Different mechanisms of mitral regurgitation in hypertrophic cardiomyopathy: A clinical case and literature review

Michela Molisana; Adelina Selimi; Germana Gizzi; Simone D'Agostino; Umberto Ianni; Vito Maurizio Parato

doi:10.3389/fcvm.2022.1020054

Different mechanisms of mitral regurgitation in hypertrophic cardiomyopathy: A clinical case and literature review

Front Cardiovasc Med. 2022 Oct 28:9:1020054. doi: 10.3389/fcvm.2022.1020054. eCollection 2022.

Authors

Michela Molisana¹, Adelina Selimi^{2

3}, Germana Gizzi¹, Simone D'Agostino¹, Umberto Ianni¹, Vito Maurizio Parato^{1

3}

Affiliations

¹ Cardiology and Cardiac Rehabilitation Unit, Madonna del Soccorso Hospital, San Benedetto del Tronto, Italy.
² Cardiology and Arrhythmology Clinic, University Hospital "Umberto I-Lancisi-Salesi", Ancona, Italy.
³ Politecnica delle Marche University, School of Medicine, Ancona, Italy.

Abstract

Background: 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.

Case summary: 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.

Conclusion: 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.

Keywords: LVOT obstruction; case report; chordal rupture; hypertrophic cardiomyopathy; mitral regurgitation; mitral valve flail; review; systolic anterior motion (SAM).

Publication types

Review