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

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...

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Bibliographic Details
Published in:ESC Heart Failure 2020-04, Vol.7 (2), p.721-726
Main Authors: Yamamoto, Masahiro, Takashio, Seiji, Nakashima, Naoya, Hanatani, Shinsuke, Arima, Yuichiro, Sakamoto, Kenji, Yamamoto, Eiichiro, Kaikita, Koichi, Aoki, Yoko, Tsujita, Kenichi
Format: Article
Language:English
Subjects:
Adult congenital heart disease
Blood pressure
Cardiomyopathy
Cardiovascular disease
Case Report
Congenital diseases
Defects
Double‐chambered right ventricle
Electrocardiography
Heart
Magnetic resonance imaging
Mutation
Noonan syndrome
Obstructive hypertrophic cardiomyopathy
Orthopedics
Patients
Peptides
Pharmacological therapy
Pulmonary arteries
Spinal cord
Surgery
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Summary: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.
ISSN:2055-5822
2055-5822
DOI:10.1002/ehf2.12650