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. 2007 Apr;92(4):351–356. doi: 10.1136/adc.2006.097469

Table 2 Cardiovascular manifestations of Marfan's syndrome11,13,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38.

Lesion/feature Symptoms Frequency Complications Comments
Aortic root dilatation None 60–80%11,28,30,31,37 Aortic dissection Dissection rare in children <10 years old
Pulmonary artery dilatation None 76%35 Dissection rare Diagnostic feature in those <40 years old
Mitral regurgitation/prolapse/annular calcification Palpitations 52–68%29 Arrhythmias Regurgitation may be intermittent
Chest pain dyspnoea Endocarditis
Ventricular dysfunction
Descending aorta dilatation None Aortic dissection Rare in childhood
Endothelial dysfunction/abnormal aorta elasticity None 80–100%32,36 Increased vascular stiffness May contribute to dissection risk
Tricuspid valve prolapse None unless severe 4%30 May progress requiring repair Severe disease uncommon except in infantile type
36% in infantile type37
Left ventricular dysfunction Dyspnoea Up to 100% Diastolic. May be progressive to systolic dysfunction May occur despite normal valves
Reduced exercise tolerance Severity varies26,27
Arrhythmias Palpitations Up to 20–30%23,24,25 May cause sudden death Associated with ventricular dilatation
Collapse
Chest pain
Coronary artery aneurysm Myocardial infarction <1%34 Only described in adults
Atrial septal defect None 4%38 May need surgical repair More common than in normal population