Mortality in patients with ventricular septal defect in Sweden: a national register study.

OBJECTIVES

Ventricular septal defect (VSD) is the most common congenital heart defect. Much remains unclear about the long-term prognosis for VSD patients. This study aimed to investigate the all-cause and cardiovascular mortality in patients with VSD compared with controls without congenital heart disease in Sweden.

METHODS

Swedish National Patient Register was used to identify all VSD patients born between 1970 and 2017. Mortality data were collected from the Swedish National Cause of Death register. Patients with associated complex congenital heart disease or non-congenital VSD were excluded. The VSD cases were matched by age and sex with 10 controls without congenital heart disease for each case.

RESULTS

A total of 22 855 VSD patients were included. The hazard of death for the entire VSD cohort, including patients with syndromes and associated non-complex congenital cardiac lesions, was increased compared with matched controls without congenital heart disease, with a HR of 6.8 (95% CI 6.1 to 7.6) for ages 0-17 years and HR of 3.1 (95% CI 2.5 to 4.0) for ages >18 years, during a mean follow-up of 14.3 years (±11.1). In isolated, non-syndromic VSD, the hazard of death was still elevated compared with controls, regardless of whether the VSD had been repaired in childhood or not. The HR for death for unrepaired VSDs was 4.8 (95% CI 4.1 to 5.6) for ages 0-17 years and 2.1 (95% CI 1.5 to 3.0) for ages >18 years and for VSDs repaired before 18 years of age HR for death was 2.9 (95% CI 1.2 to 7.1) for ages 0-17 years and 8.2 (95% CI 2.2 to 30.4) for ages >18 years.

CONCLUSIONS

Mortality rates were three times higher in adult VSD patients compared with matched controls. Mortality risk was highest in VSD cohorts that included patients with syndromes and congenital valvular lesions, highlighting VSD patients at extra risk of adverse outcomes.