Aortopathies in adult congenital heart disease and genetic aortopathy syndromes: management strategies and indications for surgery

Learning objectives Review the epidemiology, histology, pathophysiology and genetics of congenital heart disease-associated aortopathies and genetic aortopathy syndromes. Review and describe appropriate imaging strategies for diagnosis and follow-up appropriate pharmacotherapeutic strategies and indications for aortic surgery considerations relevant to pregnancy considerations relevant to sports participation in patients with congenital heart disease-associated aortopathy or a genetic aortopathy syndrome. Introduction Progressive proximal aortic (ie, aortic root and ascending aortic) dilatation is frequently found in adults with unrepaired or repaired congenital heart disease (CHD). With the growing number of patients with adult CHD (ACHD), physicians increasingly find themselves challenged with the management of proximal aortic dilatation in this heterogeneous patient population. Data on the natural history of CHD-associated aortic dilatation, including risk of dissection, rupture and aortic insufficiency, are scarce. These issues, essentially informing decisions regarding surveillance, pharmacotherapy and surgery, are better defined...
Source: Heart - Category: Cardiology Authors: Tags: Education in Heart Source Type: research