Rare Case of Unileaflet Mitral Valve

Abstract

Unileaflet mitral valve is the rarest of the congenital mitral valve anomalies and is usually life threatening in infancy due to severe mitral regurgitation (MR). In most asymptomatic individuals, it is mostly due to hypoplastic posterior mitral leaflet. We present a 22-year-old male with palpitations, who was found to have an echocardiogram revealing an elongated anterior mitral valve leaflet with severely hypoplastic posterior mitral valve leaflet appearing as a unileaflet mitral valve without MR. Our case is one of the 11 reported cases in the literature so far. We hereby review those cases and conclude that these patients are likely to be at risk of developing worsening MR later in their lives.

Hypoplastic posterior mitral valve leaflet: A case report and review of the literature

“…7,10,11,14,15 All of the surgical procedures were performed successfully with no postoperative reported complications and patients often reported the absence of the initial symptoms on regular follow-up. In 6 patients including the current case, a decision was made to medically treat the patient with a follow-up visit every 6 months, 8,9,12,[16][17][18] while there was no information regarding the faith of 3 patients from the same family reported by Kanagala et al 4 Although hypoplasia of the PML is an abnormality that is mostly present in infancy and childhood with symptomatic mitral regurgitation, 19,20 Left atrial enlargement and subsequent development of atrial fibrillation may hasten the process of annular dilatation. They reported this congenital anomaly as a consequence of developmental arrest at the stage of chordae and leaflets conversion from muscular to thin connective structures.…”

“…All of the surgical procedures were performed successfully with no postoperative reported complications and patients often reported the absence of the initial symptoms on regular follow‐up. In 6 patients including the current case, a decision was made to medically treat the patient with a follow‐up visit every 6 months, while there was no information regarding the faith of 3 patients from the same family reported by Kanagala et al …”

Surgical Repair of a Unileaflet Mitral Valve: A Rare Congenital Abnormality and a Novel Surgical Approach

“… 3 1 45/F Dyspnea, severe MR Elongated AMVL, hypoplastic posterior leaflet Not attempted, patient refused Annual follow-up Shah et al. 21 1 22/M Palpitations, incidental finding of ULMV Elongated AMVL Not attempted, medical management Annual follow-up Candan et al. 22 1 47/M Asymptomatic ULMV Not attempted, medical management ND Bar et al.…”

MitraClip for High-Risk Patients with Significant Mitral Insufficiency: Shall We Unreservedly Recommend It?

“…4,5 In contrast, congenital malformations exist but are rather rare. [6][7][8] It is currently understood to perform surgical reconstruction as early as possible in case of significant mitral regurgitation to avoid nonreversible consequences such as persistent left atrial fibrillation because of atrial dilatation exceeding 50 mm, fixed reduction of left ventricular function, and secondary pulmonary hypertension leading to right heart disturbances. [9][10][11][12][13] The prudent surgeon always intends to reconstruct the valve with appropriate measures typically comprising leaflet reconstruction and annuloplasty with rings.…”