Hemodynamics Assessment with Four-Dimensional Flow MRI for a Case of Total Cavopulmonary Connection with Extracardiac Conduit Kinking and Protein-Losing Enteropathy

DOI Web Site 6 References
  • Fujita Shuhei
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine
  • Yamagishi Masaaki
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine
  • Miyazaki Takako
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine
  • Maeda Yoshinobu
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine
  • Itatani Keiichi
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine
  • Taniguchi Satoshi
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine
  • Hongu Hisayuki
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine
  • Hoshino Shinsuke
    Department of Pediatrics, Shiga University of Medical Science
  • Somura Junpei
    Department of Pediatrics, Shiga University of Medical Science
  • Yaku Hitoshi
    Department of Cardiovascular Surgery, Kyoto Prefectural University of Medicine

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  • 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

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Abstract

<p>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.</p>

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