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Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children

1
Department of Cardiology, The Children’s Memorial Health Institute, 04-730 Warsaw, Poland
2
Department of Medical Genetics, The Children’s Memorial Health Institute, 04-730 Warsaw, Poland
3
Departmentof Cardiomyopathies, Cardiovascular Magnetic Resonance Unit, National Institute of Cardiology, 04-682 Warsaw, Poland
*
Author to whom correspondence should be addressed.
Academic Editor: George E. Louridas
Cardiogenetics 2021, 11(4), 191-203; https://doi.org/10.3390/cardiogenetics11040020
Received: 8 June 2021 / Revised: 22 August 2021 / Accepted: 18 October 2021 / Published: 22 October 2021
Background: Left ventricular noncompaction (LVNC) is a genetically determined cardiomyopathy that occurs following a disruption of endomyocardial morphogenesis. The purpose of this study was to identify the clinical characteristics and genetic profile of children with LVNC. Methods: From February 2008 to July 2020, a total of 32 children (median 11.5 years) with LVNC were prospectively enrolled and followed up for a median of 4.02 years. Diagnosis was made based on characteristic features of LVNC in echocardiography and cardiovascular magnetic resonance (CMR). Patients’ clinical symptoms, family history, ECG, Holter ECG, and genetic tests were also evaluated. Results: The most common presenting symptom was heart failure (31% of children). ECG abnormalities were noted in 56% of patients. The most prominent features were ventricular arrhythmias, sinus bradycardia, and paroxysmal third-degree atrioventricular block. Most of the patients (94%) met the criteria for LVNC and CMR confirmed this diagnosis in 82% of cases. The molecular etiology was found in 53% of children. Conclusion: Although heart failure and arrhythmias were very frequent in our study group, thromboembolic events and genetic syndromes were rare. For the accurate and reliable assessment of children with LVNC, it is necessary to get to know their family history and detailed clinical profile. View Full-Text
Keywords: left ventricular noncompaction; cardiomyopathy; heart failure; arrhythmia; conduction disturbances; molecular etiology; children left ventricular noncompaction; cardiomyopathy; heart failure; arrhythmia; conduction disturbances; molecular etiology; children
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MDPI and ACS Style

Paszkowska, A.; Mirecka-Rola, A.; Piekutowska-Abramczuk, D.; Ciara, E.; Mazurkiewicz, Ł.; Bieganowska, K.; Ziółkowska, L. Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children. Cardiogenetics 2021, 11, 191-203. https://doi.org/10.3390/cardiogenetics11040020

AMA Style

Paszkowska A, Mirecka-Rola A, Piekutowska-Abramczuk D, Ciara E, Mazurkiewicz Ł, Bieganowska K, Ziółkowska L. Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children. Cardiogenetics. 2021; 11(4):191-203. https://doi.org/10.3390/cardiogenetics11040020

Chicago/Turabian Style

Paszkowska, Agata, Alicja Mirecka-Rola, Dorota Piekutowska-Abramczuk, Elżbieta Ciara, Łukasz Mazurkiewicz, Katarzyna Bieganowska, and Lidia Ziółkowska. 2021. "Spectrum of Clinical Features and Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children" Cardiogenetics 11, no. 4: 191-203. https://doi.org/10.3390/cardiogenetics11040020

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