Tricuspid atresia

Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic or an absence of the right ventricle. more...

Because of the lack of an A-V connection, an atrial septal defect (ASD) must be present to maintain blood flow. Also, since there is a lack of a right ventricle there must be a way to pump blood into the pulmonary arteries, and this is accomplished by a ventricular septal defect (VSD).

Blood is mixed in the left atrium. Because the only way the pulmonary circulation receives blood is through the VSD, a patent ductus arteriosus is usually also formed to increase pulmonary flow.

Clinical manifestations

• progressive cyanosis
• poor feeding
• tachypnea over the first 2 weeks of life
• holosystolic murmur due to the VSD
• superior axis and left ventricular hypertrophy (since it must pump blood to both the pulmonary and systemic systems)
• normal heart size

Treatment

• PGE1 to maintain patent ductus arteriosus
• modified Blalock-Taussig shunt to maintain pulmonary blood flow by placing a Gortex conduit between the subclavian artery and the pulmonary artery.
• cavopulmonary anastomosis (hemi-Fontan or bidirectional Glenn) to provide stable pulmonary flow
• Fontan procedure to redirect inferior vena cava and hepatic vein flow into the pulmonary circulation

External Links

• Tricuspid Atresia information from Seattle Children's Hospital Heart Center

Read more at Wikipedia.org