An Adult Case of D-TGA with PAH and Irreversible Hepatic Failure after Mustard Operation

DOI
  • Ueda Hironobu
    Department of Cardiology, Iwate Medical University School of Medicine
  • Takahashi Shin
    Department of Cardiology, Iwate Medical University School of Medicine
  • Sasaki Koto
    Department of Cardiology, Iwate Medical University School of Medicine
  • Koizumi Junichi
    Department of Cardiovascular Surgery, Iwate Medical University School of Medicine
  • Morino Yoshihiro
    Department of Cardiology, Iwate Medical University School of Medicine

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Other Title
  • 肺高血圧による肝不全を合併したMustard術後の完全大血管転位の成人例

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Prolonged pulmonary hypertension (PH) after Mustard operation for dextro-transposition of the great arteries (D-TGA) results in dysfunction of the functional pulmonic ventricle, the so-called anatomical left ventricle (aLV), and congestive liver cirrhosis. We report our experience with this in an adult. Case: A 38-year-old man had been diagnosed with D-TGA and ventricular septal defect after birth, and following the Blalock-Hanlon procedure and pulmonary artery banding in the neonatal period, Mustard operation and ventricular septal defect closure were performed at 6 years of age. PH was identified by cardiac catheterization at 13 and 21 years of age. At 38 years of age, when he visited our hospital, he had edema of the lower limbs, deep cyanosis, and severe hypoxemia with an oxygen saturation of 68% on room air. The patient’s condition was classified as Child-Pugh class grade B due to thrombocytopenia, elevated total bilirubin, and coagulation abnormalities. Cardiac catheterization revealed increased venous pressure and Mustard route pressure gradient, along with combined post-and pre-capillary PH. In the subsequent course of treatment, aLV dysfunction associated with PH, portal hypertension following congestive cirrhosis, and exacerbation of pulmonary arteriovenous fistula were identified as the causes of hypoxia. Subsequently, the patient died of multiple organ failure.Conclusion: D-TGA cases are often fatal due to pre-capillary PH and Mustard root stenosis after Mustard operation, in addition to anatomical right ventricular dysfunction, which is functionally a systemic ventricle. PH treatment requires early intervention and continuous management into adulthood.

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