PMID- 32979348 OWN - NLM STAT- MEDLINE DCOM- 20210924 LR - 20210924 IS - 1931-3543 (Electronic) IS - 0012-3692 (Linking) VI - 159 IP - 3 DP - 2021 Mar TI - Characteristics and Long-term Outcomes of Pulmonary Venoocclusive Disease Induced by Mitomycin C. PG - 1197-1207 LID - S0012-3692(20)34738-3 [pii] LID - 10.1016/j.chest.2020.09.238 [doi] AB - 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 MMC was withdrawn for all patients, and 14 patients received specific pulmonary arterial hypertension (PAH) therapies.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. CI - Copyright (c) 2020 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. FAU - Certain, Marie-Caroline AU - Certain MC AD - Department of Respiratory and Intensive Care Medicine, National French Reference Constitutive Center for Rare Pulmonary Diseases, Hopital Francois Mitterrand, Burgundy University Hospital, Dijon, France. FAU - Chaumais, Marie-Camille AU - Chaumais MC AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France; Assistance Publique-Hopitaux de Paris (AP-HP), Department of Pharmacy, Hopital Bicetre, Le Kremlin-Bicetre, France; University of Paris-Saclay, Faculty of Pharmacy, Chatenay Malabry, France. FAU - Jais, Xavier AU - Jais X AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France; University of Paris-Saclay, School of Medicine, Le Kremlin-Bicetre, France. FAU - Savale, Laurent AU - Savale L AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France; University of Paris-Saclay, School of Medicine, Le Kremlin-Bicetre, France. FAU - Seferian, Andrei AU - Seferian A AD - AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France. FAU - Parent, Florence AU - Parent F AD - AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France. FAU - Georges, Marjolaine AU - Georges M AD - Department of Respiratory and Intensive Care Medicine, National French Reference Constitutive Center for Rare Pulmonary Diseases, Hopital Francois Mitterrand, Burgundy University Hospital, Dijon, France; University of Bourgogne Franche-Comte, School of Medicine, Dijon, France; INSERM UMR 123-1, LNC Faculty of Medicine and Pharmacy, Dijon, France. FAU - Favrolt, Nicolas AU - Favrolt N AD - Department of Respiratory and Intensive Care Medicine, National French Reference Constitutive Center for Rare Pulmonary Diseases, Hopital Francois Mitterrand, Burgundy University Hospital, Dijon, France. FAU - Bourdin, Arnaud AU - Bourdin A AD - Department of Respiratory Diseases, Hopital Arnaud de Villeneuve, Montpellier University Hospitals, Montpellier, France; University of Montpellier, School of Medicine, Montpellier, France; INSERM U1046, Montpellier, France. FAU - Boissin, Clement AU - Boissin C AD - Department of Respiratory Diseases, Hopital Arnaud de Villeneuve, Montpellier University Hospitals, Montpellier, France. FAU - Cottin, Vincent AU - Cottin V AD - Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Hopital Louis Pradel, Lyon, France; Claude-Bernard Lyon 1 University, University of Lyon, INRA, UMR754, Lyon, France; UMR 754, Lyon, France. FAU - Traclet, Julie AU - Traclet J AD - Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Hopital Louis Pradel, Lyon, France. FAU - Renard, Sebastien AU - Renard S AD - Department of Cardiology, Assistance Publique-Hopitaux de Marseille (AP-HM), Hopital de la Timone, Aix-Marseille University, Marseille, France. FAU - Noel, Violaine AU - Noel V AD - Internal Medicine Department, Hopital Robert-Debre, Reims, France. FAU - Picard, Francois AU - Picard F AD - Department of Cardiology, University Hospital of Bordeaux, Bordeaux, France. FAU - Girerd, Barbara AU - Girerd B AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France; University of Paris-Saclay, School of Medicine, Le Kremlin-Bicetre, France. FAU - Ghigna, Maria-Rosa AU - Ghigna MR AD - Department of Pathology, Hopital Marie Lannelongue, Le Plessis-Robinson, France. FAU - Perros, Frederic AU - Perros F AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France. FAU - Sitbon, Olivier AU - Sitbon O AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France; University of Paris-Saclay, School of Medicine, Le Kremlin-Bicetre, France. FAU - Bonniaud, Philippe AU - Bonniaud P AD - Department of Respiratory and Intensive Care Medicine, National French Reference Constitutive Center for Rare Pulmonary Diseases, Hopital Francois Mitterrand, Burgundy University Hospital, Dijon, France; University of Bourgogne Franche-Comte, School of Medicine, Dijon, France; INSERM UMR 123-1, LNC Faculty of Medicine and Pharmacy, Dijon, France. FAU - Humbert, Marc AU - Humbert M AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France; University of Paris-Saclay, School of Medicine, Le Kremlin-Bicetre, France. FAU - Montani, David AU - Montani D AD - INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hopital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hopital Bicetre, Le Kremlin-Bicetre, France; University of Paris-Saclay, School of Medicine, Le Kremlin-Bicetre, France. Electronic address: david.montani@aphp.fr. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20200924 PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Antibiotics, Antineoplastic) RN - 50SG953SK6 (Mitomycin) SB - IM CIN - Chest. 2021 Mar;159(3):910-911. PMID: 33678275 MH - Antibiotics, Antineoplastic/administration & dosage/adverse effects MH - Cardiac Catheterization/methods/statistics & numerical data MH - Female MH - France/epidemiology MH - Functional Status MH - Humans MH - *Hypertension, Pulmonary/diagnosis/etiology/physiopathology MH - *Lung/blood supply/diagnostic imaging MH - Male MH - Middle Aged MH - *Mitomycin/administration & dosage/adverse effects MH - Patient Care Management/methods MH - Pharmacovigilance MH - Prognosis MH - Pulmonary Circulation/drug effects MH - *Pulmonary Veno-Occlusive Disease/chemically induced/diagnosis/mortality/physiopathology MH - Pulmonary Wedge Pressure MH - Registries/statistics & numerical data MH - Survival Analysis MH - Withholding Treatment OTO - NOTNLM OT - mitomycin C OT - pharmacovigilance OT - pulmonary hypertension OT - pulmonary venoocclusive disease EDAT- 2020/09/27 06:00 MHDA- 2021/09/25 06:00 CRDT- 2020/09/26 20:08 PHST- 2020/05/19 00:00 [received] PHST- 2020/08/05 00:00 [revised] PHST- 2020/09/16 00:00 [accepted] PHST- 2020/09/27 06:00 [pubmed] PHST- 2021/09/25 06:00 [medline] PHST- 2020/09/26 20:08 [entrez] AID - S0012-3692(20)34738-3 [pii] AID - 10.1016/j.chest.2020.09.238 [doi] PST - ppublish SO - Chest. 2021 Mar;159(3):1197-1207. doi: 10.1016/j.chest.2020.09.238. Epub 2020 Sep 24.