RT Journal Article
SR Electronic
T1 Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 630
OP 640
DO 10.1183/09031936.00093111
VO 40
IS 3
A1 Vincent Cottin
A1 Sergio Harari
A1 Marc Humbert
A1 Hervé Mal
A1 Peter Dorfmüller
A1 Xavier Jaïs
A1 Martine Reynaud-Gaubert
A1 Grégoire Prevot
A1 Romain Lazor
A1 Camille Taillé
A1 Jacques Lacronique
A1 Sabrina Zeghmar
A1 Gérald Simonneau
A1 Jean-François Cordier
A1 the Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM“O”P)
YR 2012
UL http://erj.ersjournals.com/content/40/3/630.abstract
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.