An unusual cause of respiratory distress in an infant.

Abstract

Partial anomalous venous connection (PAPVC) is a congenital cardiovascular defect where one or more (but not all) of the pulmonary veins return anomalously back to the right atrium, either via a direct or indirect connection. It often occurs with other cardiac defects, most commonly a secundum atrial septal defect. Individuals with a large degree of shunting will present with dyspnea, fatigue, and, in some cases, heart failure. Clinical associations and variants of PAPVC include scimitar syndrome, pseudo-scimitar or meandering right pulmonary vein, sinous venosus defects, malposition of the septum primum, and Turner syndrome. The patient in this case, a previously healthy, 6-month-old, full-term male, presented to the emergency department for evaluation of respiratory distress and wheezing. The infant was first seen in his pediatrician's office, where he was noted to be tachypneic and wheezing. He was feeding without difficulty, voiding well, and was active and playful. The patient had passed critical congenital heart disease screening after his birth and prior to discharge, and the family history was negative for any respiratory or cardiac conditions. Cardiac magnetic resonance imaging is becoming the mode of choice for diagnosis of PAPVC. The definitive treatment is surgical correction, but surgery is not indicated in all cases, especially if the patient is asymptomatic and the degree of shunting is small. Patients with isolated PAPVC who undergo surgical correction have good long-term outcomes. In this case, the patient underwent the Warden procedure, which causes an increased risk of superior vena cava stenosis or obstruction to the right atrium. This patient will require lifelong follow-up to assess for new onset pulmonary venous obstruction.