Natural History of Hypertrophic Cardiomyopathy in Noonan Syndrome With Multiple Lentigines.

ABSTRACT:

Background

We aimed to examine clinical features and outcomes of consecutive molecularly characterized patients with Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy.

Methods

A retrospective, longitudinal multicenter cohort of consecutive children and adults with a genetic diagnosis of Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy between 2002 and 2019 was assembled. We defined a priori 3 different patterns of left ventricular remodeling during follow-up: (1) an increase in ≥15% of the maximal left ventricular wall thickness (MLVWT), both in mm and z-score (progression); (2) a reduction ≥15% of the MLVWT, both in mm and z-score (absolute regression); (3) a reduction ≥15% of the MLVWT z-score with a stabl

SUBMITTER: Monda E 

PROVIDER: S-EPMC11791648 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature