Tricuspid atresia
From Wikipedia, the free encyclopedia
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|---|---|
| Anterior (frontal) view of the opened heart. White arrows indicate normal blood flow. (Tricuspid valve labeled at bottom left.) | |
| ICD-10 | Q22.4 |
| ICD-9 | 746.1 |
| OMIM | 605067 |
| MedlinePlus | 001110 |
| eMedicine | med/2313 |
| MeSH | D018785 |
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.
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.
[edit] 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
[edit] 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
[edit] External links
- Tricuspid Atresia information from Seattle Children's Hospital Heart Center
