
This study investigated the clinical characteristics and outcomes of individuals with Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy. Three patterns of left ventricular remodeling were identified: progression, absolute regression, and relative regression. The findings revealed a high survival rate, with freedom from the primary endpoint at 92.7% after one year and 80.9% at five years. Patients with higher maximal left ventricular wall thickness z-scores exhibited reduced survival. Absolute regression was the most common type of left ventricular remodeling observed. These results enhance our understanding of the natural course of left ventricular hypertrophy in Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy, aiding clinicians in risk stratification and predicting clinical outcomes.
Like
Save
Share