Tricuspid atresia

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Tricuspid atresia
Classification and external resources
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 (undersized) or absent 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 arteriosus usually also persists to increase pulmonary flow.

Clinical manifestations

progressive cyanosis
poor feeding
tachypnea over the first 2 weeks of life
holosystolic murmur due to the VSD
left axis deviation on electrocardiography 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 Gore-Tex 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

Congenital heart defects (Q20–Q24, 745–746)

Cardiac shunt/
heart septal defect

Aortopulmonary septal defect	R→L: Double outlet right ventricle Taussig–Bing syndrome Transposition of the great vessels dextro levo Persistent truncus arteriosus Aortopulmonary window

Atrial septal defect	L→R: Sinus venosus atrial septal defect Lutembacher's syndrome

Ventricular septal defect	L→R and R→L: Eisenmenger's syndrome R→L, with other conditions: Tetralogy of Fallot

Atrioventricular septal defect	L→R: Ostium primum

Valvular heart disease/
heart chambers

Right	pulmonary valves stenosis insufficiency tricuspid valves stenosis atresia Ebstein's anomaly Hypoplastic right heart syndrome Uhl anomaly

Left	aortic valves stenosis insufficiency bicuspid mitral valves stenosis regurgitation Hypoplastic left heart syndrome

Other

M: HRT

anat/phys/devp

noco/cong/tumr, sysi/epon, injr

proc, drug (C1A/1B/1C/1D), blte

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