Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients.

@article{Cottin2012PulmonaryHI,
  title={Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients.},
  author={Vincent Cottin and Sergio Harari and Marc Humbert and Herv{\'e} Mal and Peter Dorfm{\"u}ller and Xavier Ja{\"i}s and Martine Reynaud-Gaubert and Gr{\'e}goire Pr{\'e}v{\^o}t and Romain Lazor and Camille Taill{\'e} and Jacques Lacronique and Sabrina Zeghmar and G{\'e}rald Simonneau and Jean-François Cordier},
  journal={The European respiratory journal},
  year={2012},
  volume={40 3},
  pages={
          630-40
        }
}
This retrospective, multicentre study evaluated patients with lymphangioleiomyomatosis (LAM) and pre-capillary pulmonary hypertension (PH) by right heart catheterisation. It was conducted in 20 females with a mean ± SD age of 49 ± 12 yrs and a mean ± SD time interval between LAM and PH diagnoses of 9.2 ± 9.8 yrs. All, except for one patient, were receiving supplemental oxygen. 6-min walking distance was mean ± SD 340 ± 84 m. Haemodynamic characteristics were: mean pulmonary artery pressure (PAP… CONTINUE READING
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