Surgical Evaluation of Congenital-Heart-Disease-Associated Pulmonary Hypertension

Abstract

Congenital heart disease (CHD) is the most common birth defect, with an incidence of approximately 0.8\% of live births. Improvements in surgery have altered the impact of CHD enormously, not only improving mortality in many conditions but also allowing some infants to survive into adulthood when previously they would have died within the first few days after birth. This increased survival has led to a huge increase in the number of survivors of surgery for CHD in childhood now reaching adulthood. This group of ``grown-up CHD{''} (GUCH) patients have distinct needs outside both pediatric and adult cardiology environments and are best served by specialists in this field who are trained to understand the long-term complications these patients face. Pulmonary arterial hypertension (PAH) associated with CHD (CHD-PAH) is relatively common, occurring in 5-10\% of patients. It may complicate both simple and more complex lesions and may occur early or late in the natural or operative history of the condition. It results in significant morbidity and early mortality of patients compared with those without evidence of PAH. This chapter discusses the evaluation of CHD patients with dynamic PAH where surgical correction of lesions may be safely undertaken. Surgical or interventional correction is desirable, particularly early in the natural history of CHD before advanced changes in the pulmonary vasculature are established, provided there is evidence to support reversibility of PAH afterward, although this may be difficult to predict in some difficult or marginal patients. Special consideration is given to established, moderate to severe PAH, in particular patients with Eisenmenger syndrome. Discussion of operability most often arises in older children and adults with large shunts and therefore this chapter concentrates on this group of patients. Consideration is also given to advanced therapies, including transplantation, and the importance of anesthetic and intensive care management in patients with CHD-PAH.