1212Gene-based risk stratification for cardiac disorders in LMNA mutation carriers

Clinical disease presentation and ECG characteristics of LMNA mutation carriers

OBJECTIVE Mutations in the LMNA gene encoding lamins A and C of the nuclear lamina are a frequent cause of cardiomyopathy accounting for 5-8% of familial dilated cardiomyopathy (DCM). Our aim was to study disease onset, presentation and progression among LMNA mutation carriers. METHODS Clinical follow-up data from 27 LMNA mutation carriers and 78 patients with idiopathic DCM without an LMNA m...

Cardiac imaging for risk stratification in diabetes.

World wide, 200 million individuals currently have diabetes, and projections by the World Health Organisation suggest that its prevalence will exceed 300 million by 2025 and 360 million by 2030. More than 90% of these individuals will have type 2 diabetes. Management guidelines in Europe and the USA consider type 2 diabetes to be a cardiovascular disease equivalent. These patients have a 2–4-fo...

Risk stratification in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated desmosomal mutation carriers.

BACKGROUND We investigated the role of phenotypic characteristics in stratifying the risk of sustained ventricular arrhythmias in patients harboring arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated mutations. METHODS AND RESULTS Clinical, electrocardiographic, and arrhythmic outcome (composite measure of first occurrence of sustained ventricular tachycardia/resuscitated su...

Risk Stratification for Cardiac Arrhythmia Patients

Increased ventilatory response to exercise in symptomatic and asymptomatic LMNA mutation carriers: a follow-up study.

BACKGROUND LMNA mutations are an important cause of cardiomyopathy often leading to cardiac arrhythmias, heart failure and even heart transplantation. An increasing number of asymptomatic mutation carriers are identified, as family members of the index patients are screened. Our aim was to study the disease progression in asymptomatic LMNA mutation carriers and in patients with symptomatic card...