OBJECTIVES: We sought to assess outcomes in a series of young patients with 
Marfan syndrome and to define the prevalence of ventricular arrhythmias in this 
patient population.
BACKGROUND: While sudden death is a well-recognized outcome in Marfan syndrome, 
ventricular arrhythmias are not well described.
METHODS: Patients were followed with echocardiography, electrocardiography, and 
ambulatory electrocardiography. The prevalence and associated factors for 
ventricular dysrhythmias were defined.
RESULTS: Seventy patients with Marfan syndrome diagnosed at birth to 52 years 
were followed for a period of up to 24 years. All patients had cardiovascular 
involvement and were started on medical therapy. No patient died from aortic 
dissection, while 4% died from arrhythmias. Ventricular arrhythmias were present 
in 21% and were associated with increased left ventricular size, mitral valve 
prolapse, and abnormalities of repolarization.
CONCLUSIONS: Cardiac complications are rare in young patients with Marfan 
syndrome receiving medical therapy and close clinical follow-up. Sudden death 
still occurs, and appears more common in patients with a dilated left ventricle. 
Left ventricular dilation may predispose to alterations of repolarization and 
fatal ventricular arrhythmias.