Tangled: Unraveling a Rare Combination of Multiple Coronary Artery Fistulas and Severe Aortic Stenosis in a 70-Year-Old Man

Abstract

INTRODUCTION: Coronary artery fistulas (CAFs) are rare congenital or acquired anomalies that connect one or more coronary arteries to a cardiac chamber or a great vessel without an intervening capillary bed. Most are asymptomatic and are usually incidental findings on echocardiography or angiography for an unrelated cause. Occasionally, complications occur, which include endocarditis, thrombosis, steal syndrome, and heart failure.Here, we present a rare case of multiple CAFs draining to the main pulmonary artery in a patient with severe aortic stenosis (AS) presenting a unique management dilemma. CASE PRESENTATION: Five weeks before admission, a 70-year-old man with good baseline functional capacity, independent on all activities of daily living, developed exertional dyspnea, orthopnea, and paroxysmal nocturnal dyspnea. His symptoms persisted for a week prompting consult at another institution where he was managed as a case of acute heart failure and given diuretics, which resolved his symptoms. His two-dimensional (2D) echocardiogram showed eccentric left ventricular hypertrophy with adequate segmental and global wall motion and contractility, a left ventricular ejection fraction of 62% by the Simpson method, normal right ventricular diameter with adequate wall motion and contractility, dilated left atrial dimensions with a left atrial volume index of 50.98 mL/m2, moderate mitral regurgitation, normal right atrial diameter, thickened mitral valve leaflets without restriction of motion, thickened and calcified aortic valve, severe AS (aortic valve area of 0.37 cm2, mean gradient of 78.77 mm Hg, and peak gradient of 124.88 mm Hg), severe aortic regurgitation, mild tricuspid and pulmonic regurgitation, and normal pulmonary artery pressure (13 mm Hg by tricuspid regurgitant jet).