title: Left ventricular noncompaction: A rare indication for pediatric heart transplantation.
abstract: 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.
authors.affiliation: Serviço de Cardiologia Pediátrica, Hospital Pediátrico Integrado, Centro Hospitalar de São João, E.P.E., Porto, Portugal. Electronic address: marianamagalhaes21@hotmail.com.
authors.forename: Mariana
authors.initials: M
authors.lastname: Magalhães
authors.affiliation: Serviço de Cardiologia Pediátrica, Hospital Pediátrico Integrado, Centro Hospitalar de São João, E.P.E., Porto, Portugal.
authors.forename: Patrícia
authors.initials: P
authors.lastname: Costa
authors.affiliation: Serviço de Cardiologia Pediátrica, Hospital Pediátrico Integrado, Centro Hospitalar de São João, E.P.E., Porto, Portugal.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Vaz
authors.affiliation: Serviço de Cirurgia Cardiotorácica, Centro Hospitalar de São João E.P.E., Porto, Portugal.
authors.forename: José
authors.initials: J
authors.lastname: Pinheiro Torres
authors.affiliation: Serviço de Cardiologia Pediátrica, Hospital Pediátrico Integrado, Centro Hospitalar de São João, E.P.E., Porto, Portugal.
authors.forename: José Carlos
authors.initials: JC
authors.lastname: Areias
mesh_terms: D001145:Arrhythmias, Cardiac; D009202:Cardiomyopathies; D002648:Child; D016027:Heart Transplantation; D006352:Heart Ventricles; D006801:Humans; D008297:Male; D009206:Myocardium
keywords: Insuficiência cardíaca refractária; Left ventricular noncompaction; Pediatric heart transplantation; Refractory heart failure; Transplante cardíaco pediátrico; Ventrículo esquerdo não compactado