State-of-the art review: Noncompaction cardiomyopathy in pediatric patients.
Heart Fail Rev
; 27(1): 15-28, 2022 01.
Article
em En
| MEDLINE
| ID: mdl-33715140
ABSTRACT
Noncompaction cardiomyopathy (NCCM) is a disease characterized by hypertrabeculation, commonly hypothesized due to an arrest in compaction during fetal development. In 2006, NCCM was classified as a distinct form of cardiomyopathy (CMP) by the American Heart Association. NCCM in childhood is more frequently familial than when diagnosed in adulthood and is associated with other congenital heart diseases (CHDs), other genetic CMPs, and neuromuscular diseases (NMDs). It is yet a rare cardiac diseased with an estimated incidence of 0.12 per 100.000 in children up to 10 years of age. Diagnosing NCCM can be challenging due to non-uniform diagnostic criteria, unawareness, presumed other CMPs, and presence of CHD. Therefore, the incidence of NCCM in children might be an underestimation. Nonetheless, NCCM is the third most common cardiomyopathy in childhood and is associated with heart failure, arrhythmias, and/or thromboembolic events. This state-of-the-art review provides an overview on pediatric NCCM. In addition, we discuss the natural history, epidemiology, genetics, clinical presentation, outcome, and therapeutic options of NCCM in pediatric patients, including fetuses, neonates, infants, and children. Furthermore, we provide a simple classification of different forms of the disease. Finally, the differences between the pediatric population and the adult population are described.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Tromboembolia
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Cardiopatias Congênitas
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Cardiomiopatias
Tipo de estudo:
Diagnostic_studies
Limite:
Adult
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Child
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Humans
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Infant
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Newborn
Idioma:
En
Revista:
Heart Fail Rev
Assunto da revista:
CARDIOLOGIA
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Holanda