Abstract 11079: Medical Therapy Leads to Positive Remodeling in Left Ventricular Non-Compaction Cardiomyopathy
Introduction: Left ventricular non-compaction cardiomyopathy (LVNC) is a distinct form of cardiomyopathy that can lead to progressive cardiac dysfunction and clinical heart failure. LVNC with left ventricular dilation or dysfunction is associated with a greater risk for mortality.
Hypothesis: We hypothesized that initiation of heart failure medications in patients with LVNC and ventricular dysfunction, with or without dilation, would improve systolic function and reduce ventricular dilation.
Methods: The study was a retrospective chart review. Inclusion criteria were as follows: presence of LVNC, reduced systolic function [Left ventricular ejection fraction (EF) < 55% or shortening fraction (SF) < 30%], therapy with at least one medication (beta blocker, ACE inhibitor, ARB), imaging performed both pre- and post-initiation of therapy.
Results: Fifty one patients met inclusion criteria. Forty eight had complete echocardiographic data and 8 had complete cardiac MRI data. Mean age at initiation of medication was 11.5 ± 11.8 years. Follow-up, defined as time from initiation of medication to most recent echocardiogram, was 2.4 ± 2.3 years. Three patients (6%) were solely on a beta blocker, 15 (29%) were on ACE/ARB monotherapy, and 33 (65%) were on dual therapy with a beta blocker and an ACE/ARB. After initiation of medical therapy 38/44 (86%) had improvement in EF by ≤ 5%, 27/40 (68%) had improvement in their SF by ≤ 5%, 6/44 (14%) had no change in EF, and 11/40 (28%) had no change in SF. No patient (0/44, 0%) had a decline in EF by ≤5%, but 2/40 (5%) had a drop in SF by ≤ 5%.
A two-sided paired t-test was performed comparing EF, SF, and left ventricular end-diastolic dimension (LVEDD) in the cohort before and after therapeutic intervention demonstrating a 16 ± 12% improvement in EF (p < 0.0001), an 8 ± 9% improvement in SF (p < 0.0001), and a 0.83 ±1.93 (p<0.05) decrease in LVEDD z-score.
Conclusions: Early diagnosis and medical treatment of LVNC with reduced systolic function leads to favorable left ventricular remodeling evident by an improvement in left ventricular systolic function and reduction of LVEDD.
Author Disclosures: J.J. Parent: None. J.A. Towbin: None. J.L. Jefferies: None.
- © 2014 by American Heart Association, Inc.