Paediatrics

In many cases, congenital heart disease is diagnosed during pregnancy. However, a diagnosis may sometimes only be confirmed after the birth.

Congenital heart disease may initially be suspected during a routine ultrasound scan of the baby in the womb. Specialist ultrasound, called foetal echocardiography, will then be carried out at around 18 to 22 weeks of the pregnancy to try to confirm the exact diagnosis.

This may also be undertaken if there’s a family history of congenital heart disease or where there’s an increased risk. Echocardiography is a type of ultrasound scan, where high-frequency sound waves are used to create an image of the heart.

However, it’s not always possible to detect heart defects, particularly mild ones, using foetal echocardiography.

It’s sometimes possible to diagnose a baby with congenital heart disease shortly after birth if some of the characteristic signs or symptoms of congenital heart disease, such as a blue tinge to the skin (cyanosis), are present.

Your baby’s heart will be checked as part of the newborn physical examination. The examination involves observing your baby, feeling their pulse, and listening to their heart with a stethoscope. Heart murmurs are sometimes picked up.

However, some defects don’t cause any noticeable symptoms for several months or even years.

Echocardiography is often used to check the inside of the heart. Heart problems that were missed during foetal echocardiography can sometimes be detected as a child develops.

An electrocardiogram (ECG) is a test that measures the heart’s electrical activity. Electrodes are placed on the skin around the heart and are connected to a computer. The computer analyses the electrical signals produced by the heart to assess how well it’s beating.

A chest X-ray of the heart and lungs can be used to check whether there’s an excess amount of blood in the lungs, or whether the heart is larger than normal. Both may be signs of heart disease.

Pulse oximetry is a test that measures the amount of oxygen present in the blood.

The test involves placing a special sensor on the fingertip, ear or toe that sends out light waves. A computer is connected to the sensor and measures how the light waves are absorbed.

Oxygen can affect how the light waves are absorbed, so by analysing the results, the computer can quickly determine how much oxygen is present in the blood.

Paediatrics is a branch of medicine that manages medical conditions affecting infants, children and young people. Traditionally in the UK Paediatrics covers birth to approximately 18 years of age. However some branches cover before birth and pediatricians may occasionally continue treat patients over the age of 18. Some patients may transition to adult services between 16-18 years of age.

Paediatrics can be divided into 4 main areas:

General Paediatrics – a hospital role covering children from birth to the age of 16. Most paediatricians have this generalist role

Neonatology – this role specialises in looking after new born babies. It is usually based in an intensive care unit developed to provide care for premature babies or those with problems at birth

Community paediatrics – these doctors are based in the community and look after children with developmental, social or behavioural problems and those with a physical disability

Paediatric Cardiology – this is a small area which is a specialty in its own right. These doctors diagnose and treat children with cardiac (heart) conditions

Paediatric cardiologists are doctors who diagnose and treat children with cardiac (heart) conditions. They work with patients antenatally (before birth), through childhood and into adulthood.

Paediatric cardiology is concerned with diseases of the heart in the growing and developing individual. As well as expertise in heart disease, paediatric cardiologists also need a thorough grounding in general paediatrics, in order to provide all-round patient care.

Paediatric cardiologists broadly treat congenital heart disease (present at birth), arrhythmias (variations in heart rhythm) and disturbances of circulatory function.

The initial assessment performed by the paediatric cardiologist might start with a physical examination using a stethoscope, after which more detailed investigations may be suggested (ECG, ultrasound & heart scan).

Patients often present with complex diagnostic and medical problems and after the initial assessment the paediatric cardiologist then chooses an optimal management plan. They work closely with a wide range of specialists as part of a multidisciplinary team to assess and treat patients.

Paediatric cardiologists play a vital role in the teaching of medical students, doctors. GPs, nurses and paramedical staff. Most are also involved in research.

These include:

  • cardiovascular collapse in infancy
  • cardiac failure in infants and children
  • cyanosis in the newborn and beyond (where the skin has a bluish tinge, due to lung or circulatory problems)
  • children with heart murmurs
  • children and adolescents with chest pain, palpitations, or fainting
  • patients with congenital heart disease
  • cardiovascular abnormalities in neonatal intensive care
  • fetal cardiac abnormalities
  • paediatric cardiac transplantation
  • assessment of children prior to cardiac surgery
  • assessment of children with cardiac disease who are undergoing non-cardiac surgery
  • management of critically ill children with cardiac problems

Surgery on the heart first began in 1896 when a stab wound to the heart was sutured preventing death from haemorrhage. However little progress occurred over the next 50 years due to the difficulty of operating inside the heart without stopping it.

Heart surgery today is divided into 2 fields, congenital and acquired. Congenital surgery deals with defects that are present at birth while acquired, deals with defects that occur in a previously normal heart. The 2 are sometimes referred to as paediatric (congenital) and adult cardiac surgery but that definition is less accurate since many children operated on are surviving into adulthood sometimes need further surgery. Occasionally a defect present at birth may not declare itself until adult life and is best operated by a surgeon trained in the recognition and correction of such problems. A few children born with normal hearts may develop diseases of the heart that require an operation and this will come under the care of a congenital (paediatric) cardiac surgeon.

All heart surgery is divided into either closed or open. Closed heart surgery refers to any operation on the heart that does not need a pump to take over the function of the heart during the operation. Open heart surgery (80% cases) is any operation where a pump (Heart-Lung Bypass Machine) is used.

Congenital heart surgery began in 1939 when a surgeon in Boston USA tied off a persistent arterial duct (PDA). It was followed in 1944 by an operation to relieve Coarctation of the aorta (Craaford in Stockholm), and the Blalock-Taussig shunt at Johns Hopkins Hospital in Baltimore. Later in 1947/8 Brock and Holmes-Sellors in London performed operations to treat stenosed (narrowed) valves. All these operations were enormous steps at the time but were limited by the unavailability of the heart lung machine so were closed heart operations.

In 1953 the first operation inside the heart was accomplished when John Gibbons in Philadelphia successfully closed an ASD utilising a heart lung machine that he had developed. However the machine was not reliable and he was unable to repeat the success. Other surgeons persisted with research and development and in 1955 a machine was available to allow open heart surgery to become a reality. This introduced a period of massive and rapid progress such that today there is virtually no baby born with a heart defect for whom some form of operation is not possible. However at the same time many defects are so complex that the operations can never achieve a normal circulation. Approximately 5,500 operations for congenital heart defects are performed each year in the UK and Ireland of which about 1,000  patients are over 16 years old. Every operation is reported to a central monitoring organisation, NICOR (National Institute for Cardiovascular Outcomes Research) to ensure standards are maintained at a high level. Currently 30-day survival is just over 97% irrespective of the underlying condition making the UK and Ireland one of the safest places in the World to have such surgery.

Congenital Cardiac Surgery is now a formal Sub-Specialty of Cardiothoracic Surgery. This means that trainee surgeons who wish to pursue a career in Congenital will need to spend at least two years of their final training within a dedicated Congenital Programme. Trainees must have passed their FRCS(CTh) and hold an NTN in Cardiothoracic Surgery. They must then apply for one of the UK training programmes for Sub-Specialty Training. All consist of two paired centres with the trainee spending one year in each. The posts are interviewed for at the same time as national recruitment, once a year. Currently, the recognised programmes are (a) Great Ormond Street and Birmingham Children’s Hospital, (b) Evelina (Guy’s and St Thomas’) and the Royal Brompton Hospital and (c) Alder Hey Liverpool and Freeman, Newcastle.

After 6 years in training and having passed the FRCS(CTh) the trainee will be awarded a CCT (Certificate of Completion of Training) which will allow them to enter the GMC register as a “Specialist”. Without that specialist registration they cannot be appointed to a NHS consultant position. If pursuing a career in Congenital Cardiac Surgery the trainee will have also completed their two-year Sub-Specialty training and will receive their CCT in not only cardiothoracic surgery in general, but also in the Sub-Specialty of Congenital.

In summary anyone wishing to become a congenital cardiac surgeon will spend at least 10 years after graduation from medical school before they become a consultant in the NHS.

The Future

The increasing survival into adulthood of children with complex cardiac defects has brought new challenges. For some the repaired heart may begin to fail early. For them an operation may enable recovery but for others if medication does not help then cardiac transplant may be the only option. This is limited by donor organ availability which already cannot meet national need. One possibility may be genetic modulation of other mammalian hearts though that has been disappointing thus far. A more realistic solution may be the development of a totally artificial heart that can be implanted. The current success of heart surgery owes a great deal to the technological advances in material science and diagnostics as it does to the greater knowledge of cardiac physiology and biochemistry. These technological advances may well overcome the current difficulties that prevent widespread application of totally implantable artificial hearts.

[Content provided by Professor David Anderson, Consultant Heart Surgeon and Professor of Children’s Heart Surgery, St Thomas’ Hospital – last updated 6 March 2017] Taken from https://www.bcca-uk.org/pages/page_box_contents.asp?pageid=929&navcatid=244 [13 August 2018].

COVID-19 Advice for North West CHD Network: Advice for patients and families affected by congenital heart disease and children with other heart conditions pleaseclick here