From the Guidelines
Rare causes of right heart failure include arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia (ARVD), which is a genetic condition where heart muscle is replaced by fatty tissue, weakening right ventricular function, as noted in a study published in the European Heart Journal 1.
Causes of Right Heart Failure
Other rare causes of right heart failure include:
- Congenital heart defects such as Ebstein's anomaly and atrial septal defects, which create abnormal blood flow patterns that overload the right ventricle
- Carcinoid heart disease, which occurs when vasoactive substances from carcinoid tumors damage heart valves, particularly affecting the tricuspid and pulmonary valves
- Cardiac amyloidosis, which involves protein deposits in heart tissue that stiffen the heart walls
- Endomyocardial fibrosis, which causes scarring of the heart's inner lining, restricting filling
- Certain medications like some chemotherapy agents (particularly anthracyclines), appetite suppressants (fenfluramine, dexfenfluramine), and ergot alkaloids, which can cause right heart damage
- Radiation therapy to the chest, which can lead to delayed cardiac fibrosis
- Sarcoidosis, which may cause granulomas in heart tissue, disrupting electrical conduction and contractility
- Thyroid disorders (both hyperthyroidism and hypothyroidism), which can indirectly affect right heart function
Diagnosis and Treatment
Diagnosis typically requires echocardiography, cardiac MRI, and sometimes myocardial biopsy, as outlined in a report from the American College of Cardiology/American Heart Association task force on clinical data standards 1. Treatment must target the underlying cause while providing supportive care with diuretics, oxygen therapy, and in advanced cases, consideration of right ventricular assist devices, as recommended by the American Heart Association 1. Key considerations in managing right heart failure include:
- Evaluating and managing the underlying cause of right ventricular dysfunction
- Assessing and addressing signs and symptoms of heart failure, such as edema, fatigue, and shortness of breath
- Utilizing pharmacological and mechanical interventions to support right ventricular function and overall cardiac output.
From the Research
Rare Causes of Right Heart Failure
- Right heart failure can be caused by various factors, including ischemia, elevated pulmonary arterial pressure, or volume overload 2
- Pulmonary hypertension due to left ventricular failure, pulmonary arterial hypertension, chronic lung conditions, thromboemboli, or idiopathic conditions can also lead to right heart failure 2
- Other rare causes of right heart failure include severe pulmonary hypertension that is unresponsive to vasodilators, which can be treated with combination therapy of oral pulmonary vasodilators such as sildenafil and bosentan 3
Diagnosis and Management
- Diagnosis of right heart failure involves evaluation with electrocardiogram, laboratory analysis, and imaging to assess cardiac function and end-organ injury 2
- Management of right heart failure focuses on treating the underlying condition, optimizing oxygenation and ventilation, treating arrhythmias, and understanding the patient's hemodynamics with bedside ultrasound 2
- Treatment options for right heart failure include pharmacotherapies, mechanical circulatory support devices, and combination therapy with oral pulmonary vasodilators such as sildenafil and bosentan 4, 5, 3
Treatment Options
- Combination therapy with sildenafil and bosentan has been shown to improve symptoms and hemodynamics in patients with pulmonary arterial hypertension 3, 6
- However, adding bosentan to sildenafil therapy was not superior to sildenafil monotherapy in delaying the time to the first morbidity/mortality event in one study 6
- Mechanical circulatory support devices and pharmacotherapies to reduce afterload can also improve outcomes in patients with right heart failure 4, 5