Pulmonary hypertension in lymphangioleiomyomatosis: Characteristics in 20 patients

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.