title: Medical Therapy Leads to Favorable Remodeling in Left Ventricular Non-compaction Cardiomyopathy: Dilated Phenotype.
abstract: Left ventricular non-compaction cardiomyopathy (LVNC) is a distinct and heterogeneous entity that can lead to progressive cardiac dysfunction and heart failure. LVNC with dilation and/or dysfunction is associated with a greater mortality risk. We hypothesized that initiation of heart failure medications in patients with LVNC and ventricular dysfunction or dilation would improve systolic function and result in favorable left ventricular remodeling. The study was a retrospective chart review. Inclusion criteria included: presence of LVNC, reduced systolic function or ventricular dilation, therapy with at least one medication (beta blocker, angiotensin-converting-enzyme inhibitor, angiotensin II receptor blocker), imaging pre- and post-initiation of therapy. Fifty-one patients met inclusion criteria. Mean age at initiation of medication was 11.5 ± 11.8 years. Follow-up was 2.4 ± 2.3 years. Three patients (6 %) were solely on a beta blocker, 15 (29 %) on ACE/ARB monotherapy, and 33 (65 %) on dual therapy. At follow-up 45/51 patients (88 %) had improvement in ejection fraction/shortening and 6/51 (12 %) had no change. Ejection fraction, shortening fraction, and left ventricular end-diastolic dimension in the cohort before and after therapeutic intervention demonstrated a 16 ± 12 % improvement in ejection fraction (p < 0.0001), an 8 ± 9 % improvement in shortening fraction (p < 0.0001), and a 0.83 ± 1.93 (p < 0.05) decrease in left ventricular end-diastolic z-score. Early diagnosis and medical treatment of LVNC with reduced systolic function and/or dilation leads to favorable remodeling evident by an improvement in ventricular systolic function and reduction of ventricular end-diastolic dimensions.
authors.affiliation: Indiana University School of Medicine, Riley Hospital at Indiana University Health, 705 Riley Hospital Drive, RR 127, Indianapolis, IN, 46202, USA. jjparent@iu.edu.
authors.forename: John J
authors.initials: JJ
authors.lastname: Parent
authors.affiliation: Division of Pediatric Cardiology, The Heart Institute, Le Bonheur Children's Hospital, Memphis, TN, USA.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: John L
authors.initials: JL
authors.lastname: Jefferies
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis* / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010641:Phenotype; D012189:Retrospective Studies; D013599:Systole; D018487:Ventricular Dysfunction, Left / Q000503:physiopathology*; D016277:Ventricular Function, Left / Q000187:drug effects*; D020257:Ventricular Remodeling; D055815:Young Adult
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors
keywords: Cardiomyopathy; Heart failure; Left ventricular non-compaction; Reverse remodeling