C oronary sinus (CS) ostial atresia is a rare entity, known to be associated with a variety of congenital heart diseases. [1][2][3][4][5][6] Generally, an anomalous decompressing vein drains the coronary venous blood. Any obstruction in the path of coronary venous drainage can lead to coronary venous hypertension and myocardial infarction (MI). We report a case of obstructed totally anomalous pulmonary venous (TAPV) return with CS ostial atresia, which led to venous MI. This, to our knowledge, has never before been reported.
Case ReportIn March 2013, a 12-week-old girl with a history of failure to thrive presented in extremis with cardiogenic shock. Her physical examination showed that she had tachypnea, poor systemic perfusion, cyanosis, and bibasilar rales. An emergency echocardiogram revealed a membranous obstruction at the junction of the brachiocephalic vein and the superior vena cava (Fig. 1) and TAPV drainage of a supracardiac type-together with a dilated CS, a large atrial septal defect with right-to-left shunting, and pulmonary hypertension.Emergency cardiac catheterization, performed to relieve the obstruction, confirmed the presence of TAPV drainage through a vertical vein into the left brachiocephalic vein, which was obstructed at its junction with the right superior vena cava (gradient, 19 mmHg). This CS ostial atresia resulted in severe pulmonary hypertension and retrograde drainage, via a connecting vein, to the pulmonary venous confluence (Fig. 2). Balloon angioplasty at the site of the obstructive membrane substantially relieved the hypertension.During the procedure, the patient developed sustained monomorphic ventricular tachycardia secondary to an MI, as evidenced by troponin levels elevated to 130 ng/mL and by diffuse ST-segment changes on electrocardiogram. The infarction was most likely associated with the transient elevation of coronary pressure upon balloon inflation.The patient was noted on her echocardiogram to have global qualitative left ventricular dysfunction, and her myocardial function recovery was closely observed in the intensive care unit. Her cardiac enzyme levels returned to baseline, and her left ventricular function improved substantially. A cardiac computed tomogram, obtained a week later to better delineate the postoperative pulmonary venous anatomy, confirmed the above findings (Fig. 3).Two weeks later, the patient underwent surgical repair, which comprised anastomosis of the venous confluence to the left atrium, unroofing of the CS to the left atrium, ligation of the vertical vein and the connecting vein from the CS to the confluence,