At 1.6m-tall with long hair and fair skin, Ms Esther Lum* appears to be an average 25-year-old Singaporean. So normal, in fact, that one would never be able to tell that she was born with ALCAPA - an extremely rare congenital heart disease where the left coronary artery rises from the pulmonary artery rather than the aorta. She is just one of an increasing number of adult Singaporeans with congenital heart disease, who have grown up to find they face a host of new challenges as adult sufferers.

Congenital heart disease affects about 0.81 per cent of total live births in Singapore, with some 300 newborn cases detected annually. The survival rate for children born with congenital heart disease is now close to 93 per cent as compared to 25 per cent 50 years ago, which means that there are now many adults living with the condition referred to as adult congenital heart disease (ACHD).

Despite their improved life expectancy, ACHD patients face many issues. Many mistakenly believe that they are "cured" after surgical procedures done in their childhood and are unaware of possible developments later in adulthood. Ms Lim's case is such an example. When she was five years old, she underwent surgery to re-connect her left coronary artery to its rightful point of origin. Said Ms Lum: "I thought that it was over and done with after my first surgery. But during my last two check-ups, the doctor noticed that my heart was expanding and showing some leakage (backflow of blood) from one of the heart valves. Because of this, she told me to prepare myself for follow-up surgery in the next five to 10 years." Ms Lum's physician, Dr Tan Ju Le, Senior Consultant at the Department of Cardiology and Director, Adult Congenital Heart Diseases, National Heart Centre Singapore said: "Very few congenital heart defects are cured; most are palliated or repaired."

Dr Tan said the diversity and spectrum of congenital heart defects is quite different from adult cardiology, which deals mostly with coronary artery disease and heart failure. "Congenital heart disease is not just one but many types of defects with varying degrees of severity. Not all ACHD patients require surgery. For example, patients with a small hole in the heart with no symptoms may not need any intervention. Another patient with the same heart defect but with enlargement of the heart chambers or increment in lung pressure may need intervention. So treatment has to be individualised,"

Whether or not additional surgery is required, ACHD patients need lifelong follow-up sessions and regular check-ups. Problems that may surface later in life include palpitations, breathlessness due to heart failure and constant fever from infective endocarditis (infection of the heart valves).

For female patients, there are also issues related to pregnancy, contraception and risk to offspring. "I can remember on several occasions telling some of my young female patients with very complex cyanotic congenital heart defects that they should avoid pregnancy as it carries a very high risk, not only for them but for their babies as well," said Dr Tan. These patients are subsequently referred to the Cardiac Pregnancy Clinic for contraception counselling.

On a positive note, Dr Tan has helped one such patient and her husband to successfully apply for child adoption. Said Dr Tan: "We're here to help them lead as normal a life as possible. They cannot change what they are born with but they can certainly choose to live their life to the fullest."

Through the ACHD programme, patients also learn to handle other social, medical, financial and psychological issues. For example, they may have difficulties getting insured for a life policy.

Despite an impending surgical procedure, patients like Ms Lum remain optimistic. She believes her positive outlook on life is something she learned since transferring to the ACHD programme two years ago. "I felt at ease with Dr Tan the moment we met," she recalled. "It's reassuring to know that with support from the ACHD programme, someone is always there for you."

*Patient's name has been changed for privacy.