Abstract
Background: Left ventricular noncompaction (LVNC) is a distinct cardiomyopathy characterized by the presence of a two-layer myocardium with prominent trabeculation and deep intertrabecular recesses. The diagnosis of LVNC can be challenging because the diagnostic criteria are not uniform. The aim of our study was to evaluate echocardiographic and CMR findings in a group of children with isolated LVNC. Methods: From February 2008 to July 2021, pediatric patients under 18 years of age at the time of diagnosis with echocardiographic evidence of isolated LVNC were prospectively enrolled. The patients underwent echocardiography and contrast-enhanced cardiovascular magnetic resonance (CMR) with late gadolinium enhancement to assess myocardial noncompaction, ventricular size, and function. Results: A total of 34 patients, with a median age of 11.9 years, were recruited. The patients were followed prospectively for a median of 5.1 years. Of the 31 patients who met Jenni’s criteria in echocardiography, CMR was performed on 27 (79%). Further comprehensive analysis was performed in the group of 25 patients who met the echocardiographic and CMR criteria for LVNC. In echocardiography, the median NC/C ratio in systole was 2.60 and in diastole 3.40. In 25 out of 27 children (93%), LVNC was confirmed by CMR, according to Petersen’s criteria, with a median NC/C ratio of 3.27. Conclusions: (1) Echocardiography precisely identifies patients with LVNC. (2) Echocardiography is a good method for monitoring LV systolic function, but CMR is indicated for the precise assessment of LV remodeling and RV size and function, as well as for the detection of myocardial fibrosis.
Highlights
Left ventricular noncompaction (LVNC) is described as a distinct cardiomyopathy characterized by a two-layer myocardium with prominent trabeculation, deep intertrabecular recesses, and a thin compacted myocardial layer
In 25 out of 27 children (93%), LVNC was confirmed by cardiovascular magnetic resonance (CMR), according to Petersen’s criteria, with a median NC/C ratio of 3.27 (IQR, 2.56, 3.76) and on average 5.1 ± 1.5 noncompacted segments
Echocardiography is a good method for monitoring LV systolic function, but CMR is indicated for precise assessment of the left ventricular morphology and enlargement; 4
Summary
Left ventricular noncompaction (LVNC) is described as a distinct cardiomyopathy characterized by a two-layer myocardium with prominent trabeculation, deep intertrabecular recesses, and a thin compacted myocardial layer. LVNC was classified as a primary cardiomyopathy by the American Heart Association in 2006 [1] but remains unclassified by the European Society of Cardiology [2]. It typically involves the left ventricle, involvement of the right ventricle (RV) has been reported [3]. Methods: From February 2008 to July 2021, pediatric patients under 18 years of age at the time of diagnosis with echocardiographic evidence of isolated LVNC were prospectively enrolled. Further comprehensive analysis was performed in the group of 25 patients who met the echocardiographic and CMR criteria for LVNC. Conclusions: (1) Echocardiography precisely identifies patients with LVNC. (2) Echocardiography is a good method for monitoring LV systolic function, but CMR is indicated for the precise assessment of LV remodeling and RV size and function, as well as for the detection of myocardial fibrosis
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