家族性肥厚型心肌病一家系的调查分析

目的:分析一家族性肥厚型心肌病的特点。方法:对我院就诊的一肥厚型心肌病大家系进行临床调查研究,分析其临床特点,绘制家系图谱。结果:该家系为连续四代遗传,家系成员共35例,患者11例,猝死3例,死亡2例。有1例患者房颤及脑梗塞,2例患者行永久性起搏器植入术,猝死年龄最小3岁,符合肥厚型心肌病高发病率、高猝死率、发病年龄早等特点。结论:家族性肥厚型心肌病详细的家系调查有助于了解疾病全貌,更好地揭示其遗传规律。

Investigation and Analysis of One Family with Familial Hypertrophic Cardiomyopathy Pedigree

Objective:To analysis the clinical features of a familial hypertrophic cardiomyopathy.Methods:A family with Hypertrophic cardiomyopathy history was investigated and the clinical features were analyzed in our hospital.Results:The disease was continued for 4 generations in this family. There 11 cases out of 35 family members. Among themthere were 3 cases of sudden death and 2 cases of death. One patient had atrial fibrillation and cerebral infarction. Permanent pacemaker had been implanted into 2 patients. The minimum age of death was 3. All of these conform to the characteristics of high incidence, high death rate, and early onset in patients.Conclusion:By investigating whole family of familial hypertrophic cardiomyopathy in detail, we will gain more knowledge about this disease and better revealing its genetic regularity.