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

被引：4

作者：

Yamamoto, Masahiro ^{[1
]}

Takashio, Seiji ^{[1
]}

Nakashima, Naoya ^{[1
]}

Hanatani, Shinsuke ^{[1
]}

Arima, Yuichiro ^{[1
]}

Sakamoto, Kenji ^{[1
]}

Yamamoto, Eiichiro ^{[1
]}

Kaikita, Koichi ^{[1
]}

Aoki, Yoko ^{[2
]}

Tsujita, Kenichi ^{[1
]}

机构：

[1] Kumamoto Univ, Ctr Metab Regulat Hlth Aging, Grad Sch Med Sci, Dept Cardiovasc Med, Kumamoto, Japan

[2] Tohoku Univ, Dept Med Genet, Sch Med, Sendai, Miyagi, Japan

来源：

ESC HEART FAILURE | 2020年 / 7卷 / 02期

基金：

日本学术振兴会;

关键词：

Adult congenital heart disease; Double-chambered right ventricle; Obstructive hypertrophic cardiomyopathy; Pharmacological therapy; Noonan syndrome; CARDIOVASCULAR MAGNETIC-RESONANCE;

D O I：

10.1002/ehf2.12650

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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 beta-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.

引用

页码：721 / 726

页数：6

共 15 条

[1] An uncommon variant of double-chambered right ventricle masquerading as double-chambered left ventricle [J].