Molisana Michela, Selimi Adelina, Gizzi Germana, D'Agostino Simone, Ianni Umberto, Parato Vito Maurizio
Cardiology and Cardiac Rehabilitation Unit, Madonna del Soccorso Hospital, San Benedetto del Tronto, Italy.
Cardiology and Arrhythmology Clinic, University Hospital "Umberto I-Lancisi-Salesi", Ancona, Italy.
Front Cardiovasc Med. 2022 Oct 28;9:1020054. doi: 10.3389/fcvm.2022.1020054. eCollection 2022.
Abnormalities of the mitral valve (MV) apparatus are typical features of hypertrophic cardiomyopathy (HCM). These abnormalities include leaflet elongation, thick leaflets, displacement of papillary muscle, and systolic anterior motion (SAM) of the MV anterior leaflet. Mitral valve chordal rupture associated with HCM is a rare but serious issue capable of change the clinical apparence and the prognosis of the patient.
A 57-year-old lady with a history of diabetes, dyslipidemia, and a previous single episode of atrial fibrillation (treated with pharmacological cardioversion), presented to the Emergency Department for worsening dyspnea (New York Heart Association Classification class IV). A trans-thoracic echocardiogram (TTE) showed a significant, septal, and asymmetric left ventricular hypertrophy (basal anteroseptal wall diastolic thickness of 19 mm) with normal left ventricle systolic function. A SAM of AML was evident together with a left ventricular outflow tract gradient of 56 mmHg at rest, rising to 136 mmHg during the Valsalva maneuver. In addition, there was evidence of moderate to severe mitral regurgitation (MR) with an anteriorly directed jet, not very typical of MR related to SAM. A 2D-3D trans-esophageal echocardiogram (2D-3D TEE) revealed a combined MR mechanism based on PML degenerative prolapse with P2-flail from ruptured chordae with related eccentric anteriorly directed regurgitant jet, together with a second regurgitant posteriorly directed jet, related to SAM of AML. The patient underwent MV repair together with septal myectomy, with a good final outcome.
Pre-operative echocardiography (both TTE and 2D-3D TEE) is an essential tool in order to detect different MV abnormalities in patients with HCM. These types of patients should never be treated by septal reduction alone. Surgical MV repair or replacement, together with septal myectomy, may be the preferred approach.
二尖瓣（MV）装置异常是肥厚型心肌病（HCM）的典型特征。这些异常包括瓣叶延长、瓣叶增厚、乳头肌移位以及MV前叶的收缩期前向运动（SAM）。与HCM相关的二尖瓣腱索破裂是一个罕见但严重的问题，能够改变患者的临床表现和预后。
一名57岁女性，有糖尿病、血脂异常病史，既往有单次房颤发作（经药物复律治疗），因呼吸困难加重（纽约心脏协会心功能分级IV级）就诊于急诊科。经胸超声心动图（TTE）显示显著的、间隔性的、不对称的左心室肥厚（基底前间隔壁舒张期厚度为19mm），左心室收缩功能正常。AML的SAM明显，静息时左心室流出道压差为56mmHg，瓦尔萨尔瓦动作时升至136mmHg。此外，有中度至重度二尖瓣反流（MR）的证据，反流束向前，不太符合与SAM相关的MR特点。二维-三维经食管超声心动图（2D-3D TEE）显示MR的综合机制，基于PML退行性脱垂伴P2瓣叶因腱索破裂而连枷样改变，伴有相关的偏心向前反流束，以及第二个向后反流束，与AML的SAM有关。患者接受了MV修复术和间隔心肌切除术，最终预后良好。
术前超声心动图检查（TTE和2D-3D TEE）是检测HCM患者不同MV异常的重要工具。这类患者绝不应仅通过间隔减容治疗。手术MV修复或置换，联合间隔心肌切除术，可能是首选方法。
