Tricuspid Atresia

Updated: Sep 25, 2016
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Richard A Lange, MD, MBA  more...
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Overview

Background

Tricuspid atresia is the third most common form of cyanotic congenital heart disease, with a prevalence of 0.3-3.7% in patients with congenital heart disease. The deformity consists of a complete lack of formation of the tricuspid valve with absence of direct connection between the right atrium and right ventricle.

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Pathophysiology

Three types of tricuspid atresia are described, depending on the associated relationship of the great vessels. In type I, the great arteries are related normally; in type II, the great arteries are d-transposed; and in type III, the great arteries are l-transposed. The types are further subclassified according to the presence or absence of ventricular septal defects and pulmonary valve pathology. [1, 2]

Other cardiovascular anomalies occur in 15-20% of patients with tricuspid atresia. Most of the associated anomalies relate to transposition of the great vessels. A persistent left superior vena cava anomaly is observed in 15% of patients.

With the absence of the tricuspid valve and no continuity between the right atrium and right ventricle, venous blood returning to the right atrium can exit only by an intra-atrial communication. Because of the obligatory right-to-left shunt at the level of the atria, saturation of the left atrial blood is diminished.

The intracardiac blood flow in tricuspid atresia further depends on the presence or absence of pulmonary arterial pathology. [3] In the absence of pulmonary atresia or pulmonary valve stenosis, the volume of blood to the lungs may be normal with normal oxygenation occurring, resulting in reduced cyanosis. In contrast, with accompanying pulmonary artery or valve stenosis, pulmonary blood flow is reduced, resulting in increased cyanosis.

Pulmonary obstruction occurs most often in patients with tricuspid atresia and normally related great arteries. Patients with d-transposed great arteries and tricuspid atresia generally have unobstructed pulmonary blood flow.

The left ventricle comprises most of the ventricular mass in tricuspid atresia. Because of volume overload (the left ventricle receives all the venous return) and persistent hypoxemia, decreased ventricular function may result in fibrosis, decreased ejection fraction, mitral annular dilatation, and mitral insufficiency.

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Epidemiology

United States data

The frequency of tricuspid atresia is 2.9% in autopsy series.

Race-, sex-, and age-related demographics

No racial predilection is apparent.

Considering all forms of tricuspid atresia, no sexual predilection exists. Males present more frequently with transposed great vessels than females.

The anomaly is congenital and is evident at birth.

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Prognosis

Mortality/Morbidity

Depending on the degree of obstruction and associated anomalies, tricuspid atresia may be lethal at birth. Without repair, the patient rarely survives to adulthood.

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