Imaging Features of Pediatric Left Ventricular Noncompaction Cardiomyopathy in Echocardiography and Cardiovascular Magnetic Resonance

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.

Genetic Profile of Left Ventricular Noncompaction Cardiomyopathy in Children—A Single Reference Center Experience

“…LV enlargement was diagnosed when the LVEDd z-score was greater than 2; LV systolic function impairment was defined as an LVEF of 55% or less. The methodology of the echocardiographic examination was described in detail in an earlier publication [ 19 ].…”

“…Late gadolinium enhancement (LGE) images were obtained 10–15 min after the intravenous administration of 0.1 mmol/kg of gadobutrol (Gadovist, Bayer, Berlin, Germany) and were visually evaluated for areas of myocardial hyperintensity visible in two distinct planes. The extent of LGE was quantitatively assessed and determined as a percentage of the LV total mass, as described in our earlier study [ 19 ].…”

Clinical Presentation of Left Ventricular Noncompaction Cardiomyopathy and Bradycardia in Three Families Carrying HCN4 Pathogenic Variants

“…A total of six children from four families were enrolled and followed prospectively for the median of 12 (6-13) years. The median age of patients was 11 (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16) years. Clinical characteristics of the study patients are presented in Table 1.…”

“…Echocardiography, including two-dimensional, Doppler and M-mode imaging, was performed at rest, as previously described [ 15 ], using Philips Epiq7 (Philips Medical Systems, Bothell, WA, USA). Echocardiographic images included parasternal long- and short-axis and apical two-, three- and four-chamber views.…”

Can left ventricular entropy by cardiac magnetic resonance late gadolinium enhancement be a prognostic predictor in patients with left ventricular non-compaction?

“…Therefore, CMR plays a crucial role in the diagnosis, risk stratification, and treatment of patients with LVNC. 5 , 6 Positive late gadolinium enhancement (LGE) and LV systolic dysfunction [left ventricular ejection fraction (LVEF) <50%] have been used to determine the prognosis of patients with LVNC. 3 However, some investigations have discovered that even individuals with negative LGE and normal LVEF can develop MACE.…”