Abstract: SA-PO961

Kidney Transplantation in a Patient with Severe Pulmonary Hypertension on Macitentan Therapy

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Kommana, Sandhya L, Harbor ULCA MEDICAL CENTER, Harbor City, California, United States
  • Lum, Erik Lawrence, UCLA Ronald Reagan Medical Center, Westwood, California, United States
Background

Pulmonary Hypertension (PH) is associated with a significant reduction in patient survival and graft function following kidney transplantation. Patients with severe pulmonary hypertension, defined as a 6 minute walk test < 300 meters or ventricular systolic pressure (RVSP) > 55 mmHg, are at increased risk for peri-operative complications and is considered a contraindication to kidney transplantation. Here we report a case with severe PH on maintenance Macitentan (Endothelin receptor antagonist) therapy who underwent successful kidney transplantation.

Methods

A 65-year-old male with ESRD secondary to autoimmune vasculitis was evaluated for kidney transplantation. His past medical history was notable for dermatomyositis, interstitial lung disease, pulmonary hypertension, and coronary artery disease.

Two years prior to evaluation he was noted to have increasing dyspnea. A CT scan of the chest revealed pulmonary fibrosis. Pulmonary function tests revealed restrictive lung disease with FEV1 75% predicted, FEV1/FVC 57% and significant DLCO impairment of 28%. An Echocardiogram showed RVSP of 70 mmHg and was started on nocturnal oxygen therapy. His symptoms continued to worsen and was started on Macitentan, with improvement in his pulmonary pressures (32 mmHg) and nocturnal oxygen was discontinued.

One year prior to transplantation he developed worsening dyspnea as a result of pulmonary edema in association with progression of his renal disease and was initiated on hemodialysis. His symptoms improved and he was cleared for kidney transplantation.

The patient underwent living unrelated kidney transplantation without complications. His Macitentan was continued during the perioperative period. We used caution not to use Diltiazem or Fluconazole postoperatively due to drug interaction with Macitentan. He is currently 3 months post kidney transplantation and has good allograft function with S Cr of 1.2 mg/dl.

Conclusion

The prevalence of pulmonary hypertension in ESRD patients has been estimated between 9-15% and when severe may preclude patients from getting kidney transplantation. However, the recent development of effective pharmaceutical therapies for PH may improve these outcomes. To our knowledge, this is the first reported case where kidney transplantation was performed in a patient with pulmonary hypertension on drug therapy.