title: Orthotropic heart transplantation for adult congenital heart disease: a case with heterotaxy and dextrocardia.
abstract: A 41-year-old male with heterotaxy (left isomerism) and dextrocardia composed by single ventricle, absent inferior vena cava, bilateral superior vena cava (SVC), common atrioventricular valve has received orthotopic heart transplantation (HTx) after long waiting period as Status-1. Reconstructions of bilateral SVC and hepatic vein route were successful without use of prosthetic material, and the donor heart was placed in the left mediastinum. In spite of satisfactory early recovery, the patient expired 4 months after transplantation mainly from fungal infection which developed following humoral rejection. HTx for adult patients with complex congenital heart disease is demanding in technical as well as pre- and post-transplant management, and indication should be critically determined.
authors.affiliation: Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Yamadaoka 2-2, Suita, Osaka, 565-0871, Japan. hmatsudahuhs@gmail.com.|Higashi Takarazuka Satoh Hospital, Nagaochou 2-1, Takarazuka, Hyogo, 665-0873, Japan. hmatsudahuhs@gmail.com.
authors.forename: Hikaru
authors.initials: H
authors.lastname: Matsuda
authors.affiliation: Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Yamadaoka 2-2, Suita, Osaka, 565-0871, Japan.
authors.forename: Norihide
authors.initials: N
authors.lastname: Fukushima
authors.affiliation: Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Yamadaoka 2-2, Suita, Osaka, 565-0871, Japan.|National Cerebral and Cardiovascular Center, Osaka, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Ichikawa
authors.affiliation: Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Yamadaoka 2-2, Suita, Osaka, 565-0871, Japan.
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Sawa
mesh_terms: D000328:Adult; D003914:Dextrocardia / Q000000981:diagnostic imaging / Q000601:surgery; D017809:Fatal Outcome; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000601:surgery*; D016027:Heart Transplantation / Q000379:methods*; D006352:Heart Ventricles / Q000503:physiopathology; D059446:Heterotaxy Syndrome / Q000601:surgery; D006801:Humans; D008297:Male; D011859:Radiography / Q000379:methods; D014682:Vena Cava, Inferior / Q000002:abnormalities; D014683:Vena Cava, Superior / Q000002:abnormalities
keywords: Adult congenital heart disease; Dextrocardia; Heart transplantation; Heterotaxy; Humoral rejection