title: Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients.
abstract: 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.
authors.affiliation: Hospices Civils de Lyon, 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.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Sergio
authors.initials: S
authors.lastname: Harari
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Hervé
authors.initials: H
authors.lastname: Mal
authors.forename: Peter
authors.initials: P
authors.lastname: Dorfmüller
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.forename: Martine
authors.initials: M
authors.lastname: Reynaud-Gaubert
authors.forename: Grégoire
authors.initials: G
authors.lastname: Prevot
authors.forename: Romain
authors.initials: R
authors.lastname: Lazor
authors.forename: Camille
authors.initials: C
authors.lastname: Taillé
authors.forename: Jacques
authors.initials: J
authors.lastname: Lacronique
authors.forename: Sabrina
authors.initials: S
authors.lastname: Zeghmar
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
mesh_terms: D000328:Adult; D001944:Breath Tests; D002248:Carbon Monoxide / Q000032:analysis; D006328:Cardiac Catheterization; D005080:Exercise Test; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000503:physiopathology* / Q000628:therapy; D018192:Lymphangioleiomyomatosis / Q000401:mortality / Q000503:physiopathology* / Q000628:therapy; D008875:Middle Aged; D010100:Oxygen / Q000097:blood / Q000627:therapeutic use; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D014655:Vascular Resistance / Q000502:physiology
chemical_list: D002248:Carbon Monoxide; D010100:Oxygen