Management of Aortic Dissection in Marfanoid young patient with Ebstein anomaly: Rare Case Report

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DOI: 10.21522/TIJMD.2013.04.01.Art004

Authors : Naresh Sen


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.


[1.]   Ades L. Guidelines for the diagnosis and management of Marfan syndrome. Heart Lung Circ. 2007;16:28–30.

[2.]   Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Warnes CA, Dearani JA. Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly: survival and functional improvement. J Am Coll Cardiol. 2012 Jun 5. 59(23):2101-6.

[3.]   Attie F, Casanova JM, Zabal C, Buendía A, Miranda I, Rijlaarsdam M. Ebstein's anomaly. Clinical profile in 174 patients. Arch Inst Cardiol Mex. 1999 Jan-Feb. 69(1):17-25..

[4.]   Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: second of two parts. N Engl J Med. 2000;342:334–342.

[5.]   Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Effect of operation for Ebstein anomaly on left ventricular function. Am J Cardiol. 2008 Dec 15. 102(12):1724-7.

[6.]   Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Functional status after operation for Ebstein anomaly: the Mayo Clinic experience. J Am Coll Cardiol. 2008 Aug 5. 52(6):460-6.

[7.]   Dean JCS. Management of Marfan syndrome. Heart. 2002;88:97–103.

[8.]   Groves ER. Epidural analgesia for labour in a patient with Ebstein’s anomaly. Can J Anaesth 1995;42(1):77-79.

[9.]   McGoldrick KE. Eye, ear, nose, and throat diseases. In: Fleisher LA, editor. Anesthesia and Uncommon Disease. 5th ed. Philadelphia: Saunders Elsevier; 2006. pp. 6–7.

[10.]   Meijboom LJ, Nolle GJ, Mulder BJM. Prevention of cardiovascular complications in the Marfan syndrome. Vas Disc Prevent. 2004;1:79–86.

[11.]   Mesrobian RB, Epps JL. Midtracheal obstruction after Harrington rod placement in a patient with Marfan’s syndrome. Anesth Analg. 1986;65:411–413.

[12.]   Misa VS, Pan PH. Evidence based case report for analgesic and anaesthetic management of parturient with Ebstein’s anomly and Wolff-Parkinson- White syndrome. Int J Obstet Anesth 2007;16:77-81.

[13.]   Oh AY, Kim YH, Kim BK, et al. Unexpected tracheomalacia in Marfan syndrome during general anesthesia for correction of scoliosis. Anesth Analg. 2002;95:331–332.

[14.]   Oliver WC, Lynch JJ. Congenital heart disease. In: Fleisher LA, editor. Anesthesia and Uncommon Disease. 5th ed. Philadelphia: Saunders Elsevier; 2006. pp. 115–118.

[15.]   Robinson PN, Godfrey M. The molecular genetics of Marfan syndrome and related microfibrillopathies. J Med Genet. 2000;37:9–25.

[16.]   Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan’s syndrome. N Engl J Med. 1994;330:1335–1341.