Thanks to advances in medical care, premature death is now less common than in the past for Australians with congenital heart disease.

For most adults with congenital heart disease death is now more likely from non-cardiac related causes and patients with simple defects can expect to live into their seventies, according to a review of more than 3000 patients by Dr Christopher Yu at Concord Repatriation General Hospital, Sydney.

The findings come from a six-year follow up study of 3068 adult patients with congenital heart disease in the hospitals’ database.

Almost half (47%) had simple defects, 33.5% had moderate defects and 17.8% had complex defects. Overall the median age of death was 54 years, with 92% of patients surviving to 50 years of age and 88% to age 60.

Heart failure was the most common cause of death (17%) in adults with congenital heart disease, but non-cardiac deaths (especially malignancy and infections) outnumbered cardiac-related deaths, particularly in adults with simple defects.

In the simple defect group the mortality rate was 0.4 deaths per 100 patient years, which was lower than rates seen in precious studies of adults with congenital heart disease. The median age of death was 70 years of age, which was nine years less than the average age of death for the Australian population.

Survival rates were significantly worse with increasing complexity of congenital heart disease, with median age of death being 51 years for moderate complexity defects and 34 years for complex defects.

Publishing their findings in Heart, Dr Yu and colleagues note that perioperative mortality rates were low at around 5%, likely reflecting improved operating techniques and specialist cardiac anaesthesia.

“Over 50% of our perioperative deaths were due to high-risk redo operations or extraction of device leads, also recognised to be high risk,” they write.

They say their findings have shown for the first time that many patients with simple congenital heart disease live significantly longer and are more likely to die from non cardiac causes.

However premature death is still common among patients with more complex congenital heart disease and the study “highlights target areas for improving mortality rates in a contemporary adult congenital heart disease population,” they conclude.