SESSION TYPE: Cardiovascular Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Pulmonary veno-occlusive disease (PVOD) is an uncommon variant of primary pulmonary artery hypertension (PAH) and is a rare but fatal complication of hematopoeitic stem cell transplant (HSCT). It often goes unrecognized antemortem.
CASE PRESENTATION: 63-year-old man with history of Burkitt’s lymphoma s/p chemotherapy in 2003 followed by autologous HSCT in 2005. In January 2011, he was diagnosed with MDS and received chemotherapy followed by an allogeneic HSCT. He was admitted to the ICU a year later for acute hypoxic respiratory failure due to large bilateral pleural effusions. He was found to have pulmonary hypertension with PASP 76 mmHg on echo. He was treated with broad spectrum antimicrobials and bilateral chest tubes. Pleural fluid was positive for HHV-6, as was serum. He was started on Foscarnet. Cardiac MRI upon discharge from the ICU showed LVEF of 62%, RVEF of 48%, and mild to moderate RVE. Rheumatology work-up was negative. Two weeks later, he was readmitted to the ICU with recurrent respiratory failure. Echocardiogram showed persistent pulmonary hypertension confirmed by a PA catheter. Serial measurements showed improved hemodynamics with inhaled iloprost. Despite response to pulmonary vasodilator, he developed septic shock secondary to pneumonia leading to his death. Autopsy revealed PVOD.
DISCUSSION: Pulmonary complications after HSCT are predominantly infectious in etiology.PVOD in the post-HSCT phase as it is in this case, is only described in few case-reports. High dose preparative chemotherapy;namely, Busulfan-based regimens, and radiation before transplantation are thought to be the culprit behind this patient's vasculopathy. The diagnosis as challenging as it appears, should be based on high clinical suspicion, an integrated assessment incorporating high resolution computed tomography (septal lines, ground-glass opacities and lymph node enlargement), pulmonary function testing (lower DLCO), arterial blood gas analysis (lower PaO(2) at rest) and bronchoalveolar lavage (occult alveolar hemorrhage).Lung biopsy (or post-mortem examination) remains the definitive mean of diagnosis. Despite an understanding of the underlying vascular inflammation, endothelial activation leading to venular hypertrophy and obstruction, there is no definite treatment recommendation. Lung transplantation has traditionally been the treatment of choice.
CONCLUSIONS: Much is still unknown about PVOD. Although uncommon, it should be considered in the differential diagnosis of pulmonary hypertension that develops post-HSCT.
1) Mandel et al.Pulmonary Veno-occlusive disease.Am. J. Respir. Crit. Care Med. November 1, 2000 vol. 162 no. 5 1964-1973
2) Bunte et al.Pulmonary veno-occlusive disease following hematopoietic stem cell transplantation: a rare model of endothelial dysfunction.Bone Marrow Transplantation .January 28, 2008 .41 677-686.
DISCLOSURE: The following authors have nothing to disclose: Hala Moukhachen, Prabalini Rajendram, Sanjay Chawla, Nina Raoof, Kaye Hale, Louis Voigt, Stephen Pastores, Neil Halpern
No Product/Research Disclosure InformationCritical Care Medicine Service. Department of Anesthesiology and Critical Care Medicine, Memorial Sloan-Kettering Cancer Center, New York, NY