This case highlights the rare coexistence of left ventricular noncompaction cardiomyopathy and subaortic membranous stenosis in a young patient presenting with severe heart failure.
Prompts evaluation for subaortic stenosis in LV noncompaction with reduced LVEF; leaves open management guidelines for this rare presentation.
Left ventricular noncompaction cardiomyopathy (LVNC) is a relatively rare primary genetic cardiomyopathy and increased in the frequency of detection. LVNC is characterized by prominent wall trabeculations and intertrabecular recesses that communicate with the ventricular cavity. It could be in isolated form or coexists with other congenital heart diseases including valvular heart disease. The prevalence of adult LVNC ranges from 0.01% to 0.27%. This present case is a 19-year-old male patient who presents as a cardiology outpatient with progressive dyspnea for one month. Physical examination revealed tachycardia and third heart sound on auscultation. A complete left bundle branch block was detected on electrocardiography, and chest X-ray showed an enlarged cardiac shadow (cardiomegaly). Echocardiography revealed left ventricular systolic dysfunction (LVEF: 25%), a noncompact layer, hypertrabeculation, and subaortic membranous stenosis with P-mean of 32 mmHg. The patient had started heart failure management and scheduled cardiac resynchronization therapy (CRT) for life-saving and recommended to search for either left ventricular assist device (LVAD) or heart transplantation where he can. In conclusion, the symptoms of heart failure and cardiac arrhythmias should be considered significant in apparently healthy young patients. Besides, intensive medical treatment has indicated the implantation of cardiac resynchronization therapy (CRT) “life-saving” and advanced cases of heart transplantation.
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Ahmed et al. (2021) studied this question.
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