Total Anomalous Pulmonary Venous Connection Workup

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

Assess and improve (as possible) the oxygenation, acid-base status, and hemogram status in newborns or young infants with total anomalous pulmonary venous connection (TAPVC) in preparation for surgery.



ECG reveals significant right ventricular hypertrophy in most of these patients, usually with a qR pattern in the right chest leads by age 5-7 days. Right atrial enlargement rarely occurs in these younger patients.


Imaging Studies

Chest radiography

In patients with total anomalous pulmonary venous connection with pulmonary venous obstruction, chest radiographs reveal a normal heart size with a diffuse reticular pattern fanning out from the hilum.

When the pulmonary veins are unobstructed, the heart is enlarged (right atrial and right ventricular enlargement), and pulmonary markings reveal active increase in size of the pulmonary hilar and midzone vessels.

Magnetic resonance imaging

MRI serves to confirm the diagnosis in patients with total anomalous pulmonary venous connection (especially in those with associated lung disease).


Selective pulmonary vein or pulmonary artery angiography may precisely reveal a vessel's anatomy.


Echocardiographic findings, which are usually definitive, have been vital in pinpointing the exact cardiac defect. Hyaline membrane disease may demonstrate similar findings initially. In this setting, ECG helps identify right ventricular hypertrophy in patients with total anomalous pulmonary venous connection, especially in premature babies, particularly because premature babies usually have a greater level of left ventricular force.

Echocardiography of the precordium in patients with total anomalous pulmonary venous connection reveals right ventricular and pulmonary artery volume loading with flattened or paradoxic septal motion on M-mode imaging. Apical and subcostal 4-chamber views usually best identify individual pulmonary veins and their confluence in patients with total anomalous pulmonary venous connection. Then, using multiple views, the common pulmonary vein can usually be tracked to its point of entry to the systemic venous system or to the coronary sinus.

Subcostal long- and short-axis views can also help evaluate size and flow patterns across the foramen ovale.

Zhang et al evaluated the value of echocardiography in the diagnosis of different types of anomalous pulmonary venous connections (APVCs) that were identified and confirmed by surgery or computerized tomography angiography (CTA): 51 total anomalous pulmonary venous connections (TAPVCs) and 33 partial anomalous pulmonary venous connections (PAPVCs). The 51 TAPVCs were classified according to the Darling method-type I (41.1%), type II (52.9%), type III (1.9%), and type IV (3.9%). The sensitivity of echocardiography in the diagnosis of APVCs was 97.6%; of the TAPVCs correctly diagnosed by echocardiography, the diagnostic accuracy of classification was 94%. [5]

Total anomalous pulmonary venous connection may be difficult to diagnose, especially in an ill newborn on a ventilator, 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 ultrasonography greatly aids in the diagnosis of individual pulmonary veins and in analysis of the abnormal flow pattern across the atrial septal defect

Color-flow mapping may be helpful in finding individual pulmonary veins and confirming whether they enter the left atrium. Color-flow ultrasonography may also be used to assess directional flow at the foramen ovale. In patients with total anomalous pulmonary venous connection, flow across the atrial septum predominantly occurs from the right to left.

Altogether, echocardiography with additional color Doppler can help make the diagnosis in the vast majority of patients with total anomalous pulmonary venous connection. In patients with pulmonary inflow obstruction, further diagnostic studies may be needed.

With fetal echocardiography, an attempt should be made to see the individual pulmonary veins, but most consistent diagnostic findings in total anomalous pulmonary venous connection have involved a confluence (chamber) behind the left atrium or a vertical vein.



In some patients with multiple sites of pulmonary venous connection, cardiac catheterization serves to better define sites of pulmonary venous obstruction, when other associated cardiac defects are present (ie, pulmonary atresia), and to directly measure foramen ovale size when surgery is delayed.