Lung Transplantation for Idiopathic Pulmonary Arterial Hypertension: Steps in the Right Direction
In the last decade, there have been remarkable advances in the medical management of patients with idiopathic pulmonary arterial hypertension (IPAH). These therapies have improved the quality of life, saved many lives and in some cases obviated the need for lung transplantation. However, the reality is that the majority of patients with IPAH, even with access to the best medical therapy, continue to progress and will require lifesaving lung transplantation.
Lung transplantation for PAH has traditionally been viewed as a higher up front surgical risk lung transplant to perform, but with excellent long term survival and quality of life. The concern in the current management strategy for patients with PAH is, that by virtue of the seriousness of the condition, they tend to have the highest mortality on the lung transplant wait list and then, with the practice of listing them after they begin to fail first, second and sometimes third line medical therapies, they are in even poorer medical condition and generally deteriorating very rapidly. With a limited supply of donor organs, once can see that this might exacerbate the problem of wait list mortality in this particular patient population that cannot afford to wait. One can also appreciate then, that when the UNOS Lung Allocation Score (LAS) was initially introduced, this caused some concern regarding the potential for disadvantaging IPAH patients on the list. The LAS score was designed with the intent to optimize utilization of the scarce resource - donor lungs - by allocation of lungs to patients who have the best potential for a good outcome after transplantation, balancing mortality on the waitlist with survival probability after transplant.
- Received May 16, 2013.
- Accepted May 17, 2013.