心血管疾患と診断

What an Adult Cardiologist Should Know about Cyanotic Congenital Heart Disease?

Abstract

P. Syamasundar Rao

The prevalence of congenital heart defects (CHDs) in adults has increased during the last decade such that now there are more adults with CHD than children. Advances in the diagnosis and management of CHD appear to be the reason for such a phenomenon. In this review only cyanotic CHD are addressed. The majority are likely to be those that have undergone corrective or palliative surgery, although rarely uncorrected defects may present in adulthood. Pathophysiologic effects of right to left shunt associated with cyanotic CHD are reviewed. Brief description of the anatomy of the most common cyanotic CHD, namely, tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, total anomalous pulmonary venous connection and tricuspid atresia is presented followed by its management at presentation. The residual defects and their long-term effects along with management strategies for each of the above defects during adulthood are reviewed. Adults who did not have surgical correction in childhood should undergo surgical correction, but they may have a higher risk than seen in children.