Congenital heart disease (CHD) comprises of a colourful spectrum of anomalies resulting from abnormal development of the heart segments and the great arteries during foetal life. CHD is the most common birth defect and its incidence ranges from 6 to 13 per 1000 live births affecting approximately 1 neonate in every 120-166 births. It is estimated that about 1,310,000 babies are born with CHD worldwide each year. CHD prevalence in the population has grossly increased as survival to adulthood now reaches 90-95% in developed countries from less than 20% in the presurgical era. In the 2020s, the number of grown-ups living with CHD is expected to surpass the neonatal/infant CHD incidence. About 15% of CHD patients who previously underwent cardiac surgery will require subsequent re-operations (e.g., conduit exchange, valve repairs/replacements, operation for acquired hearts disease, etc.).
Majority of the surgical procedures for congenital heart disease involves implantation of some material in the form of patches, conduits and/or valves. This makes congenital cardiac surgery a reconstructive surgery. Little patient undergoing these operations will grow and develop after surgery and they will grow out their valve or conduit implants. Currently available biomaterials lack the potential of growth, and conduits, valves also derange over time surrendering patients to reoperations. At present, a neonate/infant with conduit or valve implantation undergoes 2-3 reoperations before reaching adulthood for structural failure or for simply growing out the implant. Availability of viable, growing biomaterials could cancel reoperations from the start, and that could entail with significant public health benefit and improved quality-of-life. This is a huge public health burden. Cardiac surgery for Grown-Up Congenital Heart (GUCH) or Adult Congenital Heart Disease (ACHD) patients currently represents 20% of the entire congenital cardiac surgery activity and most these procedures are reoperations.
Treatment for these patients usually requires special expertise and the involvement of a team of allied specialists, e.g., adult congenital cardiologist, congenital cardiac surgeon, internist, anaesthesiologist, haematologist, social workers, etc. Treating patients with ACHD has a significant public health aspect and it is a commitment for life for which specialized services, GUCH or ACHD teams have been formed worldwide.
Patients qualify in three main groups:
- Reoperations of previously implanted valves and/or conduits that can be also performed by using minimally-invasive methods and in selected patients by catheter-based procedures. A small percentage of patient with unresolved or ongoing cardiac problems will need surgery to optimise their myocardial performance, e.g., ventricular synchronization therapy, implantation of a pacemaker and automated implantable defibrillator.
- Newly-discovered congenital cardiac problems. These patients present with an anomaly which is typically dealt with during the paediatric age. Some defects, e.g., ASDs meeting anatomical criteria could be closed by a device at catheter-based procedure or larger ones require surgical closure with a patch. These procedures could be performed with minimally-invasive methods.
- Cardiac surgery for acquired heart disease. These patients undergoing various procedures require the expertise of a congenital cardiac surgeon to help in the aspects of unusual anatomy, previous procedures and special aspects of care.
A/Prof Laszlo Kiraly
Head & Senior Consultant, Department of Cardiac, Thoracic and Vascular Surgery
National University Heart Centre, Singapore
Group Chief, Cardiothoracic Surgery, NUHS
Asst Prof Winn Maung Maung Aye
Consultant, Department of Cardiac, Thoracic and Vascular Surgery
National University Heart Centre, Singapore
Dr Senthil Kumar Subbian
Consultant, Department of Cardiac, Thoracic and Vascular Surgery
National University Heart Centre, Singapore
