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 37(2): 126-132 (2021)

症例報告Case Report

心房内血流転換を併施し心内修復術を行った内臓錯位症候群・左側相同,鏡像型右胸心,完全型房室中隔欠損,単心房,下大静脈欠損,両側上大静脈の一例Intra-Atrial Rerouting of Anomalous Systemic Venous Connection and Septation of Common Atrium in Intracardiac Repair of Complete Atrioventricular Septal Defect with Left Isomerism and Dextrocardia: A Case Report

1北海道大学大学院医学研究院循環器呼吸器外科Department of Cardiovascular and Thoracic Surgery, Hokkaido University Graduate School of Medicine ◇ Hokkaido, Japan

2北海道大学大学院医学研究院小児科Department of Pediatrics, Hokkaido University Graduate School of Medicine ◇ Hokkaido, Japan

3手稲渓仁会病院心臓血管外科Department of Cardiovascular Surgery, Teine Keijinkai Hospital ◇ Hokkaido, Japan

4帯広厚生病院小児科Department of Pediatrics, Obihiro Kosei Hospital ◇ Hokkaido, Japan

受付日:2020年8月25日Received: August 25, 2020
受理日:2020年11月23日Accepted: November 23, 2020
発行日:2021年8月1日Published: August 1, 2021

内臓錯位症候群・左側相同に関連する心疾患では下大静脈欠損や両側上大静脈などの体静脈還流異常や単心房を合併することも多く,二心室修復の際には体静脈の再建も要する.今回我々は,内臓錯位症候群・左側相同,鏡像型右胸心,房室中隔欠損,単心房,両側上大静脈,下大静脈欠損,半奇静脈接続の4歳女児に対し,心房内血流転換を含めた心内修復術を行った.術前CTでは体静脈の心外再建には不向きな心大血管形態で,共通肺静脈腔の存在から心房内血流転換が適していると判断し,右上大静脈の血流を左側心房へ心房内血流転換しつつ,房室中隔欠損に対してmodified one patch法で共通房室弁の分割と心房中隔作成を行った.術後検査では体静脈や肺静脈の狭窄は認めず,経過良好で11日目に自宅退院となった.共通肺静脈腔を呈している場合は,心房内血流転換は体静脈還流異常に対して有用な術式となりうる.

Left isomerism is frequently associated with anomalous systemic venous connection and common atrium, requiring systemic venous abnormality correction and common-atrium septation in biventricular repair. Our case was a 4-year-old girl diagnosed with left isomerism, dextrocardia, complete atrioventricular septal defect, common atrium, bilateral superior vena cava, and interruption of the inferior vena cava with hemiazygos vein connection. She underwent intracardiac repair with intra-atrial rerouting. Preoperative computed tomography suggested that extracardiac reconstruction of the anomalous systemic vein was inappropriate, and showed that all the pulmonary veins drain to the common chamber in the common atrium. Thus, the blood flow from the right superior vena cava was diverted into the left side of the common atrium through an intra-atrial tunnel. Complete atrioventricular septal defect was repaired with modified one-patch method, and the patch divided the common atrium into the right and left atria. She was discharged on postoperative day 11 without obstruction in systemic and pulmonary venous return. Therefore, if all the pulmonary veins drain to the common chamber, intra-atrial rerouting may effectively correct anomalous systemic venous connection in the septation of the common atrium.

Key words: congenital heart disease; anomalous systemic venous connection; common atrium; left isomerism; complete atrioventricular septal defect

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