TY - JOUR
T1 - Heart transplant outcomes in patients with left ventricular non-compaction cardiomyopathy
AU - Al-Kindi, Sadeer G.
AU - El-Amm, Chantal
AU - Ginwalla, Mahazarin
AU - Hoit, Brian D.
AU - Park, Soon J.
AU - Oliveira, Guilherme H.
N1 - Publisher Copyright:
© 2015 International Society for Heart and Lung Transplantation.
PY - 2015/6/1
Y1 - 2015/6/1
N2 - 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.
AB - 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.
KW - UNOS
KW - adult
KW - cardiomyopathy
KW - heart transplantation
KW - non-compaction
KW - pediatric
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U2 - 10.1016/j.healun.2014.11.005
DO - 10.1016/j.healun.2014.11.005
M3 - Article
C2 - 25572453
AN - SCOPUS:84930084133
SN - 1053-2498
VL - 34
SP - 761
EP - 765
JO - Journal of Heart and Lung Transplantation
JF - Journal of Heart and Lung Transplantation
IS - 6
ER -