Key result
Orthotopic heart transplantation succeeds in pediatric LVNC after prior transcoronary stem cell therapy.
Why the study?
Isolated left ventricular noncompaction is a rare disease with debated morphological diagnostic criteria and limited data on treatment outcomes including heart transplantation.
Case Report (n=1)
This case report highlights that isolated left ventricular noncompaction can lead to decompensated heart failure requiring and successfully treated by orthotopic heart transplantation in a pediatric patient.
End-stage IVNC may warrant transplant evaluation; leaves open need for prospective registries on prognosis and screening.
The isolated noncompaction of the left ventricle (IVNC) or “spongy myocardium” is a rare disease. IVNC is characterized by an excessively prominent trabecular meshwork of myocardium and deep intertrabecular recesses due to the arrest in the compaction process of the myocardial fibers in the absence of other structural heart diseases. IVNC can occur in sporadic and in familial patterns. The clinical symptoms depend on the extent of noncompacted cardiac segment. IVNC can lead to decompensated heart failure and may be successfully treated with heart transplantation. The morphological criteria for the diagnosis of this entity remain the subject of debate and are usually based on the findings of the imaging studies. Due to rarity of the disease, we are reporting a pathological finding in a recipient heart. This 9-year-old girl had undergone transcoronary stem cell implantation previously, before undergoing successful orthotropic heart transplantation.
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Betigeri et al. (2009) conducted a case report in Isolated left ventricular noncompaction (n=1). Orthotopic heart transplantation was evaluated. Orthotopic heart transplantation was successfully performed in a 9-year-old girl with isolated left ventricular noncompaction who had previously undergone transcoronary stem cell implantation.
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