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

純型肺動脈閉鎖（pulmonary atresia with intact ventricular septum：以下PAIVS）の治療方針の決定には右室の大きさ，肺動脈の閉鎖様式に加え，冠動脈が右室内腔と繋がる異常交通（類洞交通）の有無や程度が重要とされる．今回胎児期に類洞交通を評価できたPAIVSの一例を経験したので報告する．
超音波検査にて胎児の右室が小さいことを指摘され妊娠23週に紹介となった．初診時の超音波検査にてPAIVSと診断した．また右室心尖部に心筋を貫通する両方向性血流を認め，類洞交通の存在が疑われた．その後冠動脈を起始部から系統的に描出することで，左冠動脈前下行枝からの大きな類洞交通であることを確認した．右室が高度の低形成でかつ大きな類洞交通があることから単心室修復が必要となることが予想された．児は出生後，心臓超音波検査，心臓血管造影にて同様の診断がなされ，単心室修復に至った．
胎児超音波で冠動脈を起始部から系統的に観察することで，類洞交通の評価が可能となりそれにより，出生前から治療方針の検討ができ，家族への説明に有用であった．

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．

著者英表記誤記あり　(誤)Takeshi Kaji→(正)Takashi Kaji

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