What signs and symptoms of cardiovascular dysfunction can be seen on inspection of the child?
Consider the following 2 cases and discuss the questions below before reading the tutorial. Show
Case AA 3 month old baby presents with poor feeding and failure to thrive. She was born at term after a normal pregnancy. Mum describes that the baby always breathes very fast, that she takes a long time to feed and gets very sweaty. On examination the baby is pink, malnourished and breathless. She has a respiratory rate of 50 and has marked intercostal and sub-costal recession. She has a loud pan-systolic murmur and her liver is palpable at 2cm. A CXR shows increased pulmonary vascular markings and a large heart (>60% of the thoracic diameter). Case BA 9 year old girl presents with fever and chest pain. Her mother informs you that she had a sore throat a few weeks ago for which she did not receive any treatment. On examination you notice that her right knee and left elbow are swollen and painful. On auscultation of her heart you hear a loud pansystolic murmur. Questions:
Tutorial:Heart disease in children may be congenital or acquired. This tutorial will consider the different types of heart disease in children and the recognition and investigation of children with heart disease. 1. Congenital heart diseasei. Structural heart defectsThe incidence of congenital heart disease (CHD) varies between 5 and 8/1000 live births. The cause of most congenital heart disease is unknown, but is likely to be related to genetic defects, teratogens (such as maternal alcohol or drugs, including anti-epileptics or warfarin), or maternal disease (rubella, diabetes). The incidence of different types of structural heart disease varies between populations, but ventricular septal defect (VSD) is the most common lesion in all populations. The incidence of different types of CHD is shown in table 1 below. Table 1. The incidence of types of CHD in the UK ii. Genetic defectsRecognisable chromosomal abnormalities are present in 25% of children with CHD. The diagnosis of a chromosomal abnormality should lead to active assessment for CHD. The most common chromosomal abnormality is Down’s syndrome (trisomy 21) – 40% of children with Down’s syndrome have CHD, most commonly atrioventricular septal defect (AVSD) or VSD. Genetic defects associated with cardiac lesions are shown in table 2 below: Table 2. Genetic defects associated with cardiac lesions iii. Classification of structural defectsIt is useful to classify congenital heart disease according to the pathophysiology of the major heart lesion, in particular, whether the lesion is associated with cyanosis (‘blue’) or is acyanotic (‘pink’), also whether the lesion is associated with abnormal flow between the cardiac chambers (abnormal ‘shunt’), obstruction to flow, abnormal connections of the major vessels or abnormal mixing. Table 3. Classification of common congenital heart lesions iv. Duct dependent circulationIn utero, the placenta is the main site for gas exchange for the developing foetus and the blood flow to the foetal lung is minimal. Blood from the right ventricle bypasses the lungs and passes directly from the pulmonary artery to the aorta via a foetal vessel called the arterial duct. After birth, a number of changes occur in transition from the foetal to the newborn circulation including expansion of the lungs (which reduces pulmonary vascular resistance) and closure of the arterial duct, so that blood now perfuses the lungs. There are certain severe congenital cardiac lesions that are only compatible with life if the arterial duct remains open – the circulation is said to be ‘duct dependent’. These babies typically present with acute collapse as the duct closes within the first 5 days of life (the differential diagnosis is septic shock). Examples of duct dependent circulations include critical coarctation of the aorta (duct dependent systemic circulation) or pulmonary atresia (duct dependent pulmonary circulation). In critical coarctation there is extreme narrowing of the aorta just where the arterial duct joins the aorta, and blood supply to the lower half of the body is only possible if it passes from the pulmonary artery to the descending aorta via the duct. In pulmonary atresia, the only blood supply to the lungs is that which passes from the aorta to the pulmonary artery via the duct. Continued survival of these babies requires infusion of prostglandin E1 (prostin) to keep the duct open until urgent cardiac surgery is possible. NB prostin also causes pyrexia and apnoeas and these babies may require ventilation while on prostin infusions. v. Eisenmenger’s syndromeLesions associated with left to right shunt, such as AVSD, cause high pulmonary blood flow and congestive cardiac failure. The normal physiological response to high pulmonary blood flow is for the pulmonary vascular resistance to increase. With time, the pulmonary vascular resistance will exceed the systemic vascular resistance, and the flow across the shunt will reverse (Eisenmenger’s syndrome). Clinically, this is associated with an initial improvement in symptoms of cardiac failure as pulmonary blood flow reduces, followed by increasing cyanosis as the shunt reverses to become right to left. Surgical closure of the shunt is not possible at this stage as the resistance to flow through the pulmonary circulation is too high and right ventricular failure will occur. Individuals with Eisenmenger’s syndrome are deeply cyanosed, may develop haemoptysis, endocarditis or cerebral abscess, and will eventually die from cardiac failure. Eisenmenger’s syndrome occurs before the age of 1 year in children with very high pulmonary blood flow, such as unrepaired AVSD, but may occur at the age of 40-50 years in adults with unrepaired ASD who have had moderately increased pulmonary blood flow over many years. Corrective cardiac surgery to avoid Eisenmenger’s must be undertaken before the onset of pulmonary vascular disease, the ideal age dependent on the severity of the underlying lesion. vi. Further details and diagrams of the normal heart and common congenital heart lesions.See the following web links for further details of congenital heart lesions:
vii. Arrhythmias in childrenAnother form of congenital heart disease in children may be the presence of abnormal conduction pathways that lead to the development of cardiac arrhythmias, particularly supraventricular tachycardia. Supraventricular tachycardia may be caused by abnormal conduction pathways such as the Wolf-Parkinson-White syndrome, characterised on the ECG by a short P- R interval and an abnormal delta wave on the upstroke of the QRS complex. The child may develop sudden episodes of tachycardia associated with heart rates of >240beats/min, up to 300beats/min. These appear on ECG as narrow complex rhythms. The child may feel faint and uncomfortable during an arrhythmia. The arrhythmia may terminate spontaneously or be terminated by vagal manoeuvres such as brief immersion of the face in ice cold water, carotid sinus massage (massage to the carotid artery in the neck), or in older children, a Valsalva manoeuvre. Specific treatment is with rapid bolus of intravenous adenosine, or if this fails, synchronised cardioversion. Beta blockers may be used to prevent further episodes of tachyarrhythmia – many children will grow out of these episodes with time. Transcatheter radiofrequency ablation of the accessory pathway is undertaken in specialist centres for older children whose symptoms are not controlled by drugs. Supraventricular tachycardia must be differentiated from ventricular tachycardia (VT). In VT the rate is often slower (<200 beats/min), with wide complexes on the ECG with no association between the QRS complex and the P wave. The child is often symptomatic, presenting with acute collapse. Ventricular tachyarrhythmias are often associated with underlying heart disease such as cardiomyopathy. Torsades de Pointes is a particular form of sinusoidal ventricular arrhythmia that occurs in the rare congenital long QT syndrome. If a child with ventricular tachycardia is acutely symptomatic they will require electrical cardioversion; if not, then treatment with an antiarrhythmic drug such as flecainide or amiodarone, or in the case of Torsades de Pointe, intravenous magnesium. Bradycardia. Heart rate less than 90/minute. May be congenital (complete heart block) or acquired following infection (myocarditis, rheumatic fever) or cardiac surgery (damage to the conduction pathways). 2. Acquired Heart DiseaseAcquired heart disease is much more common in adults than in children, but a there are number of diseases affecting the heart that may be acquired during childhood. i. Rheumatic heart diseaseRheumatic fever is uncommon in the developed world, but still occurs with an incidence of 1-2/1000 in developing countries. Rheumatic fever is due to an abnormal immune response to the Lancefield Group A streptococcus (Streptococcus pyogenes), which results in a pancarditis that may lead to permanent damage to the heart and heart valves. The left sided valves are most commonly affected, the mitral valve more commonly than the aortic valve. Mitral regurgitation is seen initially; mitral stenosis may develop over time. Approximately 8 million children are affected by rheumatic heart disease worldwide. The typical picture is of a child 5-15 years of age who suffers from an untreated streptococcal throat infection; 1-5 weeks later they present with a fever, swollen joints and signs of cardiac disease (breathlessness due to heart failure). There is no specific test for rheumatic fever, but the diagnosis is based on the presence of two major criteria, or one major and two minor, in a child with evidence of a recent streptococcal throat infection. Table 4. Criteria for diagnosis of rheumatic fever. Major criteria
Minor criteria
Treatment of acute rheumatic fever The most important treatment of acute rheumatic fever is prevention, associated with improvements in socioeconomic conditions. Once acute rheumatic fever has occurred, treatment includes penicillin to eradicate the streptococcal infection, high dose aspirin as an anti inflammatory drug, or steroids if the cardiac disease is severe. Heart failure is treated with diuretics, and urgent heart valve replacement may occasionally be required. Long-term antibiotics throughout childhood are essential to prevent recurrences and to reduce the risk of subsequent valvular heart disease. Penicillin is commonly given as a monthly intramuscular injection. Heart surgery may be required at a later stage to correct long-term damage to the heart valves. ii. Infective endocarditisChildren who have congenital heart defects or who have rheumatic heart disease may develop infective endocarditis in which the endocardial tissue is infected by organisms such as Streptococcus viridans, Staphylococcus aureus, or Staphylococcus epidermidus. The child presents with general malaise, arthralgia, fever, splenomegaly and the signs of their underlying heart disease. The classical signs of splinter haemorrhages, petechiae, haemorrhagic lesions (Janeway lesions), tender Osler’s nodes, or Roth’s spots (retinal haemorrhage) are not always seen in children. Microscopic haematuria is common, as is anaemia and a raised ESR. Serial blood cultures may reveal the causative organism. Infective endocarditis may affect the heart valves and cause severe heart failure and death. Large lumps of infected material (vegetations) may embolise or cause abscesses in the aortic root, brain, lung, kidney or spleen. Treatment of endocarditis is at least 6 weeks of antibiotics to clear the infection. Surgery may occasionally be required to remove large clumps of infected material or treat the effects of severe valve damage. It is important to prevent the development of endocarditis in susceptible individuals by administering prophylactic antibiotics, for instance when undergoing dental or genitourinary procedures that may be associated with a bacteraemia. iii. Kawasaki diseaseKawasaki disease is a rare disease that primarily occurs in children under the age of 5 years. It is an inflammatory condition associated with skin rashes, fever, cracked lips and peeling of the skin of the hands and feet. It causes inflammation of blood vessels including the coronary arteries that may result in coronary aneurysms and myocardial infarction. Treatment is symptomatic with anti-inflammatory agents (e.g. high dose aspirin) iv. Other causes of acquired heart disease in childrenInfective pericarditis may occur secondary to septicaemia or pulmonary infection. Viral infection may result in cardiomyopathy. AIDS related myocarditis is seen in end stage disease. Heart failure may also be a late manifestation of severe anaemia or severe malnutrition 3. Recognising Cardiac Disease in ChildrenAlthough rare in children, is essential that the anaesthetist recognises the presence of heart disease prior to surgery:
Children rarely present with the symptoms classically associated with heart disease in adults (chest pain, shortness of breath, swollen ankles) – rather they present with a variety of symptoms such as failure to thrive, frequent chest infections, or unexplained ‘funny turns’. A careful history and examination is key, as are special investigations such as CXR, ECG and pulse oximetry. An echocardiogram is the gold standard investigation required to confirm the diagnosis. Cardiac catheterisation is used as a diagnostic tool to answer specific questions, for instance to measure the pulmonary vascular resistance, or investigate the anatomy of the pulmonary vessels. i. HistoryThere are important features to identify in the history including pregnancy and birth history, any cardiac symptoms, and a general enquiry: Pregnancy – maternal disease, drug and alcohol intake Birth history – history of prematurity associated with PDA. Birth asphyxia associated with persistent foetal circulation (persistent pulmonary hypertension of the newborn, PPHN) Cardiac symptoms
General enquiry – this may reveal other symptoms suggestive of a complex congenital disorder such as Down’s syndrome, a family history of cardiac disease, or symptoms suggestive of acquired heart disease such as rheumatic fever or endocarditis ii. ExaminationThe child should be systematically examined and in particular for features of cardiac disease following the standard routine of inspection, palpation, percussion and auscultation: Inspection
Palpation
Percussion
Auscultation
Table 5. Cardiac murmurs in children Innocent cardiac murmurs. The commonest murmur heard in children is the functional, innocent or physiological heart murmur, which is heard in 10% of normal children. The classical innocent murmur in children is the known as the ‘Still’s’ murmur. Innocent murmurs may also be due to flow murmurs associated with increased cardiac output, heard in children with a fever or anaemia. The heart is structurally normal in all children with an innocent murmur. A murmur in a child may be classified as innocent if the child has no other signs or symptoms of cardiac disease, and the murmur has certain characteristic features:
iii. Investigations Special investigations include the CXR, ECG, echocardiography and cardiac catheterisation. Chest X-Ray. In looking at the CXR in a child, always consider the age of the patient and if the film was taken in the sitting or lying position. The CXR should be evaluated systematically: A – Adequacy and alignment The film should be sufficiently penetrated to just visualise the disc spaces of the lower thoracic vertebrae through the heart shadow. At least 5 anterior rib ends should be seen above the diaphragm on the right hand side. Alignment can be assessed by ensuring that the medial ends of both clavicles are equally spaced about the spinous processes of the upper thoracic vertebrae. B – Bones Check the ribs, clavicles and vertebrae. (Rib notching is sometimes seen in severe coarctation of the aorta) C – Cartilage and Soft Tissues Lungs: Compare side to side and upper, middle and lower third of the chest. Look for pleural effusions, pneumothorax, vascular markings that are increased or decreased (plethoric or oligaemic), fluid in the fissures, white lung areas which could be consolidation or haemorrhage. Heart: Look at the size of the heart, is it enlarged? Is the shape unusual? (see below). In normal infants the heart is up to 60% of the thoracic diameter, 50% thereafter. Remember that a normal cardiac shadow does not rule out cardiac disease. The upper mediastinum: In children under the age of 18 months, the normal thymus may simulate superior mediastinal widening (above the level of the carina). D – Diaphragms The boarder between the heart and the diaphragm and the diaphragm and the ribs (cardiophrenic and costophrenic angles) should be clear on both sides. Loss of definition of the left diaphragm behind the heart suggests left lower lobe collapse, an abnormal hump suggests diaphragmatic rupture, a hazy diaphragm suggests effusion or collapse in the bordering lung segment, and an elevated diaphragm suggests phrenic nerve palsy. There are some classical appearances of the CXR in children:
The electrocardiogram (ECG)The ECG may be useful to investigate rhythm and conduction abnormalities, as well as assessing chamber hypertrophy and strain. Interpretation of the paediatric ECG is complex and must take the child’s age into account, with comparison to tables of normal values. EchocardiographyEchocardiography is a form of cardiac imaging that uses reflection of ultrasound pulses from interfaces between tissue planes. It can be used to generate detailed real time images of the cardiac anatomy. Doppler ultrasound may be used to estimate pressure gradients across valves and VSDs. Echocardiography has become the standard investigation for all patients with valvular heart disease, congenital heart disease, myocardial and pericardial disease, and in assessing myocardial function. Cardiac CatheterisationCardiac catheterisation is used to answer specific diagnostic questions in children with congenital heart disease. A catheter can be passed into the heart chambers under X-ray control to measure intracardiac pressures and oxygen saturations, or for radiological imaging by injection of contrast media. Interventional cardiology is a growing speciality that provides definitive treatment for a growing number of conditions, for instance closure of ASD or PDA by insertion of occlusion devices, balloon dilatation of pulmonary stenosis, or diathermy ablation of abnormal conduction pathways. Further readingPaediatric cardiology: an introduction. Archer N, Burch M. Chapman Hall Medical. Philadelphia 1998 Answers to Questions.Most answers are covered in the tutorial text.
What are the signs and symptoms of cardiac failure in a child?Symptoms may include:. Visible swelling of the legs, ankles, eyelids, face, and (occasionally) abdomen.. Abnormally fast breathing.. Shortness of breath or labored breathing.. Fatigue.. Nausea.. Falling asleep when feeding or becoming too tired to eat.. Lack of appetite.. What is the common finding for children with a cardiac problem?Signs in older children can include becoming easily short of breath, easily tiring, or fainting during exercise or activity. They may also have swelling in the hands, the ankles and the feet. Your pediatrician may also hear an abnormal heartbeat or abnormal heart sound called a murmur during a checkup.
What are the signs and symptoms of cardiovascular?What are the symptoms of cardiovascular heart disease?. chest pain.. pain, weakness or numb legs and/or arms.. breathlessness.. very fast or slow heartbeat, or palpitations.. feeling dizzy, lightheaded or faint.. fatigue.. swollen limbs.. What are 3 warning signs or symptoms of cardiovascular disease?Symptoms. Chest pain, chest tightness, chest pressure and chest discomfort (angina). Shortness of breath.. Pain in the neck, jaw, throat, upper belly area or back.. Pain, numbness, weakness or coldness in the legs or arms if the blood vessels in those body areas are narrowed.. |