Conference: 2010 International PHA Conference and Scientific Sessions
Release Date: 06.24.2010
Presentation Type: Abstracts
Le R, Fenstad E, Maradit-Kremers H, McCully R, Frantz R, McGoon M, Kane G.
Mayo Clinic, Rochester, MN, USA
BACKGROUND: Pulmonary arterial hypertension (PAH) is a severe disease presenting most often with significant shortness of breath secondary to pulmonary vascular remodeling and associated right ventricular dysfunction. The majority of PAH patients present with multiple comorbid illnesses at the time of diagnosis. PAH has a very high mortality, often less than a decade.
METHODS: Single center cohort study of 489 patients with WHO group 1 PAH first seen between 1/1/1995 and 12/31/2005. All patients completed a standardized assessment of functional status at the time of diagnosis including questions regarding difficulty performing activities of dressing, bathing, etc.; data was compiled retrospectively to ascertain PAH severity at the time of diagnosis.
RESULTS: The mean age of patients included was 53 years (SD=15); 44.4% (364/489) were female; 49% (238/489) had idiopathic PAH. The majority, 55.6% (272/489), presented with WHO functional class III. On initial six-minute walk (6MW) an average distance of 330.3 meters (SD=124.5) was achieved. By echocardiography the average right ventricular systolic pressure was estimated at 85.3 mmHg (SD=24.8). Right atrial pressure by cardiac catheterizations averaged 11.7 mmHg (SD=5.9) whereas mean PA pressure averaged 51.7 mmHg (SD=12.8). Questions addressing the patient’s ability to perform basic activities including dressing [HR 2.47 (95% CI 1.65– 3.57)], bathing [1.70 (1.06– 2.60)], housekeeping [1.45 (1.08-1.94)], using the toilet [2.54 (1.20–4.68)], and eating [2.10 (1.04–3.77)] when adjusted for age and gender were significantly associated with increased 5-year mortality. Functional assessment data tended to be associated with lower 6MW distances and a higher WHO functional class but with similar PA pressures. Functional assessments provided independent prognostic information in multivariate analysis as exemplified by difficulty walking [1.86 (1.03 – 3.2)] modeled with WHO functional class [1.13 (0.9–1.48)] and 6MW distance [0.994 (0.992–0.996)].
CONCLUSIONS: Patient perceived functional status by standardized assessment provided incremental prognostic benefit in multivariate models greater than WHO functional class and 6MW distance despite similar PA pressures. Assessment of a patient’s ability to perform activities of daily living should be included in the initial evaluation of patients presenting with PAH to more accurately assess their disease severity and the prognostic implication.