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 34(4): 197-204 (2018)

症例報告Case Report

4D flow MRIを用いた血行動態評価が有用であったTCPC術後導管屈曲,蛋白漏出性胃腸症の1例Hemodynamics Assessment with Four-Dimensional Flow MRI for a Case of Total Cavopulmonary Connection with Extracardiac Conduit Kinking and Protein-Losing Enteropathy

1京都府立医科大学附属小児医療センター小児心臓血管外科Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine ◇ Kyoto, Japan

2滋賀医科大学小児科Department of Pediatrics, Shiga University of Medical Science ◇ Shiga, Japan

3京都府立医科大学心臓血管外科Department of Cardiovascular Surgery, Kyoto Prefectural University of Medicine ◇ Kyoto, Japan

受付日:2018年5月14日Received: May 14, 2018
受理日:2018年7月4日Accepted: July 4, 2018
発行日:2018年12月20日Published: December 20, 2018

Four-dimensional flow magnetic resonance imaging (4D flow MRI)による血流可視化によって3次元的な血流の拍動がとらえられ,さらに3次元血流速度分布の流体力学的な解析はwall share stressやflow energy loss等の心血管系への力学的なストレスを定量可能とし,先天性心疾患の治療方針決定への応用が期待される.今回心外導管型Fontan術後の導管の屈曲と蛋白漏出性胃腸症(protein-losing enteropathy: PLE)のため循環動態の把握が治療方針に関わる症例に対して4D flow MRIでの血流解析を行ったため報告する.症例は,右室型単心室,肺動脈閉鎖の14歳男児であった.2歳時に心外導管total cavopulmonary connection (18 mm expanded polytetrafluoroethyleneグラフト)を施行し,術後3年目にPLEを発症,ステロイド依存状態となった.CTで心外導管中央に石灰化を伴う屈曲を認め,再手術適応評価のため精査を行った.カテーテル検査では屈曲部での圧較差は認めず,平均肺動脈圧とRVEDPの上昇を認め,等容拡張期の圧低下の遅れを認めた.4D flow MRIでは導管屈曲部および心室内での血流加速はなく,flow energy lossは有意でなかった.この結果より導管交換のみではPLE改善が見込めないことが示唆され,心室の拡張障害に対する内科的治療を先行する方針となった.

Four-dimensional flow magnetic resonance imaging (4D flow MRI) visualizes three-dimensional pulsatile blood flow and allows quantification of the mechanical stress to the cardiovascular system. We report a case of total cavopulmonary connection (TCPC) with a kinked extracardiac conduit in a 14-year-old boy with protein-losing enteropathy (PLE), for which a 4D flow MRI assessment of hemodynamics proved useful. Aged 2 years, the patient underwent extracardiac TCPC with an 18-mm expanded polytetrafluoroethylene conduit for a single right ventricle and pulmonary atresia. He developed PLE 3 years later, which was controlled with steroids. Aged 14 years, computed tomography revealed a kinked conduit with calcification. Cardiac catheterization showed no drop in pressure in the kinked portion, but the mean pulmonary arterial and right ventricular end-diastolic pressures were elevated. A systemic right ventricular pressure curve demonstrated a slow pressure decrease in the isovolumic relaxation phase, with a prolonged time constant, and 4D flow MRI demonstrated no flow acceleration through the kinked portion or in the systemic ventricle, with sufficient low-flow energy loss. We decided initially to optimize the patient’s medication to improve diastolic dysfunction, and then to perform a conduit exchange in the future once the steroid dose was reduced.

Key words: 4D flow MRI; protein-losing enteropathy; total cavopulmonary connection; hemodynamics; flow energy loss

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