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 32(3): 215-220 (2016)

症例報告Case Report

2回の肺静脈狭窄解除術を経てFontan型手術に到達し得た下心臓型総肺静脈還流異常を合併した単心室の1例Fontan Completion after Release of Refractory Pulmonary Venous Obstruction: A Case Report of Univentricular Heart with Infracardiac Total Anomalous Pulmonary Venous Connection

1兵庫県立尼崎総合医療センター心臓血管外科Departments of Cardiovascular Surgery, Hyogo Prefectural Amagasaki General Medical Center ◇ Hyogo, Japan

2兵庫県立尼崎総合医療センター小児循環器内科Pediatric Cardiology, Hyogo Prefectural Amagasaki General Medical Center ◇ Hyogo, Japan

受付日:2015年8月26日Received: August 26, 2015
受理日:2016年2月24日Accepted: February 24, 2016
発行日:2016年5月1日Published: May 1, 2016

無脾症候群では多様な心奇形を呈する.そのほとんどが単心室であり,Fontan型手術を目標として治療を行うことが多いが,肺動脈,肺静脈の問題を合併することがあり予後が悪い.今回,下心臓型総肺静脈還流異常を合併した無脾症候群の低体重出生時に対する2回の肺静脈狭窄解除を経験した.生後8日2,097 gで初回手術(総肺静脈還流異常修復,BTシャント変法,PDA離断部肺動脈形成)を行った.肺静脈狭窄が進行し,外科的に肺静脈狭窄解除を行ったが,術後肺静脈再狭窄が進行した.そこでBTシャント変法追加による肺血流量の増加と外科的肺静脈狭窄の再解除を連続して行い,肺静脈血流の確保を計画した.その後,段階的に肺動脈形成を行うことで,Fontan型手術に到達した.計画的な肺血流の調整が肺静脈狭窄の進行予防と肺動脈発育に寄与したと考えられた.

Patients with asplenia syndrome usually have various cardiac malformations. Although most of these patients are Fontan candidates, some cannot achieve Fontan completion because of pulmonary artery and vein problems. We report a case of asplenia syndrome with Fontan completion achieved after pulmonary venous obstruction (PVO) was repaired twice. The patient underwent her first operation (repair of total anomalous venous connection, modified Blalock–Taussig [BT] shunt, patent ductus arteriosus division, and pulmonary artery plasty) at 8 days of age (body weight: 2,097 g). As the pulmonary venous orifice was obstructed postoperatively, we performed surgical PVO release, but left PVO occurred again. We created a left modified BT shunt to increase pulmonary flow to the left pulmonary artery and preserve pulmonary vein flow, and performed consecutive PVO release . Subsequently, Fontan completion was achieved through a bidirectional Glenn operation with additional flow from the systemic to the pulmonary artery. Planned adjustment of pulmonary blood flow can be an effective way to prevent progression of PVO and promote pulmonary artery growth.

Key words: pulmonary venous obstruction; asplenia; total anomalous pulmonary venous connection; Fontan

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