Adult Congenital Heart

It is estimated that between 362 and 4,090 adults per million people are living with CHD, although this varies by region. About half of these individuals are women, many of whom are of childbearing age. Pregnancy can carry increased risks for women with CHD, so it is very important to have specialist assessment and careful monitoring before and during pregnancy.

There are many different types of congenital heart disease seen in adults. Some people have long-term effects following childhood heart surgery, such as leaking valves or narrowed blood vessels. Others may have abnormalities of the heart valves, including conditions like a bicuspid aortic valve or Ebstein anomaly. Some people have structural problems such as holes in the heart, including Atrial septal defect or Ventricular septal defect, or a persistent connection between blood vessels known as Patent ductus arteriosus. Other conditions include abnormalities of the blood vessels, such as Coarctation of the aorta, or more complex conditions like Transposition of the Great Arteries and Tetralogy of Fallot, and often having had a heart operation at childhood. Some individuals may also develop Pulmonary hypertension.

Adults with congenital heart disease often experience symptoms such as shortness of breath, tiredness, and reduced ability to exercise. Some people may also notice palpitations or feel their heart racing. The condition is usually diagnosed through a combination of a physical examination and tests such as heart scans, ECGs, or more advanced imaging like MRI or CT scans.

Treatment depends on the type and severity of the condition. Some mild cases do not require treatment and only need regular check-ups to monitor the heart. Other patients may need medications to control symptoms, procedures using catheters to repair problems without open surgery, or in some cases, heart surgery. The goal of treatment is to improve symptoms, prevent complications, and support a good quality of life.

Living with congenital heart disease usually requires lifelong follow-up, ideally in a specialist centre with experience in adult congenital heart conditions. It is also important to reduce the risk of complications such as Infective endocarditis, an infection of the inner lining of the heart. Patients are often advised to maintain good dental hygiene and may need antibiotics before certain medical or dental procedures, depending on their condition.

Surgery is an important treatment option for some people with adult congenital heart disease (ACHD). The aim of surgery in adult congenital heart diseases is to improve how the heart works, relieve symptoms, and prevent future complications. Whenever possible, surgeons try to repair the heart using the patient’s own tissue, such as the pericardium (the natural lining around the heart), or use well-tested biological (often bovine) or synthetic materials like Gore-Tex. Repairing the heart is usually preferred because it helps preserve the heart’s natural function. However, if a valve is too damaged to repair, it may need to be replaced with an artificial valve. These replacement valves can be mechanical (which are long-lasting but usually require lifelong blood-thinning medication) or biological (made from animal or human tissue and usually do not require long-term anticoagulation). In some younger patients, particularly those with problems affecting the aortic valve or aortic root, a specialised operation called the Ross procedure may be recommended. In this operation, the patient’s own pulmonary valve is moved to replace the diseased aortic valve. The missing pulmonary valve is then replaced with a donor valve (from another human, known as a homograft) or a tissue valve.

With advances in pediatric cardiology and cardiac surgery, an increasing number of patients with congenital heart disease are surviving well into adulthood. As this population ages, they are now encountering not only the long-term consequences of their underlying congenital conditions but also the onset of acquired cardiovascular diseases more typical of older adults, such as coronary artery disease, valvular heart disease, and aortic aneurysms. This evolving clinical landscape presents unique challenges, as these patients may require complex therapeutic interventions to their hearts (both surgical and percutaneous) superimposed on previously repaired congenital defects or in the context of newly diagnosed congenital abnormalities in adulthood. Consequently, their care demands a highly specialized, multidisciplinary approach that integrates expertise in both congenital and acquired heart disease to optimize outcomes.

For some patients with Some adults with congenital heart disease can be treated without open-heart surgery using minimally invasive procedures called percutaneous (catheter-based) techniques. These procedures are performed by specially trained doctors called interventional cardiologists. Instead of making large cuts, they insert a thin tube (catheter) into a blood vessel, usually in the leg, and guide it up to the heart using imaging. Through this catheter, doctors can place small devices inside the heart to treat certain problems. For example, these devices can be used to close holes in the heart, open up narrowed heart valves, or in some cases implant replacement valves. Because these procedures are less invasive than surgery, recovery is often quicker, and the risks may be lower for suitable patients. For some people with specific types of holes in the heart, such as a Atrial septal defect (a hole between the upper chambers of the heart), catheter-based closure is often the preferred treatment, especially when there are no other associated heart problems. Your heart specialist will assess whether this approach is suitable for you based on your individual condition.

Adult congenital heart disease can be complex, with more than 30 different types of conditions, many of which affect the structure and function of the heart in different ways. The onset of acquired cardiovascular diseases more typical of older adults that require therapies on these patients adds another level of complexity. This means that care can be challenging and requires specialist expertise. For this reason, patients should be referred to specialist adult congenital heart centres where experienced teams can provide the most appropriate care. In these centres, patients benefit from a multidisciplinary team approach, which includes cardiologists, cardiac surgeons, specialist nurses, and other healthcare professionals. Care typically involves a detailed initial assessment, clear explanations of the condition, discussion of treatment options, and access to advanced procedures when needed. Just as importantly, patients receive ongoing support, counselling, and regular monitoring to help them manage their condition and maintain the best possible quality of life.

Many children born with congenital heart disease require heart surgery or catheter-based procedures during infancy, childhood, or adolescence to repair abnormalities in the structure of the heart and blood vessels. These operations may include closure of holes within the heart, repair or replacement of heart valves, reconstruction of blood vessels, or more complex procedures for conditions affecting the chambers of the heart and circulation. Thanks to major advances in cardiac surgery, anaesthesia, imaging, and intensive care, the vast majority of these children now survive into adulthood and lead active and fulfilling lives. However, congenital heart disease is often a lifelong condition, and some patients may require additional procedures later in life because previous repairs may gradually wear out, valves may become leaky or narrowed, or changes in the heart and circulation develop over time.

As adults, patients with congenital heart disease may also develop common acquired heart conditions such as coronary artery disease, aortic aneurysms, valve disease, heart rhythm problems, or heart failure, similar to the general population. In some patients, these conditions may develop earlier because of the long-term effects of congenital heart disease or previous heart surgery. Treatment can therefore become more complex, particularly in patients who have undergone multiple operations in the past or who have unusual heart anatomy. Some adults may require repeat surgery for their congenital heart condition alone, while others may need combined procedures to treat both their congenital heart disease and newly acquired adult cardiac disease at the same time, such as coronary artery bypass surgery together with valve or aortic surgery.

Careful planning, advanced imaging, and a multidisciplinary approach are essential in these situations. For this reason, these operations are best performed in specialist Centers where Congenital Heart Disease Specialist (congenital cardiologists and congenital cardiac surgeons) work along with Adult Cardiac Specialist (adult cardiologist and adult specialist cardiac surgeons), imaging specialists, and specialist cardiac anaesthetists and intensivist work closely together to provide highly specialised and personalised care.