An Autopsy Case of Eisenmenger Syndrome with Pheochromocytoma

DOI Web Site 10 References
  • Miura Fumitake
    Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine
  • Yamamoto Yohei
    Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine
  • Shimada Jun
    Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine
  • Kitagawa Yosuke
    Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine
  • Tsuruga Kazushi
    Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine
  • Otani Katsuki
    Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine
  • Takahashi Toru
    Hirosaki University School of Health Sciences and Graduate School of Health Sciences
  • Yonesaka Susumu
    Hirosaki University School of Health Sciences and Graduate School of Health Sciences
  • Kato Noriko
    Department of Anatomic Pathology, Hirosaki University School of Medicine and Graduate School of Medicine
  • Ito Etsuro
    Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine

Bibliographic Information

Other Title
  • 褐色細胞腫を合併したEisenmenger症候群の剖検例

Search this article

Abstract

<p>We report a rare case of a patient with pheochromocytoma (PCC) and Eisenmenger syndrome (ES). The patient was a female born to healthy parents. Although double outlet right ventricle (DORV) was diagnosed in the neonatal period, she displayed ES pathology from infancy. At the age of 30 years, PCC complications were diagnosed. An operation was contraindicated because of high perioperative risk. She died at the age of 32 years owing to advanced heart failure and renal failure. Her pathologic anatomy revealed DORV, severe pulmonary vascular obstruction, PCC in the left adrenal gland, multiple paraganglioma (PGL) in the bladder, and cyanotic nephropathy. In recent years, hypoxia has been reported as a risk factor for PCC/PGL. In our case, PCC/PGL was associated with DORV and ES. The diagnosis of PCC/PGL in congenital heart disease patients tends to be delayed as the symptoms of PCC/PGL overlap with those of heart disease. The harmful effects of hypertension and/or tachycardia on cardiac hemodynamics due to PCC were difficult to manage as she was contraindicated for surgery. PCC/PGL should be considered as a complication of congenital heart disease.</p>

Journal

References(10)*help

See more

Keywords

Details 詳細情報について

Report a problem

Back to top