Hypoplasia of the posterior mitral leaflet: A rare cause of mitral regurgitation in adulthood

Abstract

Hypoplasia of the posterior mitral valve leaflet (PMVL) is a rare congenital heart disease, usually presenting in infancy and childhood with severe mitral regurgitation, either in isolation or associated with other cardiac lesions. We report a case of a 69-year-old woman with recent-onset exertional dyspnea and severe mitral regurgitation. Two- and three-dimensional transesophageal echocardiography showed severe hypoplasia of the PMVL, confirmed by surgical inspection.

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

“…3,5,6,13 In 5 cases, where mitral valve repair was not feasible, mitral valve was surgically replaced with a biological prosthetic valve. 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.…”

“…While varying degrees of mitral insufficiency were reported in all patients, only 11 (53%) patients had a detectable systolic murmur which was associated with the presence of severe regurgitation of mitral valve. Frank pulmonary edema was reported in 2 (10%) patients, and documented pulmonary hypertension was diagnosed in one patient; however, there was no reliable information regarding the presence of milder forms of pulmonary congestion from all available reports.…”

“…As shown in Table , the majority of clinical signs and symptoms, as well as electrocardiographic finding in these patients, were directly related to the severity of the mitral valve insufficiency. Left atrial enlargement was present in most (64%) of symptomatic patients and those who had more than moderate degrees of mitral regurgitation . In 4 patients (36%), atrial fibrillation was detected as a part of the reentry phenomenon secondary to the atrial dilatation.…”

“…Surgical repair of the mitral valve with or without chordal transfer was performed if it was technically feasible . In 5 cases, where mitral valve repair was not feasible, mitral valve was surgically replaced with a biological prosthetic valve . 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.…”

“…Left atrial enlargement was present in most (64%) of symptomatic patients and those who had more than moderate degrees of mitral regurgitation. [5][6][7][8][9][10][11] In 4 patients (36%), atrial fibrillation was detected as a part of the reentry phenomenon secondary to the atrial dilatation. All patients underwent either transthoracic or transesophageal echocardiography.…”