药物与手术治疗肥厚型心肌病伴室壁瘤的疗效对比分析

Management of Hypertrophic Cardiomyopathy with Left Ventricular Apical Aneurysms: Prognosis and Comparison of Medical and Surgical Treatment

目的探讨肥厚型心肌病伴室壁瘤的发生率、临床特点、治疗和预后。方法连续收集在我院诊治的1844例肥厚型心肌病患者,通过超声、心脏核磁和左室造影筛选室壁瘤,对其临床资料进行分析和随访。结果肥厚型心肌病合并室壁瘤患者22例(患病率1.1%),其中11例患者反复发作持续性室速,9例伴有附壁血栓。随访4.5±3.0年后,18例无手术患者左心扩大、收缩功能下降,其中11例发生心脏性死亡、进展性心衰或卒中;另4例经室壁瘤切除术后左室功能逆转,无不良事件发生。2例瘤内有存活心肌的室壁瘤患者发生心脏性猝死,另1例手术后存活。结论肥厚型心肌病伴室壁瘤的主要临床表现是持续性室速和附壁血栓,其预后极差,室壁瘤切除术可有效改善预后,而瘤部位存活心肌可能是其心脏性猝死的预测因子和早期手术指征。

Objective This study was aimed to evaluate the prevalence, clinical course, therapy, and prognosis of hypertrophic cardiomyopathy （HCM） patients with left ventricular apical aneurysms （LVAAs）. Methods From 2002 to 2013, 1844 HCM patients were consecutively recruited at Fuwai Hospital. Medical history, including data from echocardiography, cardiac magnetic resonance, and cardiac nuclear imaging was analyzed. Results LVAAs were identified in 22 HCM patients （1.1%）, with their average age at 50.4±13.8 years old in study entry. Recurrent sustained ventricular tachycardia and mural thrombus were relatively observed in 11 and 9 patients. The segments with perfusion defect in nuclear imaging （n=12） were in accordance with that of transmural fibrosis in magnetic resonance （n=19）. Left ventricular remodeling with cavity dilation and systolic dysfunction was substantially shown in medical treated patients （n=18） but was reversed by aneurysmectomy （n=4）. During 4.5±3.0 years of follow-up, 11 medical patients experienced adverse clinical events （annual event rate, 11.1 % year）, but no adverse event in surgical patients. Two of three patients with viable myocardium in aneurysm experienced cardiac death and the residual one who underwent surgery stably survived. Conclusion HCM patients with LVAAs are associated with substantial cardiovascular complications and aneurysmectomy is an effective therapy to improve prognosis. Aneurysmal viability may early predict a worse outcome and put forward urgent requirement of aneurysmectomy for HCM patients.