Pulmonary hypertension in lymphangioleiomyomatosis: Characteristics in 20 patients

Vincent Cottin, Sergio Harari, Marc Humbert, Hervé Mal, Peter Dorfmüller, Xavier Jaïs, Martine Reynaud-Gaubert, Grégoire Prevot, Romain Lazor, Camille Taillé, Jacques Lacronique, Sabrina Zeghmar, Gérald Simonneau, Jean François Cordier

Research output: Contribution to journalArticlepeer-review


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) 32±6 mmHg, cardiac index 3.5±1.1 L·min-1·m-2and pulmonary vascular resistance (PVR) 376±184 dyn·s·cm-5. Mean PAP was >35 mmHg in only 20% of cases. The forced expiratory volume in 1 s was 42±25%, carbon monoxide transfer factor was 29±13%, and arterial oxygen tension (Pa,O2) was 7.4±1.3 kPa in room air. Mean PAP and PVR did not correlate with Pa,O2. In six patients who received oral pulmonary arterial hypertension (PAH) therapy, the PAP decreased from 33±9 mmHg to 24±10 mmHg and the PVR decreased from 481±188 dyn·s·cm-5 to 280±79 dyn·s·cm- 5. The overall probability of survival was 94% at 2 yrs. Pre-capillary PH of mild haemodynamic severity may occur in patients with LAM, even with mild pulmonary function impairment. PAH therapy might improve the haemodynamics in PH associated with LAM. Copyright

Original languageEnglish
Pages (from-to)630-640
Number of pages11
JournalEuropean Respiratory Journal
Issue number3
Publication statusPublished - Sep 1 2012


  • Interstitial lung disease
  • Lymphangioleiomyomatosis
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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