title: Heart transplant outcomes in patients with left ventricular non-compaction cardiomyopathy.
abstract: BACKGROUND
  Left ventricular non-compaction cardiomyopathy (LVNCC) is a rare disease that starts in utero and may progress to heart failure (HF), sometimes requiring orthotopic heart transplantation (OHT). There are limited data addressing characteristics of LVNCC patients that require OHT and their outcomes. We therefore sought to investigate the characteristics and outcomes of LVNCC patients treated with OHT.
  
  
  METHODS
  We queried the United Network for Organ Sharing (UNOS) database for all patients listed for OHT with LVNCC as the primary heart failure etiology between 2000 and 2013. We examined their characteristics at listing and outcomes after OHT and compared the findings with those of patients with idiopathic cardiomyopathy (IDCMP).
  
  
  RESULTS
  We identified 113 patients (43 adults and 70 pediatrics) with LVNCC of 45,298 patients (0.25% overall, 0.11% of adults and 1.0% of pediatrics) listed for OHT in this time period. Most were male children with mean age at listing of 16.9 years. Compared with the overall IDCMP cohort, patients with LVNCC were younger, had higher use of inotropes and extracorporeal membrane oxygenation (ECMO), and were more often listed as UNOS Status 1A with shorter waiting time. However, when adjusted for age, gender and ethnicity, these differences disappeared. During transplant listing, 8 (7.9%) died, 5 (5.0%) improved and avoided transplant, 3 (3.0%) became too sick for transplant and 78 (77.2%) underwent OHT. There was a non-significant trend toward longer cardiac allograft survival in patients with LVNCC (10.6 vs. 9.4 years; log-rank test, p = 0.068). Patients with LVNCC had similar outcomes to other IDCMP patients, except for more post-transplant infections (50.0% vs. 21.6%, p < 0.05).
  
  
  CONCLUSIONS
  LVNCC patients undergoing heart transplantation are mostly pediatric and predominantly bridged to transplant with inotropes or ECMO. Despite having more post-transplant infections, their survival is similar to that of other IDCMP patients.
authors.affiliation: Advanced Heart Failure and Transplant Center, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center and Case Western Reserve University, Cleveland, Ohio.
authors.forename: Sadeer G
authors.initials: SG
authors.lastname: Al-Kindi
authors.affiliation: Advanced Heart Failure and Transplant Center, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center and Case Western Reserve University, Cleveland, Ohio.
authors.forename: Chantal
authors.initials: C
authors.lastname: El-Amm
authors.affiliation: Advanced Heart Failure and Transplant Center, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center and Case Western Reserve University, Cleveland, Ohio.
authors.forename: Mahazarin
authors.initials: M
authors.lastname: Ginwalla
authors.affiliation: Advanced Heart Failure and Transplant Center, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center and Case Western Reserve University, Cleveland, Ohio.
authors.forename: Brian D
authors.initials: BD
authors.lastname: Hoit
authors.affiliation: Advanced Heart Failure and Transplant Center, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center and Case Western Reserve University, Cleveland, Ohio.
authors.forename: Soon J
authors.initials: SJ
authors.lastname: Park
authors.affiliation: Advanced Heart Failure and Transplant Center, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center and Case Western Reserve University, Cleveland, Ohio. Electronic address: guilherme.oliveira@uhhospitals.org.
authors.forename: Guilherme H
authors.initials: GH
authors.lastname: Oliveira
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002311:Cardiomyopathy, Dilated / Q000453:epidemiology / Q000401:mortality* / Q000601:surgery*; D002316:Cardiotonic Agents; D002648:Child; D002675:Child, Preschool; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000401:mortality* / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D055815:Young Adult
chemical_list: D002316:Cardiotonic Agents
keywords: UNOS; adult; cardiomyopathy; heart transplantation; non-compaction; pediatric