• dilatation of the ascending aorta with or without aortic
regurgitation and involving at least the sinuses of Valsalva; or
• dissection of the ascending aorta
• mitral valve prolapse with or without mitral valve regurgitation
• dilatation of the main pulmonary artery in the absence of valvular
or peripheral pulmonic stenosis or any other obvious cause,
below the age of 40 years
• calcification of the mitral annulus below the age of 40 years; or
• dilatation or dissection of the descending thoracic or abdominal
aorta before the age of 50 years.
For the cardiovascular system to be involved a major criterion or
only 1 of the minor criteria must be present.
Why Might a Person with Marfan SyndromeDevelop an Arrhythmia?
1. Unrelated to Marfan Syndrome
Arrhythmia with a Normal Heart
• caffeine, alcohol, stress, anxiety, lack of sleep, stimulants
• Electrolyte abnormalities (low K+, low Mg++)
Arrhythmia with Underlying Heart Disease
• HTN, CAD, Cardiomyopathy, CHF
• Conduction disease
• Valvular heart disease
2. Related to Marfan Syndrome
Valvular Heart Disease
• Mitral valve prolapse
• Mitral regurgitation
• Aortic insufficiency
Mitral Valve Prolapse and Arrhythmias
1. MVP occurs in 3-5% of general population
2. MVP occurs in >60% of people with the Marfan syndrome
3. Vast majority of people with MVP are asymptomatic
Mitral Valve Prolapse and Arrhythmia
1. Spectrum of arrhythmias have been observed in pts with MVP
PAC’s, PVC’s, WPW, SVT, Ventricular arrhythmias
Can be related to associated cardiac dysfunction: (MR, LAE, LVE)
2. Mechanism of arrhythmia unclear:
Related to mechanical stretch on mitral valve?
Related to underlying cardiomyopathy?
Related to autonomic dysfunction?
3. MVP and Sudden Death
Very rare complication of MVP
Associated with: Severe MR, Severe Valvular deformity, LV
enlargement and dysfunction, Complex ventricular arrhythmia,
Long QT, Abnormal EKG (inferior ST-T), syncope