Total Anomalous Pulmonary Venous Connection Differential Diagnoses

Updated: Dec 29, 2020
  • Author: Allen D Wilson, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Diagnostic Considerations

Important considerations

Total anomalous pulmonary venous connection (TAPVC) may be difficult to diagnose, especially in ill newborns on ventilation, if views of the atrial septum are difficult to obtain or if the common pulmonary vein is small or at an obtuse angle to the left atrial back wall. The addition of color Doppler imaging greatly aids in diagnosis in individual pulmonary veins and in analysis of the abnormal flow pattern across the atrial septal defect. Usually, the need for surgery in these patients is not debatable, although timing can be an issue.

The risk of recurrent pulmonary vein stenosis or anastomosis at the anastomosis site between the common pulmonary vein and the left atrium must be explained to families.

Other problems to be considered


In newborns, also consider the following conditions:

  • Tachypnea

  • Cyanosis

  • Signs of pulmonary venous congestion

  • Cor triatriatum

  • Mitral stenosis

  • Hypoplastic left heart syndrome

  • Coarctation or interrupted aortic arch

  • Transposition of the great vessels

  • Persistent fetal circulation

Infants (age usually >6 wk)

Right ventricular volume load and pulmonary hypertension may indicate any of several heart defects, including the following:

  • Large ventricular septal defect

  • Common arteriovenous canal

  • Truncus arteriosus

  • Single ventricle

Children older than 1 year

Also consider large atrial septal defect as well as common atrium partial anomalous pulmonary venous connection.

Differential Diagnoses