日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

We describe a male infant who was diagnosed in utero with pulmonary atresia and an intact ventricular septum (PA-IVS) and diagnosed with aortocoronary atresia (ACA) by cardiac catheterization after birth. He was delivered at full term and appeared normal for gestational age. Electrocardiography findings on day 3 of life showed ST depression while crying, and right ventricle-dependent coronary circulation (RVDCC) was suspected. Cardiac catheterization findings confirmed PA-IVS and ACA. We evaluated the blood flow of sinusoidal communication (SC) during the clinical course using pulsed-wave Doppler echocardiography. He underwent a Blalock–Taussig shunt and an ascending aorta-to-right ventricle shunt (Ao-RV shunt) on day 41 of life. At the time of delivery, SC flow was antegrade from the right ventricle (RV) to the intramyocardium at systole and retrograde from the intramyocardium to the RV at diastole. Antegrade SC flow became biphasic after the Ao-RV shunt and increased at end-diastole. Furthermore, the increased oxygenation of SC blood due to the Ao-RV shunt helped to improve the coronary circulation. This novel Ao-RV shunt approach is suitable for treating PA-IVS and ACA.