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. 2016 Mar;9(2):161-70.
doi: 10.1161/CIRCOUTCOMES.115.002154. Epub 2016 Mar 8.

Adult Congenital Heart Disease Patients Experience Similar Symptoms of Disease Activity

Ari M Cedars  1 , Ada Stefanescu Schmidt  2 , Craig Broberg  2 , Ali Zaidi  2 , Alexander Opotowsky  2 , Jasmine Grewal  2 , Joseph Kay  2 , Ami B Bhatt  2 , Eric Novak  2 , John Spertus  2 ; Alliance for Adult Research in Congenital Cardiology (AARCC)
Affiliations

Adult Congenital Heart Disease Patients Experience Similar Symptoms of Disease Activity

Ari M Cedars et al. Circ Cardiovasc Qual Outcomes. 2016 Mar.

Abstract

Background: There is a lack of objective data on the symptoms characterizing disease activity among adults with congenital heart disease (ACHD). The purpose of this study was to elicit the most important symptoms from patients across the spectrum of ACHD and to examine whether reported symptoms were similar across the spectrum of ACHD as a foundation for creating a patient-reported outcome measure(s).

Methods and results: We constructed a 39-item survey using input from physicians specializing in ACHD to assess the symptoms patients associate with disease activity. Patients (n=124) prospectively completed this survey, and the results were analyzed based on underlying anatomy and disease complexity. A confirmatory cohort of patients (n=40) was then recruited prospectively to confirm the validity of the initial data. When grouped based on underlying anatomy, significant differences in disease-related symptom rankings were found for only 6 of 39 symptoms. Six symptoms were identified which were of particular significance to patients, regardless of underlying anatomy. Patients with anatomy of great complexity experienced greater overall symptom severity than those with anatomy of low or moderate complexity, attributable exclusively to higher ranking of 5 symptoms. The second patient cohort had symptom experiences similar to those of the initial cohort, differing in only 5 of 39 symptoms.

Conclusions: This study identified 6 symptoms relevant to patients across the spectrum of ACHD and remarkable homogeneity of patient experience, suggesting that a single disease-specific patient-reported outcome can be created for quality and outcome assessments.

Keywords: adult congenital heart disease; disease-specific symptoms; outcome assessment; patient-reported outcome; quality of life.

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Figures

Figure 1
Figure 1
Symptom survey developed by adult congenital heart disease specialists which was provided to patients.
Figure 1
Figure 1
Symptom survey developed by adult congenital heart disease specialists which was provided to patients.
Figure 2
Figure 2
Graphical representation of the standardized multiplied impact and frequency scores from the test (blue bars) and confirmatory (red bars) cohorts. Standardization was performed by averaging the multiplied impact and frequency score for the total questionnaire and subtracting it from the multiplied impact and frequency score for each symptom. Statistically significant differences are indicated by asterisks. Numbers correspond with the symptoms in the same order in which they are found in Table 6.
See this image and copyright information in PMC

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