Autopsy results and factors associated with sudden cardiac death in young individuals with congenital heart disease – a nationwide study

Sudden cardiac death (SCD) is a leading cause of mortality among individuals with congenital heart disease (CHD), and risk stratification remains challenging. This study aimed to describe the underlying structural cardiac abnormalities in a national cohort of SCD victims with CHD, their socioeconomic status, and interactions with the healthcare system before death. The Swedish study of Sudden Cardiac Death in the Young, 2000–2010, included SCD victims under 36 years, along with population-based controls and their parents. Of 903 SCD victims, 39 with autopsy-defined CHD were included in this study, together with 195 controls. Information on socioeconomic variables and healthcare contacts was gathered from Swedish national registers. The median age for SCD was 24 years, and 64% were male. The CHD was undiagnosed before death in 31% of the cases, of whom 8 had coronary anomalies. Moderate to complex CHD was observed in 41%. Structural abnormalities of the ventricles were prevalent, with left ventricular hypertrophy present in 56% and fibrosis in 64%. The cases had a higher frequency of hospital admissions within 6 months before SCD compared to controls (OR 14.1,95% CI 3.80–52.44), <i>p</i> &lt; 0.001. No socioeconomic differences were observed. This study identified a broad spectrum of underlying anatomical defects, with ventricular structural abnormalities being a common autopsy finding. The majority of cases had moderate to severe lesions. An increased frequency of healthcare contacts prior to death was noted, which may be a variable needing more attention as a predictor for a higher risk of SCD.