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Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients

2012; European Respiratory Society; Volume: 40; Issue: 3 Linguagem: Inglês

10.1183/09031936.00093111

1399-3003

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

Eosinophilic Disorders and Syndromes

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 ( P a,O 2 ) was 7.4±1.3 kPa in room air. Mean PAP and PVR did not correlate with P a,O 2 . 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.