Introduction

Heart disease causes dread in parents and carers. This is usually because their experience of heart disease is in adults whose ischaemic heart disease causes chest pain, peripheral oedema or sudden death. In developed countries cardiac problems in children are usually congenital and present with heart murmur, breathlessness or cyanosis. Recent advances have made congenital heart disease an eminently treatable condition, and many children with murmurs have normal hearts and need no treatment at all. Do not forget when talking to families how frightening talk of heart problems will be.

History

Serious heart disease in children presents with poor growth, breathlessness, pallor or cyanosis. In babies this causes feeding problems – breaking off in mid-feed, with panting or ‘grunting’ respiration and sometimes sweating. Murmurs (e.g. ventricular septal defect, VSD) may not be evident at birth, but may be apparent by the time of the 8-week check. Older children with heart disease may lack energy and tire easily.

Ask about any episodes of cyanosis, pallor or distress. Check for a family history of cardiac problems in children. Reassure the family that the grandfather’s recent heart attack is unlikely to be relevant. Ask about any previous treatments or heart operations.

Examination

Make a non-threatening start by feeling the radial and then the brachial pulse. Remember that the pulse is faster in infants than in older children and adults. Some irregularity of the pulse (sinus arrhythmia) is normal in healthy children. The character of the pulse may be abnormal in aortic stenosis (low volume) or patent ductus arteriosus (PDA; collapsing or ‘bounding’ pulse). Palpating the femoral pulses feels intrusive to the child – leave it until the end, but do not forget to do it. Radiofemoral delay is not of use in paediatrics as most children are too small for the delay to be perceptible.

Ideally the examination can now proceed through inspection of the praecordium, palpation, auscultation and percussion, but in an active toddler you may need to grab opportunities to examine as they present. Remember that children who have had cardiac catheterization may have impalpable pulses and small scars in the groin or antecubital fossa over the femoral or brachial artery. Listen to the heart sounds at each of the four sites shown in Figure 9.1. At each site listen carefully for first and second heart sounds. In the pulmonary area it should be possible to hear the pulmonary and aortic components separately. An extra sound at the apex following the second sound is the third sound. This is usually physiological in children. In children with a cardiac failure there may be a gallop rhythm produced by third and fourth sounds. Finally, listen for heart murmurs. Try to decide where the murmur is best heard, how loud it is and whether it is a pansystolic (i.e. beginning with, and not separate from, the first heart sound) or an ejection systolic murmur. Diastolic murmurs are rare in childhood, but listen carefully all the same.

Figure 9.1 Auscultation points.

Blood pressure measurement is important in children, but is best left until last as it may be distressing. In infants, blood pressure can be measured using an automated blood pressure machine – palpation techniques are unreliable. In older children the blood pressure can be measured using a stethoscope in the normal way, but the correct size cuff must be used. The cuff should cover two-thirds of the length of the upper arm, and the bladder should encircle at least 80% of the arm. Centile charts for blood pressure are available in the UK. Blood pressure should be measured in the right arm in almost all circumstances, and both arms if there are thoracotomy scars.

The following scheme is recommended.

Investigations

A chest X-ray will allow assessment of cardiac size and pulmonary vasculature. An ECG may give information on the relative size of the cardiac chambers and on cardiac strain. Characteristic abnormalities are seen with some congenital heart defects, e.g. superior QRS axis and ostium primum atrial septal defect (ASD) or tricuspid atresia. Transcutaneous oxygen saturation, ideally in the right arm, will more reliably detect cyanosis. If saturation is less than 95%, in the absence of a sufficient alternative explanation, echocardiography is indicated. An echocardiogram allows accurate diagnosis of cardiac anatomy and a useful assessment of cardiac function. Cardiac catheterization accesses the heart through a catheter usually inserted into the femoral artery. This technique enables therapy, e.g. balloon atrial septostomy, to create an artificial ASD in transposition of the great vessels, as well as aiding diagnosis.

It is likely that motion-tolerant pulse oximetry of newborn infants will be introduced during the currency of this textbook, following the publication of a major trial in over 20 000 newborn infants, in November 2011, which demonstrated effectiveness in early detection of severe congenital heart disease. The UK National Screening Committee is currently considering its nationwide introduction.