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A 26 year old English teacher with Marfan syndrome had been lost to follow up for two years in which time his aortic root had dissected and increased in diameter from 4.4 cm to 8.5 cm, without symptoms.
He had previously had mitral valve repair for valve prolapse, and there was a strong family history of early sudden death. He had attended annual follow up from the age of 14 years until two years ago when he went to teach abroad. On previous visits his systolic blood pressure had never exceeded 110 mm Hg and aortic root measurements by echocardiography had been steady at 4.4 cm. He was not on β blockers because of asthma. He re-attended after returning to the country.
Although he was asymptomatic, on clinical examination signs of severe aortic regurgitation were found. He was admitted and magnetic resonance imaging (MRI) was performed. It showed (panel A) a flask shaped aneurysm (An) confined to the aortic root and lower ascending aorta with a maximum diameter of 8.5 cm, and a dissection flap (white arrow). The turbulent aortic regurgitant jet (black arrow head) represents severe aortic incompetence. The patient underwent emergency aortic root replacement with a St Jude valved conduit, and made a full recovery. The postoperative MRI (panel B) showed good surgical result, the signal void from the metal ring of the St Jude valve (large arrow head) and the distal suture line of the conduit (paired small arrow heads).
This case illustrates that aortic dissection in Marfan syndrome can be asymptomatic and vigilant follow up is essential for such patients.
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