• Marie-Caroline Certain, Marie-Camille Chaumais, Xavier Jaïs, Laurent Savale, Andrei Seferian, Florence Parent, Marjolaine Georges, Nicolas Favrolt, Arnaud Bourdin, Clément Boissin, Vincent Cottin, Julie Traclet, Sébastien Renard, Violaine Noel, François Picard, Barbara Girerd, Maria-Rosa Ghigna, Frédéric Perros, Olivier Sitbon, Philippe Bonniaud, Marc Humbert, and David Montani.
• Department of Respiratory and Intensive Care Medicine, National French Reference Constitutive Center for Rare Pulmonary Diseases, Hôpital François Mitterrand, Burgundy University Hospital, Dijon, France.
• Chest. 2021 Mar 1; 159 (3): 1197-1207.

BackgroundPulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) predominantly characterized by pulmonary vein and capillary involvement. An association between chemotherapy, in particular mitomycin C (MMC), and PVOD has been reported.Research QuestionWhat are the characteristics of MMC-induced PVOD, and what is the prognosis for patients with MMC-induced PVOD?Study Design And MethodsWe report the clinical, functional, radiologic, and hemodynamic characteristics at diagnosis and outcomes of patients with PVOD from the French PH Registry after exposure to MMC. The results are expressed as the median (minimum-maximum).ResultsFrom June 2011 to December 2018, 17 incident cases of MMC-induced PVOD were identified. At diagnosis, these patients had severe clinical and functional impairment, with 12 patients having a New York Heart Association (NYHA) functional class of III or IV and a 6-min walk distance of 220 (0-465) m. Right heart catheterization confirmed severe precapillary PH with a mean pulmonary artery pressure of 38 (30-52) mm Hg, a cardiac index of 2.2 (1.5-4) L/(min × m2), and pulmonary vascular resistance of 8.3 (5.1-14.5) Wood units. The diffusing capacity of the lungs for carbon monoxide was markedly decreased at 31% (20%-51%) of the theoretical values associated with severe hypoxemia. MMC was withdrawn for all patients, and 14 patients received specific pulmonary arterial hypertension (PAH) therapies. Among these patients, mild but statistically insignificant improvements were observed in NYHA functional class (P = .10), 6-min walk distance (P = .09), and pulmonary vascular resistance (-4.7 Wood units; P = .052) at reassessment (median delay of 4.8 months). Three patients experienced pulmonary edema requiring the cessation or reduction of PAH treatment. The median overall survival was 20 months, and the 6-, 12-, and 24-month survival rates were 76%, 58%, and 18%, respectively.InterpretationPVOD after MMC treatment is a rare but life-threatening complication associated with a poor prognosis despite MMC withdrawal and PAH-specific therapy.Copyright © 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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