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肥厚性梗阻性心肌病:外科治疗及结果

[Hypertrophic obstructive cardiomyopathy: surgical treatment and results].

作者信息

Schulte H D, Lösse B

出版信息

Herz. 1985 Apr;10(2):102-11.

PMID:4039290
Abstract

The natural history of hypertrophic obstructive cardiomyopathy is characterized, in particular, in younger patients by sudden cardiac death, in the majority of patients by progressive congestive heart failure [14, 26, 27, 33-36]. With the aid of beta-adrenergic receptor blockers, calcium channel blockers and antiarrhythmic agents the clinical outlook for most of the patients can be improved [17, 19, 23, 28]. Patients who do not respond to medical treatment can be regarded as possible candidates for surgical revision of the left ventricular outflow tract. In consideration of surgical treatment, two morphologic and hemodynamic forms of the disease must be differentiated: the typical subaortic muscular hypertrophy in the region of the outflow tract and the atypical, more mid-ventricular muscular hypertrophy with systolic entrapment of a distal portion of the ventricle in which frequently a typical subaortic hypertrophy also may be present [15, 43]. As a result of septal subvalvular hypertrophy which may substantially compromise left ventricular ejection during systolic contraction, in hypertrophic obstructive cardiomyopathy there is a deviation of the left ventricular major axis, dyscoordination in the sequence of contraction, hypertrophy of the left ventricular walls, diminished diastolic compliance and, to a varying extent, mitral regurgitation. Diagnostically useful findings are a mid-systolic murmur at the aortic valve, bisferiens carotis pulse, systolic pressure gradient between the left ventricle and the aorta and a postextrasystolic paradoxical pressure response. Echocardiographically, asymmetric septal hypertrophy, systolic anterior motion of the anterior mitral valve leaflet, premature mid-systolic closure of the aortic valve and an enlarged left atrium will be detected. Surgical intervention is indicated for patients with class III clinical symptoms (NYHA) not amenable to medical treatment and pressure gradients between left ventricle and aorta, at rest or postextrasystolically, in excess of 50 mm Hg. An overview of the variety of surgical approaches and procedures employed for hypertrophic obstructive cardiomyopathy is provided by Table 1. From initial attempts to eliminate the outflow tract obstruction by myotomy, the subvalvular myectomy was developed in which an about 1 cm wide section of muscle is resected; this procedure can be carried out from a transaortic, transatrial or transventricular approach [4, 9, 21, 22, 24, 38, 39, 47]. Under the assumption that the anterior motion of the anterior mitral valve leaflet contributes to systolic obstruction, some authors recommended reconstructive procedures or replacement of the mitral valve.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

肥厚性梗阻性心肌病的自然病史具有如下特点

在年轻患者中,尤其以心源性猝死为特征;在大多数患者中,则以进行性充血性心力衰竭为特征[14, 26, 27, 33 - 36]。借助β - 肾上腺素能受体阻滞剂、钙通道阻滞剂和抗心律失常药物,大多数患者的临床预后可以得到改善[17, 19, 23, 28]。对药物治疗无反应的患者可被视为左心室流出道手术矫正的可能候选者。考虑到手术治疗,必须区分该疾病的两种形态学和血流动力学形式:流出道区域典型的主动脉瓣下肌性肥厚,以及非典型的、更偏向心室中部的肌性肥厚,伴有心室远端部分的收缩期嵌顿,其中也可能经常存在典型的主动脉瓣下肥厚[15, 43]。由于间隔瓣下肥厚在收缩期收缩时可能严重损害左心室射血,在肥厚性梗阻性心肌病中存在左心室长轴偏移、收缩顺序不协调、左心室壁肥厚、舒张顺应性降低以及不同程度的二尖瓣反流。诊断上有用的发现包括主动脉瓣处的收缩中期杂音、双峰脉、左心室与主动脉之间的收缩期压力梯度以及早搏后矛盾性压力反应。超声心动图检查可发现不对称性间隔肥厚、二尖瓣前叶收缩期前向运动、主动脉瓣过早的收缩中期关闭以及左心房扩大。对于纽约心脏协会(NYHA)III级临床症状且药物治疗无效、静息或早搏后左心室与主动脉之间压力梯度超过50 mmHg的患者,建议进行手术干预。表1提供了用于肥厚性梗阻性心肌病的各种手术方法和程序的概述。从最初试图通过肌切开术消除流出道梗阻开始,发展出了瓣下肌切除术,即切除约1 cm宽的一段肌肉;该手术可通过经主动脉、经心房或经心室途径进行[4, 9, 21, 22, 24, 38, 39,

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