Tricuspid atresia

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Tricuspid atresia
Synonyms
Pronounce	N/A
Specialty	N/A
Symptoms	Cyanosis, dyspnea, fatigue, poor feeding
Complications	Heart failure, arrhythmias, stroke
Onset	At birth
Duration	Lifelong
Types	N/A
Causes	Congenital heart defect
Risks	Family history, genetic disorders
Diagnosis	Echocardiogram, cardiac catheterization
Differential diagnosis	Tetralogy of Fallot, transposition of the great arteries
Prevention	None
Treatment	Surgery, medications
Medication	Prostaglandin E1, diuretics
Prognosis	Variable, depends on surgical outcomes
Frequency	Rare
Deaths

Congenital heart defect

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.

See also

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