Management of Acute Aortic and Pulmonary Complications in Marfan Syndrome

Initial Diagnostic Approach

The American Heart Association recommends simultaneous imaging with chest X-ray and CT angiography of the chest to confirm pneumothorax and exclude Type A or Type B aortic dissection in patients with Marfan syndrome presenting with sharp ipsilateral chest pain and dyspnea 1, 2
The American College of Cardiology suggests that aortic dissection can present with pleuritic chest pain mimicking pneumothorax, and therefore, imaging should not be delayed for physical examination findings alone 1
Assessment for hemodynamic instability, such as hypotension and tachycardia, is crucial in patients with suspected aortic dissection or tension pneumothorax, as recommended by the American Heart Association 2

The American College of Cardiology recommends emergency surgical repair with composite graft replacement for Type A aortic dissection in Marfan syndrome, regardless of aortic diameter 2, 4
The American Heart Association suggests that Type B dissection is initially managed medically with aggressive blood pressure control targeting systolic BP <120 mmHg using intravenous beta-blockers 2, 5

The American College of Cardiology recommends starting beta-blocker and angiotensin receptor blocker in maximally tolerated doses to reduce aortic dilation rate in patients with Marfan syndrome 2, 5
The American Heart Association suggests targeting heart rate <60-70 bpm and systolic BP <120 mmHg to reduce hemodynamic stress on the aortic wall 5

The American College of Cardiology recommends performing echocardiography at 6 months after the initial event to establish the rate of aortic enlargement in patients with Marfan syndrome 1, 3
The American Heart Association suggests annual echocardiography if the aortic root is <4.5 cm and stable, with increased frequency to every 6 months if the diameter is ≥4.5 cm or growth rate ≥0.5 cm/year 2, 3

The American College of Cardiology recommends prophylactic aortic root replacement when the diameter reaches ≥5.0 cm (external diameter measurement) in patients with Marfan syndrome 2, 4
The American Heart Association suggests that earlier surgery at ≥4.5 cm is reasonable with additional risk factors, including family history of dissection at <5.0 cm, rapid growth ≥0.5 cm/year, severe aortic regurgitation, or planned pregnancy 2, 4