All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Tricuspid valve atresia
Medical expert of the article
Last reviewed: 07.07.2025
Tricuspid atresia is the absence of the tricuspid valve associated with right ventricular hypoplasia. Associated abnormalities are common and include atrial septal defect, ventricular septal defect, patent ductus arteriosus, and transposition of the great vessels. Symptoms of tricuspid atresia include cyanosis and signs of heart failure. The second heart sound is single, the nature of the murmur depends on the presence of associated defects. Diagnosis is based on echocardiography or cardiac catheterization. Radical treatment is surgical correction. Endocarditis prophylaxis is recommended.
Tricuspid atresia accounts for 5-7% of all congenital heart defects. In the most common type (approximately 50%), there is a ventricular septal defect (VSD) and pulmonary artery stenosis, and blood flow to the lungs is reduced. At the atrial level, a right-to-left shunt of blood develops, causing cyanosis. In the remaining 30%, there is transposition of the great vessels with a normal pulmonary valve, and blood enters the pulmonary circulation directly from the left ventricle, which usually results in heart failure.
[ Symptoms of tricuspid atresia
Severe cyanosis is usually present at birth. Signs of heart failure may appear by 4 to 6 weeks of age.
Physical examination usually reveals a single 2nd heart sound and a holosystolic or early systolic murmur of intensity 2-3/6 of a ventricular septal defect in the 3rd-4th intercostal space to the left of the sternum. Systolic thrill is rarely detected in the presence of pulmonary artery stenosis. Diastolic murmur at the apex is heard if pulmonary blood flow is increased. With prolonged cyanosis, clubbing may form.
Diagnosis of tricuspid atresia
The diagnosis is suggested on the basis of clinical data, taking into account chest X-ray and ECG, the exact diagnosis is established on the basis of two-dimensional echocardiography with color Doppler.
In the most common form, the X-ray shows the heart to be of normal size or slightly enlarged, the right atrium is enlarged, and the pulmonary pattern is poor. Sometimes the heart shadow resembles that of tetralogy of Fallot (a boot-shaped heart, a narrow waist of the heart due to the pulmonary artery segment). The pulmonary pattern may be enhanced, and cardiomegaly may be observed in infants with transposition of the great vessels. The ECG shows a leftward deviation of the electrical axis of the heart and signs of left ventricular hypertrophy. Hypertrophy of the right atrium or hypertrophy of both atria is also common.
Cardiac catheterization is usually necessary to clarify the anatomy of the defect before surgery.
Treatment of tricuspid atresia
In neonates with severe cyanosis, prostaglandin E1 infusions [0.05-0.10 mcg/(kg x min)] are administered to reopen the ductus arteriosus prior to planned cardiac catheterization or surgical correction.
Balloon atrial septostomy (Rashkind procedure) may be performed as part of primary catheterization to increase the right-to-left shunt if the interatrial communication is inadequate. Some neonates with transposition of the great vessels and signs of heart failure require medical treatment (eg, diuretics, digoxin, ACE inhibitors).
Radical treatment of tricuspid atresia includes step-by-step correction: soon after birth, Blalock-Taussig anastomosis is performed (connection of the artery of the systemic circulation and the pulmonary artery using a GoreTex tube); at the age of 4-8 months, a bidirectional shunting operation is performed - the Glenn operation (an anastomosis between the superior vena cava and the right pulmonary artery) or the hemiFontan operation (creation of a bypass path for blood flow between the superior vena cava and the central part of the right pulmonary artery by forming an anastomosis between the right atrial appendage and the pulmonary artery using a patch sutured in the area of the upper part of the right atrium); by the age of 2 years, a modified Fontan operation is performed. This approach has increased the early postoperative survival rate to more than 90%; the survival rate after 1 month is 85%, the 5-year survival rate is 80%, and the 10-year survival rate is 70%.
All patients with tricuspid atresia, whether or not the defect has been corrected, should receive endocarditis prophylaxis before dental or surgical procedures that may result in bacteremia.