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: Vincent Cottin, Sergio Harari, Marc Humbert, Hervé Mal, Peter Dorfmüller, Xavier Jaïs, Martine Reynaud-Gaubert, Grégoire Prevot, Romain Lazor, Camille Taillé, Jacques Lacronique, Sabrina Zeghmar, Gérald Simonneau, Jean-François Cordier, Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P)
• Journal: The European respiratory journal (Eur Respir J) Vol. 40 Issue 3 Pg. 630-40 (Sep 2012) ISSN: 1399-3003 [Electronic] England
• PMID: 22362861 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Carbon Monoxide
• Oxygen

Topics

• Adult
• Breath Tests
• Carbon Monoxide (analysis)
• Cardiac Catheterization
• Exercise Test
• Female
• Hemodynamics
• Humans
• Hypertension, Pulmonary (mortality, physiopathology, therapy)
• Lymphangioleiomyomatosis (mortality, physiopathology, therapy)
• Middle Aged
• Oxygen (blood, therapeutic use)
• Respiratory Function Tests
• Retrospective Studies
• Vascular Resistance (physiology)