815 Lung Transplantation for Children in Europe: A 10-Year Multi-Center Austrian-German Experience

Abstract

Development of PVS following LTx has not been described. We present four infant LTx recipients with severe PVS, all progressing to death. Methods and Materials: We reviewed patient records at our center from 1993-2011. Eighty-five patients less than 2 yr of age received bilateral LTx. Infant pulmonary function studies, chest imaging, echocardiography, bronchoscopy and biopsy were performed on a regular schedule. Results: Four (4.7%) children (all transplanted since 2008) developed diffuse PVS. Pre-LTx diagnoses included two surfactant disorders and two with primary PVS. All received immunosuppression consisting of tacrolimus, mycophenolate (MMF) and prednisone. Average age at LTx was 10 mos; mean time to PVS diagnosis was 429 d. Diagnosis of PVS was initially made by cardiac angiography and confirmed at open lung biopsy or autopsy. Despite medical and catheter-based intervention, PVS rapidly progressed to death in all four (mean time from diagnosis to death 108 d). Three subjects demonstrated intermittent donor-specific antibodies but histology and immunohistochemistry for C4D did not reflect antibodymediated rejection. No infectious agent was identified. All received treatment for both humoral and cellular rejection without improvement. No diffuse PVS has been noted post-LTx in the 308 children over 2 yrs of age transplanted at our center. Conclusions: Diffuse PVS is a previously unreported complication of infant LTx with unclear etiology. The only recent change in our protocol has been the transition of immunosuppression from cyclosporine/azathioprine to tacrolimus/MMF. PVS should be considered and increased surveillance with echocardiography encouraged in infant LTx recipients with an otherwise unexplained development of pulmonary graft dysfunction.