PT  - JOURNAL ARTICLE
AU  - Cottin, Vincent
AU  - Harari, Sergio
AU  - Humbert, Marc
AU  - Mal, Hervé
AU  - Dorfmüller, Peter
AU  - Jaïs, Xavier
AU  - Reynaud-Gaubert, Martine
AU  - Prevot, Grégoire
AU  - Lazor, Romain
AU  - Taillé, Camille
AU  - Lacronique, Jacques
AU  - Zeghmar, Sabrina
AU  - Simonneau, Gérald
AU  - Cordier, Jean-François
AU  - , 
TI  - Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients
AID  - 10.1183/09031936.00093111
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - 630--640
VI  - 40
IP  - 3
4099  - http://erj.ersjournals.com/content/40/3/630.short
4100  - http://erj.ersjournals.com/content/40/3/630.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - 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−2 and 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.