Acute Aortic Dissection Determines the Fate of Initially Untreated Aortic Segments in Marfan Syndrome

Study Questions:

What are the incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome?

Methods:

This was a retrospective analysis of 86 consecutive patients with Marfan syndrome who underwent 136 aortic surgeries.

Results:

Primary presentation was acute aortic dissection (AAD) in 36% (77% type A, 23% type B) and aneurysmal disease in 64% of patients. In patients without AAD, 11% required surgery on primarily nontreated aortic segments (five of six patients experienced type B dissection during follow-up), whereas in patients after AAD, 48% underwent surgery of initially nontreated aortic segments (42% of patients with type A and 86% of those with type B dissection; p = 0.0002). Among patients with acute type B dissection, mean diameter at the time of dissection was 37 ± 8.5 mm (range 19-50 mm). Mean diameter at the time of surgery was 64 ± 10.6 mm (41-80 mm), indicating an average growth rate of 16 ± 11.4 mm/6 months after onset of pain. Sixty-seven percent of patients underwent surgery within the first year of conventionally “uncomplicated” type B aortic dissection.

Conclusions:

The authors concluded that uncomplicated type B dissection in patients with Marfan syndrome is a strong predictor for the need of reintervention in primarily nontreated aortic segments.

Perspective:

The results from this analysis are sobering in that, despite screening and prophylactic surgery, over one-third of Marfan syndrome patients in this cohort initially presented with acute dissection. Perhaps the most important finding from this analysis is that initially ‘uncomplicated’ type B aortic dissection may not be entirely ‘uncomplicated,’ and warrants very close follow-up. The authors of this analysis convincingly demonstrated that type B dissection in patients with Marfan syndrome is strongly associated with a subsequent need for aortic surgery and largely driven by the rate of aneurysmal growth.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD & Pediatrics, Congenital Heart Disease

Keywords: Aortic Aneurysm, Thoracic, Marfan Syndrome, Pain


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