Abstract

A 20-days-old female newborn was referred to our hospital because of persistent respiratory distress and a mild central cyanosis. On echocardiography from the suprasternal notch all 4 pulmonary veins could be visualized entering a common chamber posterior to the left atrium. This chamber communicated with a left vertical vein which drained through a large innominate vein into the right superior vena cava. The diagnosis of supracardiac type of TAPVD was confirmed the following day by angiography. The child was successfully operated and the postoperative course was uneventful. Cardiac catheterization carries an increased risk in patients with TAPVD in case of pulmonary hypertension due to pulmonary-venous obstruction. In experienced centers echocardiography has become the definite diagnostic method with a high level of sensitivity and specificity in patients with atrial sitlus solitus, unifocal pulmonary venous connection, and no evidence of other major congenital cardiac defects. With continuously improving knowledge an increasing number of newborns will benefit from improved ability to perform surgical repair without prior cardiac catheterization.

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