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 39(1): 18-24 (2023)

症例報告Case Report

右室依存性冠循環を伴った右心低形成症候群に対する上行大動脈右室短絡術前後の冠血流評価Evaluation of Coronary Perfusion Using Pulsed-Wave Doppler in Hypoplastic Right Heart Syndrome with Right Ventricle-Dependent Coronary Circulation Before and After Aorta-To-Right Ventricle Shunt

1大阪母子医療センター小児循環器科Department of Pediatric Cardiology, Osaka Women’s and Children’s Hospital ◇ Osaka, Japan

2大阪母子医療センター心臓血管外科Department of Cardiovascular Surgery, Osaka Women’s and Children’s Hospital ◇ Osaka, Japan

受付日:2022年7月28日Received: July 28, 2022
受理日:2023年1月11日Accepted: January 11, 2023
発行日:2023年2月1日Published: February 1, 2023

症例は在胎39週5日,2,910 gにて出生した男児で,肺動脈閉鎖,右室低形成,心室中隔欠損症,左冠動脈起始部閉鎖,右室依存性冠循環(RVDCC)と診断された.冠血流の評価に,類洞交通の血流パターンをフォローした.初回手術前は,収縮期に右室から左室心筋へ,拡張期に左室心筋から右室へ向かう2相性血流であった.月齢1に左Blalock–Taussigシャント術を施行後,循環動態が不安定となり心筋虚血が疑われた.類洞交通の血流パターンは,収縮期に右室から左室心筋へ,拡張早期に左室心筋から右室へ,拡張後期に右室から左室心筋へ向かう3相性パターンに変化していた.月齢2に上行大動脈右心室短絡術(Ao-RVシャント)を施行し臨床症状の改善を認めた.術後の類洞交通血流パターンは術前と同じ3相性パターンであったが,拡張早期にAo-RVシャントを通して酸素化された血液が右室に流れ,拡張後期に右室から左室心筋へ灌流することで虚血が改善したと推察される.Ao-RVシャントは,RVDCCを伴う心筋虚血に対し有用な治療であり,類洞交通の血流パターンを評価することは心筋虚血の予測に有用と考えられる.

We present a male infant diagnosed with pulmonary atresia, hypoplastic right ventricle, ventricular septal defect, atresia of the orifice of the left coronary artery, and right ventricle-dependent coronary circulation. We assessed the blood flow in the sinusoidal communication (SC) utilizing pulsed-wave Doppler echocardiography to calculate coronary perfusion during the clinical course. At the time of delivery, the SC blood flow had a biphasic pattern; antegrade from the right ventricle (RV) to the left ventricle (LV) intramyocardium during systole and retrograde from the LV intramyocardium to the RV during diastole. The patient received a Blalock–Taussig (BT) shunt method at the age of 1 month. Following the BTS method, myocardial ischemia developed, and the SC blood flow showed a triphasic pattern; antegrade from the RV to the LV intramyocardium during systole, retrograde from the LV intramyocardium to the RV during early diastole, and antegrade from the RV to the LV intramyocardium during late diastole. The increase in diastolic RV pressure after the BTS method caused a reversal of the late diastolic blood pressure between the RV and LV myocardium, which was considered to induce a flow of non-oxygenated flow through the SC, leading to LV myocardial ischemia. An aorta-to-right ventricle (Ao-RV) shunt method was conducted at the age of 2 months. The SC blood flow revealed the same triphasic pattern, but the myocardial ischemia vanished. We hypothesize that the increased oxygenation of the SC blood flow during late diastole from the Ao-RV shunt enhanced the coronary circulation. Evaluation of SC blood flow patterns is effective for predicting myocardial ischemia.

Key words: right ventricle-dependent coronary circulation; aorta-right ventricle shunt; pulmonary atresia; sinusoidal communication; pulsed-wave Doppler method

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