ORIGINAL ARTICLE Medical Therapy Leads to Favorable Remodeling in Left Ventricular Non-compaction Cardiomyopathy: Dilated Phenotype John J. Parent 1 • Jeffrey A. Towbin 2 • John L. Jefferies 3 Received: 15 September 2015 / Accepted: 15 December 2015 Ó Springer Science+Business Media New York 2015 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 mor- tality 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 retro- spective chart review. Inclusion criteria included: presence of LVNC, reduced systolic function or ventricular dilation, ther- apy 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 interven- tion 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. Keywords Cardiomyopathy Á Left ventricular non- compaction Á Heart failure Á Reverse remodeling Introduction Left ventricular non-compaction cardiomyopathy (LVNC) is a heterogeneous disease with phenotypic and genotypic variation [7, 18]. Regardless of this variation clinical manifestations that concern clinicians and result in poor patient outcomes include clinical heart failure, arrhythmias, and thromboembolic phenomena. LVNC with dilation or dysfunction, i.e., LVNC-dilated phenotype (LVNC-DCM) is associated with a greater risk for mortality than the other forms of LVNC [2, 5, 17, 20, 22]. Many clinicians treat LVNC-DCM similar to pure dilated cardiomyopathy (DCM); however, no previous report has evaluated the outcomes of this strategy [13]. Our institution has a large referral population for all types of cardiomyopathy, including LVNC, from regional, national, and international sources. This referral base has created a central location for near uniform treatment of a large number of patients with LVNC-DCM. Our treatment strategy employs a combination of beta blockade and angiotensin-converting enzyme (ACEi) or receptor (ARB) & John J. Parent jjparent@iu.edu Jeffrey A. Towbin Jtowbin1@uthsc.edu John L. Jefferies John.Jefferies@cchmc.org 1 Indiana University School of Medicine, Riley Hospital at Indiana University Health, 705 Riley Hospital Drive, RR 127, Indianapolis, IN 46202, USA 2 Division of Pediatric Cardiology, The Heart Institute, Le Bonheur Children’s Hospital, Memphis, TN, USA 3 The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA 123 Pediatr Cardiol DOI 10.1007/s00246-015-1330-2