With Expert Care, Patients With Adult Congenital Heart Disease Are Leading Better, Longer Lives
Posted on 18 September 2018
Congenital heart disease is usually considered a debilitating condition that severely limits a patient’s quality of life. But that is changing, says Mediclinic Panorama cardiologist Dr Lou Hofmeyr.
Congenital heart disease (CHD) is the most common form of birth defect worldwide. Usually, this condition presents as an abnormality within the structure of a newborn baby’s heart, which may affect the heart chambers or valves, or the surrounding arteries or veins.
Disrupted blood flow can have a significant impact on the growth and development of a young child. Most defects result in varying degrees of obstructions in blood flow towards or inside the heart and can cause blood to flow through the organ in a way that disrupts other bodily functions, according to the Heart and Stroke Foundation SA.
The results of these defects can range widely, from minor symptoms to life-threatening heart malfunctions – which is why most experts consider congenital heart disease serious enough to warrant urgent intervention, and even transplant surgery, soon after birth.
Dr Lou Hofmeyr, a cardiologist at Mediclinic Panorama who completed a fellowship in Adult Congenital Heart Disease (ACHD) at The Prince Charles Hospital in Brisbane, Australia, in 2013, says the treatment of children with congenital heart problems has improved so much, that many of them grow up deep into old age.
“We see kids with CHD grow into their 60s and 70s,” he says. “This means they outgrow the care their paediatricians used to provide. And as they grow older, the condition continues to affect them, so it will complicate other issues as they become adolescents and young adults.”
This is where a cardiologist can help.
“Kids with congenital heart issues need specialist assistance,” says Dr Hofmeyr. “This is a complicated disease. There are some simpler defects that can be cured when patients are young, such as atrial septal defect, but others – like transposition of the great arteries or tetralogy of Fallot – might require surgeries in childhood, if the child is strong enough, as well as regular follow-up visits long into adulthood. We try to work as well as we can with what we’ve got.”
Despite the condition being present from birth, it is seldom that doctors can say for sure that it has been inherited, he says. “Congenital heart disease begins when there is an issue with the early development of the foetus. During the first 25 and 35 days after conception, the baby’s heart revolves and rotates from a tube shape into fused folds, and if there is an error or delay in that process, the child will develop some variation of CHD.”
Naturally, there are varying stages of severity – but doctors do not fully understand the mechanism responsible for these developmental defects. “We do understand, more or less, how the heart evolves into shape, and how the walls and valves develop, but when a child is born with CHD, we can’t always give a concrete picture of what went wrong, and why.”
Dr Hofmeyr says the patients he sees with ACHD are unique and inspiring. “Most of the patients I see have had one or two surgeries in their lives, some of them have had six or seven – as children. The tenacity and determination of these people are humbling to see.”
Their strength of mind comes to the fore when someone with ACHD decides to try to have a child. Adult congenital heart disease brings amplified risks – for both mom and baby – during pregnancy, but these are case-specific, says Dr Hofmeyr.
“It depends on the defect, really: in many cases, we will advise a woman with ACHD not to try to fall pregnant, and in other cases, the defect may be minor enough that we will point out the risks, and help to manage the process carefully.”
A woman in that position has a team to lean on, he says. “A cardiologist plays a large role in managing a patient’s heart disease, of course, but when that patient falls pregnant, she will be advised by an extensive team of paediatricians, obstetricians, and others. We will make decisions for the benefit of the patient together, with a lot of planning and input from different sides.”
That multidisciplinary approach has led to some good outcomes in challenging circumstances. Recently, two patients with ACHD were able to give birth after being carefully monitored throughout their respective pregnancies by Dr Hofmeyr and other specialists.
“It’s important to stress here that these patients are incredibly committed,” he says. “This is a very humbling business. They face incredible challenges, and they never cease to amaze us with their tenacity and positivity.”