Characteristics and long-term outcomes of pulmonary veno-occlusive disease induced by Mitomycin-C
2021
Certain, Marie-Caroline | Chaumais, Marie-Camille | Jaïs, Xavier | Savale, Laurent | Seferian, Andrei | Parent, Florence | Georges, Marjolaine | Favrolt, Nicolas | Bourdin, Arnaud | Boissin, Clément | Cottin, Vincent | Traclet, Julie | Renard, Sébastien | Noel, Violaine | Picard, François | Girerd, Barbara | Marie-Rosa, Ghigna | Perros, Frédéric | Sitbon, Olivier | Bonniaud, Philippe | Humbert, Marc | Montani, David | Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon] ; Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon) | CHU Dijon ; Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon) | Centre de référence maladies rares des maladies pulmonaires rares de l’adulte (CHU Dijon) (CRMR des maladies pulmonaires rares de l’adulte) ; Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon) | Hypertension pulmonaire : physiopathologie et innovation thérapeutique (HPPIT) ; Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay | Centre Chirurgical Marie Lannelongue (CCML) | Hôpital Bicêtre [AP-HP, Le Kremlin-Bicêtre] ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP) | Université Paris-Saclay | Université Bourgogne Franche-Comté [COMUE] (UBFC) | Lipides - Nutrition - Cancer [Dijon - U1231] (LNC) ; Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement | Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp) ; Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS) | Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier) | Infections Virales et Pathologie Comparée - UMR 754 (IVPC) ; École Pratique des Hautes Études (EPHE) ; Université Paris Sciences et Lettres (PSL)-Université Paris Sciences et Lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL) ; Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE) | Hôpital Louis Pradel [CHU - HCL] ; Hospices Civils de Lyon (HCL) | Hôpital de la Timone [CHU - APHM] (TIMONE) | Hôpital Robert Debré ; Hôpital Robert Debré ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital universitaire Robert Debré [Reims] (CHU Reims) | Centre Hospitalier Universitaire de Bordeaux (CHU Bordeaux)
International audience
Afficher plus [+] Moins [-]anglais. BACKGROUND: Pulmonary 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 QUESTION: What are the characteristics of MMC-induced PVOD, and what is the prognosis for patients with MMC-induced PVOD?STUDY DESIGN AND METHODS: We 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).RESULTS: From 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 x m(2)), 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.INTERPRETATION: PVOD after MMC treatment is a rare but life-threatening complication associated with a poor prognosis despite MMC withdrawal and PAH-specific therapy.
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