Double-chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome

Masahiro Yamamoto, Seiji Takashio, Naoya Nakashima, Shinsuke Hanatani, Yuichiro Arima, Kenji Sakamoto, Eiichiro Yamamoto, Koichi Kaikita, Yoko Aoki, Kenichi Tsujita

Research output: Contribution to journalArticlepeer-review

Abstract

We present a case of double-chambered right ventricle (DCRV) complicated by hypertrophic obstructive cardiomyopathy (HOCM) in KRAS mutation-associated Noonan syndrome. The diagnosis was incidental and made during diagnostic testing for an intradural extramedullary tumour. Spinal compression, if not surgically treated, may cause paralysis of the extremities. We decided to pursue pharmacological therapy to control biventricular obstructions and reduce the perioperative complication rate. We initiated treatment with cibenzoline and bisoprolol; the doses were titrated according to the response. After 2 weeks, the peak pressure gradient of the two RV chambers decreased from 101 to 68 mmHg, and the LV peak pressure gradient decreased from 109 to 14 mmHg. Class 1A antiarrhythmic drugs and β-blockers decreased the severe pressure gradients of biventricular obstructions caused by DCRV and HOCM. The patient was able to undergo surgery to remove the intradural extramedullary tumour, which was diagnosed as schwannoma.

Original languageEnglish
Pages (from-to)721-726
Number of pages6
JournalESC Heart Failure
Volume7
Issue number2
DOIs
Publication statusPublished - 2020 Apr 1

Keywords

  • Adult congenital heart disease
  • Double-chambered right ventricle
  • Noonan syndrome
  • Obstructive hypertrophic cardiomyopathy
  • Pharmacological therapy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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