770 LONG-TERM PROGNOSIS IN LVNC CARDIOMYOPATHY: A SINGLE-CENTRE EXPERIENCE
Authors: Principi Samuele , Alfieri Michele , Paolini Federico , Bastianoni Gianmarco , Coraducci Francesca , Brugiatelli Leonardo , Torselletti Lorenzo , Stronati Giulia , Barbarossa Alessandro , Ciliberti Giuseppe , Russo Antonio Dello , Guerra Federico 
Published: December 2022

Background

left ventricular non-compaction (LVNC) cardiomyopathy is an often underdiagnosed and under-classified disease deriving from the incomplete development of ventricular myocardium. Clinical presentations may be variable and uncommon, ranging from an apparent lack of functional anomalies to heart failure, ventricular arrhythmias and, in some cases, even ischemic stroke. Despite great improvements in diagnostic performance there is still a widespread lack of evidence regarding the prognosis and management of affected patients.

Methods

all consecutive patients admitted to our Cardiology Institution from October 2009 to August 2022 fulfilling LVNC criteria by echocardiography or cardiovascular magnetic resonance (CMR) or both, were consecutively enrolled. CMR has been performed wherever possible. All patients underwent a complete cardiological visit, a 12-lead ECG and echocardiography at baseline, whereas at follow-up, if a complete visit was not possible, information regarding patients’ endpoints was acquired through telephonic contact. Additional diagnostic exams or implantation of a cardiac device were also performed if indicated. The primary endpoint was a composite of at least one between: sustained ventricular arrhythmias, an appropriate ICD intervention and sudden cardiac death. Secondary endpoints included supraventricular arrhythmias, unplanned cardiac hospitalizations, acute decompensated, chronic heart failure and ischemic stroke. Risk predictor analyses were not performed as the overall event rates were low and the risk for type II error was high.

Results

forty patients (26 males; age 45±17) were prospectively enrolled and followed up for a median of five years. CMR and echocardiography were overall agreeing on the majority of the diagnoses, with 62.5% of patients meeting the echo criteria and 70% of patients meeting the CMR criteria for LVNC. The incidence of the primary endpoint was 1.8% per years. Male gender and late gadolinium enhancement (LGE) were correlated with an increased incidence of the primary endpoint, while LVEF, NC/C or functional status were not associated with a significantly increased risk of the composite endpoint. HF diagnosis was the most common endpoint (6.1% annual incidence). The annual incidence of supraventricular arrhythmias was 3.0% and the annual incidence of stroke was 0.7%. Twenty-four patients (60%) experienced at least one hospitalization during follow-up. Unplanned hospitalizations represented 20% of all hospitalizations and were mainly HF-related. Planned hospitalizations were performed for elective procedures such as atrial fibrillation cardioversion, ablation, coronary angiography or diagnostic check-ups.

Discussion

in patients with LVNC, there is an increased incidence of cardiac-related outcomes than in the general population; furthermore, male gender and myocardial fibrosis are associated with increased risk of events. This trend highlights the importance of a prompt diagnosis and, obviously, of a correct knowledge of such disease.