Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients.

Détails

ID Serval
serval:BIB_829C5CEF72F1
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients.
Périodique
European Respiratory Journal
Auteur(s)
Cottin V., Harari S., Humbert M., Mal H., Dorfmüller P., Jaïs X., Reynaud-Gaubert M., Prevot G., Lazor R., Taillé C., Lacronique J., Zeghmar S., Simonneau G., Cordier J.F.
Collaborateur(s)
Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P)
Contributeur(s)
Crestani B., Günther S., Hirschi S., Khouatra C.
ISSN
1399-3003 (Electronic)
ISSN-L
0903-1936
Statut éditorial
Publié
Date de publication
2012
Volume
40
Numéro
3
Pages
630-640
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Résumé
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.
Mots-clé
Adult, Breath Tests, Carbon Monoxide/analysis, Cardiac Catheterization, Exercise Test, Female, Hemodynamics, Humans, Hypertension, Pulmonary/mortality, Hypertension, Pulmonary/physiopathology, Lymphangioleiomyomatosis/mortality, Lymphangioleiomyomatosis/physiopathology, Middle Aged, Oxygen/blood, Oxygen/therapeutic use, Respiratory Function Tests, Retrospective Studies, Vascular Resistance/physiology
Pubmed
Web of science
Open Access
Oui
Création de la notice
01/11/2012 19:41
Dernière modification de la notice
08/05/2019 21:13
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