Lung transplantation for idiopathic pulmonary arterial hypertension: steps in the right direction.

I n the past 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 IPAH 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 IPAH 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, in general, are deteriorating very rapidly. With a limited supply of donor organs, onc can see that this might exacerbate the problem of waitlist mortality in this particular patient population that cannot afford to wait. One can also appreciate then, that when the United Network for Organ Sharing Lung Allocation Score (LAS) was initially introduced, this caused some concern regarding the potential for placing IPAH patients on the list at a disadvantage. The LAS score was designed with the intent to optimize the use of the scarce resource, donor lungs, by the 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. To optimally treat patients with IPAH, the treating physicians in the IPAH and lung transplant community need to (1) optimize medical care, (2) optimize timing of listing for transplant , and (3) optimize management on the lung transplant waitlist to maximize the likelihood of successful lifesaving lung transplantation in the context of available donor organs and current listing guidelines and practices. 1. Optimizing medical care of IPAH patients: This is a large and growing field in which major advances have been made with the development of intravenous infusion therapies of prostacyclin and related analogs and other vasoactive drugs. As further research is conducted in the fundamental pathophysiology and underlying mechanisms of IPAH, additional advances have been and continue to be made with the …