Abstract

Abstract Background: Left ventricular noncompaction (LVNC) is a rare type of cardiomyopathy. The core clinical feature is heart failure that responds poorly to treatments. Case presentation: A 58-year-old woman received various treatments (including metoprolol, benazepril, torasemide, spirolactone, and digoxin) for 4 years for LVNC, but responded poorly. Upon presentation, transthoracic echocardiogram (ECHO) showed 26% left ventricular ejection fraction (LVEF) and class IV diastolic dysfunction. Upon cardiac magnetic resonance imaging (CMRI), the ratio of noncompacted versus compacted myocardium was 3.9. She received guideline-recommended treatments that included sacubitril-valsartan (100 mg/day) in addition to β-blocker, torasemide, spirolactone, digoxin, and isosorbide. Symptoms and signs improved rapidly, and she was discharged 1 week later. Sacubitril-valsartan dosage was adjusted to 200 mg/day 4 weeks later. She remained in relatively good health thereafter. At the last follow-up 16 months later, LVEF was 51% on ECHO. CMRI showed the significantly reduced ratio of 2.8 in noncompacted versus compacted myocardium. Conclusions: Sacubitril-valsartan therapy may result in reverse remodeling and improve long-term outcomes in LVNC patients.

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