Acar J, Vahanian A, Passeleau M, Gabay D, Vernant P, Corone P, Guérin F
Arch Mal Coeur Vaiss. 1984 Apr;77(4):397-404.
The aim of this cooperative study was to analyse the clinical profile of subvalvular aortic stenosis (SVAS) in adults. Thirty one cases were collected : patients aged 17 to 60 years (average 34 years). The diagnosis was confirmed in all patients either at surgery (30 cases) or at autopsy (1 case). The overall incidence of SVAS in patients with fixed obstruction to left ventricular ejection operated in the same period was 3.5%. Associated congenital malformations were detected in 15% of cases but intracardiac shunts were rare. Adult SVAS is a purer malformation than in children, probably because of spontaneous and surgical selection. The anatomical type of stenosing lesion in this series was a subaortic ring or membrane; 45% of patients, usually over 40 years of age (7 out of 9) had localised septal hypertrophy at the level of the diaphragmatic obstruction. The clinical presentation was that of mixed aortic disease with associated stenosis and regurgitation. Over 80% of patients had aortic incompetence diagnosed clinically (26 out of 31 cases) or on aortography (24 out of 25 cases); the regurgitant flow was assessed as mild in 15 cases, moderate in 6 cases and severe in 3 cases. There were obvious valvular lesions at surgery in 19 cases, especially in patients over 40 years of age (8 out of 9 cases). The mechanism was variable : non-specific (8 cases), destructive (6 cases), rheumatic (2 cases) or congenital (3 cases). Bacterial endocarditis was particularly common (26% of cases). Adult SVAS is a particularly difficult clinical diagnosis : the electrocardiogram and chest X-ray are not very informative.(ABSTRACT TRUNCATED AT 250 WORDS)
这项合作研究的目的是分析成人主动脉瓣下狭窄(SVAS)的临床特征。共收集了31例病例:患者年龄在17至60岁之间(平均34岁)。所有患者均在手术时(30例)或尸检时(1例)确诊。同期接受左心室射血固定梗阻手术的患者中,SVAS的总体发病率为3.5%。15%的病例检测到相关先天性畸形,但心内分流很少见。与儿童相比,成人SVAS是一种更纯粹的畸形,可能是由于自发选择和手术选择的原因。本系列中狭窄病变的解剖类型为主动脉下环或膜;45%的患者,通常年龄超过40岁(9例中的7例)在膈肌梗阻水平有局限性室间隔肥厚。临床表现为伴有相关狭窄和反流的混合性主动脉疾病。超过80%的患者临床上(31例中的26例)或主动脉造影时(25例中的24例)被诊断为主动脉瓣关闭不全;反流程度评估为轻度15例、中度6例、重度3例。19例手术时有明显瓣膜病变,尤其是40岁以上患者(9例中的8例)。机制各不相同:非特异性(8例)、破坏性(6例)、风湿性(2例)或先天性(3例)。细菌性心内膜炎尤为常见(26%的病例)。成人SVAS是一种特别难的临床诊断:心电图和胸部X线检查提供的信息不多。(摘要截选至250字)