Tricuspid atresia

Tricuspid atresia
Anterior (frontal) view of the opened heart. White arrows indicate normal blood flow. (Tricuspid valve labeled at bottom left.)
Classification and external resources
Specialty	medical genetics
ICD-10	Q22.4
ICD-9-CM	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, so other defects must occur to maintain blood flow. Because of the lack of an A-V connection, an atrial septal defect (ASD) must be present to fill the left ventricle with blood. 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). The causes of Tricupsid atresia are unknown.^[1]

An atrial septal defect (ASD) and a ventricular septal defect (VSD) must both be present to maintain blood flow-from the right atrium, the blood must flow through the ASD to the left atrium to the left ventricle and through the VSD to the right ventricle to allow access to the lungs

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

References

↑ http://www.cdc.gov/ncbddd/heartdefects/tricuspid-atresia.html

External links

Tricuspid Atresia (TA) - Stanford Children's Health
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

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