Objectives. To study the incidence, pathogenesis and symptoms preceding sudden cardiovascular death amongst 15-35-year olds without substance abuse in Sweden during 1992-99. Design. This was a register study of a national database of forensic medicine, Rattsbase. Clinical details were obtained from forensic, police and medical records and from interviews with family members. Setting. The whole nation of Sweden. Subjects. Individuals having suffered a sudden cardiac death. Results. We found 181 cases of sudden cardiovascular death in a nationwide database, Rattsbase, in 15-35-year olds, of which 132 (73%) were male and 49 (27%) were female, and a rather stable incidence of 0.93 per 100 000 per year. Preceding symptoms were seen in half of the cases. The most common forensic diagnoses were: no structural abnormality (21.0%), coronary atherosclerosis (17.7%), dilated cardiomyopathy (12.2%), hypertrophic cardiomyopathy (10.5%) and myocarditis (10.5%). Conclusion. Sudden cardiovascular death was uncommon in the young, but the incidence was not decreasing. Postmortem diagnoses were often difficult to establish. There was a high frequency of structurally normal hearts. Because premortal cardiac-related symptoms are relatively common and treatment methods are developing, we should learn to recognize early symptoms of heart disease. To identify individuals at risk, further studies of preceding symptoms, life-style factors and electrocardiogram (ECG) changes are needed.

GI symptoms are common in patients with MD, may be the initial symptoms, and are often considered to be the most disabling consequence of the disease.

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Abstract. Mörner S, Lindqvist P, Mellberg C, Olofsson B-O,

This study shows that the heart is often affected in young patients with congenital myotonic dystrophy. The specialised conduction system is often affected and so too is the myocardium, causing impaired systolic function.