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Heart transplant outcomes in patients with left ventricular non-compaction cardiomyopathy
Sadeer G Al-Kindi1, Chantal El-Amm1, Mahazarin Ginwalla1
1Advanced Heart Failure and Transplant Center, Harrington Heart and Vascular Institute, University Hospitals Case Medical Center and Case Western Reserve University, Cleveland, Ohio.
Insights
Left ventricular non-compaction cardiomyopathy (LVNCC) patients undergoing heart transplantation are typically pediatric and often require mechanical support. Outcomes after transplant are similar to other cardiomyopathy patients, despite increased post-transplant infections.
Area of Science:
- Cardiology
- Transplantation Medicine
- Genetics
Background:
- Left ventricular non-compaction cardiomyopathy (LVNCC) is a rare congenital heart defect.
- It can lead to heart failure (HF) and necessitate orthotopic heart transplantation (OHT).
- Limited data exist on LVNCC patients requiring OHT and their post-transplant outcomes.
Purpose of the Study:
- To investigate the characteristics of LVNCC patients listed for OHT.
- To analyze the outcomes of LVNCC patients following OHT.
- To compare LVNCC patients with those suffering from idiopathic cardiomyopathy (IDCMP).
Main Methods:
- Utilized the United Network for Organ Sharing (UNOS) database.
- Included patients listed for OHT with LVNCC as the primary etiology (2000-2013).
- Compared LVNCC patients with IDCMP cohort.
Main Results:
- Identified 113 LVNCC patients (0.25% of all OHT listings).
- LVNCC patients were younger, male-predominant, and more frequently bridged with ECMO or inotropes.
- Post-transplant survival was similar to IDCMP, but with higher rates of infection (50.0% vs. 21.6%).
Conclusions:
- LVNCC patients undergoing OHT are predominantly pediatric.
- Mechanical support (inotropes, ECMO) is common for bridging these patients to transplant.
- Despite increased infections, LVNCC patients achieve similar long-term survival post-OHT compared to IDCMP patients.
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.
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