International journal of cardiology · 2025
Geographic variations in the prevalence of inherited cardiac diseases and in the incidence of related sudden death.
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Ramos López Agustín, Sabater Molina María, Gil Ortuño Cristina, Olmo Conesa María Del Carmen, Muñoz Esparza Carmen, Ramos López Noemí, Nicolás Rocamora María Elisa, López Cuenca David, Hernández Del Rincón Juan Pedro, Gimeno Blanes Juan Ramón
Аннотация
BACKGROUND: Inherited cardiac diseases have an estimated prevalence (Pe) of 1:400 individuals. Global and specific condition prevalence are based on a few publications including small cohorts of cases. The aim was to calculate their relative prevalence and the incidence of related sudden death (SD) in a Spanish autonomous community.
METHODS: 2889 patients (51.2 ± 19.1 years old, 64 % men) with diagnosis of inherited cardiomyopathy or channelopathy evaluated between 2003 and 19 were included. The observed prevalence (Po) and prevalence by health care area (HCA) were calculated and compared to the estimated prevalences (Po/Pe ratio). Besides, 571 cases of related SD (44.6 ± 18.4, 78 % men) were included, and their global (and per HCA) annual incidence were calculated.
RESULTS: The Po of hypertrophic cardiomyopathy (HCM) was 1.1 ‰ (95 %CI:1.10-1.11), dilated cardiomyopathy (DCM) 0.45 ‰ (95 %CI:0.44-0.45), and arrhythmogenic cardiomyopathy (ACM) 0.13 ‰ (95 %CI:0.12-0.13). The Po of Brugada syndrome (BS) was 0.22 ‰ (95 %CI:0.2241-0.2248), and long QT syndrome (LQTS) was 0.06 ‰ (95 %CI:0.062-0.063). Significant differences were observed in the distribution of the across HCA. The Po was lower than expected for all inherited diseases except for DCM (Po/Pe:1.25). The Po/Pe for HCM and BS were 0.54 and 0.47. Those of ACM and LQTS were strikingly low (0.17 and 0.15). The overall annual rate of SD related to inherited cardiac diseases was 2.28 per 100,000 (95 %CI:2.10-2.48), showing significant variations by HCA.
CONCLUSIONS: The Po of DCM in our region is higher than the Pe from literature, while the Po of ACM and LQTS appears to be clearly lower than reported. There is significant variability in the Po of inherited cardiac disease and the annual rate of SD across HCA.
METHODS: 2889 patients (51.2 ± 19.1 years old, 64 % men) with diagnosis of inherited cardiomyopathy or channelopathy evaluated between 2003 and 19 were included. The observed prevalence (Po) and prevalence by health care area (HCA) were calculated and compared to the estimated prevalences (Po/Pe ratio). Besides, 571 cases of related SD (44.6 ± 18.4, 78 % men) were included, and their global (and per HCA) annual incidence were calculated.
RESULTS: The Po of hypertrophic cardiomyopathy (HCM) was 1.1 ‰ (95 %CI:1.10-1.11), dilated cardiomyopathy (DCM) 0.45 ‰ (95 %CI:0.44-0.45), and arrhythmogenic cardiomyopathy (ACM) 0.13 ‰ (95 %CI:0.12-0.13). The Po of Brugada syndrome (BS) was 0.22 ‰ (95 %CI:0.2241-0.2248), and long QT syndrome (LQTS) was 0.06 ‰ (95 %CI:0.062-0.063). Significant differences were observed in the distribution of the across HCA. The Po was lower than expected for all inherited diseases except for DCM (Po/Pe:1.25). The Po/Pe for HCM and BS were 0.54 and 0.47. Those of ACM and LQTS were strikingly low (0.17 and 0.15). The overall annual rate of SD related to inherited cardiac diseases was 2.28 per 100,000 (95 %CI:2.10-2.48), showing significant variations by HCA.
CONCLUSIONS: The Po of DCM in our region is higher than the Pe from literature, while the Po of ACM and LQTS appears to be clearly lower than reported. There is significant variability in the Po of inherited cardiac disease and the annual rate of SD across HCA.