Recent progress in medical therapies has diminished the role of transplantation in the management of PPH during the past decade. Drug therapy is not effective in some patients, responses to therapy are not sustained over time in others, and drug side effects eventually limit the benefits of treatment in a few more. Lung transplantation therefore ultimately is the only alternative for patients whose PPH is severe and cannot be managed medically. Choosing the right patient as a transplant candidate and the right time to make the initial referral to a transplant center are the crucial initial steps in the transplantation process, and the long waiting time before transplantation must be integrated into this decision. The outcome of lung and heart-lung transplantation for PHH has been good but sobering. Functional recovery has been excellent, but long-term survival results have been limited by the high prevalence of chronic allograft rejection.