Abstract
This retrospective, multicenter study evaluated patients with lymphangioleiomyomatosis (LAM) and precapillary pulmonary hypertension (PH) by right heart catheterisation.
It was conducted in 20 females with a mean age of 49±12 years and a mean time interval between LAM and PH diagnoses of 9.2±9.8 years. All except 1 were receiving supplemental oxygen. Six-min walk distance was 340±84 m. Haemodynamic characteristics were: mean pulmonary arterial pressure (PAP) 32±6 mmHg, cardiac index 3.5±1.1 L.min.m−2 and pulmonary vascular resistance (PVR) 376±184 dyn.s.cm−5. Mean PAP was >35 mmHg in only 20% of cases. 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 on room air. Mean PAP and PVR did not correlate with Pa,O2. In 6 patients who received oral pulmonary arterial hypertension (PAH) therapy, the mean PAP decreased from 33±9 to 24±10 mmHg and PVR from 481±188 to 280±79 dyn.s.cm−5. The overall probability of survival was 94% at 2 years.
Precapillary PH of mild haemodynamic severity may occur in patients with LAM, even with mild pulmonary function impairment. PAH therapy might improve haemodynamics in PH associated with LAM.
- ERS