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
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 33(3): 249-255 (2017)
doi:10.9794/jspccs.33.249

症例報告Case Report

成人期に一期的Fontan型手術を行った心尖下大静脈同側の1例Primary Fontan Procedure in an Adult Patient with Apicocaval Juxtaposition

1福島県立医科大学医学部心臓血管外科Department of Cardiovascular Surgery, Fukushima Medical University ◇ Fukushima, Japan

2福島県立医科大学医学部小児科Department of Pediatrics, Fukushima Medical University ◇ Fukushima, Japan

受付日:2017年2月6日Received: February 6, 2017
受理日:2017年4月24日Accepted: April 24, 2017
発行日:2017年5月1日Published: May 1, 2017
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Fontan型手術の適応は拡大し,条件も低年齢化したが,成人期に拡大可能か不明である.手術未施行の成人例は長期低酸素と容量負荷から低心機能と心拡大を伴うため,心尖下大静脈同側例へのTotal cavo-pulmonary connection(TCPC)手術では,導管ルートに配慮が必要となる.心尖下大静脈同側の成人例に一期的Fontan型手術を施行した.症例は,48歳男性で,小児期に右室性単心室と診断されたがFontan型手術に至らなかった.息切れと動悸から就労困難となった.診断は両大血管右室起始,右室性単心室,肺動脈弁と弁上部狭窄を認めた.精査し手術適応と判断した.手術は,肺動脈形成およびTCPC手術を行った.圧迫回避目的に導管は心尖同側心房内を通した.術後経過は良好で,術後32日目に退院し,退院後3ヶ月で職場に復帰した.成人期単心室症例に再評価を行い,一期的TCPCを施行した.成人期TCPCは有用な術式であるが,適応には慎重な判断を要する.心尖下大静脈同側で心拡大を伴う成人例に心房内ルートは有用であった.

The Fontan procedure is predominantly performed in children with a functionally univentricular heart. It remains unclear as to whether the Fontan procedure can be applied for adult patients. Furthermore, the optimal conduit route remains unknown for adults with apicocaval juxtaposition. Adult Fontan candidates are likely to have dilated ventricles with poor cardiac function caused by long-term hypoxia and ventricular volume overload. Consequently, an ideal course from the inferior vena cava to the pulmonary artery should be created with caution to avoid the formation of kink or collapse of the conduit. A 48-year-old man with a double outlet right ventricle, single right ventricle, along with valvular and supravalvular pulmonary stenosis, presented with exertional dyspnea and palpitation. The patient showed situs inversus with the cardiac apex and inferior vena cava on the left side. Preoperative catheterization data showed low pulmonary vascular resistance and pulmonary artery pressure because of valvular, supravalvular, and bilateral pulmonary artery stenosis. Primary fenestrated total cavopulmonary connection (TCPC), concomitant with pulmonary artery plasty, was performed. The conduit was constructed via an intra-atrial route to avoid being compressed posteriorly by the dilated ventricle. The postoperative course was uneventful, and the patient was discharged 32 days after surgery. His exercise tolerance improved gradually thereafter. He returned to his previous job 3 months after surgery. Our case demonstrates that adult Fontan candidates whose preoperative conditions satisfy appropriate criteria are capable of undergoing primary TCPC. However, surgical indications for adult TCPC should be carefully determined. In our present case, the intra-atrial conduit route was useful for an adult patient with apicocaval juxtaposition.

Key words: Fontan procedure; adult congenital heart disease; total cavopulmonary connection; apicocaval juxtaposition; intraatrial conduit

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This page was last modified on 2017-06-05T14:34:29.844+09:00


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