Prevalence and clinical correlates of ascending aortic dilatation in patients with noncompaction cardiomyopathy.

Ascending aortic (AoAsc) dilatation can lead to acute aortic syndromes and has been described in various familial cardiac diseases. Its prevalence and clinical significance in patients with noncompaction cardiomyopathy (NCCM) are however unknown. Establishing the prevalence can facilitate recommendations on routine screening in NCCM. In this cross-sectional cohort study based on the Rijnmond Heart Failure/Cardiomyopathy Registry, the patient were enrolment between 2014 and 2021. All NCCM patients (n = 109) were age and sex matched with 109 dilated cardiomyopathy (DCM) patients as controls. The aortic diameters were measured through the parasternal long-axis transthoracic echocardiographic view at the sinuses of valsalva (SoV-Ao), sinotubular junction (STJ) and ascending aorta (AscAo). Dilatation was defined using published criteria adjusted for body surface area (BSA), sex, and age. Median age of age-sex matched NCCM and DCM patients was 45[31-56] vs. 45 [31-55] years with 53% males in both groups. NCCM patients had more familial hereditary patterns and genetic variants (55% vs. 24%, p < 0.001). DCM patients had more heart failure and left ventricular dysfunction (ejection fraction 34 ± 11 vs. 41 ± 12, p = 0.001). Ascending aortic dilatation was present in 8(7%) patients with NCCM and 5(5%) patients with DCM (p = 0.46). All dilatations were classified as mild. In conclusion, in this cross-sectional cohort study the prevalence of ascending aortic dilatation in NCCM patients was 7%, which were only mild dilatations and not significantly different from an age-sex matched cohort of DCM patients. Routine aortic dilatation screening therefore does not seem warranted in patients with NCCM.

Clinical outcomes of implantable cardioverter-defibrillator therapy in noncompaction cardiomyopathy: a systematic review and meta-analysis.

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.