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Canadian Journal of Cardiology

Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk of Death or Heart Transplantation

Published:February 02, 2022DOI:https://doi.org/10.1016/j.cjca.2022.01.030

      Abstract

      Background

      Left ventricular noncompaction (LVNC) is associated with genetic and phenotypic variability that influences outcomes. We aimed to identify risk factors for death or heart transplantation (HTx) in a paediatric LVNC cohort.

      Methods

      We reviewed patients < 18 years of age (2001-2018) with LVNC, either isolated (I-LVNC) or with dilated phenotype (D-LVNC), and at least mildly reduced left ventricular ejection fraction (EF). Patients with dilated cardiomyopathy (DCM) were included as control subjects. Descriptive statistics, multivariate analysis, and time-to-event analysis were used.

      Results

      We included 188 patients, 34 (18%) with I-LVNC, 37 (20%) with D-LVNC, and 117 (62%) with DCM. Overall median age at diagnosis was 1.08 years (interquartile range [IQR] 0.22-10.65) and median follow-up was 1.4 years (IQR 0.2-5.2) years. I-LVNC patients’ median baseline LVEF was 47%, compared with 33% for D-LVNC, and 21% for DCM (P < 0.0001); 62% of I-LVNC patients developed moderate to severe LV dysfunction during follow-up. The incidence of death or transplantation was 43.6% in the overall cohort. Freedom from death or HTx at 10 years after diagnosis was 88.6% (95% CI 76%-100%) for I-LVNC, 47% (95% CI 29%-65%) for D-LVNC, and 42.3% (95% CI 33%-52%) for DCM. On multivariable analysis, baseline LVEF and LV end-diastolic diameter (LVEDD) z-score were associated with death or transplantation. Patients with a baseline LVEDD z-score > 4 and moderate to severe LV dysfunction had a transplantation-free survival of 38%.

      Conclusions

      Baseline LV dilation and systolic dysfunction were independently associated with progression to death or HTx in LVNC patients.

      Résumé

      Contexte

      La non-compaction du ventricule gauche (NCVG) est associée à une variabilité génétique et phénotypique déterminant les issues de la maladie. Notre objectif était de définir les facteurs de risque de décès ou de greffe du cœur dans une cohorte d’enfants atteints de NCVG.

      Méthodologie

      Notre étude portait sur des patients âgés de moins de 18 ans (2001-2018), présentant une NCVG isolée (NCVG-I) ou dilatée (NCVG-D) associée à une fraction d’éjection ventriculaire gauche (FEVG) au moins légèrement réduite. Des patients atteints de myocardiopathie dilatée ont servi de sujets témoins. Des statistiques descriptives, une analyse multivariée et une analyse du temps écoulé avant la survenue d’un événement ont été utilisées.

      Résultats

      Nous avons inclus 188 patients, soit 34 (18 %) atteints de NCVG-I, 37 (20 %) atteints de NCVG-D, et 117 (62 %) atteints de myocardiopathie dilatée. L’âge médian global au moment du diagnostic était de 1,08 an (intervalle interquartile [IIQ] : 0,22-10,65); le suivi médian était de 1,4 an (IIQ : 0,2-5,2). La FEVG médiane initiale des patients atteints de NCVG-I était de 47 % comparativement à 33 % et à 21 % chez ceux atteints, respectivement, de NCVG-D et de myocardiopathie dilatée (p < 0,0001); 62 % des patients atteints de NCVG-I ont ensuite présenté une dysfonction modérée à sévère du ventricule gauche durant le suivi. L’incidence des décès ou des greffes du cœur était de 43,6 % dans l’ensemble de la cohorte. L’absence de décès ou de greffe du cœur 10 ans après le diagnostic a été observée chez 88,6 % (IC à 95 % : 76 %-100 %) des patients atteints de NCVG-I, 47 % (IC à 95 % : 29 %-65 %) de ceux atteints de NCVG-D et 42,3 % (IC à 95 % : 33 %-52 %) de ceux atteints de myocardiopathie dilatée. Dans l’analyse multivariée, la FEVG au début de l’étude et le score z du diamètre télédiastolique du ventricule gauche ont été associés au décès ou à une greffe du cœur. Les patients qui présentaient au début de l’étude un score z du diamètre télédiastolique du ventricule gauche > 4 et une dysfonction modérée à sévère du ventricule gauche présentaient un taux de survie sans greffe de 38 %.

      Conclusions

      La dysfonction systolique et la dilatation du ventricule gauche au début de l’étude étaient indépendamment associés à l’évolution vers le décès ou une greffe du cœur chez les patients atteints de NCVG.
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      Linked Article

      • Left Ventricular Noncompaction in Children: Is It a Tale of Two Diseases?
        Canadian Journal of CardiologyVol. 38Issue 6
        • Preview
          Left ventricular noncompaction cardiomyopathy (LVNC) is not as well understood as other common cardiomyopathies in children, such as hypertrophic cardiomyopathy, idiopathic dilated cardiomyopathy, or restrictive cardiomyopathy. Typical cardiac morphology of the left ventricle is a smooth-walled surface with the right ventricle having more course trabeculations. LVNC represents a variant in which the endomyocardial surface is heavily trabeculated. There are defined imaging criteria to distinguish LVNC from variations in a normal heart in which the trabeculations are more prominent.
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