Key result
Heart transplantation successfully treats a child with severe refractory heart failure from isolated LVNC.
Why the study?
Isolated left ventricular noncompaction is a rare congenital cardiomyopathy with no specific therapy, and cases refractory to medical treatment may require heart transplantation.
Case Report (n=1)
Heart transplantation can be a successful treatment for severe, refractory heart failure secondary to isolated left ventricular noncompaction in pediatric patients.
Answers are grounded in this paper and cite their sources.
May support transplantation in select pediatric isolated LVNC with refractory failure; leaves open need for larger outcome studies.
Isolated left ventricular noncompaction is a rare congenital cardiomyopathy, characterized morphologically by a dilated left ventricle, prominent trabeculations and deep intertrabecular recesses in the ventricular myocardium, with no other structural heart disease. It is thought to be secondary to an arrest of normal myocardial compaction during fetal life. Clinically, the disease presents with heart failure, embolic events, arrhythmias or sudden death. Current diagnostic criteria are based on clinical and imaging data and two-dimensional and color Doppler echocardiography is the first-line exam. There is no specific therapy and treatment is aimed at associated comorbidities. Cases refractory to medical therapy may require heart transplantation. The authors describe a case of severe and refractory heart failure, which was the initial presentation of isolated left ventricular noncompaction in a previously healthy male child, who underwent successful heart transplantation.
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Magalhães et al. (2016) conducted a case report in Isolated left ventricular noncompaction (n=1). Heart transplantation was evaluated. Heart transplantation was successfully performed in a male child presenting with severe and refractory heart failure as the initial manifestation of isolated left ventricular noncompaction.
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