胎児期から類洞交通を確認できた純型肺動脈閉鎖の一例

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  • Prenatal Diagnosis of Ventriculocoronary Communication in a Fetus with Pulmonary Atresia with Intact Ventricular Septum : a Case Report

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Abnormal coronary connections between the ventricles and aorta can have significant impacts on myocardial perfusion and consequently on the approach for surgical intervention．Presence of ventriculocoronary communication（VCC），or ventriculo-coronary fistula，may be critical to peri- and postpartum management．We report the prenatal diagnosis of VCC associated with pulmonary atresia with intact ventricular septum（PAVIS）． A24-year-old woman was referred to our institution at 23＋3weeks’ gestation because of an abnormally small right ventricle（RV）in the fetus．Fetal echocardiography showed a small RV with trivial diastolic filling flow，an intact ventricular septum，and a hypoplastic pulmonary trunk with retrograde perfusion through the ductus arteriosus．Color and pulsed Doppler sonography revealed bidirectional flow at the right ventricular apex，which was confirmed as large VCC between the left anterior descending（LAD）coronary artery and RV by delineating coronary arteries from the aortic origin with color Doppler sonography． A3，070g male infant was vaginally delivered at 40＋1 weeks’ gestation．Postnatal echocardiography，computed tomography and angiography confirmed PAIVS and large VCC between the LAD coronary artery and RV．A BIalock-Taussig shunt was placed on postnatal day 11．Subsequently，a Glenn shunt was inserted at the age of 13 months，and Fontan procedure was performed at the age of 3 years． In summary，we accurately diagnosed fetal PAIVS and VCC accurately by meticulous echocardiography．Thus，we could provide appropriate counseling to parents and optimize peri- and postpartum management．

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