Double Lung and Heart-Lung Transplantation for Congenital Heart Disease with Eisenmenger Syndrome and Idiopathic Pulmonary Arterial Hypertension

Abstract

Patients with pulmonary hypertension who develop concomitant refractory end-stage lung disease and/or chronic end-stage heart disease should undergo evaluation to determine if they are candidates for double lung (DLTx) or heart-lung transplantation (HLTx). Pulmonary hypertension is the indication for approximately 4.5% of total lung transplants. The most common indication for HLTx is complex congenital heart disease (CHD) with Eisenmenger syndrome. HLTx is also indicated in patients with idiopathic pulmonary arterial hypertension and severe right ventricular (RV) failure. Patients with pulmonary hypertension represent a heterogenous group not only in terms of mechanism leading to the development of pulmonary hypertension but also regarding the presence and degree of right and/or left ventricular dysfunction. The choice between double lung transplant (DLTx) and HLTx is based on the etiology, clinical presentation, and other factors. In this chapter, we will discuss the treatment of patients with CHD with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, including the surgical option of DLTx and HLTx.