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.
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
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.
Aortic dissection, marfan syndrome, ebstein anomaly.
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