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You are here: Contents > 2017 > Volume 26 Number 5 September 2017 > CASE REPORTS > Bentallís Procedure in Pediatric Mixed Connective Tissue Disease Syndrome: Management of Pediatric Aortic Aneurysm - A Brief Review

Bentallís Procedure in Pediatric Mixed Connective Tissue Disease Syndrome: Management of Pediatric Aortic Aneurysm - A Brief Review

Krithika Ramaprabhu1,2, Om Prakash1, Noveen Davidson1, Sanjay Bhalero1, Satish Radhakrishnan1, Robert Coelho1

1MIOT Hospitals, Manapakkam, Chennai, India
2Electronic correspondence: krithika25589@gmail.com

Mixed connective tissue disease (MCTD) syndrome in children may lead to large aortic aneurysms, which in turn pose a difficult surgical problem. Valve-sparing root replacement is not always a viable option as the disease process invariably affects the aortic valve leaflets. Among pediatric patients, the Ross procedure is contraindicated on account of weakness of the pulmonary root, while Bentall surgery is the ‘gold standard’ treatment of aortic

 

aneurysm, with reproducible and excellent long-term results. The case is presented of a three-year-old girl with a large thoracic aortic aneurysm in whom Bentall’s surgery was performed, with a good result. The present patient, with MCTD syndrome, was too young to have undergone aortic root replacement with a composite mechanical valved graft.

The Journal of Heart Valve Disease 2017;26:610-612


Bentall’s Procedure in Pediatric Mixed Connective Tissue Disease Syndrome: Management of Pediatric Aortic Aneurysm - A Brief Review

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