Bicoronary-pulmonary fistulae and severe mitral valve regurgitation – uncommon cause of myocardial ischemia – a case report

Abstract Bicoronary - pulmonary artery fistulae are rare conditions. Their association with mitral valve prolapse is even rarer and randomly reported. This association is important to be recognized in clinical practice because of the differential diagnose problems. Closing the coronary fistulae and mitral valve replacement during the same surgical procedure is probably the optimal management of these patients. We report a case involving the correction of congenital bicoronarypulmonary artery fistulae and mitral valve replacement within the same surgical procedure in a 56 years old female patient with angina and clinical signs of left ventricular failure associating the fistulae to severe mitral regurgitation due to mitral valve prolapse. Past medical history revealed autoimmune thyroiditis, atrial fibrillation, mitral and tricuspid valve regurgitation. At admission physical examination revealed stable vital signs, irregular tachycardia with significant pulse and a mitral regurgitation systolic murmur. ECG showed atrial fibrillation, no ischemia. Echocardiography revealed severe mitral regurgitation, prolapse of anterior and posterior mitral leaflets, moderate tricuspid valve regurgitation, and mild pulmonary hypertension. Coronary angiogram showed no significant lesions of the epicardial vessels but high flow congenital bicoronary-pulmonary fistulae (right coronary artery and left coronary artery to main pulmonary artery). Surgical correction of the congenital bicoronarypulmonary fistulae was performed simultaneously with mitral valve replacement in the same session. Postsurgical evolution was uneventful. Post-procedural ECG showed atrial fibrillation with a controlled heart rate, postoperative echocardiography showed normal functional and normal positioned prosthetic mitral mechanical valve, and rather normal left ventricle function. Coexistence of bicoronary-pulmonary fistulae and mitral valve insufficiency due to prolapse in a symptomatic patient with angina pectoris is a very rare clinical entity. Solving both abnormalities within the same surgical procedure was the optimal management for this patient.