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Pulmonary Artery Pressure in Lymphangioleiomyomatosis^*

An Echocardiographic Study

Angelo M. Taveira-DaSilva, MD, PhD; Olanda M. Hathaway, CRNP; Vandana Sachdev, MD; Yukitaka Shizukuda, MD, PhD; Charles W. Birdsall, RDCS and Joel Moss, MD, PhD

^* From the Pulmonary-Critical Care Medicine Branch (Drs. Taveira-DaSilva and Moss, and Ms. Hathaway) and Cardiovascular Branch (Drs. Sachdev and Shizukuda, and Mr. Birdsall), National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD. Dr. Shizukuda is currently at the Division of Cardiovascular Diseases, University Hospital, University of Cincinnati Medical Center, Cincinnati, OH.

Correspondence to: Angelo M. Taveira-DaSilva, MD, PhD, Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Building 10, Room 6D05, MSC 1590, Bethesda, MD 20892-1590; e-mail: dasilvaa{at}nhlbi.nih.gov

Abstract

Background: Exercise-induced hypoxemia is frequent in patients with lymphangioleiomyomatosis (LAM) and could be associated with pulmonary hypertension. The aims of this study were to determine the prevalence of pulmonary hypertension in patients with LAM, to identify physiologic parameters associated with its occurrence, and to evaluate the effect of oxygen on response to exercise.

Methods: Studies were performed in 120 patients. Complete data, including exercise echocardiography, pulmonary function testing, and standard cardiopulmonary exercise testing, were obtained in 95 patients.

Results: Resting pulmonary artery pressure (PAP) was 26 ± 0.7 mm Hg (mean ± SEM). Eight patients had pulmonary hypertension (43 ± 3 mm Hg), and two patients had right ventricular dilatation. Ninety-five patients exercised (room air, n = 64; oxygen, n = 31) to a power of 58 ± 2 W (49% of predicted) and an estimated peak oxygen uptake of 938 ± 30 mL/min (56% of predicted). Sixty-one patients had a decline in arterial oxygen saturation (SaO₂) > 3%, and 56 patients had an elevation in PAP > 40 mm Hg. Peak exercise PAP was negatively correlated with exercise SaO₂ (p = 0.0005). Multivariate analysis showed that exercise SaO₂ was the best predictor of exercise PAP (p = 0.012).

Conclusions: Although resting pulmonary hypertension is rare in patients with LAM, a rise in PAP at low exercise levels occurs frequently, in part related to exercise-induced hypoxemia. Optimization of oxygen administration during activities of daily living should be undertaken in patients with LAM to prevent hypoxemia and exercise-induced pulmonary hypertension.

Key Words: exercise • hypoxemia • pulmonary function tests • pulmonary hypertension