Characteristics and long-term outcomes of pulmonary veno-occlusive disease induced by Mitomycin-C.

We reported 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, with12 patients having a New York Heart Association Functional Class (NYHA FC) of III or IV and a 6-minute walk distance (6MWD) of 220 (0-465) meters. Right heart catheterization confirmed severe precapillary PH with a mean pulmonary artery pressure of 38 (30-52) mmHg, cardiac index of 2.2 (1.5-4) L.min-1.m-2 and pulmonary vascular resistance (PVR) of 8.3 (5.1-14.5) Wood units. The diffusing capacity 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 MMC was withdrawn for all patients, and 15 patients received specific pulmonary arterial hypertension (PAH) therapies. Among these patients, mild but statistically insignificant improvements were observed in NYHA FC (p=0.10), 6MWD (p=0.09) and PVR (-4.7 WU, p=0.052) 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:...
Source: Chest - Category: Respiratory Medicine Authors: Tags: Chest Source Type: research