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目的:分析46例致心律失常性右心室心肌病(ARVC)的临床特征,以期提高ARVC的诊断率,减少误诊率。方法:选择符合1994年欧洲心脏病协会诊断标准的ARVC患者46例,分析其临床表现、相关检查、诊治经过。结果:46例患者年龄3个月～75岁,男35例,女11例;临床症状为心悸、头晕者30例(65.2%),有心力衰竭症状者21例(45.7%),17例(37.0%)有晕厥史,3例(6.5%)有猝死家族史。28例(60.9%)静息心电图表现为平均QRS时程延长(>110ms),22例(47.8%)有epsilon(ε)波;46例患者均记录到不同类型的心律失常,同时并发3种及3种以上心律失常者23例(50.0%),持续性室性心动过速38例(82.6%)。全部患者入院时超声心动图检查平均右心室舒张末期内径(35.8±8.1)mm,左心房舒张末期内径(34.2±11.0)mm,左心室舒张末期内径(53.6±11.4)mm,左心室射血分数(44±20)%,共有16例患者行磁共振检查,2例患者行CT检查,其中显示右心房室扩大7例,并发右心室瘤样突出4例,有右心室脂肪浸润9例。6例患者行核素心血池显像均提示右心室扩大,右心室心功能重度受损,右心室射血分数(RVEF)(22±5)%。9例心脏移植患者病理检查提示左、右心室均有不同程度的心肌细胞萎缩、变性、坏死、纤维和脂肪组织大片的浸润,1例为右心室局灶性纤维组织增厚。10例首诊为扩张型心肌病,1例首诊为未分类心肌病,1例首诊为三尖瓣下移畸形,首诊误诊率为26.1%。结论:ARVC临床表现多样而复杂,误诊率高,应加强对ARVC的再认识以提高ARVC的诊断率。
Objective: To analyze the clinical features of 46 cases of arrhythmogenic right ventricular cardiomyopathy (ARVC) in order to improve the diagnosis rate of ARVC and reduce the misdiagnosis rate. Methods: Forty - six ARVC patients who met the diagnostic criteria of the European Association for Cardiology in 1994 were selected and their clinical manifestations, related examinations, diagnosis and treatment were analyzed. Results: 46 patients aged 3 months to 75 years old, 35 males and 11 females; Clinical symptoms were palpitations, dizziness in 30 cases (65.2%), heart failure symptoms in 21 cases (45.7%), 17 cases 37.0%) had a history of syncope, and 3 (6.5%) had a sudden family history of sudden death. Resting electrocardiogram showed prolonged QRS duration (> 110 ms) in 28 patients (60.9%) and epsilon (ε) wave in 22 patients (47.8%). All of the 46 patients had different types of arrhythmias and three concurrent 23 cases (50.0%) had more than 3 types of arrhythmia and 38 cases (82.6%) had persistent ventricular tachycardia. All patients underwent echocardiography with mean right ventricular end diastolic diameter (35.8 ± 8.1) mm, left atrial end-diastolic diameter (34.2 ± 11.0) mm, left ventricular end diastolic diameter (53.6 ± 11.4) mm, left ventricular ejection fraction (44 ± 20)%. A total of 16 patients underwent magnetic resonance imaging. Two patients underwent CT examination, including 7 cases of right atrium enlargement, 4 cases of right ventricular tumor-like protrusion and 9 cases of right ventricular fat infiltration. Radionuclide imaging showed that right ventricular enlargement, severe right ventricular dysfunction and right ventricular ejection fraction (RVEF) (22 ± 5)% in 6 patients. The pathological examination of 9 cases of heart transplantation patients showed that there was atrophy, degeneration, necrosis of cardiomyocytes, large infiltration of fibrous and adipose tissue in left and right ventricle, and thickening of the right ventricular focal fibrosis. The first diagnosis was dilated cardiomyopathy in 10 cases, the first diagnosis was unclassified cardiomyopathy, the first diagnosis was tricuspid regurgitation, the first diagnosis was misdiagnosed as 26.1%. CONCLUSIONS: The clinical manifestations of ARVC are diverse and complicated, and the rate of misdiagnosis is high. Re-cognition of ARVC should be strengthened to improve the diagnostic rate of ARVC.