Abstract:

Background: Marfan syndrome is an autosomal dominant connective tissue disorder characterized by a combination of clinical manifestations in different organ systems. Patients with Marfan syndrome (MFS) whose lifetimes are extended may be encountered as acute abdomen cases (Aortic dissection) apart from the obligatory reasons and emergencies arising naturally out of their disease, as in the case reported. Ebstein’s anomaly is a rare congenital heart disease; in which there is a downward displacement of the tricuspid valve into the right ventricle.

Case Report: 25-year-old Indian male shopkeeper, 47 kg in weight and 176 cm tall was admitted in emergency department with acute abdominal pain associated with dyspnea. Long arms and legs, arachnodactyly, kyphoscoliosis, pectus excavatum were found to be present in the physical examination. According to chest radiography, density increase in the left apical field (hyperluscency) (thought to be secondary to bullous structure), transpiring in the ascendent aorta, a rightward shift of the heart (due to the effect of the kyphoscoliosis and pectus excavatum), and thoracolumbar scoliosis (kyphoscoliosis) with a rightward aperture were detected. In addition, according to the echocardiographic examination, Ebstein’s anomaly, Aortic Dissection, Tricuspid Regurgitaion, pulmonary hypertension, and inferior deficiency of mitral, aorta, and tricuspid valves were present. Moreover, a right bundle block (secondary to Ebstein’s anomaly) was observed in the electrocardiographic examination. Finally he was shifted to cardiothoracic surgery unit for surgical repair of aortic dissection and ebstein anomaly.

Conclusion: For cases of Marfan Syndrome (MFS), the risks of which have been detected through a detailed cardiac examination including echocardiography, we believe that they can be operated as emergency cases with close anesthesia management. Mostly ascending aorta is involved with marfan syndrome but in this case evidence of ebstein anomaly is an interesting point towards cardiovascular surgery and learning about management and post op care.

Keywords: Aortic dissection, marfan syndrome, ebstein anomaly.

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