Tricuspid Atresia Workup

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

Laboratory Studies

The following laboratory studies may be useful:

  • CBC count: Because of cyanosis in this population, polycythemia may be present.
  • Prothrombin time and activated partial thromboplastin time: Findings from these studies may be abnormal secondary to the polycythemia.
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Imaging Studies

Chest radiography

Cardiomegaly is usually present, with a prominent right heart border that reflects enlargement of the right atrium.

In 80% of patients, pulmonary vascular markings are diminished because of diminished pulmonary blood flow. Pulmonary vascular markings may be increased when pulmonary flow is not obstructed.

A right aortic arch may be observed in 3-8% of cases.

Tricuspid atresia. Frontal chest radiograph in a c Tricuspid atresia. Frontal chest radiograph in a child with tricuspid atresia and a nonrestrictive ventricular septal defect. There is pulmonary plethora. Note the prominent right atrium.
Tricuspid atresia. Frontal chest radiograph in a c Tricuspid atresia. Frontal chest radiograph in a child with tricuspid atresia and a nonrestrictive ventricular septal defect, mild pulmonary plethora and, atypically, a right aortic arch (arrow). Note enlarged right atrium and the typical rounded configuration of the left cardiac apex. In the absence of the right ventricle, the left ventricle becomes hypertrophied and dilated, causing the development of a more rounded cardiac apex.
Tricuspid atresia. Frontal chest radiograph in an Tricuspid atresia. Frontal chest radiograph in an adult with untreated tricuspid atresia. Increased pulmonary blood flow through a nonrestrictive ventricular septal defect has been tolerated for years but has led to the development of pulmonary hypertension, as shown by the large proximal pulmonary arteries (arrows) and pruned distal pulmonary arteries. The development of pulmonary hypertension prevents conventional surgical treatment.

Echocardiography

The diagnosis of tricuspid atresia can be reliably established with this noninvasive method.

The basic anatomy, size of the atrial septal defect, relationship of the great vessels, degree of pulmonary blood flow, ventricular function, and valvular function can be easily ascertained using a combination of M-mode, 2-dimensional, and color-flow echocardiography. [4]

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Other Tests

Electrocardiography

Sinus rhythm is generally present, with tall P waves indicative of atrial enlargement.

First-degree atrioventricular block may be observed.

Because of the origin of the left bundle branch from a common bundle, the frontal plane QRS axis may be leftward or superior.

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Procedures

Cardiac catheterization

In infants, the primary use of cardiac catheterization is to determine the source and reliability of pulmonary blood flow and, in particular, to assess the status of the patent ductus arteriosus. If a restrictive atrial septal defect is present, then balloon septostomy can be performed in this setting.

In the older population, arteriography is used to define details important in surgical management such as number and relationship of vena cavae, size of the pulmonary arteries, pulmonary artery resistance, mitral valve competency, and definition of prior operative procedures.

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