Transcatheter Palliation of a Young Infant With Obstructed Supracardiac Total Anomalous Pulmonary Venous Connection

Abstract

Obstructed total anomalous pulmonary venous connection is a life-threatening pediatric cardiac emergency. Infants usually present in critical condition with marked respiratory distress, severe metabolic acidosis, and central cyanosis. Urgent cardiac surgical intervention, despite its high risk, is necessary in order to save the life of the patient. A two-month-old female infant presented to our tertiary care hospital with dense cyanosis and metabolic acidosis. She required mechanical ventilation, but her oxygen saturation did not improve. Her 2D transthoracic echocardiography revealed obstructed supracardiac total anomalous pulmonary venous connection with adequate interatrial communication and severe pulmonary hypertension. After discussion with the family and pediatric cardiac surgical team, it was decided to offer her transcatheter relief of obstructive ascending channel. She underwent successful balloon angioplasty of stenosed levoatrial cardinal vein (vertical vein) with remarkable improvement in blood flow and vessel caliber. She was extubated and her oxygen saturation rose from the high seventies to low eighties immediately after the procedure. She is scheduled for cardiac surgical repair within the next few days. Transcatheter angioplasty is a workable option in stabilizing very sick young infants with obstructed total anomalous pulmonary venous connection, especially supracardiac ones.