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) inKRAS 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 obstruc tions 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.

Source: ESC Heart Failure - February 27, 2020 Category: Cardiology Authors: Masahiro Yamamoto, Seiji Takashio, Naoya Nakashima, Shinsuke Hanatani, Yuichiro Arima, Kenji Sakamoto, Eiichiro Yamamoto, Koichi Kaikita, Yoko Aoki, Kenichi Tsujita Tags: Case Report Source Type: research