Treatment Options for Arrhythmogenic Right Ventricular Cardiomyopathy and Pulmonary Hypertension

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a rare heart condition characterized by the replacement of right ventricular myocardial tissue with fibrofatty tissue. While the exact cause remains unknown and there is currently no cure, several treatment strategies are available to manage symptoms and improve quality of life.

Oxygen Therapy

In cases where the patient's condition allows, supplemental oxygen therapy is often the first line of treatment. Oxygen helps alleviate symptoms related to low blood oxygen levels and can reduce the strain on the heart, particularly in patients with associated pulmonary hypertension.

Pharmacological Treatments

Medications to Improve Heart Function and Rhythm

Several drug classes are commonly used in the management of ARVC and associated pulmonary hypertension. Angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) can help improve ventricular remodeling. Beta-blockers are effective in controlling heart rate and improving long-term outcomes. Spironolactone, a potassium-sparing diuretic, helps reduce fluid buildup and lessen the workload on the heart. Digoxin may also be used to control heart rate and potentially slow the progression of myocardial fibrosis.

Targeted Pulmonary Hypertension Therapies

To specifically address elevated pulmonary artery pressure, calcium channel blockers, endothelin receptor antagonists, and prostaglandins may be prescribed. These medications help lower pulmonary vascular resistance and improve overall hemodynamics.

Interventional and Surgical Approaches

When conservative treatments fail to provide sufficient relief, more invasive options may be considered. Catheter-based interventions or implantation of cardioverter-defibrillators (ICDs) can help manage life-threatening arrhythmias. In advanced cases, heart or heart-lung transplantation may be necessary for eligible patients.

Supportive and Preventive Care

Supportive care plays a crucial role in managing ARVC. Patients are advised to strengthen their immune system, avoid respiratory infections, and attend regular follow-up appointments. Lifestyle modifications, including avoiding intense physical exertion, are often recommended to reduce the risk of sudden cardiac events.

Understanding the Patient Demographics and Risks

ARVC has a strong genetic component, with approximately 30% of cases showing familial inheritance. The condition predominantly affects individuals between the ages of 7 and 40, making it especially relevant in younger populations. Common clinical manifestations include ventricular tachycardia, progressive right ventricular dilation, refractory right heart failure, and an increased risk of sudden cardiac death. The disease often leads to right ventricular dysfunction, followed by enlargement of the right ventricle and atrium, which in turn contributes to elevated pulmonary artery pressure and further cardiac strain.