Tricuspid Atresia
Overview
Plain-Language Overview
Tricuspid Atresia is a rare heart condition present at birth where the valve between the right atrium and right ventricle is missing or abnormally developed. This defect affects the heart's ability to pump blood properly, especially to the lungs where blood gets oxygenated. Because the blood cannot flow normally through the right side of the heart, the body may not get enough oxygen, leading to cyanosis or a bluish tint to the skin. The condition involves the cardiovascular system and often requires other heart defects, like a hole between the atria, to allow blood to circulate. Symptoms usually appear early in life and can include difficulty breathing, poor feeding, and fatigue. Without treatment, this condition can cause serious health problems due to insufficient oxygen delivery to the body.
Clinical Definition
Tricuspid Atresia is a congenital heart defect characterized by the complete absence or agenesis of the tricuspid valve, resulting in no direct communication between the right atrium and right ventricle. This leads to an underdeveloped or hypoplastic right ventricle and obligates blood flow to bypass the right ventricle via an atrial septal defect or patent foramen ovale. The pathophysiology involves right-to-left shunting of blood, causing systemic hypoxemia and cyanosis. It is often associated with other cardiac anomalies such as ventricular septal defect and pulmonary stenosis or atresia. The defect significantly impairs pulmonary blood flow and oxygenation, making it a critical cause of neonatal cyanotic congenital heart disease. Diagnosis and management require understanding the altered hemodynamics and compensatory mechanisms.