O'rourke R A, Crawford M H
Curr Probl Cardiol. 1976 Mar-Apr;1(1):1-60. doi: 10.1016/s0146-2806(76)80006-0.
The midsystolic click-late systolic murmur syndrome is a complex entity with variable manifestations that involves a primary process causing myxomatous degeneration of the mitral valve leaflet(s) and subsequent systolic mitral valve leaflet prolapse. Other cardiac diseases may cause mitral valve prolapse and regurgitation associated with a midsystolic click that mimics this primary syndrome. The prolapsing mitral valve leaflet(s) syndrome occasionally may be familial. Most patients are asymptomatic but some complain of chest pain, palpitation, dyspnea or fatigue. Prolapsing mitral valve leaflet(s) can be distinguished from other causes of systolic clicks and mitral regurgitation murmurs by the characteristic movement of the clikmurmur complex in systole with various hemodynamic interventions. The clinical diagnosis usually can be confirmed by echocardiography, which demonstrates the abnormally prolapsdrome usually is minimal but can be progressive and lead to the need for prosthetic valve replacement. Most symptomatic patients can be managed medically but some require cardiac catheterization to evaluate the possibility of coexistent coronary artery disease, to assess the degree of mitral regurgitation and to evaluate other associated cardiac lesions. All patients with this syndrome should receive antibiotic prophylaxis prior to any surgical or dental procedures. Those patients suspected of having arrhythmias should be evaluated by continuous ambulatory ECG monitoring and dangerous arrhythmias probably should be treated. The prognosis usually is excellent, but sudden death and rapidly progressive mitral regurgitation due to ruptured chordae tendineae have been reported. Although more than a decade has elapsed since the midsystolic click-late systolic murmur syndrome was first recognized, much remains to be learned about this common but complex clinical entity.
收缩中期喀喇音-收缩晚期杂音综合征是一种表现多样的复杂病症，涉及导致二尖瓣叶黏液瘤样变性及随后二尖瓣叶收缩期脱垂的原发性过程。其他心脏疾病可能导致二尖瓣脱垂和反流，并伴有类似该原发性综合征的收缩中期喀喇音。二尖瓣叶脱垂综合征偶尔可能具有家族性。大多数患者无症状，但有些患者会主诉胸痛、心悸、呼吸困难或疲劳。通过收缩期喀喇音-杂音复合征在各种血流动力学干预下的特征性运动，可将二尖瓣叶脱垂与其他导致收缩期喀喇音和二尖瓣反流杂音的原因区分开来。临床诊断通常可通过超声心动图得以证实，超声心动图可显示异常脱垂。该综合征通常病情较轻，但可能会进展，导致需要进行人工瓣膜置换。大多数有症状的患者可通过药物治疗，但有些患者需要进行心导管检查，以评估是否并存冠状动脉疾病、评估二尖瓣反流的程度以及评估其他相关心脏病变。所有患有该综合征的患者在进行任何外科手术或牙科手术前均应接受抗生素预防。怀疑有心律失常的患者应通过连续动态心电图监测进行评估，对于危险的心律失常可能应予以治疗。预后通常良好，但曾有因腱索断裂导致猝死和快速进展性二尖瓣反流的报道。尽管自首次认识到收缩中期喀喇音-收缩晚期杂音综合征已过去十多年，但对于这个常见但复杂的临床病症仍有许多有待了解之处。
