Abstract

We report a unique presentation of isolated congenital cleft mitral valve complicating cardiogenic shock from acute myocardial infarction. Isolated cleft mitral valve is an uncommon diagnosis that can have significant clinical implications, especially if not recognized in patients presenting to the catheterization lab with acute myocardial infarction and cardiogenic shock. A review of this rare diagnosis including the options and timing of therapeutic interventions, which can include MitraClip, is important for publication.The case is of a patient who presented with an anterior acute ST elevation myocardial infarction. Despite early coronary revascularization and conventional support, refractory cardiogenic shock ensued requiring mechanical circulatory support escalation to Veno-arterial extracorporeal membrane oxygenation. Subsequently, left ventriculography revealed a massively dilated left atrium and severe mitral regurgitation raising concerns for a mechanical mitral valve complication. The patient was taken to the operating room for possible mitral valve surgery, but a preoperative transesophageal echocardiogram revealed an isolated posterior cleft mitral valve. Since the patient had stabilized on mechanical circulatory support, emergent surgery was deferred. The patient successfully recovered during index hospitalization with mechanical circulatory support and discharged on guideline directed medical therapy.In conclusion, isolated cleft mitral valve is a rare diagnosis that can often be underrecognized without comprehensive 3-dimensional transesophageal echocardiography evaluation. If diagnosed early with significant regurgitation, surgical treatment results in good outcomes and preservation of LV systolic function. Percutaneous correction of a CMVL with MitraClip has been described and may offer an alternative approach for high risk surgical patients.

Highlights

  • Cleft mitral valve leaflet (CMVL) is the most common cause of congenital mitral regurgitation (MR)

  • CMVL results from congenital mitral hypoplasia and is most often associated with other congenital heart defects, usually endocardial cushion defects [1, 2]

  • This case describes a patient presenting with acute myocardial infarction (AMI) who, despite early emergent revascularization, developed refractory cardiogenic shock (CS) necessitating mechanical circulatory support (MCS)

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Summary

Background

Cleft mitral valve leaflet (CMVL) is the most common cause of congenital mitral regurgitation (MR). A pre-operative transesophageal echocardiography (TEE) revealed markedly reduced global LV systolic function, an isolated cleft posterior mitral valve leaflet (CMVL) and severe MR (Fig. 3, Supplementary Video 3, Supplementary Video 4). He was discharged fully ambulatory on hospital day 10 with guideline-directed medical therapy He had multiple “heart failure” hospitalizations over the 6 months and presented with low output heart failure, severe MR and severe global LV systolic dysfunction (LAD still patent by coronary angiography). He eventually received a HeartMate 3 Left Ventricular Assist Device (LVAD) which dramatically reduced the MR and resulted in clinical stability

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