Large Isolated Major Aortopulmonary Collateral Artery Causing Severe Pulmonary Hypertension in an Infant: A Rare and Challenging Diagnosis

Major Aortopulmonary Collateral Artery (MAPCA) as an isolated congenital anomaly, without evidence of any structural heart disease, is a very rare observation. Previously published reports indicate that symptomatic infants with large isolated MAPCA usually present with congestive heart failure or recurrent respiratory tract infections. To the best of our knowledge, the present case of an infant with large isolated MAPCA is a unique case with a diagnostic dilemma due to presentation with severe pulmonary hypertension as a predominant sign. The infant was managed successfully by percutaneous obliteration with amplatzer vascular plugs, along with perioperative and postoperative sildenafil.