- Tricuspid atresia
Name = Tricuspid atresia
Caption = Anterior (frontal) view of the opened heart. White arrows indicate normal blood flow. (Tricuspid valve labeled at bottom left.)
ICD10 = ICD10|Q|22|4|q|20
ICD9 = ICD9|746.1
OMIM = 605067
MedlinePlus = 001110
eMedicineSubj = med
eMedicineTopic = 2313
MeshID = D018785
Tricuspid atresia is a form of
congenital heart diseasewhereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplasticor an absence of the right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the heart to be unable to properly oxygenate the rest of the blood in the body. Because of this, the body does not have enough oxygen to live, and steps must be taken to keep the child alive.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 arteriosusis usually also formed to increase pulmonary flow.
tachypneaover the first 2 weeks of life
holosystolicmurmur due to the VSD
superior axisand left ventricular hypertrophy(since it must pump blood to both the pulmonary and systemic systems)
* "normal" heart size
PGE1to maintain patent ductus arteriosus
Blalock-Taussigshunt to maintain pulmonary blood flow by placing a Gortex conduitbetween the subclavian arteryand the pulmonary artery.
* cavopulmonary anastomosis (
hemi-Fontanor bidirectional Glenn) to provide stable pulmonary flow
Fontan procedureto redirect inferior vena cavaand hepatic veinflow into the pulmonary circulation
* [http://heartcenter.seattlechildrens.org/conditions_treated/tricuspid_atresia.asp Tricuspid Atresia information] from Seattle Children's Hospital Heart Center
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