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CLINICAL STUDY: PULMONARY HYPERTENSION

Pulmonary function in primary pulmonary hypertension

^* Division of Respiratory and Critical Care Physiology and Medicine and Division of Cardiology, Department of Medicine, Research and Education Institute, Harbor–UCLA Medical Center, Torrance, California, USA

Manuscript received May 31, 2002; revised manuscript received November 16, 2002, accepted December 4, 2002.

^* Reprint requests and correspondence: Dr. James E. Hansen, St. John’s Cardiovascular Research Center, 1124 West Carson Street, Box 405, Torrance, California 90509-2910, USA.
jimandbev{at}cox.net

OBJECTIVES: The study was done to ascertain the degree to which abnormalities in resting lung function correlate with the disease severity of patients with primary pulmonary hypertension (PPH).

BACKGROUND: Patients with PPH are often difficult to diagnose until several years after the onset of symptoms. Despite the seriousness of the disorder, the diagnosis of PPH is often delayed because it is unsuspected and requires invasive measurements. Although PPH often causes abnormalities in resting lung function, these abnormalities have not been shown to be statistically significant when correlated with other measures of PPH severity.

METHODS: Resting lung mechanics and diffusing capacity for carbon monoxide DL_CO were assessed in 79 patients whose findings conformed to the classical diagnostic criteria of PPH and who had no evidence of secondary causes of pulmonary hypertension. These findings were correlated with severity of disease as assessed by cardiac catheterization, New York Heart Association (NYHA) class, and cardiopulmonary exercise testing.

RESULTS: When PPH patients were first evaluated at our referral clinic, the DL_CO and lung volumes were decreased in approximately three-quarters and one-half, respectively. The decreases in DL_CO, and to a lesser extent lung volumes, correlated significantly with decreases in peak oxygen uptake (reflecting maximum cardiac output), peak oxygen pulse (reflecting maximum stroke volume), and anaerobic threshold (reflecting sustainable exercise capacity) and higher NYHA class.

CONCLUSIONS: Patients with PPH commonly have abnormalities in lung mechanics and DL_CO levels that correlate significantly with disease severity. These measurements can be useful in evaluating patients with unexplained dyspnea and fatigue.

Abbreviations and Acronyms   CPET   cardiopulmonary exercise test   DLCO   diffusing capacity of the lung for carbon monoxide or gas transfer index   FEV1   forced expiratory volume in 1 second   FVC   forced vital capacity   MVV   maximum voluntary ventilation   NYHA   New York Heart Association   %pred   percent predicted   PPH   primary pulmonary hypertension   TLC   total lung capacity   VA'   effective alveolar volume

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