A Case of Pulmonary Hypertension and Intrapulmonary Shunt Caused by Porto-Pulmonary Shunt with Noonan Syndrome

• Nagata Yoshihiro

  Department of Pediatrics, Okazaki City Hospital

• Kato Taichi

  Department of Pediatrics, Graduate School of Medicine, University of Nagoya

• Makita Satoru

  Department of Pediatric Surgery, Graduate School of Medicine, University of Nagoya

• Ikeda Maiko

  Department of Pediatrics, Okazaki City Hospital

• Fukazawa Yoshie

  Department of Pediatrics, Graduate School of Medicine, University of Nagoya

• Kishimoto Yasuaki

  Department of Pediatrics, Graduate School of Medicine, University of Nagoya

• Numaguchi Atsushi

  Department of Emergency and Critical Care Medicine, Nagoya University Hospital

• Nagai Noriko

  Department of Pediatrics, Okazaki City Hospital

Bibliographic Information

Other Title

• 門脈体循環シャントによる肺高血圧症及び肺内シャントを合併したNoonan症候群の1例

Abstract

Portopulmonary hypertension and hepatopulmonary syndrome are distinctive diseases characterized by vasoconstriction and vasodilation, respectively. To date, only a few cases coexisting both clinical states have been reported. Here we present the Noonan syndrome patient with coexisting portopulmonary hypertension and hepatopulmonary syndrome. In this case, the patient with Noonan syndrome was followed-up by cardiac ultrasonography for mild pulmonary stenosis and patent foramen ovale. After pulmonary stenosis and patent foramen ovale improved, pulmonary arterial hypertension and hepatopulmonary syndrome developed over a half year. Coexisting portosystemic shunt was subsequently diagnosed. Administration of bosentan and sildenafil, and home oxygen therapy improved subjective symptoms. On the other hand, the findings of echocardiography had no change in estimated pulmonary arterial pressure. The existence of intrahepatic portal vein was confirmed by liver biopsy and contrast enhanced computer tomography, which provided definitive diagnosis of “congenital extrahepatic portosystemic shunt Type 2”. After the ligation of portosystemic shunt, while pulmonary arterial hypertension remained unchanged, liver function and exercise capacity improved six month after surgery. This case is the first report that shows the ligation was conducted for treatment of congenital extrahepatic portosystemic shunt Type 2 with portopulmonary hypertention and hepatopulmonary syndrome, and provides highly suggestive clinical course to establish a standard therapy for these rare complications coexisted.

Journal

• Pediatric Cardiology and Cardiac Surgery

  Pediatric Cardiology and Cardiac Surgery 31 (4), 212-219, 2015

  Japanese Society of Pediatric Cardiology and Cardiac Surgery

Keywords

• portopulmonary hypertension
• hepatopulmonary hypertension
• congenital extrahepatic portosystemic shunt Type 2
• Noonan syndrome

Details

• CRID: 1390001204671806848
• NII Article ID: 130005098183
• DOI: 10.9794/jspccs.31.212
• ISSN: 21872988
• ISSN: 09111794
• Web Site: https://www.jstage.jst.go.jp/article/jspccs/31/4/31_1953/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2015379284
• Text Lang: ja
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed