Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation

Abstract

Background: Left ventricular non-compaction (LVNC) may manifest an undulating phenotype ranging from dilated to hypertrophic appearance. It is unknown whether tissue Doppler (TD) velocities can predict adverse clinical outcomes including death and need for transplantation in children with LVNC. Methods and results: 56 children (median age 4.5 years, median follow-up 26 months) with LVNC evaluated at one hospital from January 1999 to May 2004 were compared with 56 age/sex-matched controls. Children with LVNC had significantly decreased early diastolic TD velocities (Ea) at the lateral mitral (11.0 vs 17.0 cm/s) and septal (8.9 vs 11.0 cm/s) annuli compared with normal controls (p,0.001 for each comparison). Using receiver operator characteristic curves, the lateral mitral Ea velocity proved the most sensitive and specific predictor for meeting the primary end point (PEP) at 1 year after diagnosis (area under the curve = 0.888, SE = 0.048, 95% CI 0.775 to 0.956). A lateral mitral Ea cut-off velocity of 7.8 cm/s had a sensitivity of 87% and a specificity of 79% for the PEP. Freedom from death or transplantation was 85% at 1 year and 77% at 2 years. Conclusions: TD velocities are significantly reduced in patients with LVNC compared with normal controls. Reduced lateral mitral Ea velocity helps predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation.

Left ventricular noncompaction cardiomyopathy: updated review

“…McMahon and colleagues reported a reduction of tissue Doppler velocities in children with LVNC compared with normal controls [McMahon et al 2007]. The diagnosis of isolated LVNC can be difficult because of the association with other congenital heart diseases [Madan et al 2012].…”

“…Just like the adults, noncompaction affects mostly the left ventricle but the right ventricle and septum can also be affected [Sert et al 2012]. It can manifest in different forms ranging from dilated to hypertrophic appearance [McMahon et al 2007]. Some children can present with chest pain, heart murmur, abnormal heart sound, abnormal EKG and/ or abnormal echocardiogram [Brescia et al 2013;Pignatelli et al 2003].…”

Left Ventricular Noncompaction

“…Tissue Doppler-derived indices of LV diastolic relaxation are significantly reduced in patients with LVNC, and predict children who are at risk of adverse clinical outcome, including death or the need for cardiac transplantation. 39,40 Restrictive hemodynamics identified by cardiac catheterization, as well as an initial presentation of LVNC as a restrictive cardiomyopathy in children with LVNC, have been described. 8 Diastolic dysfunction in ventricular noncompaction may be related to both abnormal relaxation and restrictive filling caused by the numerous prominent trabeculae.…”

Successful Cardiac Resynchronization Therapy in a 3-Year-Old Girl With Isolated Left Ventricular Non-Compaction and Narrow QRS Complex

“…8 Tissue Doppler velocities are significantly reduced in patients with LVNC, and help identify children with LVNC who are at risk of adverse clinical outcomes, including death, and need cardiac transplantation. 9 Successful CRT in adults with isolated LVNC has been reported recently, 10 however, the efficacy of CRT for pediatric patients with LVNC has not been reported. The use of CRT in children has gained a great deal of attention, especially in Japan where there is little chance of heart transplantation.…”