Tricuspid atresia
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| Tricuspid atresia Classification and external resources | |
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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 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-632-7753
Associate Editor-in-Chief: Keri Shafer, M.D. [2]
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Overview
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.
Pathophysiology
This defect occurs during prenatal development. 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 deviation 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
Additional Reading
- Moss and Adams' Heart Disease in Infants, Children, and Adolescents Hugh D. Allen, Arthur J. Moss, David J. Driscoll, Forrest H. Adams, Timothy F. Feltes, Robert E. Shaddy, 2007 ISBN 0781786843
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
