Palliative Care in Advanced Congenital Heart Disease: A Comprehensive Clinical Review

 Palliative Care in Advanced Congenital Heart Disease: A Comprehensive Clinical Review

Authors: Dr. Shekhar Ingle and Team, Doctor’s Forum for All 🏥

Disclaimer: This article is for educational and clinical reference purposes only. It does not replace individualized clinical judgment, local protocols, or specialist consultation. Medication doses should be verified with current formularies and adjusted to patient-specific factors.

Abstract

Congenital heart disease (CHD) encompasses a wide spectrum of structural cardiac abnormalities present at birth. Advances in paediatric cardiology and cardiac surgery have dramatically improved survival, with over 90% of children born with CHD now reaching adulthood. However, many patients with complex CHD face progressive heart failure, arrhythmias, cyanosis, pulmonary hypertension, and multi-organ dysfunction as they age. Palliative care is an essential component of comprehensive care for patients with advanced congenital heart disease, providing symptom management, psychosocial support, advance care planning, and end-of-life care. This article provides a detailed, clinically verified review of palliative care principles and symptom management in advanced CHD.

1. Introduction

Congenital heart disease affects approximately 8–10 per 1,000 live births worldwide, making it the most common congenital anomaly [1]. The spectrum ranges from simple lesions (e.g., atrial septal defect, small ventricular septal defect) that may close spontaneously or require minimal intervention, to complex lesions (e.g., hypoplastic left heart syndrome, transposition of great arteries, single ventricle physiology) requiring multiple palliative surgeries.

Despite remarkable surgical advances, many patients with complex CHD develop progressive complications including:

· Chronic heart failure

· Pulmonary hypertension

· Cyanosis and polycythaemia

· Arrhythmias (atrial and ventricular)

· Thromboembolic events

· Infective endocarditis

· Hepatic and renal dysfunction

· Cognitive and psychosocial challenges

The Fontan circulation, created for single ventricle physiology, represents a unique palliative state with inevitable long-term complications [2]. Patients with Eisenmenger syndrome, uncorrected cyanotic heart disease, or failing Fontan physiology often have a limited life expectancy and high symptom burden.

Palliative care should be integrated early and provided alongside ongoing cardiology management, not reserved for the terminal phase.

2. Disease Trajectory and Triggers for Palliative Care Referral

Palliative care referral should be considered when:

· Complex CHD with progressive heart failure (NYHA Class III–IV).

· Failing Fontan circulation with protein-losing enteropathy, plastic bronchitis, or liver disease.

· Eisenmenger syndrome with progressive cyanosis and functional decline.

· Recurrent hospitalisations (>2 per year) for heart failure, arrhythmias, or infections.

· Inotrope dependence or consideration of mechanical circulatory support/transplant.

· Recurrent arrhythmias with ICD shocks.

· Progressive renal or hepatic dysfunction.

· Significant cognitive impairment, frailty, or cachexia.

· Patient or family request for symptom management or goals-of-care discussion.

· Limited life expectancy (e.g., 6–12 months).

3. General Principles of Palliative Care in Advanced CHD

1. Concurrent care: Palliative care should be provided alongside ongoing cardiology management, including heart failure therapy, antiarrhythmics, anticoagulation, and consideration of advanced therapies.

2. Interdisciplinary team: Congenital cardiologist, palliative care physician, cardiac nurse specialist, pharmacist, physiotherapist, dietitian, psychologist, social worker, and chaplain.

3. Holistic assessment: Regular evaluation of physical, psychological, social, and spiritual needs.

4. Symptom-driven approach: Prioritise patient-reported outcomes and quality of life.

5. Communication and advance care planning: Discuss prognosis, goals, and preferences early, including ICD deactivation, place of death, and preferences for resuscitation.

6. Family-centred care: Recognise the lifelong journey of patients and families with CHD; provide support across the lifespan.

7. Medication review: Simplify polypharmacy; discontinue medications that do not provide immediate symptom benefit in the terminal phase [3].

4. Symptom Assessment and Management

4.1 Dyspnoea

Dyspnoea is the most common and distressing symptom in advanced CHD, resulting from heart failure, pulmonary hypertension, cyanosis, arrhythmias, or pleural effusions.

Management:

· Optimise heart failure therapy:

  · Diuretics for fluid overload.

  · ACE inhibitors/ARBs or sacubitril/valsartan for ventricular dysfunction (caution in Fontan circulation).

  · Beta-blockers (carvedilol, bisoprolol) for ventricular dysfunction.

  · Mineralocorticoid receptor antagonists (spironolactone).

  · SGLT2 inhibitors (empagliflozin, dapagliflozin) for heart failure with reduced ejection fraction [4].

· Oxygen: Only if hypoxaemia (SpO₂ ≤ 90%). In cyanotic CHD, baseline oxygen saturation may be chronically low; oxygen therapy should be individualised.

· Opioids: Low-dose oral or subcutaneous morphine (e.g., 2.5–5 mg every 4 hours as needed) is effective for refractory dyspnoea [5]. Start low and titrate slowly, especially in cyanotic patients.

· Benzodiazepines: May be used for associated anxiety or panic; use cautiously.

· Non-pharmacological: Fan therapy, cool air, positioning, pursed-lip breathing, and energy conservation.

· Treat pleural effusions: Thoracentesis or indwelling pleural catheter for recurrent effusions.

4.2 Fatigue and Weakness

Fatigue is pervasive in advanced CHD due to low cardiac output, cyanosis, anaemia, deconditioning, depression, and medication side effects.

Management:

· Treat reversible causes: Anaemia (iron replacement if deficient), thyroid dysfunction, depression, and medication side effects.

· Exercise: Supervised cardiac rehabilitation or graded physical activity improves functional capacity and quality of life [6].

· Energy conservation: Prioritise activities, use assistive devices, and plan rest periods.

· Psychological support: Cognitive behavioural therapy and counselling.

· Corticosteroids: Short-term dexamethasone (4–8 mg daily) may improve energy and appetite in terminal phase, but evidence is limited.

4.3 Pain

Pain in advanced CHD may arise from chest wall strain, musculoskeletal problems, liver capsule distension (hepatic congestion), or comorbid conditions.

Management:

· WHO analgesic ladder: Paracetamol first-line. NSAIDs should be used cautiously due to renal impairment risk and potential interaction with anticoagulants.

· Opioids: Morphine or oxycodone for moderate-to-severe pain. Fentanyl patches may be preferred in stable renal function.

· Neuropathic pain: Gabapentin or pregabalin; amitriptyline for neuropathic pain but caution with cardiac arrhythmias.

· Non-pharmacological: Physiotherapy, TENS, heat/cold, and cognitive behavioural therapy.

4.4 Cyanosis and Polycythaemia

Chronic cyanosis in uncorrected or palliated cyanotic CHD leads to secondary polycythaemia, hyperviscosity, and increased thrombotic risk.

Management:

· Hydration: Encourage adequate oral fluids to prevent dehydration and hyperviscosity.

· Phlebotomy: Routine phlebotomy is not recommended. It should be reserved for symptomatic hyperviscosity (headache, dizziness, visual disturbances) with haematocrit >65%, and performed with volume replacement [7].

· Iron deficiency: Treat with oral iron, as iron deficiency causes microcytic polycythaemia and increases stroke risk.

· Anticoagulation: Indicated in selected patients with atrial fibrillation, mechanical valves, or previous thromboembolism, but bleeding risk must be assessed.

4.5 Arrhythmias

Arrhythmias are common in advanced CHD and may be atrial (atrial fibrillation, atrial flutter, intra-atrial re-entrant tachycardia) or ventricular (ventricular tachycardia, ventricular fibrillation).

Management:

· Atrial arrhythmias: Rate control with beta-blockers or digoxin; rhythm control with amiodarone or catheter ablation in selected patients. Anticoagulation for atrial fibrillation based on risk scores.

· Ventricular arrhythmias: Beta-blockers, antiarrhythmics, ICD placement for secondary prevention. ICD shocks can be distressing; discuss deactivation in advanced disease.

· Palliative approach: In the terminal phase, antiarrhythmics may be continued if they provide symptom benefit, but ICD deactivation should be discussed to prevent painful shocks.

4.6 Protein-Losing Enteropathy and Plastic Bronchitis (Fontan Circulation)

These are devastating complications of Fontan circulation associated with poor prognosis.

Protein-losing enteropathy (PLE):

· Characterised by loss of protein in the stool, leading to hypoalbuminaemia, oedema, ascites, and malnutrition.

· Management: Diuretics, albumin infusions, budesonide (oral), low-fat diet, and consideration of Fontan revision or transplantation.

· Palliative care: Symptom management, nutritional support, and family counselling.

Plastic bronchitis:

· Characterised by formation of thick, rubbery casts in the airways, causing airway obstruction and severe dyspnoea.

· Management: Bronchoscopic removal of casts, inhaled tissue plasminogen activator (tPA), mucolytics, and aggressive chest physiotherapy.

· Palliative care: Opioids and benzodiazepines for distressing dyspnoea and anxiety.

4.7 Depression and Anxiety

Depression and anxiety are common in adults with CHD, affecting 30–50% of patients, and are associated with worse quality of life and outcomes.

Management:

· Screening: Use PHQ-9 or HADS regularly.

· Pharmacotherapy: SSRIs (sertraline, citalopram, escitalopram) are first-line and generally safe. Avoid tricyclic antidepressants due to cardiotoxicity.

· Psychotherapy: Cognitive behavioural therapy, supportive counselling, and mindfulness.

· Anxiety: Short-term benzodiazepines for acute anxiety; consider buspirone or SSRIs for chronic anxiety.

· Support groups: CHD patient associations provide peer support.

4.8 Nausea, Anorexia, and Cachexia

Nausea may result from hepatic congestion, medications, or gastroparesis. Cardiac cachexia is associated with poor prognosis.

Management:

· Nausea: Treat reversible causes (constipation, medication side effects). Use metoclopramide (if no prolonged QT), ondansetron, or haloperidol.

· Anorexia: Small frequent meals, oral nutritional supplements, and appetite stimulants. Megestrol acetate may improve appetite but increases thromboembolic risk; use cautiously.

· Corticosteroids: Dexamethasone 4–8 mg daily can improve appetite and wellbeing in advanced disease.

· Hydration: Encourage oral fluids unless fluid-restricted.

4.9 Constipation

Common due to reduced mobility, low fibre intake, dehydration, and medications (opioids, diuretics, iron).

Management:

· Prophylaxis with opioids: Prescribe laxatives (e.g., macrogol, senna) routinely.

· Titrate laxatives to achieve regular soft stools.

· Avoid bulk-forming agents if fluid restriction or mechanical obstruction.

· Treat faecal impaction with suppositories or enemas if needed.

4.10 Sleep Disturbance

Dyspnoea, orthopnoea, nocturia, arrhythmias, and depression disrupt sleep.

Management:

· Treat underlying causes: Optimize dyspnoea control, diuretics and timing, pain control, and manage depression.

· Sleep hygiene: Regular schedule, avoid caffeine and alcohol.

· Screen for obstructive sleep apnoea, which may coexist.

· Non-benzodiazepine hypnotics: Short-term zolpidem or melatonin may be used; avoid long-term benzodiazepines.

4.11 Cognitive Impairment

Cognitive impairment may occur due to chronic hypoxaemia, stroke, or comorbidities.

Management:

· Screen for delirium and dementia.

· Treat delirium: Identify and correct causes (infection, hypoxia, medications, urinary retention). Haloperidol or risperidone may be used for agitation.

· Support family with decision-making and provide a calm environment.

5. Special Considerations in Specific CHD Conditions

5.1 Fontan Circulation

Fontan circulation is a palliative surgical strategy for single ventricle physiology. Long-term complications include:

· Heart failure

· Arrhythmias

· Thromboembolism

· Protein-losing enteropathy

· Plastic bronchitis

· Liver dysfunction (Fontan-associated liver disease)

· Renal dysfunction

Palliative approach:

· Optimise heart failure therapy.

· Manage arrhythmias.

· Anticoagulation for thromboembolism prevention.

· Manage PLE and plastic bronchitis as above.

· Monitor liver function; consider liver transplantation in selected patients.

· Discuss transplant candidacy early; provide palliative care while awaiting or if not a candidate.

5.2 Eisenmenger Syndrome

Eisenmenger syndrome results from uncorrected left-to-right shunt leading to severe pulmonary hypertension and cyanosis.

Management:

· Pulmonary vasodilators: Bosentan, sildenafil, or riociguat improve symptoms and survival [8].

· Oxygen: Long-term oxygen if hypoxaemic.

· Avoid dehydration and high altitude.

· Phlebotomy: Only for symptomatic hyperviscosity.

· Contraception: Pregnancy is high-risk and should be discouraged.

· Palliative care: Symptom management, advance care planning, and end-of-life care.

5.3 Hypoplastic Left Heart Syndrome (HLHS)

HLHS requires staged palliative surgery (Norwood, Glenn, Fontan). Long-term survival is improving but remains limited.

Palliative approach:

· Support families through staged surgeries.

· Discuss prognosis and goals of care at each stage.

· Provide psychosocial support for parents and patients.

· Manage heart failure, arrhythmias, and other complications.

6. Device Therapy and Advanced Interventions

6.1 Implantable Cardioverter-Defibrillators (ICDs)

ICDs are used for prevention of sudden cardiac death in selected patients with CHD.

· Discuss deactivation early after implantation and revisit at each clinical deterioration.

· Deactivation does not hasten death; it prevents painful shocks and allows natural death.

· Procedure: Arrange with cardiology/device clinic.

6.2 Pacemakers

Pacemakers are common in CHD. Deactivation is more complex and ethically nuanced; usually continued as it does not cause discomfort.

6.3 Mechanical Circulatory Support and Transplantation

Ventricular assist devices (VADs) and heart transplantation are options for selected patients with end-stage CHD. Palliative care should be provided while awaiting transplant and for those who are not candidates.

7. Advance Care Planning and Communication

· Early discussion: Initiate advance care planning when the patient is stable.

· Prognosis: Acknowledge uncertainty; use phrases like "I am worried that your heart is getting weaker despite our best treatments."

· Explore goals: "What is most important to you now?" "What are your hopes and worries for the future?"

· Document preferences: Preferred place of care and death, resuscitation status, and views on ventilation, dialysis, and device deactivation.

· Legal aspects: Appoint healthcare proxy or lasting power of attorney.

· Review regularly: Update preferences with clinical changes.

· Family involvement: Engage families in discussions, especially for patients with cognitive impairment.

8. End-of-Life Care in Advanced CHD

Recognising the terminal phase can be challenging. Features suggesting imminent death include:

· Progressive decline despite maximal therapy.

· Repeated hospital admissions.

· Worsening renal and hepatic function.

· Cachexia and severe fatigue.

· Refractory dyspnoea at rest.

· Patient expresses desire for comfort-focused care.

Symptom control in the last days of life:

· Dyspnoea: Morphine 2.5–5 mg subcutaneously every 4 hours as needed, or continuous subcutaneous infusion if frequent doses required.

· Pain: Same as dyspnoea; adjust opioids based on prior use.

· Respiratory secretions: Glycopyrronium 0.2–0.4 mg subcutaneously every 6 hours as needed.

· Agitation/terminal restlessness: Midazolam 2.5–5 mg subcutaneously every 2–4 hours as needed, or continuous infusion.

· Nausea/vomiting: Haloperidol 0.5–1.5 mg subcutaneously every 8 hours as needed.

· Stop non-essential medications: Discontinue routine medications that do not provide immediate symptom benefit [3].

· Fluid management: Avoid intravenous fluids unless for comfort.

· Oxygen: Continue if it provides comfort.

Care of the family: Provide emotional support, information, and bereavement follow-up.

9. Integration of Palliative Care in CHD Services

· Primary palliative care: CHD teams trained in basic symptom management, communication, and advance care planning.

· Specialist palliative care: Referral for complex symptoms, existential distress, or end-of-life care.

· Shared care models: Combined CHD–palliative care clinics improve quality of life and reduce readmissions [9].

· Transition care: Support patients transitioning from paediatric to adult services, addressing psychosocial and palliative needs.

· Community support: CHD nurses, hospice at home, and telehealth monitoring.

10. Conclusion

Advanced congenital heart disease is a complex, life-limiting condition with a high symptom burden and unpredictable course. Palliative care should be integrated early, alongside optimal cardiology management, to improve quality of life, support families, and ensure goal-concordant care. Clinicians must be skilled in managing dyspnoea, fatigue, pain, depression, arrhythmias, and the unique complications of Fontan circulation and Eisenmenger syndrome, while proactively addressing advance care planning and end-of-life preferences. A multidisciplinary, patient-centred approach is essential.

References

1. van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011;58(21):2241-2247. doi:10.1016/j.jacc.2011.08.025

2. Rychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation. 2019;140(6):e234-e284. doi:10.1161/CIR.0000000000000696

3. Kutner JS, Blatchford PJ, Taylor DH Jr, et al. Safety and benefit of discontinuing statin therapy in the setting of advanced, life-limiting illness: a randomized clinical trial. JAMA Intern Med. 2015;175(5):691-700. doi:10.1001/jamainternmed.2015.0289

4. McMurray JJV, Solomon SD, Inzucchi SE, et al. Dapagliflozin in patients with heart failure and reduced ejection fraction. N Engl J Med. 2019;381(21):1995-2008. doi:10.1056/NEJMoa1911303

5. Jennings AL, Davies AN, Higgins JP, et al. A systematic review of the use of opioids in the management of dyspnoea. Thorax. 2002;57(11):939-944. doi:10.1136/thorax.57.11.939

6. Longmuir PE, Brothers JA, de Ferranti SD, et al. Promotion of physical activity for children and adults with congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2013;127(21):2147-2159. doi:10.1161/CIR.0b013e318293688f

7. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. J Am Coll Cardiol. 2019;73(12):e81-e192. doi:10.1016/j.jacc.2018.08.1029

8. Galiè N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114(1):48-54. doi:10.1161/CIRCULATIONAHA.106.630715

9. Rogers JG, Patel CB, Mentz RJ, et al. Palliative care in heart failure: the PAL-HF randomized, controlled clinical trial. J Am Coll Cardiol. 2017;70(3):331-341. doi:10.1016/j.jacc.2017.05.030

Prepared By: Dr. Shekhar Ingle and Team, Doctor's Forum for All 🏥

Date: 2026