Restrictive Cardiomyopathy Symptoms in Later Stages

Restrictive cardiomyopathy is a condition characterized by the stiffening of the heart muscles and endocardium due to fibrosis. This stiffness impairs the heart's ability to properly relax and fill with blood, leading to compromised diastolic function. In infants, this condition can progress through distinct stages, presenting a range of symptoms as the disease advances.

Common Symptoms in the Middle Stages

As the disease progresses to the middle stages, infants may begin to show visible signs of distress. These often include a dusky or bluish skin tone, medically known as cyanosis, or a reddish complexion due to poor oxygenation. Infants may also experience difficulty breathing, persistent coughing, and episodes of hemoptysis — coughing up blood. Chest discomfort or pain may also occur as the heart struggles to function efficiently.

Progression to Advanced Stages

By the time the disease reaches its later stages, symptoms become more severe and often indicate right-sided heart failure. These may include significant enlargement of the liver, accumulation of fluid in the abdominal cavity known as ascites, swelling in the lower extremities, and distended neck veins — a sign of elevated venous pressure. Hypotension, or low blood pressure, is also commonly observed in advanced cases.

Diagnostic Findings

Imaging studies such as chest X-rays typically reveal an enlarged heart that may appear spherical or flask-shaped. This distinct appearance helps clinicians differentiate restrictive cardiomyopathy from other types of heart disease.

Prognosis and Treatment Outlook

Unfortunately, the prognosis for infants diagnosed with restrictive cardiomyopathy remains poor. Despite medical interventions, the disease often progresses to heart failure. Once heart failure develops, life expectancy is significantly reduced, with many infants succumbing to the condition within a few years following diagnosis. Early detection and comprehensive management are crucial to improving quality of life and potentially extending survival.