The clinical characteristics of 5 patients with inherited hypertrophic cardiomyopathy
10.3760/cma.j.issn.0253-3758.2009.04.008
- VernacularTitle:肥厚型心肌病样心肌病五例及其分析
- Author:
Ji-Qiang HE
1
;
Zhi-Hong HAN
;
Xue-Jun REN
;
Yue-Chun GAO
;
Xiao-Ling ZHANG
;
Teng-Yong JIANG
Author Information
1. 首都医科大学附属北京安贞医院
- Keywords:
Cardiomyopathies;
Diagnosis,differential
- From:
Chinese Journal of Cardiology
2009;37(4):320-323
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical characteristics of patients with inherited hypertrophic cardiomyopathy.Methods The clinical characteristics,electrocardiogram,serum chemistry and diagnostic methods were retrospectively investigated in 5 patients with inherited hypertrophic cardiomyopathy.Results The electrocardiograms of all patients were abnormal.with prominent left ventricular voltage and ST-T changes.One male patient with clinicopathological features of early onset,muscle weakness,ventricular preexcitation,elevations of two Serum proteins and intracytoplasmic vacuoles containing autophagic material and glycogen in biceps brachill muscle cells was diagnosed Danon's disease.Mitochondrial cardiomyopathy was diagnosed in one male patient with early onset,short PR interval and biopsy findings of ragged-red fibers in biceps brachill muscle.Three patients were diagnosed as Fabry's disease with clinical characteristics including pain and acroparesthesias,angiokeratoma and decrease of α-galactosidaseA activity.Conclusion Some of the rare inherited hypertrophic cardiomyopathy misht easily be clinically misdiagnosed as hypertrophic cardiomyopathy,systemic and careful case history inquiring and specific relevant examinations would help to make the right diagnosis in these patients.
