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Cardiology / Cardiovascular

Marfan Syndrome (Aortic Root Dilation)

ICD-10 Code
Q87.4

Advanced Clinical Criteria for Marfan Syndrome (Aortic Root Dilation).

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents for follow-up of known Marfan Syndrome (Q87.4). Reports no current chest pain, palpitations, or exertional dyspnea. Known history of aortic root dilation, currently managed with beta-blockade. Denies syncope, visual disturbances, or joint pain. Compliance with medication and activity restrictions is confirmed.

Clinical Examination Findings

General: Tall, thin habitus, dolichostenomelia, arachnodactyly. Cardiovascular: Regular rate and rhythm, S1/S2 normal, no murmurs, rubs, or gallops. Peripheral pulses symmetric. Musculoskeletal: Pectus excavatum noted, joint hypermobility present. Ocular: Lens subluxation absent on gross inspection.

Treatment Protocol

Continue current regimen of beta-blockers (e.g., Atenolol/Propranolol) for heart rate and blood pressure control to reduce aortic wall stress. Maintain target systolic BP <120 mmHg. Annual echocardiographic monitoring of aortic root diameter. Strict avoidance of contact sports and heavy isometric lifting.

Detailed clinical guide coming soon.