Abstract
Background: The prevalence of pulmonary hypertension (PH) has been estimated to 6.7% in 95 patients with lymphangioleiomyomatosis (LAM) as defined by sPAP>35 mmHg at echocardiography.
Objective: To evaluate the hemodynamic characteristics and pulmonary function in patients with LAM and PH not explained otherwise.
Methods: A retrospective multicenter study was conducted in patients with LAM with precapillary PH (mean PAP ≥25 mmHg, pulmonary artery wedge pressure <15 mmHg, and normal or decreased cardiac index at RHC).
Results: Twenty patients were studied, with a mean age of 49±12 years. The median delay between the diagnosis of LAM and PH was 6.2 years. Dyspnea was NYHA class I/II in 10%, III in 50%, and IV in 40%. A single patient had right heart failure. Six minute walk distance was 340±84 m. Hemodynamic characteristics were: mean PAP 32±6 mmHg, cardiac index 3.5±1.1 L.min.m-2, pulmonary vascular resistance (PVR) 376±184 dyn.s.cm-5, and pulmonary capillary wedge pressure 10±3 mmHg. The mean PAP was >35 mmHg in 3 cases. FVC was 76±28% of predicted, FEV1 42±25%, FEV1/FVC 47±15%, and DLco 29±13%. PaO2 was 7.4±1.3 kPa on room air. All patients were on long-term oxygen therapy. In five patients receiving bosentan, RHC after a median of 3.2 years demonstrated a median decrease of 42% in PVR. After a median follow-up of 2.9 years, one patient had died of cardiac arrest, and 5 patients had undergone lung transplantation.
Conclusion: Precapillary PH of moderate hemodynamic severity may occur in patients with LAM and severe pulmonary function impairment. Bosentan therapy might improve hemodynamic characteristics.
Support: CNMR and FP7 of the European Commission.
- © 2011 ERS