Coronary-pulmonary artery fistula with lung hypoplasia and a bicuspid aortic valve: A case report

BACKGROUND: With an incidence of less than 1%, a Coronary Artery to Pulmonary Artery fistula (CAPF) is a rare coronary anomaly that causes heart failure. It causes a left to right cardiac shunt. While guidelines favor surgical correction in symptomatic patients, we present a challenging case with multiple cardio-thoracic pathologies. CASE PRESENTATION: We present a 38-year-old obese male with persistent atrial fibrillation (AF). He presented to our hospital in decompensated heart failure and AF with rapid ventricular response. He was found to have a CAPF, a bicuspid aortic valve and left lung hypoplasia in the presence of severely reduced left ventricular systolic dysfunction. The patient subsequently underwent various cardiac testing demonstrating advanced anatomical and physiologic involvement of his CAPF, including suggested coronary steal. Despite some indications for percutaneous or surgical referral, we optimized his AF and congestive heart failure in lieu of formulating a treatment strategy for his CAPF and other abnormalities. CONCLUSION: This report illustrates a case of a young adult who presented in decompensated heart failure with newly diagnosed left ventricular systolic function and rapid AF, who had a triad of congenital defects including a CAPF, a bicuspid aortic valve and left lung hypoplasia. To the best of our knowledge, this triad of defects is unreported. This case highlights the clinical approach in the evaluation of a cardiac shunt and it's management strategies in the presence of multiple cardio-thoracic comorbidities.