Adult Congenital Heart Disease (ACHD)

An increasing number of children with congenital heart disease are surviving into adulthood due to greatly improved surgical, medical, anaesthetic and intensive care over the last few decades. The survival of congenital patients into adulthood is now close to 85% in comparison to the 15% survival rate several decades ago. This new group of patients is now entering adulthood and will need regular monitoring and sometimes further surgical interventions may also be required.

With the exception of a few conditions, ligation of isolated patent ductus arteriosus (PDA) and closure of isolated atrial septal defects (ASD) and ventricular septal defects (VSD), surgical treatment of congenital heart disease is more often palliative than curative. Repeated procedures and interventions are the norms as conduits, baffles, shunts and grafts become stenosed, blocked and incompetent with time. In addition to the medical and surgical issues, this growing population may also face specific social, psychological and behavioral problems throughout their life.

It is timely that adult congenital heart disease (ACHD) (also known as congenital heart disease in adults) is gaining recognition worldwide and in Singapore as an established subspecialty of cardiology.

The incidence of congenital heart disease (CHD) in Singapore is 0.81% of total live births based on the birth defect registry from 1994-2000. In Singapore, with an estimated population of around 4.3 million, the estimated number of adults with CHD would be in the region of 12,000 adults with an extra 300-320 cases added annually, assuming an annual birth of 37,000 – 40,000 newborns. It is estimated that in the next decade, the number of adult patients with congenital heart disease worldwide will exceed their paediatric counterparts.

Challenges

Despite their improved life expectancy, congenital patients stepping into adulthood face many issues:

• They have limited understanding of their underlying congenital defects
• Many believe that they are ‘cured’, unaware of possible sequel from their surgery and the need for further surgical procedures
• Medical and surgical issues which require life-long follow up, regular check-ups and repeated investigations and evaluation
• Issues related to pregnancy, contraception, risk to offspring need to be discussed
• Risk of endocarditis and the need for antibiotic prophylaxis
• Management of associated non-cardiac medical problems
• Social, emotional, financial, vocational, educational, psychological and lifestyle issues specific to each individual. As they enter adulthood, ACHD patients need to take charge of their lives, substituting parental overprotection in childhood with a sense of responsibility and maturity of their own

In the past, these adult patients were usually managed by paediatric cardiologists who, although excellent in interpreting and comprehending the underlying physiology and haemodynamics, were nonetheless ill-equipped in managing adult related problems like coronary artery disease, pregnancy and contraception, arrhythmias,hypertension. Research has shown that ACHD patients especially those of moderate to severe complexity do better when their care is centralised to a dedicated specialised ACHD unit.

Having survived into adulthood, ACHD patients are now facing a new set of challenges encompassing not only medical or surgical problems but extending to psychological, vocational and lifestyle issues. Congenital heart disease is a heterogenous condition with a wide spectrum of varying complexity and severity. Therefore, all ACHD patients (with the exception of those with mild isolated valvular heart disease or those with repaired patent ductus arteriosus, ventricular septal defect and atrial septal defect) should be followed up in a specialised ACHD unit with a dedicated multidisciplinary team who can anticipate the various issues particular to each individual. Only then can we provide adequate care and work towards improving the clinical outcome of this special group of patients.

Diagnosis typically starts with a physical examination by a doctor, assessing your medical history and routine tests. Other tests recommended may include:

• Electrocardiogram (ECG)
• Exercise Stress Test
• Echocardiogram
• Cardiac catherisation