Left and Right Ventricular Morphology, Function and Myocardial Deformation in Children with Left Ventricular Non-Compaction Cardiomyopathy: A Case-Control Cardiovascular Magnetic Resonance Study

Abstract

Background: Left ventricular non-compaction (LVNC) is a rare cardiomyopathy typically involving the left ventricle (LV); however, the right ventricle (RV) can also be affected. This case-control study aimed to assess the morphology and function of LV and RV in children with LVNC. Methods: Sixteen children (13 ± 3 years, six girls) with LVNC were compared with 16 sex- and age-matched controls. LV and RV morphology and function were evaluated in cardiovascular magnetic resonance (CMR) studies. Additionally, LV and RV global radial (GRS), circumferential (GCS), and longitudinal strain (GLS) were assessed using tissue-tracking analysis. Results: Patients with LVNC did not differ from the healthy controls in terms of age, height, weight, and body surface area (BSA). In total, 4/16 subjects with LVNC had mid-wall late gadolinium enhancement (LGE). Compared to the control group, patients with LVNC had higher end-diastolic volume (EDV) indexed for body surface area (BSA), lower ejection fraction (EF), and lower LV strain parameters (all p < 0.05). Children with LVNC also presented with thicker RV apical trabeculation, whereas there were no differences in RV EF and EDV/BSA between the groups. Nevertheless, children with LVNC had impaired RV GRS and GCS (both p < 0.05). Conclusions: LVNC in pediatric patients is associated with LV enlargement and impaired LV systolic function. Additionally, children with LVNC have increased RV trabeculations and subclinical impairment of RV myocardial deformation.

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.…”

“…In the literature reports, the relationship between LVNC and sinus bradycardia has been described in approximately 55% of cases, although the basis of conduction disturbances remains unclear [ 8 , 26 ]. The results of other studies demonstrated that the cause of sinus bradycardia in patients with LVNC may be the presence of a pathogenic variant in the HCN4 gene [ 11 , 27 , 28 ].…”

“…The spectrum of arrhythmias is very wide, with the most common one being ventricular or supraventricular arrhythmias and the Wolff–Parkinson–White (WPW) syndrome. Less frequently, bradyarrhythmias, including sinus bradycardia, sick sinus syndrome and varying degrees of atrioventricular (AV) block, including complete heart block, were observed in individuals with LVNC [ 5 , 6 , 7 , 8 ].…”

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

“…CMR imaging was performed using a 1.5-T scanner (Magnetom AvantoFit, Siemens, Erlangen, Germany), with a dedicated cardiac phased-array coil and electrocardiographic gating, as previously described [ 23 ]. Steady-state free precession (SSFP) cine images of the heart were acquired in the short-axis and four-, three-, and two-chamber planes with a minimum of 25 phases per cardiac cycle.…”

Left Ventricular Noncompaction in Children: The Role of Genetics, Morphology, and Function for Outcome

“…LV trabeculation was found to be correlated with reduced myocardial deformation indexes, even if left ventricular ejection fraction (LV-EF) was normal [ 67 ]. Children with LVNC and normal LV function myocardial deformation patterns measured by tissue tracking analysis are significantly impaired compared to controls [ 68 , 69 ]. Rigid body rotation (RBR) is characterized by impaired LV twist with apex and base rotating in the same direction and can be used as a measure of cardiac mechanics in echocardiography and CMR.…”