Isolated left ventricular non‐compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry

Abstract

AimsThe clinical features, prognosis, and even definition of left ventricular non-compaction (LVNC) are still the subject of much debate. The aim of this registry was to describe the clinical, echocardiographic, and prognostic features of LVNC in France. The main endpoint was to assess clinical and echocardiographic predictors of adverse outcome, defined as death or heart transplantation. Methods and resultsBetween 2004 and 2006, 154 suspected cases of LNVC were identified from a nationwide survey in France. The diagnosis of LVNC was confirmed in 105 cases by echocardiographic evaluation in a core laboratory. Clinical and echocardiographic data for the 105 cases of LVNC are presented. Left ventricular non-compaction was first detected from heart failure symptoms in 45 patients, rhythm disorders in 12, and familial screening in 8. Left ventricular ejection fraction (LVEF) was ,30% in 46% of patients, but ≥50% in 16%. The latter had less symptoms of severe heart failure (11 vs. 54%, P ¼ 0.001), but similar extension of the NC zone. During 2.33 + 1.47 years of follow-up, several complications occurred, including severe heart failure in 33 patients, transplantation in 9, ventricular arrhythmia in 7, embolic events in 9, and death in 12. Factors associated with death or heart transplantation were NYHA 3 or 4 (HR ¼ 6.69; P ¼ 0.0007), high LV filling pressures (HR ¼ 7.59; P ¼ 0.001), LVEF (HR ¼ 0.93; P ¼ 0.006), and hospitalization for heart failure (HR ¼ 13.55; P , 0.0001). ConclusionIn this large reported series of LVNC, we observed that: (i) Left ventricular non-compaction was detected by familial screening in asymptomatic patients in 8% of cases. (ii) Left ventricular non-compaction was frequently over-diagnosed by echocardiography. (iii) Patients identified as LVNC presented with a high risk of severe complications, transplantation or death and needed close follow-up.--

Clinical and prognostic relevancy of left ventricular trabeculation assessed by cardiac magnetic resonance in patients with dilated cardiomyopathy

“…However, it is still unclear whether increased trabeculation is a result of distinct cardiomyopathy or rather a sign of the remodelling of the failing heart [16][17][18][19]. Previous studies reported that a relatively high percentage of patients with idiopathic heart failure meet criteria for noncompaction [11,20]. In accordance with these studies, we found that more than half of our population had an abnormal amount of trabeculation.…”

Atualização da diretriz brasileira de insuficiência cardíaca crônica - 2012

“…Pode apresentar-se com manifestações de IC por disfunção sistólica e/ou diastólica, ou com arritmias cardíacas ou ainda com fenômenos tromboembólicos. O ecocardiograma e a ressonância magnética do coração são os exames complementares de escolha no diagnóstico 285,286 . Não existem estudos específicos no tratamento da IC nesse grupo de pacientes.…”

Left ventricular non‐compaction: Prevalence in congenital heart disease

“…[5][6][7][8][9][10][11][12][13][14][15] Typical complications include congestive heart failure, ventricular arrhythmias and thromboembolic events. [12][13][14]16,17 Recently, the association of LVNC with metabolic diseases, genetic syndromes and other cardiac abnormalities has been reported. [18][19][20] Genetic studies have shown sarcomere gene mutations in some patients with isolated LVNC similar to those with dilated or hypertrophic cardiomyopathy, and a recent study has found similar mutations in patients with Ebstein anomaly.…”