A Case Of Superior Sinus Venosus Atrial Septal Defect Associated With Ostial Atresia Of The Right Coronary Artery And Coronary-cameral Fistulae
Sujata Subramanian, Karen Wright, Charles D. Fraser, Carlos M. Mery.
Dell Children's Medical Center, Austin, TX, USA.
INTRODUCTION: We describe an unusual case of a child diagnosed with a superior sinus venosus atrial septal defect (ASD) with partial anomalous pulmonary venous return (PAPVR), who was also noted to have right coronary artery (RCA) ostial atresia and coronary-cameral fistulae to the right ventricular outflow tract.METHODS: A 9-month-old child was evaluated for murmur and poor weight gain. Echocardiogram showed a large superior sinus venosus ASD, PAPVR (right upper vein draining into the superior vena cava and the right middle pulmonary vein to the right cavo-atrial junction), a large secundum ASD and dilation of right-sided chambers. In addition, very dilated and tortuous coronary vessels were observed which prompted further work-up with a CT angiogram (CTA) and cardiac catheterization. This revealed RCA ostial atresia with retrograde filling of the RCA from the left coronary arterial system, and multiple sinusoidal and fistulous connections from the blind ending pouch of the RCA to the right ventricular cavity at the level of the infundibulum.RESULTS: Dual-coronary circulation was established by dividing the fistulae and implanting the RCA into the ascending aorta via a trapdoor incision. The superior sinus venosus ASD was repaired with the Warden approach and the secundum ASD was closed primarily. Post-operative recovery was excellent, and pre-discharge CTA showed a wide-open RCA ostium with excellent antegrade flow.CONCLUSION: Coronary abnormalities can coexist with sinus venosus ASD and PAPVR. Creation of a two-coronary circulation was possible in this child with clinical indications for repair of the sinus venosus ASD/PAPVR.
Back to 2022 Abstracts