Introduction: Noncompaction cardiomyopathy (NCCM) is characterized by left ventricular (LV) hypertrabeculation. Therefore, endocardial tracing / quantification of LV function could be cumbersome. Hypothesis: Accurate assessment of the (subclinical) LV dysfunction in NCCM patients could improve non-invasive monitoring of disease progression, risk stratification, and treatment. Method: We reviewed the echocardiographic images of 67 patients (54% male, median age 48 year [22-73]. LV function was assessed by conventional biplane disk summation, wall motion score (WMS), and global longitudinal strain (GLS). LV function was considered abnormal if LV ejection fraction (LVEF) <50% and GLS >-18,9%. Results: LV function measured by biplane vs WMS showed significantly lower LV EF with WMS (p-value: 0,0016; Figure ), while the average GLS in all patients was -11,0%, SD ±3,8 (normal values: -18,9%; p-value: p<0,001). By dichotomizing the group into LVEF <50% vs. ≥50%, biplane EF was abnormal in 68,7%, while this was with WMS 88,1% en GLS in 100% patients. In patient with EF <50% compared to ≥ 50% GLS in low biplane EF group was: -9.3% vs -14.5% (p<0.001). In the WMS EF was this respectively -10,3±3,6% versus -15.5± 1.5 (p<0.001). Conclusion: The systolic LV dysfunction in patients with NCCM noncompaction cardiomyopathy found by WMS and GLS was significantly lower compared to routine biplane EF measurement suggesting that WMS and GLS measurement are much more potential prognostic tool. Given the correct execution of WMS is limited by the assessor's experience, estimation of the LV function with GLS are probably most reliable. Future research should expel whether these results are representative in a larger group of patients and correlates with the clinically relevant endpoints.

A subgroup of patients with noncompaction cardiomyopathy (NCCM) is at increased risk of ventricular arrhythmias and sudden cardiac death (SCD). In selected patients with NCCM, implantable cardioverter-defibrillator (ICD) therapy could be advantageous for preventing SCD. Currently, there is no complete overview of outcome and complications after ICD therapy in patients with NCCM. This study sought to present an overview using pooled data of currently available studies. Embase, MEDLINE, Web of Science, and Cochrane databases were searched and returned 915 studies. After a thorough examination, 12 studies on outcome and complications after ICD therapy in patients with NCCM were included. There were 275 patients (mean age 38.6 years; 47% women) with NCCM and ICD implantation. Most of the patients received an ICD for primary prevention (66%). Pooled analysis demonstrates that the appropriate ICD intervention rate was 11.95 per 100 person-years and the inappropriate ICD intervention rate was 4.8 per 100 person-years. The cardiac mortality rate was 2.37 per 100 person-years. ICD-related complications occurred in 10% of the patients, including lead malfunction and revision (4%), lead displacement (3%), infection (2%), and pneumothorax (2%). Patients with NCCM who are at increased risk of SCD may significantly benefit from ICD therapy, with a high appropriate ICD therapy rate of 11.95 per 100 person-years and a low cardiac mortality rate of 2.37 per 100 person-years. Inappropriate therapy rate of 4.8 per 100 person-years and ICD-related complications were not infrequent and may lead to patient morbidity.

No abstract