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 for genetic aortopathy syndromes, particularly Marfan syndrome (MFS). Moreover, histopathological similarities between CHD-associated and MFS-associated aortopathies have been found.1

This review outlines CHD–associated thoracic aortic dilatation, with special focus on management and indications for surgery. Genetic aortopathy syndromes, including MFS, are addressed as these also require cardiologic follow-up of the proximal aorta. Moreover, data from studies in patients with MFS may inform management of proximal aortic dilatation in the setting of other diseases and syndromes. International guidelines on aortic disease,2–4 ACHD5 6 and valvular heart disease7 are complementary with respect to management of CHD-associated aortic dilatation, and the proposed management represents an integration of these guidelines.