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-2and 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. Copyright
| Original language | English |
|---|---|
| Pages (from-to) | 630-640 |
| Number of pages | 11 |
| Journal | European Respiratory Journal |
| Volume | 40 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Sep 1 2012 |
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Keywords
- Interstitial lung disease
- Lymphangioleiomyomatosis
- Pulmonary hypertension
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
Cite this
Pulmonary hypertension in lymphangioleiomyomatosis : Characteristics in 20 patients. / Cottin, Vincent; Harari, Sergio; Humbert, Marc; Mal, Hervé; Dorfmüller, Peter; Jaïs, Xavier; Reynaud-Gaubert, Martine; Prevot, Grégoire; Lazor, Romain; Taillé, Camille; Lacronique, Jacques; Zeghmar, Sabrina; Simonneau, Gérald; Cordier, Jean François.
In: European Respiratory Journal, Vol. 40, No. 3, 01.09.2012, p. 630-640.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Pulmonary hypertension in lymphangioleiomyomatosis
T2 - Characteristics in 20 patients
AU - Cottin, Vincent
AU - Harari, Sergio
AU - Humbert, Marc
AU - Mal, Hervé
AU - Dorfmüller, Peter
AU - Jaïs, Xavier
AU - Reynaud-Gaubert, Martine
AU - Prevot, Grégoire
AU - Lazor, Romain
AU - Taillé, Camille
AU - Lacronique, Jacques
AU - Zeghmar, Sabrina
AU - Simonneau, Gérald
AU - Cordier, Jean François
PY - 2012/9/1
Y1 - 2012/9/1
N2 - 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-2and 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. Copyright
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-2and 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. Copyright
KW - Interstitial lung disease
KW - Lymphangioleiomyomatosis
KW - Pulmonary hypertension
UR - http://www.scopus.com/inward/record.url?scp=84865594877&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84865594877&partnerID=8YFLogxK
U2 - 10.1183/09031936.00093111
DO - 10.1183/09031936.00093111
M3 - Article
VL - 40
SP - 630
EP - 640
JO - Scandinavian Journal of Respiratory Diseases
JF - Scandinavian Journal of Respiratory Diseases
SN - 0903-1936
IS - 3
ER -