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Intra- and Inter-grader Agreement for World Health Organization Functional Class Assignment Based on a Novel Questionnaire in Patients with Pulmonary Arterial Hypertension

M. Murugappan

Darren Taichman

Timothy Williamson

Rael Sundy

B. Byers

Maribeth Duncan

J. Zheng

Murali Chakinala


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Conference: 2008 International PHA Conference and Scientific Sessions

Release Date: 06.20.2008

Presentation Type: Abstracts

Murugappan M. 1, Taichman D. 2, Williamson T. 3, Sundy R. 1, Byers B. 1, Duncan M. 1, Zheng J. 1, Chakinala M. 1

1. Washington University, St. Louis, MO, USA
2. University of Pennsylvania, Philadelphia, PA, USA
3. Kansas University Medical Center, Kansas City, KS, USA

BACKGROUNDThe World Health Organization (WHO) functional classification (FC) scheme is an important assessment in pulmonary arterial hypertension (PAH). Given its subjectivity and variability across graders, a role exists for an accurate, reliable instrument to aid in FC assignment.

METHODS: Enrolled patients at Washington University completed an unencouraged 6 minute walk (6MW) with Borg dyspnea score (BDS) and a novel questionnaire that gauged their daily activity tolerance.  Interviewers assigned FC based on routine clinic evaluation while blinded to the questionnaire & 6MW distance (6MWD).  One interviewer (MC) as well as two outside pulmonary hypertension specialists (DT and TW) assigned FC based on completed questionnaires, unaware of patient identity & 6MWT results.   Intra-grader and inter-grader agreements for FC assignment (I/II vs. III vs. IV) solely based on questionnaires were assessed by weighted Kappa (K).  Agreement between FC assignment by interviewer and by questionnaire was also determined in a subset of patients.  Relationship between questionnaire-assigned FC assignment and 6MWD was assessed by analysis of variance (ANOVA). 

RESULTS53 patients were enrolled: 39 females (74%), mean age 49 (±13) yrs, diagnoses (36% Idiopathic PAH, 23% collagen vascular disease, 41% other). Based on interviews, 49% were FC I/II, 38% FC III, and 13% FC IV. 6MWD and BDS significantly differed among these groups [mean 6MWD (ft): 1441 (±237), 1035 (±317), 528 (±155), respectively (p<0.001); mean BDS: 2.7 (±2.2), 5.0 (±2.6), 7.0 (±2.9), respectively (p<0.001)]. Intra-grader agreement for FC, based on questionnaires, among individual physicians was substantial to almost perfect (K =0.75-0.86).  Overall inter-grader agreement for FC assignment based on questionnaires was moderate (= 0.53), while pair-wise agreements between clinicians’ interpretations were moderate to substantial (K = 0.48-0.71). Agreement in principal clinician’s FC assignment between interview and interpretation of questionnaires was moderate (K = 0.58).  For each physician, there was a significant relationship between 6MWD and questionnaire-based FC assignments (p<0.001). 

CONCLUSION: This pilot study revealed moderate to substantial intra-grader and inter-grader agreement for FC assignment in patients with PAH, based on a novel questionnaire.  Inferences of FC assignments based on questionnaires were significantly related to 6MWD, a well-established parameter of functional outcome in PAH.  Future studies to further refine and validate the questionnaire are warranted.