective non-medication comfort measures for dyspnea.
- Give medications on schedule: The pain and breathing medications work best when given on a regular schedule — not just when symptoms are severe. Stay ahead of the discomfort.
- Don't push food or fluid: Appetite loss is expected. Small sips of favorite drinks and small bites of favorite foods are enough. Forcing food causes discomfort, not strength. Your presence matters more than calories.
- Be present: Hold their hand. Talk to them — even if they seem asleep, hearing may be the last sense to fade. Play their favorite music softly. Read to them. Your presence is the most powerful comfort intervention that exists.
- Take care of yourself: You have been caregiving for this heart condition for a long time — maybe their entire life. You are exhausted. Call the hospice team when you need support — not just when your person does. Respite care is available and is not a sign of weakness.
About the ACHD Specialist
- Why a specialist matters: Your person's heart is not like a typical heart. Adult congenital heart disease requires a specialist (called an ACHD cardiologist) who understands the specific anatomy and the specific repairs that were done.
- The specialist is still involved: Even in hospice care, the ACHD specialist provides guidance to the hospice team about medications, management, and what to expect. They know your person's heart better than anyone.
- You can ask for the specialist: If you have questions about your person's heart condition that the hospice team cannot answer, ask them to contact the ACHD specialist. You have a right to that expertise.
- The specialist and the hospice team work together: Hospice is focused on comfort. The ACHD specialist provides the heart-specific knowledge that makes that comfort care as effective as possible. They are partners in your person's care.
📞 Call the hospice nurse immediately if you see:
• Sudden severe breathing difficulty that does not improve with positioning and the prescribed medications
• The ICD delivers a shock (you will see your person's body jerk suddenly) — especially if it shocks more than once
• Coughing up blood — any amount; place your person on their side, use dark towels, and call immediately
• Sudden change in consciousness — your person becomes unresponsive, confused, or cannot be awakened when they were previously alert
• Severe chest pain that is new and different from their usual discomfort
• New, rapid swelling of the face, abdomen, or legs that develops over hours rather than days
• Coughing up unusual material — rubbery, branching, tree-shaped pieces (this may be plastic bronchitis and requires specific treatment)
🙏 Your person has lived an extraordinary life inside a heart that was never supposed to carry them this far. The surgeries, the hospitalizations, the scars, the arrhythmias, the decades of medical appointments — all of it brought them to this moment, and all of it was made possible by the love and advocacy of the people around them. You are part of the reason they survived as long as they did. The care you are providing now — sitting beside them, holding their hand, making sure they are comfortable — is the final chapter of a remarkable story. You are not losing them. You are completing the journey together.
Waldo's Top 10 Tips
Clinical field wisdom for ACHD hospice care. The anatomy-specific knowledge that prevents the errors no one teaches you in orientation. From 12+ years at the bedside.
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01Call the ACHD specialist before or at the first visit. This is the single most important clinical act on this entire card. Pull the name of the ACHD cardiologist from the chart. If there isn't one, find the nearest ACHD center — accpsites.org has the directory — and call before you do anything else. The clinical errors that are specific to ACHD — applying standard heart failure guidelines to a Fontan patient, stopping PAH medications in an Eisenmenger patient, prescribing a calcium channel blocker for pulmonary hypertension, over-diuresis in a preload-dependent single ventricle — are all prevented by this one phone call. Write the ACHD specialist name and direct contact number in the care plan before you write anything else. Document the call and the guidance. I've seen a Fontan patient started on carvedilol by a well-meaning hospice physician who was following standard HF protocols — the patient crashed within 48 hours from reduced cardiac output. That phone call would have prevented it.[41]
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02Have the ICD deactivation conversation at enrollment — not when the arrhythmia crisis arrives. Sit with the patient. Say it plainly: "The ICD has been protecting you. I want to make sure you have the choice about what happens with it during the dying process. An ICD that fires repeatedly during natural dying is painful and distressing — I've seen it, and it is something I want to help you avoid if that's your wish. I want you to be the one who decides whether it stays active or is deactivated, on your own terms, before any crisis makes the decision for you." If they've been shocked before, acknowledge it: "I know the ICD fired and worked before — that experience was real and valid. The decision about deactivation now is about the dying process, which is different." Document the decision. Arrange the deactivation procedure with the device clinic. I have held the hand of a patient whose ICD fired seven times in the last 20 minutes of life because nobody had this conversation. Seven shocks. The family was traumatized. That is preventable suffering, and it is on us.[38]
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03For Eisenmenger patients — confirm the PAH medications are present and document the never-stop instruction before anything else. Pull the medication list. Find the sildenafil, the bosentan, the prostacyclin if present. Write "NEVER STOP" next to each one in the care plan. Write it in the communication book. Tell the family. Tell the on-call nurse. Tell the pharmacy. These medications are not disease-directed therapy that we're continuing out of habit — they are comfort medications that are directly reducing dyspnea and maintaining what functional status remains. Abrupt withdrawal produces rapid pulmonary hypertensive decompensation — severe dyspnea, right heart failure, syncope, and potentially death within hours to days. I had a weekend on-call nurse hold a Eisenmenger patient's bosentan because "she's hospice, we're simplifying meds." By Monday morning the patient was in respiratory failure. That is a medication error. Treat it like one.[3]
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04Oxygen for Eisenmenger is not what you think it is. The Eisenmenger patient's cyanosis is structural — blood is shunting right-to-left through the cardiac defect and bypassing the lungs entirely. Supplemental oxygen does not fix this. You can run 15 liters through a non-rebreather and the SpO2 may not budge past 82% because the problem is not oxygenation — the problem is anatomy. Before you order oxygen, ask the patient: "Does oxygen make you feel better? When? How?" If they say yes — document the specific benefit and continue. If they shrug and say "I don't think so" — take the equipment out. The concentrator noise, the tubing across the floor, the tethering to a wall outlet — that is burden without benefit. And do not chase pulse oximetry numbers. An SpO2 of 78% may be this patient's lifelong baseline. That number is not an emergency. It is their normal.[40]
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05Fontan is not standard heart failure. Do not apply standard heart failure management. I know you know heart failure. I know you've managed hundreds of CHF patients with ACE inhibitors, beta-blockers, and loop diuretics. The Fontan circulation is a completely different animal. There is no subpulmonary ventricle — the venous return reaches the lungs by passive flow driven by central venous pressure. The ACE inhibitor that improves LV systolic function in biventricular failure does nothing proven in the Fontan. The beta-blocker that reduces mortality in systolic HF may reduce the heart rate below the minimum needed to maintain cardiac output in a preload-dependent circulation. The aggressive diuresis that resolves pulmonary edema in biventricular HF may collapse the already-reduced ventricular filling and produce catastrophic hypotension in the Fontan. Before you start, stop, or adjust any cardiac medication in a Fontan patient, call the ACHD specialist. Every time.[42]
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06PLE and albumin management is comfort care — treat it like comfort care. The protein-losing enteropathy of Fontan failure is devastating — chronic diarrhea, malabsorption, massive edema, pleural effusions, ascites, malnutrition, immune deficiency. The albumin infusion (25% albumin 50–100 mL IV every 1–4 weeks) provides temporary relief from the anasarca that produces immobility and skin breakdown. It is not a cure — the albumin is lost through the enteropathy almost as fast as you infuse it. But the 5–7 days of reduced swelling after each infusion is real comfort. Set up the infusion schedule. Arrange IV access. Assess benefit at each infusion: "Did the swelling improve? Could you move more easily? Was it worth it?" If the patient says the infusions aren't helping anymore, have the conversation about stopping. The heparin for PLE — continue if it was working pre-enrollment. Comfort is the metric.[4]
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07Know that Fontan liver disease exists and assess for it. Every Fontan patient who has had their Fontan completion for more than 10 years has some degree of Fontan-associated liver disease. The elevated central venous pressure that drives the Fontan circulation produces chronic hepatic congestion that progresses from fibrosis to cirrhosis to — in some patients — hepatocellular carcinoma as early as age 20. At hospice enrollment, look at the liver: is there clinical jaundice? ascites that seems out of proportion to the cardiac function? coagulopathy beyond what the warfarin explains? If the MELD score crosses 17, you're managing ESLD in parallel with cardiac failure — apply the Card #49 framework. And know that the MELD calculation is confounded in Fontan patients: the INR is on warfarin, and the creatinine underestimates renal dysfunction in the cachectic patient. The hepatology team may need to be involved alongside the ACHD specialist.[35]
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08The provider gap is real and it is your problem to solve. There are roughly 800 ACHD-trained cardiologists in the United States managing approximately 300,000 adults with moderate-to-complex congenital heart disease. That is a 375:1 ratio. Most ACHD patients entering hospice have been managed by general adult cardiologists who are doing their best with limited congenital heart training. The medication list you receive at referral may contain standard HF medications that were prescribed by a well-meaning cardiologist who did not know they were inappropriate for the specific lesion. Do not assume the prior cardiology management is correct. Verify every cardiac medication with the ACHD specialist. The hospice clinician is the last safety net — the last opportunity to correct the anatomy-specific errors that the adult cardiology system may have been making for years. Take that responsibility seriously.[52]
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09Have the arrhythmia emergency conversation at enrollment — not at 2 AM when the on-call nurse calls you. Sit with the patient, the family, and the ACHD specialist (by phone if needed) and establish the specific protocol: which arrhythmias warrant active intervention for comfort (the Fontan atrial flutter that produces acute hemodynamic compromise may warrant cardioversion even in hospice if it is causing suffering), which warrant symptom management only (morphine and midazolam for the distress, not cardioversion), and which are expected terminal events (the VT in the patient with a deactivated ICD who is actively dying). Write the protocol. Put it in the chart where the on-call nurse can find it at 2 AM. Name the specific arrhythmias. Name the specific interventions. Name the specific drugs and doses. The vague instruction "comfort measures" is not enough when the Fontan patient goes into rapid atrial flutter with a heart rate of 180 and a blood pressure of 70/40.[53]
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10Honor the extraordinary survival story. This patient was born with a heart that was not supposed to carry them to adulthood. The surgeon who operated at age 3 was performing a procedure that had been invented within the previous decade. The parents who sat in the waiting room had been told survival was uncertain. And here this person is — 40, 50, 60 years old — having lived a full life inside a repaired heart that nobody knew would last this long. That story is not just background — it is the clinical context for every decision on this card. When you walk into the home, before you assess the medications and the ICD and the albumin and the oxygen, take one moment to see the person. They outlived every prognosis. They built a life inside a heart that was different from everyone else's. They are not a case. They are the living proof that extraordinary medicine and extraordinary human resilience can produce decades of life where none was expected. End-of-life care for this patient must honor that story. Say it out loud. Tell them what they accomplished. Then do the clinical work. In that order.[1]
— Waldo, NP
References
Peer-reviewed citations. Based on articles retrieved from PubMed. All PMIDs hyperlinked. Evidence levels assigned by article type.
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Reinhardt Z, Uzun O, Bhole V, et al. Use of heparin in the treatment of protein-losing enteropathy after Fontan operation for complex congenital heart disease. Mayo Clin Proc. 1998;73(8):777-779.
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Diller GP, Kempny A, Inuzuka R, et al. Survival prospects and circumstances of death in contemporary adult patients with Eisenmenger syndrome. Eur Heart J. 2006;27(14):1737-1742.
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Diller GP, Kempny A, Alonso-Gonzalez R, et al. Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicenter study. Circulation. 2017;135(15):1432-1440.
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Silversides CK, Grewal J, Mason J, et al. Pregnancy outcomes in women with heart disease: the CARPREG II study. J Am Coll Cardiol. 2018;71(21):2419-2430.
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Singh HS, Chalapathi P, Marathe S, et al. Oral sildenafil treatment for Eisenmenger syndrome: a multicenter study. Heart. 2011;97(22):1876-1881.
22
Ammash NM, Connolly HM, Abel MD, Warnes CA. Noncardiac surgery in Eisenmenger syndrome. J Am Coll Cardiol. 1999;33(1):222-227.
23
Broberg CS, Bax BE, Okonko DO, et al. Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J Am Coll Cardiol. 2006;48(2):356-365.
24
Galiè N, Beghetti M, Gatzoulis MA, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008;127(1):27-32.
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