Tricuspid atresia

Congenital heart defect

Overview

Diagram of tricuspid atresia

Tricuspid atresia is a type of congenital heart defect where the tricuspid valve is missing or abnormally developed. This defect blocks blood flow from the right atrium to the right ventricle, leading to a lack of oxygen-rich blood being pumped to the body.

Pathophysiology

In tricuspid atresia, the absence of a functional tricuspid valve results in the right atrium being unable to directly communicate with the right ventricle. Consequently, blood must find alternative pathways to reach the pulmonary circulation. Typically, this involves a patent foramen ovale or an atrial septal defect allowing blood to pass from the right atrium to the left atrium. From there, blood enters the left ventricle and is pumped into the systemic circulation.

Clinical Presentation

Patients with tricuspid atresia often present with cyanosis, which is a bluish discoloration of the skin due to low oxygen levels in the blood. Other symptoms may include difficulty breathing, fatigue, and poor growth. The severity of symptoms can vary depending on the presence and size of associated defects such as ventricular septal defects or patent ductus arteriosus.

Diagnosis

Echocardiography is the primary diagnostic tool for tricuspid atresia, allowing visualization of the heart's structure and blood flow. Additional imaging techniques, such as cardiac MRI or CT scan, may be used to provide further anatomical details. Cardiac catheterization can also be performed to assess hemodynamics and oxygen levels in different parts of the heart.

Treatment

The management of tricuspid atresia typically involves surgical intervention. Initial procedures may include the creation of a Blalock-Taussig shunt to increase pulmonary blood flow. Definitive surgical repair often involves a series of operations culminating in a Fontan procedure, which directs venous blood directly to the pulmonary arteries, bypassing the right heart.

Prognosis

The prognosis for individuals with tricuspid atresia has improved significantly with advances in surgical techniques. Long-term outcomes depend on the success of surgical interventions and the presence of any complications. Lifelong follow-up with a cardiologist specializing in congenital heart disease is essential.

Related pages

• Congenital heart defect
• Cyanotic heart defect
• Fontan procedure
• Blalock-Taussig shunt