Construct and Predictive Validity of the Kansas City Cardiomyopathy Questionnaire in Adult Congenital Heart Disease

Figures

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  Figure 1. KCCQ Scores Stratified by Anatomic Complexity Groups Median KCCQ scores were compared across anatomic complexity groups. Compared to simple/moderate complexity (89.0), KCCQ scores were significantly lower for patients with great anatomic complexity (76.6). KCCQ = Kansas City Cardiomyopathy Questionnaire.

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  Figure 2. KCCQ Scores Stratified by ACHD Physiological Stages Median KCCQ scores were compared across ACHD physiological stages. Scores progressively declined with worsening of physiological stage: 98.0 in stage A, 90.7 in stage B, 81.9 in stage C, and 72.6 in stage D. ACHD = adult congenital heart disease; other abbreviation as in Figure 1.

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  Figure 3. Freedom From Death or Hospitalization Stratified by KCCQ Score Groups Kaplan-Meier curves showing freedom from the outcome of death or all-cause hospitalization. Compared to patients scoring 76 to 100, those scoring ≤50 had significantly worse event-free survival. Abbreviation as in Figure 1.

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  Figure 4. Freedom From Death or Procedures Stratified by KCCQ Score Groups Kaplan-Meier curves showing freedom from the outcome of death or interventional cardiac procedures including cardiac surgery, cardiac catheterization intervention, electrophysiological ablation, or placement of a permanent pacemaker. Patients scoring ≤50 had significantly worse event free survival compared to patients who scored 76 to 100. Abbreviation as in Figure 1.

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  Central Illustration. Associations of KCCQ in ACHD Demonstrate Construct and Predictive Validity In a single-center, cross-sectional study of ambulatory ACHD patients (129 KCCQs total, median age 29.0 years, 41.3% female), lower KCCQ scores were associated with great anatomic complexity, advanced ACHD physiological stage, physician-reported HF, and advanced NYHA functional class thereby demonstrating construct validity. To demonstrate predictive validity, a prospective cohort study of patients with at least 6 months follow-up was performed (N = 92, median follow-up 16.3 months). ACHD patients who ultimately died, were hospitalized, or underwent an interventional cardiac procedure had significantly lower KCCQ scores, demonstrating predictive validity. These findings provide an opportunity to utilize the KCCQ to assess health status in ACHD both clinically and in future scholarly work. Abbreviations as in Figures 1 and 2.