ACUTE STANFORD TYPE A AORTIC DISSECTION PRESENTING WITH RESPIRATORY SYMPTOMS AS THE INITIAL MANIFESTATION OF SUSPECTED MARFAN SYNDROME: CASE REPORT
DOI:
https://doi.org/10.67214/ca15d605Keywords:
Aortic dissection, Bentall procedure, Marfan syndromeAbstract
Introduction: Acute Stanford type A aortic dissection is a life-threatening emergency that requires immediate diagnosis and surgical intervention. While the classic presentation involves severe chest or back pain, recognition may be delayed in patients with atypical symptoms, particularly those with underlying connective tissue disorders such as Marfan syndrome.
Case Presentation: A 46-year-old man (height 190 cm) with no prior diagnosis of connective tissue disease presented with a three-day history of fever, cough, and mild chest discomfort, without severe chest, back, or abdominal pain. Physical examination revealed a Marfanoid habitus, and the family history was notable for aortic dissection in several first-degree relatives. Computed tomography angiography of the aorta identified an acute Stanford type A (DeBakey I) dissection originating at the aortic root and extending through the ascending aorta, aortic arch, and descending thoracic aorta, with propagation toward the iliac arteries. Transthoracic echocardiography confirmed the diagnosis by revealing a markedly dilated aortic root (66–68 mm), an intimal flap at the right coronary cusp, and severe aortic regurgitation. The patient underwent urgent surgical repair using a Bentall procedure. The postoperative course was complicated by significant bleeding, requiring surgical re-exploration. Following this intervention, recovery was uneventful. The overall clinical presentation and perioperative findings were most consistent with an underlying diagnosis of Marfan syndrome.
Conclusion: This case underscores the importance of considering aortic dissection in patients with a Marfanoid phenotype and positive family history, even when typical symptoms are absent. Early diagnosis and surgical intervention are critical for improving survival in this high-risk population.
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Copyright (c) 2026 Matej Jordanoski (Author)
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