Patient Reported Outcome Measures in Adults with Fontan Circulatory Failure

Area of Science:

• Cardiology
• Adult Congenital Heart Disease (ACHD)
• Patient-Reported Outcomes (PROs)

Background:

• Patient-reported outcomes (PROs) are crucial for assessing acquired heart disease but lack definition in Adult Congenital Heart Disease (ACHD).
• Fontan circulatory failure (FCF) is a significant concern in ACHD, necessitating validated assessment tools.

Purpose of the Study:

• To evaluate the discriminatory capacity of various PRO survey tools in identifying Fontan circulatory failure (FCF).
• To determine if combining PRO tools improves the detection of FCF in ACHD patients.

Main Methods:

• Consecutive adult patients with Fontan circulation or simple shunt lesions completed a comprehensive package of PRO measures.
• PRO tools included the Kansas City Cardiomyopathy Questionnaire (KCCQ-12), EuroQol-5-dimension (EQ5D), Short Form Health Status Survey (SF-12), self-reported NYHA Functional Class, and Specific Activity Scale (SAS).
• Statistical analysis compared PRO scores between Fontan and shunt lesion patients, and between FCF and non-FCF subgroups.

Main Results:

• Fontan patients reported lower KCCQ-12 scores compared to simple shunt lesion patients (p < 0.001).
• Patients with FCF exhibited significantly lower KCCQ-12 scores than those without FCF (p = 0.002).
• The KCCQ-12 demonstrated good discriminatory capacity for FCF (c-statistic 0.75), which improved to 0.82 when combined with other PRO tools.

Conclusions:

• The KCCQ-12 questionnaire is a valuable tool for identifying FCF in ACHD.
• Combining KCCQ-12 with complementary PRO tools enhances the detection of FCF.
• Further research is needed to establish the role of PROs in guiding ACHD management strategies.