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Updated: Jun 1, 2026
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Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
Published on: October 28, 2020
Left ventricular noncompaction: analysis of a pediatric population
Marta António1, Carmen Costa, Margarida Venâncio
1Serviço de Cardiologia Pediátrica do Hospital Pediátrico de Coimbra, Coimbra, Portugal. marta.antonio@gmail.com
Summary
Left ventricular noncompaction (LVNC) in children often presents as congestive cardiac failure and can coexist with other cardiomyopathies. Further large-scale studies are needed to clarify diagnostic criteria for this rare condition.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Genetics
Background:
- Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by excessive trabeculations and deep recesses.
- Diagnosis is typically aided by echocardiography and cardiac MRI, with MRI offering high sensitivity and specificity.
Purpose of the Study:
- To characterize clinical and imaging features of pediatric LVNC.
- To assess the evolution of LVNC in children.
Main Methods:
- Retrospective chart review of five pediatric patients with LVNC.
- Follow-up at Coimbra Pediatric Hospital from January 1999 to December 2007.
- Genetic analysis for MYBPC3 gene mutations.
Main Results:
- Median age at presentation was five months; most patients were male.
- Congestive cardiac failure was the most common presentation; ventricular apical involvement occurred in all cases.
- Four patients showed improved ventricular dysfunction with therapy; one case showed a phenotype change from dilated to hypertrophic, with a MYBPC3 gene mutation identified.
Conclusions:
- Congestive cardiac failure is the primary clinical presentation of pediatric LVNC, which may co-occur with dilated or hypertrophic cardiomyopathies.
- The study highlights the need for larger prospective studies to establish definitive diagnostic criteria for pediatric LVNC.
