Adult Congenital Heart Disease (ACHD)

Adult Congenital Heart Disease (ACHD) - What it is

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.

Adult Congenital Heart Disease (ACHD) - Symptoms

Adult Congenital Heart Disease (ACHD) - How to prevent?

Adult Congenital Heart Disease (ACHD) - Causes and Risk Factors

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.

Adult Congenital Heart Disease (ACHD) - Diagnosis

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

Adult Congenital Heart Disease (ACHD) - Treatments

NHCS Adult Congenital Heart Disease (ACHD) Programme

This programme was first started in 2003 and currently offers:

ACHD clinic on every Wednesday and Thursday afternoon and on the first and third Fridays of the month in which an average of 20-30 patients are seen at each session. Besides offering care and follow up of ACHD patients, NHCS also does screening of suspected Marfan patients; close monitoring of ACHD patients during pregnancy (see Pregnancy and Women with CHD) and a monthly joint Cardiac Obstetric Clinic (every last Monday of the month) with obstetricians from Singapore General Hospital (SGH); Monthly Transition Clinic in KK Women's and Children's Hospital (KKH) run jointly with paediatric cardiologists from KKH for patients above the age of 16. The aim is to give paediatric patients a smooth transition from paediatric cardiology to adult cardiology services. An average of 100-150 teenagers with congenital heart disease are transferred from KKH to NHCS ever year. There is also a monthly Pulmonary Hypertension Clinic (every second Friday of the month) run jointly with a respiratory physician and a rheumatologist from SGH for general as well as congenital patients with severe pulmonary hypertension.
Dedicated twice weekly congenital echocardiography sessions. Dedicated weekly congenital cardiac catheterisation and intervention (particularly ASD, patent foramen ovale (PFO) and PDA device closure).
Surgical interventions including extra-cardiac Fontan procedures for uni-ventricular hearts, pulmonary homograft replacements for tetralogy of fallot patients with severe pulmonary regurgitation, Rastalli conduit for patients with pulmonary atresia.
Expertise in nuclear imaging, magnetic resonance imaging (MRI) and Multi-slice Computed Tomography (CT) imaging.
Expertise in pacing, arrhythmia treatment and ablation in congenital patients.
Expertise in cardiopulmonary and exercise testing.
Expertise in heart and lung transplantation for selected patients with end-stage congenital heart disease.
Dedicated ACHD nurse for education, support and care of ACHD patient and their families.

Adult Congenital Heart Disease (ACHD) Clinic

This clinic looks after patients with operated as well as unoperated congenital heart conditions from age 16 onwards. Adults with congenital heart disease need regular monitoring and sometimes further surgical interventions. It also screens suspected Marfan patients and monitors ACHD patients closely during pregnancy.

For more information on NHCS Adult Congenital Heart Disease (ACHD) Programme, visit here.

Adult Congenital Heart Disease (ACHD) - Preparing for surgery

Adult Congenital Heart Disease (ACHD) - Post-surgery care

Adult Congenital Heart Disease (ACHD) - Other Information

Overview

Hypertension,

Abnormal Heart Rhythm/Arrhythmia,

Atrial Septal Defect,

Patent Ductus Arteriosus,

Patent Foramen Ovale,

Tetralogy of Fallot,

Coronary Artery Disease / Coronary Heart Disease,

Ventricular Septal Defect (VSD)

Updated on 2017-03-28T16:00:00Z
Article contributed by Department of Cardiology, National Heart Centre Singapore

The information provided is not intended as medical advice. Terms of use. Information provided by SingHealth

Discover articles,videos, and guides afrom Singhealth's resources across the web. These information are collated, making healthy living much easier for everyone.