POSTERIOR MITRAL VALVE LEAFLET HYPOPLASIA: AN UNUSUAL PRESENTATION OF MITRAL REGURGITATION

Abstract

Background: Posterior mitral valve leaflet (PMVL) hypoplasia or agenesis is an extremely rare congenital abnormality. Case: 72 year old male with diastolic heart failure, permanent atrial fibrillation, and mitral regurgitation (MR) was referred to our clinic for undergoing evaluation for percutaneous mitral valve repair (PMVR). Transthoracic echocardiogram demonstrated an eccentric posteriorly directed MR. Following this a transesophageal echocardiogram (TEE) was performed that demonstrated a large anterior mitral leaflet (AML), absent PMVL and a hypoplastic posteromedial papillary muscle. The anterior mitral leaflet had degenerative changes with prolapse of the A2-A3 scallops and severe posteriorly directed MR. Decision-making: PMVL hypoplasia is a rare congenital abnormality of the mitral valve and its prevalence is estimated to be 1:8800. It is usually detected in early life, however, in our patient it was not detected until the seventh decade as he was asymptomatic from his MR. The patient had a large AML that likely had good coaptation with the posterior rim of the mitral annulus resulting in minimal regurgitation. Progressively with age and degenerative changes, the patient developed severe MR. TEE with multiplanar 3D imaging helped identify this rare congenital abnormality. This anatomy is unsuitable for PMVR and therefore surgery was recommended. Conclusion(s): PMLV is a rare congenital abnormality which results in severe MR and requires careful evaluation. [Figure presented]Copyright 2019 American College of Cardiology Foundation. All rights reserved.