Eur Respir J. 2012 Feb 23. [Epub ahead of print]

Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients.

Cottin V, Harari S, Humbert M, Mal H, Dorfmüller P, Jais X, Reynaud-Gaubert M, Prevot G, Lazor R, Taillé C, Lacronique J, Zeghmar S, Simonneau G, Cordier JF; the Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).

Hôpital Louis Pradel, Service de pneumologie - Centre de référence national des maladies pulmonaires rares et Centre de compétences de l'hypertension artérielle pulmonaire, Lyon, France; Université de Lyon, Université Lyon I, INRA, UMR754 INRA-Vetagrosup EPHE IFR 128, Lyon, France.

Abstract

This retrospective, multicenter study evaluated patients with lymphangioleiomyomatosis (LAM) and precapillary pulmonary hypertension (PH) by right heart catheterisation.It was conducted in 20 females with a mean age of 49±12 years and a mean time interval between LAM and PH diagnoses of 9.2±9.8 years. All except 1 were receiving supplemental oxygen. Six-min walk distance was 340±84 m. Haemodynamic characteristics were: mean pulmonary arterial pressure (PAP) 32±6 mmHg, cardiac index 3.5±1.1 L.min.m(-2) and pulmonary vascular resistance (PVR) 376±184 dyn.s.cm(-5). Mean PAP was >35 mmHg in only 20% of cases. 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 on room air. Mean PAP and PVR did not correlate with Pa,O2. In 6 patients who received oral pulmonary arterial hypertension (PAH) therapy, the mean PAP decreased from 33±9 to 24±10 mmHg and PVR from 481±188 to 280±79 dyn.s.cm(-5). The overall probability of survival was 94% at 2 years.Precapillary PH of mild haemodynamic severity may occur in patients with LAM, even with mild pulmonary function impairment. PAH therapy might improve haemodynamics in PH associated with LAM.

PMID:22362861