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.

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 child with tricuspid atresia and a nonrestrictive ventricular septal defect. There is pulmonary plethora. Note the prominent right atrium.

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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.

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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.

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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.^[5]

The diagnosis of cardiac disease in the fetus is increasingly made with ultrasound and fetal echocardiography because of advances in imaging technology over the last two decades.^{[6, 7, 8, 9, 10]}Yu et al reported an overall sensitivity of 75% diagnostic accuracy in detection of major CHD in the first trimester of pregnancy in a recent meta-analysis that included 18 studies and more than 26,000 fetal hearts.^[7]

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.

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.

Treatment & Management

Mai CT, Isenburg JL, Canfield MA, et al, for the National Birth Defects Prevention Network. National population-based estimates for major birth defects, 2010-2014. Birth Defects Res. 2019 Nov 1. 111(18):1420-1435. [QxMD MEDLINE Link]. [Full Text].
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Schneider AW, Blom NA, Bruggemans EF, Hazekamp MG. More than 25 years of experience in managing pulmonary atresia with intact ventricular septum. Ann Thorac Surg. 2014 Nov. 98(5):1680-6. [QxMD MEDLINE Link].
Karamlou T, Ashburn DA, Caldarone CA, et al. Matching procedure to morphology improves outcomes in neonates with tricuspid atresia. J Thorac Cardiovasc Surg. 2005 Dec. 130(6):1503-10. [QxMD MEDLINE Link].
[Guideline] Donofrio MT, Moon-Grady AJ, Hornberger LK, et al, for the American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014 May 27. 129(21):2183-242. [QxMD MEDLINE Link]. [Full Text].
Yu D, Sui L, Zhang N. Performance of first-trimester fetal echocardiography in diagnosing fetal heart defects: meta-analysis and systematic review. J Ultrasound Med. 2020 Mar. 39(3):471-80. [QxMD MEDLINE Link].
Karim JN, Bradburn E, Roberts N, Papageorghiou AT, ACCEPTS study. First-trimester ultrasound detection of fetal heart anomalies: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 2022 Jan. 59(1):11-25. [QxMD MEDLINE Link].
Ye B, Wu Y, Chen J, et al. The diagnostic value of the early extended fetal heart examination at 13 to 14 weeks gestational age in a high-risk population. Transl Pediatr. 2021 Nov. 10(11):2907-20. [QxMD MEDLINE Link]. [Full Text].
Cordisco Md A, Lozza V, Filice ME, Chiappa E. First trimester prenatal diagnosis of a severe tricuspid valve regurgitation and pulmonary valve atresia. Echocardiography. 2021 Dec. 38(12):2119-21. [QxMD MEDLINE Link].
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Wilder TJ, Ziemer G, Hickey EJ, et al. Surgical management of competing pulmonary blood flow affects survival before Fontan/Kreutzer completion in patients with tricuspid atresia type I. J Thorac Cardiovasc Surg. 2015 Nov. 150(5):1222-30.e7. [QxMD MEDLINE Link].
Alsoufi B, Schlosser B, Mori M, et al. Influence of morphology and initial surgical strategy on survival of infants with tricuspid atresia. Ann Thorac Surg. 2015 Oct. 100(4):1403-9; discussion 1409-10. [QxMD MEDLINE Link].
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Media Gallery

Tricuspid Atresia. Fontan procedure: Illustration of the atrial-to-pulmonary artery anastomosis.
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 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 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.
Tricuspid Atresia. Axial ECG-gated spin-echo MRI in an adult patient with tricuspid atresia shows the high signal from atrioventricular sulcus tissue (black arrow), replacing the tricuspid valve, and an enlarged right atrium. Note how the mitral valve orientation (white arrows) is abnormal. The right ventricular outflow chamber (R) is anterior.
Tricuspid Atresia. Axial ECG-gated spin-echo MRI (10 mm caudad to previous Image ) shows the high signal intensity from atrioventricular sulcus tissue and the restrictive ventricular septal defect (arrow) between the ventricle and the right ventricular outflow chamber. Note the dilated and rounded left ventricular cavity.
Tricuspid Atresia. Axial ECG-gated spin-echo MRI in an adolescent patient with tricuspid atresia with modified Fontan repair. The Fontan conduit (white arrow) runs from the right atrium (A) around the front of the heart towards the pulmonary artery. Note that the front of the heart is identified by the anterior atrioventricular sulcus tissue containing the signal void of the right coronary artery (black arrow).
Tricuspid Atresia. Axial ECG-gated spin-echo MRI in an adolescent patient with tricuspid atresia with modified Fontan repair (10 mm inferior to previous Image ). Thick atrioventricular sulcus tissue (arrow) is noted replacing the tricuspid valve. The ventricular septal defect has been repaired, and the ventricular septum is now intact.
Tricuspid Atresia. Apical 4-chamber 2-dimensional echocardiogram shows atrioventricular sulcus tissue (solid arrow) replacing the tricuspid valve in a patient with tricuspid atresia. Note the enlarged right atrium posterior to the abnormal atrioventricular sulcus tissue. A moderate-sized ventricular septal defect (open arrow) is noted between the ventricle (V) and outflow chamber (C).
Tricuspid Atresia. Fluoroscopic image shows a Park blade septostomy catheter with cutting blade extended in a patient with tricuspid atresia. The catheter has been passed through a restrictive atrial septal defect, which was resistant to balloon septostomy. The blade was used to make 2 cuts in the atrial septum, starting a tear, which then was completed using balloon septostomy.
Tricuspid Atresia. Frontal ventriculogram in a patient with tricuspid atresia shows the pulmonary arteries arising from a small right ventricular type outflow chamber (arrow). A restrictive ventricular septal defect and a large globular ventricle (V) are noted.
Tricuspid Atresia. Steep left anterior oblique ventriculogram in a patient with tricuspid atresia shows a restrictive ventricular septal defect (between arrows) and a typically large globular ventricle (V).
Tricuspid Atresia. Steep left anterior oblique ventriculogram in a patient with tricuspid atresia shows a larger nonrestrictive ventricular septal defect (white arrow). A typically large globular ventricle (V) is seen, which is receiving inflow from a single atrioventricular valve (mitral valve, black arrows). Note how the aorta and pulmonary arteries are superimposed, making interpretation of their attachments difficult. Angiography must be performed in multiple projections to fully define complex relationships accurately.
Tricuspid Atresia. Shallow right anterior oblique view from a ventriculogram in a patient with tricuspid atresia shows mitral regurgitation with contrast filling in both the left atrium (LA) and right atrium (RA), through the atrial septal defect. Contrast outlines the thick band of atrioventricular sulcus tissue (arrow), which is demonstrated well on cross-sectional imaging techniques.
Tricuspid Atresia. Right anterior oblique ventriculogram in a patient with tricuspid atresia shows simultaneous filling of the aorta (Ao) and pulmonary arteries (PA). Nonrestrictive ventricular septal defect was present, which necessitated pulmonary artery banding (arrow) to reduce pulmonary blood flow and protect against development of pulmonary hypertension before proceeding to a Fontan procedure.