Background: With constantly increasing life expectancy, adults with congenital heart disease (ACHD) are facing age and ACHD-related comorbidities, increasing their mortality risk. The prevalence of comorbidities among ACHD in Israel is unknown.
Method: ACHD patients (Age>18), insured by Maccabi and Clalit Health Services (2007-2011), were studied retrospectively for recorded comorbidities. Congenital disease complexity was categorized by the most severe defect (JACC. 2001; 37:1170-1175). Israeli population estimates for Standardized Morbidity Ratios (SMR) are based on the Hadera district study.
Findings: Among 15,404 ACHD patients (median age – 51, 49% males), 56.1% had a simple, 15.8% had a moderate, 5% had a complex and 23% had an unspecified defect. Comorbidity or risk-factor (dyslipidemia or hypertension) diagnoses were documented for 57.4% (95% Confidence Interval (CI): 56.2-58.6%). The most common comorbidity was cardiovascular (16.6%, 95%CI: 16.0-17.2%), including cerebrovascular disease, peripheral vascular disease or myocardial infraction, followed by pulmonary disease (13.6%, 95%CI: 13.0-14.2%). Complex patients had higher prevalence of heart failure, but lower prevalence of risk-factors (Table). Adjusted Odds Ratios (OR) for comorbidity diagnosis were significantly lower among complex compared to simple defect patients (OR=0.79, 95%CI: 0.67-0.94). Compared to simple defects, moderate (OR=1.24, 95%CI:1.04-1.49), complex (OR=1.46, 95%CI:1.12-1.90) and unspecified defects (OR=1.28, 95%CI: 1.12-1.47) were associated with increased risk of heart failure, but decreased risk of other cardiovascular comorbidities. ACHD had higher comorbidity rates compared to the reference population, especially cardiovascular (SMR=3.48, 95%CI: 3.35-3.62), hepatic (SMR=2.82, 95%CI: 2.58-3.06) and pulmonary diseases (SMR=2.18, 95%CI: 2.08-2.29). During 5.2 years median follow-up, 1360 (8.8%) patients died. Comorbidity diagnosis was associated with increased mortality risk in Cox proportional hazard model adjusted for age, sex, defect complexity, health services provider, ethnicity and district (Hazard Ratio=1.67, 95%CI: 1.48-1.89).
Conclusion: ACHD patients have higher prevalence of comorbidities compared to the general population. Comorbidities were found to increase the mortality risk among the ACHD population.