Ascending aortic dissection - Marfan syndrome

Case contributed by Dr Danilo Ferreira Maia

Presentation

A female patient experienced chest pain in the precordial region radiated to the back of sudden onset.

Patient Data

Age: 30 years
Gender: Female

Intimal flap within the ascending aorta compatible with dissection that extends into the abdominal aorta to the level of the renal arteries. The ascending aorta is dilated. 

Case Discussion

Electrocardiogram without significant changes. Chest radiography showed cardiomegaly. An echocardiogram showed dissection of the ascending aorta with significant aortic valve insufficiency and ventricular dysfunction.

The patient presented two siblings with the diagnosis of Marfan syndrome manifesting as right lens dislocation, dolichocephaly and joint hypermobility.

After CT angiography, the patient underwent implantation of a biological tube by the Bentall technique, with reimplantation of the coronary ostia.

Hospital discharge after 3 days, hemodynamically stable.

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Case information

rID: 68347
Published: 3rd Jun 2019
Last edited: 7th Jun 2019
System: Vascular, Chest
Inclusion in quiz mode: Included
Institution: Santa Casa de Belo Horizonte

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