Long-Term Prognosis of Different Subtypes of Left Ventricular Noncompaction Cardiomyopathy Patients: A Retrospective Study in China

Abstract

Left ventricular noncompaction (LVNC) is a heterogeneous cardiomyopathy that can be classified into different subtypes based on morphologic and functional features. However, the prognosis of the dilated and isolated subtypes of non-pediatric LVNC remains unknown. We retrospectively studied 101 patients with LVNC diagnosed at Peking Union Medical College Hospital from 2006 to 2022 using the Jenni criteria of transthoracic echocardiography. The patients were grouped into those with dilated LVNC (n = 64) or isolated LVNC (n = 37), and 88 patients (54 with dilated LVNC and 34 with isolated LVNC) were followed up successfully. The primary outcome was major adverse cardiovascular events (a composite of cardiovascular mortality, heart failure, severe ventricular arrhythmia, and systolic embolism). The median follow-up time was 5.24 years. The incidence of major adverse cardiovascular events was 43.2%; patients with dilated LVNC had a higher risk (adjusted hazard ratio, 4.43; 95% confidence interval, 1.24–15.81; p = 0.02) than those with isolated LVNC. None of the isolated LVNC patients had cardiovascular deaths or severe ventricular arrhythmias. The risk of systemic embolism was similar between patients with dilated and isolated LVNC. Our findings indicate that transthoracic echocardiography is a useful tool for classifying LVNC into subtypes with distinct clinical outcomes. Dilated LVNC is associated with a poor prognosis, while the isolated subtype is probably a physiological condition.

Clinical Features of Noncompaction Cardiomyopathy Across All Age Strata

“…They observed that NCM patients with MR may face a higher risk of adverse cardiovascular outcomes 4 . These observations were corroborated by Gao et al, who identi ed a signi cantly elevated risk of adverse events in NCM patients with left ventricular dilation compared to those without 13 . Consistent with these ndings, our comprehensive analysis demonstrated that across all age groups, NCM patients with MR exhibited markedly impaired left heart function and increased left heart size compared to NCM patients without valvular disease.…”

“…The European Society of Cardiology has classi ed NCM as an unclassi ed hereditary cardiomyopathy 12 .Based on the presence or absence of comorbidities, NCM was divided into isolated NCM and NCM with other concomitant congenital heart disease 12 . Strati ed by different affected sites, NCM manifests as left ventricular type, right ventricular type, and biventricular type, with the left ventricular type being the most prevalent, and isolated right ventricular involvement being exceptionally rare 12,13 . This comprehensive study revealed a predominant occurrence of NCM in conjunction with leftto-right shunt congenital heart disease, leading to increased left heart volume load-a pattern consistent with prior research ndings [14][15][16] .…”