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Multicenter Study
. 2021 Mar;159(3):1197-1207.
doi: 10.1016/j.chest.2020.09.238. Epub 2020 Sep 24.

Characteristics and Long-term Outcomes of Pulmonary Venoocclusive Disease Induced by Mitomycin C

Marie-Caroline Certain  1 , Marie-Camille Chaumais  2 , Xavier Jaïs  3 , Laurent Savale  3 , Andrei Seferian  4 , Florence Parent  4 , Marjolaine Georges  5 , Nicolas Favrolt  1 , Arnaud Bourdin  6 , Clément Boissin  7 , Vincent Cottin  8 , Julie Traclet  9 , Sébastien Renard  10 , Violaine Noel  11 , François Picard  12 , Barbara Girerd  3 , Maria-Rosa Ghigna  13 , Frédéric Perros  14 , Olivier Sitbon  3 , Philippe Bonniaud  5 , Marc Humbert  3 , David Montani  15
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Free article
Multicenter Study

Characteristics and Long-term Outcomes of Pulmonary Venoocclusive Disease Induced by Mitomycin C

Marie-Caroline Certain et al. Chest. 2021 Mar.
Free article

Abstract

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 × 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 MMC was withdrawn for all patients, and 14 patients received specific pulmonary arterial hypertension (PAH) therapies.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.

Interpretation: PVOD after MMC treatment is a rare but life-threatening complication associated with a poor prognosis despite MMC withdrawal and PAH-specific therapy.

Keywords: mitomycin C; pharmacovigilance; pulmonary hypertension; pulmonary venoocclusive disease.

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