Children who had heart surgery 20-30 years ago need to check in again, says the Heart and Stroke Foundation of Canada

Toronto – Adults who, as children, had surgery to correct congenital heart defects are failing to keep in touch with their cardiologists – and that could endanger their future health, says the Heart and Stroke Foundation of Canada.

“Research shows that people who had cardiac surgery as children should be having their hearts monitored regularly to detect irregular rhythms and the first signs of heart failure,” says Dr. Peter Liu, of Toronto’s Heart and Stroke/Richard Lewar Centre of Excellence

In Canada there are roughly 100,000 such patients and one million in the USA. The very success of surgery to repair pediatric heart defects is creating its own problems, says Dr. Liu. People are alive today as a result of the surgical advances of the ’60s and ’70s. Children born with heart defects – who would otherwise have died – have survived into adulthood.

Many such people probably feel they have been cured. They may feel too involved with family and career to see a doctor. They may even have forgotten why they had the surgery, if indeed they were ever told. But congenital heart defects are not cured – they are repaired. Any heart repair should be regularly inspected for “wear” and tear, especially when the repair is 25-30 years old, says Dr. Liu.

Cardiologist Dr. Gary Webb, a spokesperson for the Heart and Stroke Foundation, says, “Our goal now is to keep these patients in good shape for another 30 or 40 years. It can be done but early detection and treatment of problems is essential.”

Adults who have had heart repair surgery should not wait until they feel symptoms, cautions Dr. Webb. By the time symptoms appear, much of the opportunity for treatment will have been lost.

Extremely specialized testing and monitoring is required to safeguard the future health of these patients. A visit to the family doctor or to a cardiologist lacking special training in this area is not recommended.

Dr. Michael Gatzoulis of the Royal Brompton Hospital, London, England, working with Dr. Liu, examined 51 patients who 25 years ago had received the Mustard procedure in Toronto. They found that nearly a quarter of the sample were beginning to develop heart rhythm problems which, left untreated could lead to heart failure.

The Mustard procedure – developed by Dr. William Mustard with support from the Heart and Stroke Foundation of Canada– was the first operation to correct the congenital abnormality that produced “blue babies.

Blue babies are born with a defect called transposition of the great vessels, in which blood from the lungs flows back to the lungs and blood from the body flows back to the body. The two major arteries coming out of the heart, the aorta and the pulmonary artery are connected to the wrong chambers. Such babies look blue because insufficient oxygen is circulating in their bodies.

The Mustard procedure restores the circulation but reverses the direction of the blood flow in the heart. In a normal heart, de-oxygenated, blue, blood is pumped into the lungs via the right ventricle. Then it is distributed throughout the body via the left ventricle. In the Mustard procedure, blood is pumped to the lungs via the left ventricle and disseminated throughout the body via the right ventricle.

But the right ventricle is not the optimal shape to support the high pressure work performed in a normal heart by the left ventricle. Over a period of 20 or 30 years the right ventricle may begin to fail. The research conducted by Dr. Liu and Dr. Gatzoulis confirms that this is indeed the case.

The first Mustard procedure was performed in 1963 and the operation was superseded in 1987 when an arterial switch procedure was introduced which normalizes left ventricular function. The Mustard procedure reduced an 80% mortality in the first year of life to an 80% survival at age 20.

Fifteen hundred Canadian children received the procedure. Worldwide the total is approximately 46,000. “The Mustard procedure was the first to show that congenital heart defects could be repaired. It transformed the whole field. There is a wide spectrum of heart defects, over 700, and the majority can be operated on with good results,” says Dr. Gatzoulis.

By seeing the properly qualified specialist, for a series of non-invasive tests measuring the size and function of the heart, former patients will be helping not only themselves but a generation yet unborn, says the Heart and Stroke Foundation.

For more information, contact : Elissa Freeman, Heart and Stroke Foundation of Ontario, 416-489-7111 ext. 316 or Sharon Edwards, Heart and Stroke Foundation of Ontario, 416-489-7111 ext. 455.