PMID: 29762936May 16, 2018Paper

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

The Journal of Heart Valve Disease
Krithika RamaprabhuRobert Coelho

Abstract

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.

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Cardiac Aneurysm

Aneurysm refers to a bulge of the wall or lining of a vessel commonly occurring in the blood vessels at the base of the septum or within the aorta. In the heart, it usually arises from a patch of weakened tissue in a ventricular wall, which swells into a bubble filled with blood. Discover the latest research on cardiac aneurysm here.

Aortic Aneurysm

An aortic aneurysm is the weakening and bulging of the blood vessel wall in the aorta. This causes dilatation of the aorta, which is usually asymptomatic but carries the risk of rupture and hemorrhage. Find the latest research on aortic aneurysms here.