Lung Transplantation and LAM

Referral for lung transplantation should be considered when the FEV1 approaches 30 percent. Patients may be eligible based on other factors that profoundly affect quality of life, such as disabling shortness of breath or problems maintaining oxygen saturation with lesser degrees of airflow obstruction.

Both single and bilateral lung transplantation have been performed for patients with LAM and both are suitable options.  Recent data from the United Network for Organ Sharing national registry suggest a trend toward slightly higher long-term survival associated with bilateral lung transplantation compared to single lung in LAM recipients but further corroboration is necessary before definitive recommendations can be made endorsing one procedure over the other.   Prior pleurodesis or pleurectomyis not a contraindication to transplant but does complicate the surgical removal of the native lung(s).  Referral to a lung transplant center experienced in handling complex pleural dissection is advised.

One, five, and ten-year survival rates following lung transplantation for LAM are89%, 67%, and 47%, respectively.  These survival rates are better on average than rates for patients who undergo transplant for different lung diseases . LAM has been documented to recur in a small percentage (likely < 10%) of LAM lung transplant recipients and recurrence does not typically result in significant impairment in the function of the transplanted lungs.

If you are on sirolimus or everolimus for LAM, it will need to be stopped for at least a month and maybe longer before going on the transplant waiting list.  This is necessary, as these medications have been implicated in wound healing problems, specifically healing at the anastomosis (the suture line where the donor bronchus is connected to the recipient’s bronchus).  Breakdown of the anastomosis is a serious complication after lung transplantation.

A few problems specific to LAM can develop after transplant and must be watched for by the transplant team.  The development of chylous pleural effusions can occur even in women who didn’t have chyle problems before the transplant operation.  They can usually be managed with chest tube placement and sometimes medications or diet modification can help.  If a patient receives a single lung transplant, then obviously the native lung may continue to pose LAM-related problems such as pneumothoraces or chylous pleural effusions.  Angiomyolipomas, if present, can hemorrhage after lung transplantation, as they can during any major illness or surgical recovery.  This is unusual but must be considered if blood counts drop during recovery or if flank pain develops.

The transplant team will consider using sirolimus or everolimus as an immunosuppressant after the operation (not immediately but much later after the surgery has healed up), as preliminary data suggests that use of sirolimus after transplant can reduce the likelihood of developing LAM again in the new lung(s) and it can control angiomyolipomas.  More data need to be published regarding the use of sirolimus after lung transplant before firmer recommendations can be made about its use in this context.