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

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
Pediatric Cardiology and Cardiac Surgery 36(1): 72-78 (2020)
doi:10.9794/jspccs.36.72

症例報告Case Report

全冠動脈孔閉鎖を伴う純型肺動脈閉鎖症に対するAo-RV シャントの新たな治療戦略：Ao-RV シャントの有用性を類洞交通血流の変化で評価New Aorta-To-Right Ventricle Shunt Strategy for Pulmonary Atresia with Intact Ventricular Septum and Aortocoronary Atresia: Evaluation by Monitoring Sinusoidal Communication Flow

吉井 公浩1，佐藤 純1，加藤 温子1，吉田 修一朗1，武田 紹1，西川 浩1，大沢 拓哉2，櫻井 寛久2，野中 利通2，櫻井 一2，大橋 直樹1Kimihiro Yoshii1, Jun Sato1, Atsuko Kato1, Shuichiro Yoshida1, Sho Takeda1, Hiroshi Nisikawa1, Takuya Osawa2, Takahisa Sakurai2, Toshimichi Nonaka2, Hajime Sakurai2, Naoki Ohashi1

1 JCHO中京病院中京こどもハートセンター小児循環器科Department of Pediatric Cardiology, Chukyo Children Heart Center, Japan Community Health Care Organization Chukyo Hospital ◇ Aichi, Japan

2 JCHO中京病院中京こどもハートセンター心臓血管外科Department of Cardiovascular Surgery, Chukyo Children Heart Center, Japan Community Health Care Organization Chukyo Hospital ◇ Aichi, Japan

受付日：2019年5月30日Received: May 30, 2019
受理日：2019年9月20日Accepted: September 20, 2019
発行日：2020年3月1日Published: March 1, 2020
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症例は妊娠39週6日，3,052 gで自然経腟分娩にて出生．胎児期に純型肺動脈閉鎖症（PA-IVS）と診断．日齢3に啼泣時の心電図のST変化から右室依存性冠循環（RVDCC）を疑い，心臓カテーテル検査にて，全冠動脈孔閉鎖（ACA）を伴うPA-IVSと診断された．SC血流の経時変化に注視し，パルスドップラー (PW）で評価し，日齢41にBTシャントおよび上行大動脈右心室短絡術（Ao-RVシャント）を施行．出生時のSC血流は収縮期にRV（右室）内から心筋内への順行性血流と，拡張期に心筋内からRV内への逆行性血流波形であったが，術後は収縮期に順行性血流，拡張早期は逆行性血流，新たに拡張末期に順行性血流を認め，2峰性順行性血流となった．加えて，Ao-RVシャントによりSCの酸素飽和度の上昇も冠循環の改善に寄与した．Ao-RVシャントはACAを伴うPA-IVSの新たな治療戦略として有用であった．

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.

Key words: pulmonary atresia with intact ventricular septum; aortocoronary atresia; sinusoidal communication; aorta to right ventricle shunt; transthoracic echocardiography

© 2020 特定非営利活動法人日本小児循環器学会© 2020 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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