Acute Aortic Dissection Determines the Fate of Initially Untreated Aortic Segments in Marfan Syndrome
What are the incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome?
This was a retrospective analysis of 86 consecutive patients with Marfan syndrome who underwent 136 aortic surgeries.
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.
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.
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.
Keywords: Aortic Aneurysm, Thoracic, Marfan Syndrome, Pain
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