Tuesday, April 24, 2012

MITRAL VALVE DISEASE

MITRAL STENOSIS
Aetiology and pathophysiology
Mitral stenosis is almost always rheumatic in origin, although in the elderly it can be caused by heavy calcification of the mitral valve apparatus. There is also a rare form of congenital mitral stenosis.
In rheumatic mitral stenosis, the mitral valve orifice is slowly diminished by progressive fibrosis, calcification of the valve leaflets, and fusion of the cusps and subvalvular apparatus. The flow of blood from left atrium to left ventricle is restricted and left atrial pressure rises, leading to pulmonary venous congestion and breathlessness. There is dilatation and hypertrophy of the left atrium, and left ventricular filling becomes more dependent on left atrial contraction.
Any increase in heart rate shortens diastole when the mitral valve is open, and produces a further rise in left atrial pressure; situations that demand an increase in cardiac output will also increase left atrial pressure. Exercise and pregnancy are therefore poorly tolerated.
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The mitral valve orifice is normally about 5 cm2 in diastole and may be reduced to 1 cm2 or less in severe mitral stenosis. Patients usually remain asymptomatic until the stenosis is approximately 2 cm2 or less. At first, symptoms occur only on exercise; however, in severe stenosis, left atrial pressure is permanently elevated and symptoms may occur at rest. Reduced lung compliance, due to chronic pulmonary venous congestion, contributes to breathlessness and a low cardiac output may cause fatigue.
Atrial fibrillation due to progressive dilatation of the left atrium is very common. The onset of atrial fibrillation often precipitates pulmonary oedema because the accompanying tachycardia and loss of atrial contraction frequently lead to marked haemodynamic deterioration with a rapid rise in left atrial pressure. In contrast, a more gradual rise in left atrial pressure tends to cause an increase in pulmonary vascular resistance, which leads to pulmonary artery hypertension that may protect the patient from pulmonary oedema. Pulmonary hypertension may lead to right ventricular hypertrophy and dilatation, tricuspid regurgitation and right heart failure.
Less than 20% of patients remain in sinus rhythm; many of these have a small fibrotic left atrium and severe pulmonary hypertension.
All patients with mitral stenosis, and particularly those with atrial fibrillation, are at risk from left atrial thrombosis and systemic thromboembolism. Prior to the advent of anticoagulant therapy, emboli caused one-quarter of all deaths in this condition.
Clinical features
These are shown in Box 18.104.
Symptoms
18.104 CLINICAL FEATURES OF MITRAL STENOSIS
Symptoms
  • Breathlessness (pulmonary congestion)
  • Fatigue (low cardiac output)
  • Oedema, ascites (right heart failure)
  • Palpitation (atrial fibrillation)
  • Haemoptysis (pulmonary congestion, pulmonary embolism)
  • Cough (pulmonary congestion)
  • Chest pain (pulmonary hypertension)
  • Symptoms of thromboembolic complications (e.g. stroke, ischaemic limb)

Signs
  • Atrial fibrillation
  • Mitral facies
  • Auscultation
    • Loud first heart sound, opening snap
    • Mid-diastolic murmur
  • Signs of raised pulmonary capillary pressure
    • Crepitations, pulmonary oedema, effusions
  • Signs of pulmonary hypertension
    • RV heave, loud P2

Effort-related dyspnoea is usually the dominant symptom. Exercise tolerance typically diminishes very slowly over many years and patients often do not appreciate the extent of their disability. Eventually symptoms occur at rest. Acute pulmonary oedema or pulmonary hypertension can lead to haemoptysis. Systemic embolism may be a presenting feature.
Signs
The forces that open and close the mitral valve increase as left atrial pressure rises. The first heart sound (S1) is therefore often unusually loud and may even be palpable (tapping apex beat). An opening snap may be audible and moves closer to the second sound (S2) as the stenosis becomes more severe and left atrial pressure rises. However, the first heart sound and opening snap may be inaudible if the valve is heavily calcified.
Turbulent flow produces the characteristic low-pitched mid-diastolic murmur and sometimes a thrill (Fig. 18.89). The murmur is accentuated by exercise and during atrial systole (pre-systolic accentuation). Early in the disease, a pre-systolic murmur may be the only auscultatory abnormality, but in patients with symptoms, the murmur usually extends from the opening snap to the first heart sound. Coexisting mitral regurgitation causes a pansystolic murmur which radiates towards the axilla.
If pulmonary hypertension supervenes there may be a right ventricular heave at the left sternal edge (due to right ventricular hypertrophy) and accentuation of the pulmonary component of the second heart sound. Tricuspid regurgitation secondary to right ventricular dilatation causes a systolic murmur and systolic waves in the venous pulse.
The physical signs of mitral stenosis are often found before symptoms develop, and their recognition is of particular importance in pregnancy.
Investigations
18.105 INVESTIGATIONS IN MITRAL STENOSIS
ECG
  • Left atrial hypertrophy (if not in AF)
  • Right ventricular hypertrophy
Chest X-ray
  • Enlarged left atrium
  • Signs of pulmonary venous congestion
Echo
  • Thickened immobile cusps
  • Reduced valve area
  • Reduced rate of diastolic filling of LV
Doppler
  • Pressure gradient across mitral valve
  • Pulmonary artery pressure
  • Left ventricular function
Cardiac catheterisation
  • Assessment of coexisting coronary artery disease and mitral regurgitation

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Figure 18.89 Mitral stenosis: murmur and illustration of the diastolic pressure gradient between left atrium and left ventricle. (Mean gradient is reflected by the area between LA and LV in diastole.) The first heart sound is loud, there is an opening snap (OS) and mid-diastolic murmur (MDM) with pre-systolic accentuation.
Echocardiogram showing reduced opening of the mitral valve in diastole.
Colour Doppler showing turbulent flow.
The ECG (Box 18.105) may show either the bifid P waves (P mitrale) associated with left atrial hypertrophy, or atrial fibrillation. There may also be evidence of right ventricular hypertrophy (pulmonary hypertension). The chest X-ray (Fig. 18.9, p. 530) may show enlargement of the left atrium and its appendage, enlargement of the main pulmonary artery and enlargement of the upper pulmonary veins and horizontal linear shadows in the costophrenic angles.
Doppler echocardiography provides the definitive evaluation of mitral stenosis (Box 18.105 and Fig. 18.89). Cardiac catheterisation has a role in assessing coexisting mitral regurgitation and coronary artery disease.
Management
Patients with minor symptoms should be treated medically, but the definitive treatment of mitral stenosis is by balloon valvuloplasty, mitral valvotomy or mitral valve replacement. Intervention should be considered if the patient remains symptomatic despite medical treatment or if pulmonary hypertension develops.
Medical management
This consists of anticoagulants to reduce the risk of systemic embolism, a combination of digoxin, β-blockers or rate-limiting calcium antagonists to control the ventricular rate in atrial fibrillation (or to prevent a rapid ventricular rate if atrial fibrillation should develop), diuretics to control pulmonary congestion, and antibiotic prophylaxis against infective endocarditis (Box 18.125, p. 633).
Mitral balloon valvuloplasty
This is the treatment of choice if the appropriate criteria are fulfilled (Box 18.106 and Fig. 18.14, p. 533). Closed or open mitral valvotomy may be used if the facilities or expertise for valvuloplasty are not available. Patients who have undergone mitral valvuloplasty or valvotomy should receive antibiotic prophylaxis against infective endocarditis and should be followed up at 1-2-yearly intervals because restenosis may occur. Clinical symptoms and signs are a guide to the severity of mitral restenosis, but Doppler echocardiography provides a more accurate assessment.
18.106 CRITERIA FOR MITRAL VALVULOPLASTY
  • Significant symptoms
  • Isolated mitral stenosis
  • No (or trivial) mitral regurgitation
  • Mobile, non-calcified valve/subvalve apparatus on echo
  • Left atrium free of thrombus
For further information see http://www.acc.orgOpen this link in a new window" target="_blank">www.acc.org, which has comprehensive guidelines on valvular heart disease.

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18.107 CAUSES OF MITRAL REGURGITATION
  • Mitral valve prolapse
  • Dilatation of the left ventricle and mitral valve ring (e.g. coronary artery disease, cardiomyopathy)
  • Damage to valve cusps and chordae (e.g. rheumatic heart disease, endocarditis)
  • Damage to papillary muscle
  • Myocardial infarction
Mitral valve replacement
Valve replacement is indicated if there is substantial mitral reflux, or if the valve is rigid and calcified (p. 633).
MITRAL REGURGITATION
Aetiology and pathophysiology
Rheumatic disease is the principal cause of mitral regurgitation in countries where rheumatic fever is common, but elsewhere, including in the UK, other causes are more important (Box 18.107). Mitral regurgitation may also follow mitral valvotomy or valvuloplasty.
Chronic mitral regurgitation causes gradual dilatation of the left atrium with little increase in pressure and therefore relatively few symptoms. Nevertheless, the left ventricle dilates slowly and the left ventricular diastolic and left atrial pressures gradually increase as a result of chronic volume overload of the left ventricle; breathlessness and pulmonary oedema eventually supervene. In contrast, acute mitral regurgitation tends to cause a rapid rise in left atrial pressure (because left atrial compliance is normal) and marked symptomatic deterioration.
Mitral valve prolapse
This is also known as 'floppy' mitral valve and is one of the more common causes of mild mitral regurgitation. It is caused by congenital anomalies or degenerative myxomatous changes and is sometimes a feature of connective tissue disorders such as Marfan's syndrome (p. 605).
In the mildest forms of mitral prolapse, the valve remains competent but bulges back into the atrium during systole, causing a mid-systolic click but no murmur. Occasionally, multiple clicks are audible. In the presence of a regurgitant valve, the click is followed by a late systolic murmur which lengthens as the regurgitation becomes more severe. A click is not always audible and the physical signs may vary with both posture and respiration.
Progressive elongation of the chordae tendineae may lead to increasing mitral regurgitation, and if chordal rupture occurs, regurgitation may suddenly become severe. These complications are rare before the fifth or sixth decade of life.
Haemodynamically significant mitral valve prolapse can predispose to infective endocarditis and requires antibiotic prophylaxis. Mitral valve prolapse is also associated with a variety of typically benign arrhythmias, atypical chest pain and a very small risk of embolic stroke or transient ischaemic attack. Nevertheless, the overall long-term prognosis is good. An echocardiogram of mitral valve prolapse is shown in Figure 18.90.
Other causes of mitral regurgitation
Mitral valve function depends on the chordae tendineae and their papillary muscles; dilatation of the left ventricle distorts the geometry of these and may cause mitral regurgitation. Dilated cardiomyopathy and the impaired ventricular function that results from coronary artery disease are common causes of so-called 'functional' mitral regurgitation. Ischaemia or infarction of the papillary muscles may also cause mitral regurgitation. Endocarditis may lead to distortion or perforation of the valve leaflets and is an important cause of acute mitral regurgitation.
Clinical features
These are summarised in Box 18.108.
The symptoms depend on how suddenly the regurgitation develops. Chronic mitral regurgitation produces a symptom complex that is similar to that of mitral stenosis, but sudden-onset mitral regurgitation usually presents with acute pulmonary oedema.
The regurgitant jet causes an apical systolic murmur (Fig. 18.90) which often radiates into the axilla, and may be accompanied by a thrill. The first heart sound is quiet because valve closure is abnormal. Increased forward flow through the mitral valve may give rise to a loud third heart sound and even a short mid-diastolic murmur. The apex beat feels active and rocking due to left ventricular volume overload and is usually displaced to the left as a result of dilatation of the left ventricle.
18.108 CLINICAL FEATURES OF MITRAL REGURGITATION
Symptoms
  • Dyspnoea (pulmonary venous congestion)
  • Fatigue (low cardiac output)
  • Palpitation (AF, increased stroke volume)
  • Oedema, ascites (right heart failure)
Signs
  • Atrial fibrillation/flutter
  • Cardiomegaly-displaced hyperdynamic apex beat
  • Apical pansystolic murmur ± thrill
  • Soft S1, apical S3
  • Signs of pulmonary venous congestion (crepitations, pulmonary oedema, effusions)
  • Signs of pulmonary hypertension and right heart failure

Investigations
These are shown in Box 18.109 and include chest X-ray, ECG and Doppler echocardiography. Atrial fibrillation is common, as a consequence of atrial dilatation. At cardiac catheterisation the severity of mitral regurgitation may be indicated by the size of the v (systolic) waves in the left atrial or PAW pressure trace, or by left ventriculography; however, this is not always reliable, as left atrial compliance may vary. In practice, a common problem lies in deciding on the extent to which cardiac failure is due to mitral regurgitation as opposed to impaired left ventricular function.
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Figure 18.90 Mitral regurgitation: radiation of the murmur to the axilla and illustration of systolic wave in left atrial pressure. The first sound is normal or soft and merges with a pansystolic murmur (PSM) extending to the second heart sound. A third heart sound occurs with severe regurgitation. The left atrium and ventricle become dilated.
A transoesophageal echocardiogram shows an example of mitral valve prolapse, with one leaflet bulging towards the left atrium (LA; arrow).
This results in a jet of mitral regurgitation on colour Doppler (arrow).
18.109 INVESTIGATIONS IN MITRAL REGURGITATION
ECG
  • Left atrial hypertrophy (if not in AF)
  • Left ventricular hypertrophy
Chest X-ray
  • Enlarged left atrium
  • Enlarged left ventricle
  • Pulmonary venous congestion
  • Pulmonary oedema (if acute)
Echo
  • Dilated LA, LV
  • Dynamic LV (unless myocardial dysfunction predominates)
  • Structural abnormalities of mitral valve (e.g. prolapse)
Doppler
  • Detects and quantifies regurgitation
Cardiac catheterisation
  • Dilated LA, dilated LV, mitral regurgitation
  • Pulmonary hypertension
  • Coexisting coronary artery disease

Management
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18.110 MEDICAL MANAGEMENT OF MITRAL REGURGITATION
  • Diuretics
  • Vasodilators, e.g. ACE inhibitors (p. 549)
  • Digoxin if atrial fibrillation is present
  • Anticoagulants if atrial fibrillation is present
  • Antibiotic prophylaxis against infective endocarditis
Mitral regurgitation of moderate severity can be treated medically (Box 18.110). In all patients with mitral regurgitation, high afterload may worsen the degree of regurgitation and hypertension should be treated with vasodilating drugs such as ACE inhibitors. Patients should be reviewed at regular intervals because worsening symptoms, progressive radiological cardiac enlargement or echocardiographic evidence of deteriorating left ventricular function are indications for surgical intervention (mitral valve replacement or repair). Mitral valve repair can be used to treat most forms of mitral valve prolapse and offers many advantages when compared to mitral valve replacement. Indeed, it is now advocated for severe regurgitation even in asymptomatic patients because results are excellent and early repair has been shown to prevent irreversible left ventricular damage. Commonly, mitral regurgitation accompanies the ventricular dilatation and dysfunction that accompany coronary artery disease. If such patients are to undergo coronary bypass graft surgery it is common practice to repair the valve and restore mitral valve function by inserting an annuloplasty ring to overcome annular dilatation and to bring the valve leaflets closer together. A common dilemma in patients with ventricular dilatation and mitral regurgitation is to determine which of the two abnormalities is the predominant problem. If, for example, ventricular dilatation is the underlying cause of mitral regurgitation, then mitral valve repair or replacement may actually worsen ventricular function as the ventricle can no longer empty into the low-pressure left atrium.

RHEUMATIC HEART DISEASE

ACUTE RHEUMATIC FEVER
Incidence and pathogenesis
Acute rheumatic fever (ARF) usually affects children (most commonly between 5 and 15 years) or young adults, and has become very rare in Western Europe and North America. Nevertheless, it remains endemic in parts of Asia, Africa and South America, with an annual incidence in some countries of more than 100 per 100 000; it is still the most common cause of acquired heart disease in childhood and adolescence.
The condition is triggered by an immune-mediated delayed response to infection with specific strains of group A streptococci that possess antigens which may cross-react with cardiac myosin and sarcolemmal membrane protein. Antibodies produced against the streptococcal antigens mediate inflammation in the endocardium, myocardium and pericardium as well as the joints and skin. Histologically, fibrinoid degeneration is seen in the collagen of connective tissues. Aschoff nodules are pathognomonic and occur only in the heart. They are composed of multinucleated giant cells surrounded by macrophages and T lymphocytes, and are not seen until the subacute or chronic phases of rheumatic carditis.
Clinical features
Click to view full size
Figure 18.88 Clinical features of rheumatic fever. Bold labels indicate Jones major criteria. (CCF = congestive cardiac failure)
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18.102 JONES CRITERIA FOR THE DIAGNOSIS OF RHEUMATIC FEVER
Major manifestations
  • Carditis
  • Polyarthritis
  • Chorea
  • Erythema marginatum
  • Subcutaneous nodules

Minor manifestations
  • Fever
  • Arthralgia
  • Previous rheumatic fever
  • Raised ESR or CRP
  • Leucocytosis
  • First-degree AV block
PLUS
  • Supporting evidence of preceding streptococcal infection: recent scarlet fever, raised antistreptolysin 0 or other streptococcal antibody titre, positive throat culture
N.B. Evidence of recent streptococcal infection is particularly important if there is only one major manifestation.

ARF is a multisystem disorder that typically follows an episode of streptococcal pharyngitis and usually presents with fever, anorexia, lethargy and joint pains. Symptoms characteristically occur 2-3 weeks after the initial attack of pharyngitis but the patient may give no history of sore throat. Arthritis occurs in approximately 75% of patients; other features include rashes, carditis and neurological changes (Fig. 18.88). The diagnosis, according to the revised Jones criteria, is based upon two or more major manifestations, or one major and two or more minor manifestations; evidence of preceding streptococcal infection is also required (Box 18.102). Only about 25% of patients will have a positive culture for group A streptococcus at the time of diagnosis because there is a latent period between infection and presentation; serological evidence of recent streptococcal infection with a raised antistreptolysin O (ASO) antibody titre may therefore be helpful. A diagnosis of presumptive ARF can be made without evidence of preceding streptococcal infection in cases of isolated chorea or pancarditis, if other causes for these have been excluded. In cases of established rheumatic heart disease or prior ARF, a diagnosis of ARF can be made based only on the presence of multiple minor criteria and evidence of preceding group A streptococcal pharyngitis.
Carditis
This is a 'pancarditis' that involves the endocardium, myocardium and pericardium to varying degrees; its incidence declines with increasing age, ranging from 90% at 3 years to around 30% in adolescence. Carditis may manifest as breathlessness (due to heart failure or pericardial effusion), palpitations or chest pain (usually due to pericarditis or pancarditis). Other features include tachycardia, cardiac enlargement and new or changed cardiac murmurs. A soft systolic murmur due to mitral regurgitation is very common. A soft mid-diastolic murmur (the Carey Coombs murmur) is typically due to valvulitis, with nodules forming on the mitral valve leaflets. Aortic regurgitation occurs in about 50% of cases but the tricuspid and pulmonary valves are rarely involved in the acute process. Pericarditis may cause chest pain, a pericardial friction rub and precordial tenderness. Cardiac failure may be due to myocardial dysfunction and/or mitral or aortic regurgitation. ECG changes are common and include ST and T wave changes; conduction defects sometimes occur and may cause syncope.
Arthritis
This is usually an early feature that tends to occur when streptococcal antibody titres are high. It is the most common major manifestation and is characterised by acute, painful, asymmetric and migratory inflammation of the large joints (typically the knees, ankles, elbows and wrists). The joints are involved in quick succession and are usually red, swollen and tender for between a day and up to 4 weeks. The pain characteristically responds to aspirin; if it does not, the diagnosis is in doubt.
Skin lesions
Erythema marginatum occurs in less than 5% of patients. The lesions start as red macules (blotches) which fade in the centre but remain red at the edges and occur mainly on the trunk and proximal extremities but not the face. The resulting red rings or 'margins' may coalesce or overlap (Fig. 18.88).
Subcutaneous nodules occur in 5-7% of patients. They are small (0.5-2.0 cm), firm and painless, and are best felt over extensor surfaces of bone or tendons. Nodules typically appear more than 3 weeks after the onset of other manifestations and are therefore a feature that helps to confirm rather than make the diagnosis.
Other systemic manifestations are rare, but include pleurisy, pleural effusion and pneumonia.
Sydenham's chorea (St Vitus dance)
This is a late neurological manifestation that typically appears at least 3 months after the episode of ARF when all the other signs may have disappeared. It occurs in up to one-third of cases and is more common in females. Emotional lability may be the first feature and is typically followed by purposeless involuntary choreiform movements of the hands, feet or face. Speech may be explosive and halting. Spontaneous recovery usually occurs within a few months. Approximately one-quarter of patients with Sydenham's chorea will go on to develop chronic rheumatic valve disease.
Investigations
These are listed in Box 18.103. The ESR and CRP are non-specific markers of systemic inflammation, and are useful for monitoring progress of the disease. Positive throat swab cultures are obtained in only 10-25% of cases of ARF. ASO titres are normal in about one-fifth of adult cases of rheumatic fever and most cases of chorea. Echocardiography typically shows mitral regurgitation with dilatation of the mitral annulus and prolapse of the anterior mitral leaflet; other common findings are aortic regurgitation and pericardial effusion.
18.103 INVESTIGATIONS IN ACUTE RHEUMATIC FEVER
Evidence of a systemic illness (non-specific)
  • Leucocytosis, raised ESR, raised CRP
Evidence of preceding streptococcal infection (specific)
  • Throat swab culture: group A β-haemolytic streptococci (also from family members and contacts)
  • Antistreptolysin 0 antibodies (ASO titres): rising titres, or levels of > 200 U (adults) or > 300 U (children)
Evidence of carditis
  • Chest X-ray: cardiomegaly; pulmonary congestion
  • ECG: first- and rarely second-degree heart block; features of pericarditis; T-wave inversion; reduction in QRS voltages
  • Echocardiography: cardiac dilatation and valve abnormalities

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Treatment of the acute attack
A single dose of benzyl penicillin 1.2 million U i.m. or oral phenoxymethylpenicillin 250 mg 6-hourly for 10 days should be given on diagnosis to eliminate any residual streptococcal infection. If the patient is penicillin-allergic, erythromycin or a cephalosporin can be used. Treatment is then directed towards limiting cardiac damage and relieving symptoms.
Bed rest and supportive therapy
Bed rest is important as it lessens joint pain and reduces cardiac workload. The duration of bed rest should be guided by symptoms and markers of inflammation (e.g. temperature, leucocyte count and ESR) and should be continued until these have settled. Patients can then return to normal physical activity, but strenuous exercise should be avoided in those who have had carditis.
Cardiac failure should be treated as necessary. Some patients, particularly those in early adolescence, develop a fulminant form of the disease with severe mitral regurgitation and sometimes concomitant aortic regurgitation. If heart failure does not respond to medical treatment in these cases, valve replacement may be necessary and is often associated with a dramatic decline in rheumatic activity. Atrioventricular block is seldom progressive and pacemaker insertion is rarely needed.
Aspirin
This will usually relieve the symptoms of arthritis rapidly and a prompt response (within 24 hours) helps to confirm the diagnosis. A reasonable starting dose is 60 mg/kg body weight per day, divided into six doses. In adults, 100 mg/kg per day may be needed up to the limits of tolerance or a maximum of 8 g per day. Mild toxic effects include nausea, tinnitus and deafness; more serious ones are vomiting, tachypnoea and acidosis. Aspirin should be continued until the ESR has fallen and then gradually tailed off.
Corticosteroids
These produce more rapid symptomatic relief than aspirin, and are indicated in cases with carditis or severe arthritis. There is no evidence that long-term steroids are beneficial. Prednisolone, 1.0-2.0 mg/kg per day in divided doses, should be continued until the ESR is normal then tailed off.
Secondary prevention
Patients are susceptible to additional attacks of rheumatic fever if further streptococcal infection occurs, and long-term prophylaxis with penicillin should be given as benzyl penicillin 1.2 million U i.m. monthly (if compliance is in doubt) or oral phenoxymethylpenicillin 250 mg 12-hourly. Sulfadiazine or erythromycin may be used if the patient is allergic to penicillin (sulphonamides prevent infection but are not effective in the eradication of group A streptococci). Further attacks of rheumatic fever are unusual after the age of 21, at which age treatment may be stopped. However, treatment should be extended if an attack has occurred in the last 5 years, or the patient lives in an area of high prevalence or has an occupation (e.g. teaching) with high exposure to streptococcal infection. In those with residual heart disease, prophylaxis should continue until 10 years after the last episode or 40 years of age, whichever is longer. Long-term antibiotic prophylaxis prevents another attack of ARF but does not protect against infective endocarditis.
CHRONIC RHEUMATIC HEART DISEASE
Chronic valvular heart disease develops in at least half of those affected by rheumatic fever with carditis. Two-thirds of cases occur in women. Some episodes of rheumatic fever may pass unrecognised and it is only possible to elicit a history of rheumatic fever or chorea in about half of all patients with chronic rheumatic heart disease.
The mitral valve is affected in more than 90% of cases; the aortic valve is the next most frequently affected, followed by the tricuspid and then the pulmonary valve. Isolated mitral stenosis accounts for about 25% of all cases of rheumatic heart disease, and an additional 40% have mixed mitral stenosis and regurgitation.
Valve disease may be symptomatic during fulminant forms of ARF, but may remain asymptomatic for many years.
Pathology
In contrast to the destructive lytic process of ARF, the main pathological process in chronic rheumatic heart disease is progressive fibrosis. The heart valves are predominantly affected but involvement of the pericardium and myocardium may contribute to heart failure and conduction disorders. Fusion of the mitral valve commissures and shortening of the chordae tendineae may lead to mitral stenosis with or without regurgitation. Similar changes in the aortic and tricuspid valves produce distortion and rigidity of the cusps, leading to stenosis and/or regurgitation. Once a valve has been damaged, the altered haemodynamic stresses perpetuate and extend the damage, even in the absence of a continuing rheumatic process.

ASSESSING SICK CHILD.

Emergency signs include:
obstructed breathing
Severe respiratory distress
Central cyanosis
Signs of shock (cold hands; capillary refill longer than 3 seconds; weak,
Fast pulse)
Coma
Convulsions
Signs of severe dehydration in a child with diarrhea (lethargy, sunken eyes,
Very slow return after pinching the skin—any two of these).
NOTE
Children with emergency signs require immediate treatment to avert death.
PRIORITY SIGNS
Tiny baby: any sick child aged under 2 months
Temperature: child is very hot
Trauma or other urgent surgical condition
Pallor (severe)
If emergency signs are found:
• Call an experienced health professional to help if available, but do not delay
starting the treatment. Stay calm and work with other health workers who
SUMMARY OF STEPS IN EMERGENCY TRIAGE ASSESSMENT AND TREATMENT
3
1. ETAT
may be required to give the treatment, because a very sick child may need
several treatments at once. The most experienced health professional should
continue assessing the child (see Chapter 2, page 37), to identify all
underlying problems and develop a treatment plan.
• Carry out emergency investigations (blood glucose, blood smear, haemoglobin).
Send blood for typing and cross-matching if the child is in shock,
or appears to be severely anaemic, or is bleeding significantly.
• After giving emergency treatment, proceed immediately to assessing,
diagnosing and treating the underlying problem.
Tables of common differential diagnoses for emergency signs are provided
from page 20 onwards.
If no emergency signs are found, check for priority signs:
■ Tiny baby: any sick child aged under 2 months
■ Temperature: child is very hot
■ Trauma or other urgent surgical condition
■ Pallor (severe)
■ Poisoning
■ Pain (severe)
■ Respiratory distress
■ Restless, continuously irritable, or lethargic
■ Referral (urgent)
■ Malnutrition: visible severe wasting
■ Oedema of both feet
■ Burns (major)
The above can be remembered with the help of “3TPR MOB”.
These children need prompt assessment (no waiting in the queue) to determine
what further treatment is needed. Move the child with any priority sign to the
front of the queue to be assessed next. If a child has trauma or other surgical
problems, get surgical help where available.
SUMMARY OF STEPS IN EMERGENCY TRIAGE ASSESSMENT AND TREATMENT
4
1. ETAT
CHART 2. Triage of all sick children
EMERGENCY SIGNS
If any sign positive: give treatment(s), call for help, draw blood for
emergency laboratory investigations (glucose, malaria smear, Hb)
ASSESS TREAT
Do not move neck if cervical
spine injury possible
If foreign body aspiration
➤ Manage airway in choking
child (Chart 3)
If no foreign body aspiration
➤ Manage airway (Chart 4)
➤ Give oxygen (Chart 5)
➤ Make sure child is warm
➤ Stop any bleeding
➤ Give oxygen (Chart 5)
➤ Make sure child is warm
If no severe malnutrition:
➤ Insert IV and begin giving
fluids rapidly (Chart 7)
If not able to insert
peripheral IV, insert an
intraosseous or external
jugular line
(see pages 310, 312)
If severe malnutrition:
If lethargic or unconscious:
➤ Give IV glucose (Chart 10)
➤ Insert IV line and give
fluids (Chart 8)
If not lethargic or
unconscious:
➤ Give glucose orally or by
NG tube
➤ Proceed immediately to full
assessment and treatment
ANY SIGN
POSITIVE
ANY SIGN
POSITIVE
Check for
severe
malnutrition
Airway and
breathing
■ Obstructed breathing,
or
■ Central cyanosis,
or
■ Severe respiratory distress
Circulation
Cold hands with:
■ Capillary refill
longer than
3 seconds,
and
■ Weak and fast pulse
5
1. ETAT
CHART 2. Triage of all sick children (continued)
TREAT
Do not move neck if cervical
spine injury possible
➤ Manage airway (Chart 3)
➤ If convulsing, give diazepam or
paraldehyde rectally (Chart 9)
➤ Position the unconscious child (if
head or neck trauma is suspected,
stabilize the neck first) (Chart 6)
➤ Give IV glucose (Chart 10)
➤ Make sure child is warm.
If no severe malnutrition:
➤ Insert IV line and begin giving fluids
rapidly following Chart 11 and
Diarrhoea Treatment Plan C in
hospital (Chart 13, page 114)
If severe malnutrition:
➤ Do not insert IV
➤ Proceed immediately to full
assessment and treatment (see
section 1.3, page 18)
PRIORITY SIGNS
These children need prompt assessment and treatment
EMERGENCY SIGNS
If any sign positive: give treatment(s), call for help, draw blood for
emergency laboratory investigations (glucose, malaria smear, Hb)
ASSESS
Coma/convulsing
■ Coma
or
■ Convulsing (now)
Severe
dehydration
(only in child
with diarrhoea)
Diarrhoea plus
any two of these:
■ Lethargy
■ Sunken eyes
■ Very slow skin
pinch
IF COMA OR
CONVULSING
DIARRHOEA
plus
TWO SIGNS
POSITIVE
Check for
severe
malnutrition
NON-URGENT
Proceed with assessment and further treatment according to
the child’s priority
■ Tiny baby (<2 months)
■ Temperature very high
■ Trauma or other urgent surgical
condition
■ Pallor (severe)
■ Poisoning (history of)
■ Pain (severe)
■ Respiratory distress
■ Restless, continuously irritable, or
lethargic
■ Referral (urgent)
■ Malnutrition: visible severe wasting
■ Oedema of both feet
■ Burns (major)
Note: If a child has trauma or other
surgical problems, get surgical help
or follow surgical guidelines
6
1. ETAT
CHART 3. How to manage the choking infant
➤ Lay the infant on
your arm or thigh in
a head down
position
➤ Give 5 blows to the
infant’s back with
heel of hand
➤ If obstruction
persists, turn infant
over and give
5 chest thrusts with
2 fingers, one finger
breadth below nipple
level in midline
(see diagram)
➤ If obstruction
persists, check
infant’s mouth for
any obstruction
which can be
removed
➤ If necessary, repeat
sequence with back
slaps again
Back slaps
Chest thrusts
7
1. ETAT
➤ Give 5 blows to the child’s back
with heel of hand with child sitting,
kneeling or lying
➤ If the obstruction persists, go
behind the child and pass your
arms around the child’s body;
form a fist with one hand
immediately below the child’s
sternum; place the other hand over
the fist and pull upwards into the
abdomen (see diagram); repeat
this Heimlich manoeuvre 5 times
➤ If the obstruction persists, check
the child’s mouth for any
obstruction which can be removed
➤ If necessary, repeat this sequence
with back slaps again
CHART 3. How to manage the choking child
(over 1 year of age)
Heimlich manoeuvre in
a choking older child
Slapping the back to clear airway
obstruction in a choking child
8
1. ETAT
CHART 4. How to manage the airway in a child
with obstructed breathing (or who has just stopped
breathing) where no neck trauma is suspected
Child conscious
1. Inspect mouth and
remove foreign
body, if present
2. Clear secretions
from throat
3. Let child assume
position of maximal
comfort
Child unconscious
1. Tilt the head as
shown
2. Inspect mouth and
remove foreign
body, if present
3. Clear secretions
from throat
4. Check the airway by
looking for chest
movements,
listening for breath
sounds and feeling
for breath
■ OLDER CHILD
■ INFANT
Neutral position to open the airway
in an infant
Look, listen and feel for breathing
Sniffing position to open the airway
in an older child
9
1. ETAT
CHART 4. How to manage the airway in a child
with obstructed breathing (or who has just stopped
breathing) where neck trauma or possible cervical
spine injury is suspected
1. Stabilize the neck, as shown in Chart 6
2. Inspect mouth and remove foreign body, if present
3. Clear secretions from throat
4. Check the airway by looking for chest movements, listening for breath
sounds, and feeling for breath
Use jaw thrust without head tilt. Place the 4th and 5th finger behind
the angle of the jaw and move it upwards so that the bottom of the jaw
is thrust forwards, at 90° to the body
If the child is still not breathing after
carrying out the above, ventilate with
bag and mask
10
1. ETAT
Give oxygen through nasal
prongs or a nasal catheter
■ Nasal Prongs
➤ Place the prongs just inside
the nostrils and secure with
tape.
■ Nasal Catheter
➤ Use an 8 FG size tube
➤ Measure the distance from
the side of the nostril to
the inner eyebrow margin
with the catheter
➤ Insert the catheter to
this depth
➤ Secure with tape
Start oxygen flow at
1–2 litres/minute
(see pages 281–284)
CHART 5. How to give oxygen
11
1. ETAT
CHART 6. How to position the unconscious child
■ If neck trauma is not suspected:
➤ Turn the child on the side to reduce risk of aspiration.
➤ Keep the neck slightly extended and stabilize by placing cheek on
one hand
➤ Bend one leg to stabilize the body position
■ If neck trauma is suspected:
➤ Stabilize the child’s neck and keep the child lying on the back:
➤ Tape the child’s forehead and
chin to the sides of a firm board
to secure this position
➤ Prevent the neck from
moving by supporting the
child’s head (e.g. using
litre bags of IV fluid on
each side)
➤ If vomiting, turn on
the side, keeping
the head in line
with the body.
12
1. ETAT
CHART 7. How to give IV fluids rapidly for shock in a child
without severe malnutrition
➤ If the child is severely malnourished the fluid volume and rate are
different, so check that the child is not severely malnourished
Shock in child without severe malnutrition—Chart 7
Shock in child with severe malnutrition—Chart 8 (and section 1.3,
page 18)
➤ Insert an intravenous line (and draw blood for emergency laboratory
investigations).
➤ Attach Ringer's lactate or normal saline—make sure the infusion is
running well.
➤ Infuse 20 ml/kg as rapidly as possible.
Volume of Ringer's lactate
or normal saline solution
Age/weight (20 ml/kg)
2 months (<4 kg) 75 ml
2–<4 months (4–<6 kg) 100 ml
4–<12 months (6–<10 kg) 150 ml
1–<3 years (10–<14 kg) 250 ml
3–<5 years (14–19 kg) 350 ml
Reassess child after appropriate volume has run in
Reassess after first infusion: If no improvement, repeat 20 ml/kg as
rapidly as possible.
Reassess after second infusion: If no improvement, repeat 20 ml/kg as
rapidly as possible.
Reassess after third infusion: If no improvement, give blood 20 ml/kg
over 30 minutes (if shock is not caused
by profuse diarrhoea, in this case repeat
Ringer’s lactate or normal saline).
Reassess after fourth infusion: If no improvement, see disease-specific
treatment guidelines. You should have
established a provisional diagnosis by
now.
After improvement at any stage (pulse slows, faster capillary refill), go to
Chart 11, page 16.
13
1. ETAT
CHART 8. How to give IV fluids for shock in a child
with severe malnutrition
Give this treatment only if the child has signs of shock and is lethargic or has lost
consciousness:
➤ Insert an IV line (and draw blood for emergency laboratory investigations)
➤ Weigh the child (or estimate the weight) to calculate the volume of fluid to be
given
➤ Give IV fluid 15 ml/kg over 1 hour. Use one of the following solutions (in order of
preference), according to availability:
— Ringer's lactate with 5% glucose (dextrose); or
— half-normal saline with 5% glucose (dextrose); or
— half-strength Darrow’s solution with 5% glucose (dextrose); or, if these are
unavailable,
— Ringer's lactate.
Weight Volume IV fluid Weight Volume IV fluid
Give over 1 hour (15 ml/kg) Give over 1 hour (15 ml/kg)
4 kg 60 ml 12 kg 180 ml
6 kg 90 ml 14 kg 210 ml
8 kg 120 ml 16 kg 240 ml
10 kg 150 ml 18 kg 270 ml
➤ Measure the pulse and breathing rate at the start and every 5–10 minutes.
If there are signs of improvement (pulse and respiratory rates fall):
— give repeat IV 15 ml/kg over 1 hour; then
— switch to oral or nasogastric rehydration with ReSoMal (see page 179),
10 ml/kg/h up to 10 hours;
— initiate refeeding with starter F-75 (see page 184).
If the child fails to improve after the first 15ml/kg IV, assume the child has septic
shock:
— give maintenance IV fluid (4 ml/kg/h) while waiting for blood;
— when blood is available, transfuse fresh whole blood at 10 ml/kg slowly over
3 hours (use packed cells if in cardiac failure); then
— initiate refeeding with starter F-75 (see page 184);
— start antibiotic treatment (see page 182).
If the child deteriorates during the IV rehydration (breathing increases by
5 breaths/min or pulse by 15 beats/min), stop the infusion because IV fluid can
worsen the child’s condition.
14
1. ETAT
CHART 9. How to give diazepam (or paraldehyde) rectally
■ Give diazepam rectally:
➤ Draw up the dose from an ampoule of diazepam into a tuberculin (1
ml) syringe. Base the dose on the weight of the child, where possible.
Then remove the needle.
➤ Insert the syringe into the rectum 4 to 5 cm and inject the diazepam
solution.
➤ Hold buttocks together for a few minutes.
Diazepam given rectally Paraldehyde given
10 mg/2ml solution rectally
Age/weight Dose 0.1ml/kg Dose 0.3–0.4 ml/kg
2 weeks to 2 months (<4 kg)* 0.3 ml (1.5 mg) 1.0 ml
2–<4 months (4–<6 kg) 0.5 ml (2.5 mg) 1.6 ml
4–<12 months (6–<10 kg) 1.0 ml (5 mg) 2.4 ml
1–<3 years (10–<14 kg) 1.25 ml (6.25 mg) 4 ml
3–<5 years (14–19 kg) 1.5 ml (7.5 mg) 5 ml
If convulsion continues after 10 minutes, give a second dose of
diazepam rectally (or give diazepam intravenously (0.05 ml/kg =
0.25 mg/kg) if IV infusion is running).
If convulsion continues after another 10 minutes, give a third dose of
diazepam or give paraldehyde rectally (or phenobarbital IV or IM
15 mg/kg).
■ If high fever:
➤ Sponge the child with room-temperature water to reduce the fever.
➤ Do not give oral medication until the convulsion has been controlled
(danger of aspiration).
* Use phenobarbital (200 mg/ml solution) in a dose of 20 mg/kg to
control convulsions in infants <2 weeks of age:
Weight 2 kg—initial dose: 0.2 ml, repeat 0.1 ml after 30 minute
Weight 3 kg—initial dose: 0.3 ml, repeat 0.15 ml after 30 minute
if
convulsions
continue }
15
1. ETAT
CHART 10. How to give IV glucose
➤ Insert IV line and draw blood for emergency laboratory investigations
➤ Check blood glucose. If low (<2.5 mmol/litre (45 mg/dl) in a well
nourished or <3 mmol/litre (54 mg/dl) in a severely malnourished
child) or if dextrostix is not available:
➤ Give 5 ml/kg of 10% glucose solution rapidly by IV injection
Volume of 10% glucose solution
Age/weight to give as bolus (5 ml/kg)
Less than 2 months (<4 kg) 15 ml
2–<4 months (4–<6 kg) 25 ml
4–<12 months (6–<10 kg) 40 ml
1–<3 years (10–<14 kg) 60 ml
3–<5 years (14–<19 kg) 80 ml
➤ Recheck the blood glucose in 30 minutes. If it is still low, repeat
5 ml/kg of 10% glucose solution.
➤ Feed the child as soon as conscious.
If not able to feed without danger of aspiration, give:
—milk or sugar solution via nasogastric tube (to make sugar solution,
dissolve 4 level teaspoons of sugar (20 grams) in a 200-ml cup of
clean water), or
—IV fluids containing 5–10% glucose (dextrose) (see App. 4, p. 357)
Note: 50% glucose solution is the same as 50% dextrose solution or D50.
If only 50% glucose solution is available: dilute 1 part 50% glucose solution to
4 parts sterile water, or dilute 1 part 50% glucose solution to 9 parts 5% glucose
solution.
Note: For the use of dextrostix, refer to instruction on box. Generally, the strip must
be stored in its box, at 2–3 °C, avoiding sunlight or high humidity. A drop of blood
should be placed on the strip (it is necessary to cover all the reagent area). After 60
seconds, the blood should be washed off gently with drops of cold water and the
colour compared with the key on the bottle or on the blood glucose reader. (The exact
procedure will vary with different strips.)
16
1. ETAT
For children with severe dehydration but without shock, refer to diarrhoea
treatment plan C, p.114.
If the child is in shock, first follow the instructions in Charts 7 and 8
(pages 12 and 13). Switch to the present chart when the child’s pulse
becomes slower or the capillary refill is faster.
➤ Give 70 ml/kg of Ringer's lactate solution (or, if not available, normal
saline) over 5 hours in infants (aged <12 months) and over 21/2 hours
in children (aged 12 months to 5 years).
Total volume IV fluid (volume per hour)
Age <12 months Age 12 months to 5 years
Weight Give over 5 hours Give over 21/2 hours
<4 kg 200 ml (40 ml/h) —
4–6 kg 350 ml (70 ml/h) —
6–10 kg 550 ml (110 ml/h) 550 ml (220 ml/h)
10–14 kg 850 ml (170 ml/h) 850 ml (340 ml/h)
14–19 kg — 1200 ml (480 ml/h)
Reassess the child every 1–2 hours. If the hydration status is not
improving, give the IV drip more rapidly
Also give ORS solution (about 5 ml/kg/hour) as soon as the child can
drink; this is usually after 3–4 hours (in infants) or 1–2 hours (in children).
Weight Volume of ORS solution per hour
<4 kg 15 ml
4–6 kg 25 ml
6–10 kg 40 ml
10–14 kg 60 ml
14–19 kg 85 ml
Reassess after 6 hours (infants) and after 3 hours (children). Classify
dehydration. Then choose the appropriate plan (A, B, or C, pages 120,
117, 114) to continue treatment.
If possible, observe the child for at least 6 hours after rehydration to be
sure that the mother can maintain hydration by giving the child ORS
solution by mouth.
CHART 11. How to treat severe dehydration in an
emergency setting after initial management of shock
17
1. ETAT
1.2 Notes for the assessment of emergency
and priority signs
■ Assess the airway and breathing (A, B)
Does the child’s breathing appear obstructed? Look and listen to determine if
there is poor air movement during breathing.
Is there severe respiratory distress? The breathing is very laboured, the child
uses auxiliary muscles for breathing (shows head nodding), is breathing very
fast, and the child appears to tire easily. Child is not able to feed because of
respiratory distress.
Is there central cyanosis? There is a bluish/purplish discoloration of the tongue
and the inside of the mouth.
■ Assess circulation (for shock) (C)
Check if the child’s hand is cold? If so
Check if the capillary refill time is longer than 3 seconds. Apply pressure to
whiten the nail of the thumb or the big toe for 3 seconds. Determine the time
from the moment of release until total recovery of the pink colour.
If capillary refill takes longer than 3 seconds, check the pulse. Is it weak and
fast? If the radial pulse is strong and not obviously fast, the child is not in
shock. If you cannot feel a radial pulse of an infant (less than 1 year old), feel
the brachial pulse or, if the infant is lying down, the femoral pulse. If you
cannot feel the radial pulse of a child, feel the carotid. If the room is very cold,
rely on the pulse to determine whether the child may be in shock.
■ Assess for coma or convulsions or other abnormal mental status (C)
Is the child in coma? Check the level of consciousness on the AVPU scale:
A alert,
V responds to voice,
P responds to pain,
U unconscious.
If the child is not awake and alert, try to rouse the child by talking or shaking
the arm. If the child is not alert, but responds to voice, he is lethargic. If there
is no response, ask the mother if the child has been abnormally sleepy or
difficult to wake. Look if the child responds to pain, or if he is unresponsive to
a painful stimulus. If this is the case, the child is in coma (unconscious) and
needs emergency treatment.
Is the child convulsing? Are there spasmodic repeated movements in an
unresponsive child?
ASSESSMENT OF EMERGENCY AND PRIORITY SIGNS
18
1. ETAT
EMERGENCY TREATMENT FOR THE CHILD WITH SEVERE MALNUTRITION
■ Assess for severe dehydration if the child has diarrhoea (D)
Does the child have sunken eyes? Ask the mother if the child’s eyes are more
sunken than usual.
Does a skin pinch go back very slowly (longer than 2 seconds)? Pinch the skin
of the abdomen halfway between the umbilicus and the side for 1 second, then
release and observe.
■ Assess for priority signs
While assessing for emergency signs, you will have noted several possible
priority signs:
Is there any respiratory distress (not severe)?
Is the child lethargic or continuously irritable or restless?
This was noted when you assessed for coma.
Note the other priority signs (see page 5).
1.3 Notes for giving emergency treatment to the child
with severe malnutrition
During the triage process, all children with severe malnutrition will be identified
as having priority signs, which means that they require prompt assessment
and treatment.
A few children with severe malnutrition will be found during triage assessment
to have emergency signs.
• Those with emergency signs for “airway and breathing” and “coma or
convulsions” should receive emergency treatment accordingly (see charts
on pages 4–16).
• Those with signs of severe dehydration but not shock should not be
rehydrated with IV fluids. This is because the diagnosis of severe dehydration
is difficult in severe malnutrition and is often misdiagnosed. Giving IV fluids
puts these children at risk of overhydration and death from heart failure.
Therefore, these children should be rehydrated orally using the special
rehydration solution for severe malnutrition (ReSoMal). See Chapter 7 (page
179).
• Those with signs of shock are assessed for further signs (lethargic or
unconscious). This is because in severe malnutrition the usual emergency
signs for shock may be present even when there is no shock.
— If the child is lethargic or unconscious, keep warm and give 10% glucose
5 ml/kg IV (see Chart 10, page 15), and then IV fluids (see Chart 8, page
13, and the Note given below).
19
1. ETAT
CHILDREN PRESENTING WITH EMERGENCY CONDITIONS
— If the child is alert, keep warm and give 10% glucose (10 ml/kg) by
mouth or nasogastric tube, and proceed to immediate full assessment
and treatment. See Chapter 7 (page 173) for details.
Note: When giving IV fluids, treatment for shock differs from that for a wellnourished
child. This is because shock from dehydration and sepsis are likely
to coexist and these are difficult to differentiate on clinical grounds alone.
Children with dehydration respond to IV fluids (breathing and pulse rates fall,
faster capillary refill). Those with septic shock and no dehydration will not
respond. The amount of fluid given should be guided by the child’s response.
Avoid overhydration. Monitor the pulse and breathing at the start and every
5–10 minutes to check if improving or not. Note that the type of IV fluid also
differs in severe malnutrition, and the infusion rate is slower.
All severely malnourished children require prompt assessment and treatment
to deal with serious problems such as hypoglycaemia, hypothermia, severe
infection, severe anaemia and potentially blinding eye problems. It is equally
important to take prompt action to prevent some of these problems, if they
were not present at the time of admission to hospital.
1.4 Diagnostic considerations of children presenting with
emergency conditions
The following text provides guidance for the approach to the diagnosis and the
differential diagnosis of presenting conditions for which emergency treatment
has been provided. After you have stabilized the child and provided emergency
treatment, determine the underlying cause of the problem, to be able to provide
specific curative treatment. The following lists and tables provide some guidance
which help with the differential diagnosis, and are complemented by the tables
in the symptom-specific chapters.
1.4.1 Child presenting with an airway or severe breathing problem
History
• Onset of symptoms: slowly developing or sudden onset
• Previous similar episodes
• Upper respiratory tract infection
• Cough
— duration in days
• History of choking
• Present since birth, or acquired
• Immunization history
— DTP, measles (continued on page 21)
20
1. ETAT
CHILD PRESENTING WITH AN AIRWAY OR SEVERE BREATHING PROBLEM
Table 1. Differential diagnosis of the child presenting with an airway
or severe breathing problem
Diagnosis or underlying cause In favour
Pneumonia —Cough with fast breathing and fever
—Development over days, getting worse
—Crepitations on auscultation
Asthma —History of recurrent wheezing
—Prolonged expiration
—Wheezing or reduced air entry
—Response to bronchodilators
Foreign body aspiration —History of sudden choking
—Sudden onset of stridor or respiratory distress
—Focal reduced air entry or wheeze
Retropharyngeal abscess —Slow development over days, getting worse
—Inability to swallow
—High fever
Croup —Barking cough
—Hoarse voice
—Associated with upper respiratory tract infection
Diphtheria —Bull neck appearance of neck due to enlarged lymph
nodes
—Red throat
—Grey pharyngeal membrane
—No DTP vaccination
Table 2. Differential diagnosis of the child presenting with shock
Diagnosis or underlying cause In favour
Bleeding shock —History of trauma
—Bleeding site
Dengue shock syndrome —Known dengue outbreak or season
—History of high fever
—Purpura
Cardiac shock —History of heart disease
—Enlarged neck veins and liver
Septic shock —History of febrile illness
—Very ill child
—Known outbreak of meningococcal infection
Shock associated with severe —History of profuse diarrhoea
dehydration —Known cholera outbreak
21
1. ETAT
CHILD PRESENTING WITH SHOCK
• Known HIV infection
• Family history of asthma
Examination
• Cough
— quality of cough
• Cyanosis
• Respiratory distress
• Grunting
• Stridor, abnormal breath sounds
• Nasal flaring
• Swelling of the neck
• Crepitations
• Wheezing
— generalized
— focal
• Reduced air entry
— generalized
— focal
1.4.2 Child presenting with shock
History
• Acute or sudden onset
• Trauma
• Bleeding
• History of congenital or rheumatic heart disease
• History of diarrhoea
• Any febrile illness
• Known dengue outbreak
• Known meningitis outbreak
• Fever
• Able to feed
Examination
• Consciousness
• Any bleeding sites
• Neck veins
• Liver size
• Petechiae
• Purpura
22
1. ETAT
1.4.3 Child presenting with lethargy, unconsciousness or
convulsions
History
Determine if there is a history of:
• fever
• head injury
• drug overdose or toxin ingestion
• convulsions: How long do they last? Have there been previous febrile
convulsions? Epilepsy?
In the case of an infant less than 1 week old, consider:
• birth asphyxia
• birth injury.
Examination
General
• jaundice
• severe palmar pallor
• peripheral oedema
• level of consciousness
• petechial rash.
Head/neck
• stiff neck
• signs of head trauma, or other injuries
• pupil size and reactions to light
• tense or bulging fontanelle
• abnormal posture.
Laboratory investigations
If meningitis is suspected and the child has no signs of raised intracranial
pressure (unequal pupils, rigid posture, paralysis of limbs or trunk, irregular
breathing), perform a lumbar puncture.
In a malarious area, prepare a blood smear.
If the child is unconscious, check the blood glucose. Check the blood pressure
(if a suitable paediatric cuff is available) and carry out urine microscopy if
possible .
It is important to determine the length of time a child has been unconscious
and his/her AVPU score (see page 17). This coma scale score should be
CHILD PRESENTING WITH LETHARGY, UNCONSCIOUSNESS OR CONVULSIONS
23
1. ETAT
CHILD PRESENTING WITH LETHARGY, UNCONSCIOUSNESS OR CONVULSIONS
Table 3. Differential diagnosis of the child presenting with lethargy,
unconsciousness or convulsions
Diagnosis or underlying cause In favour
Meningitis a,b —Very irritable
—Stiff neck or bulging fontanelle
—Petechial rash (meningococcal meningitis only)
Cerebral malaria (only in —Blood smear positive for malaria parasites
children exposed to —Jaundice
P. falciparum transmission; —Anaemia
often seasonal) —Convulsions
—Hypoglycaemia
Febrile convulsions (not likely —Prior episodes of short convulsions when febrile
to be the cause of —Associated with fever
unconsciousness) —Age 6 months to 5 years
—Blood smear normal
Hypoglycaemia (always seek —Blood glucose low; responds to glucose treatment c
the cause, e.g. severe malaria,
and treat the cause to prevent
a recurrence)
Head injury —Signs or history of head trauma
Poisoning —History of poison ingestion or drug overdose
Shock (can cause lethargy or —Poor perfusion
unconsciousness, but is —Rapid, weak pulse
unlikely to cause convulsions)
Acute glomerulonephritis with —Raised blood pressure
encephalopathy —Peripheral or facial oedema
—Blood in urine
—Decreased or no urine
Diabetic ketoacidosis —High blood sugar
—History of polydipsia and polyuria
—Acidotic (deep, laboured) breathing
a The differential diagnosis of meningitis may include encephalitis, cerebral abscess or tuberculous
meningitis. If these are common in your area, consult a standard textbook of paediatrics for further
guidance.
b A lumbar puncture should not be done if there are signs of raised intracranial pressure (see pages 149,
316). A positive lumbar puncture is one where there is cloudy CSF on direct visual inspection. CSF
examination shows an abnormal number of white cells (>100 polymorphonuclear cells per ml). A cell
count should be carried out, if possible. However, if this is not possible, then a cloudy CSF on direct
visual inspection could be considered positive. Confirmation is given by a low CSF glucose
(<1.5 mmol/litre), high CSF protein (>0.4 g/litre), organisms identified by Gram stain or a positive
culture, where these are available.
c Low blood glucose is <2.5 mmol/litre (<45 mg/dl), or <3.0 mmol/litre (<54 mg/dl) in a severely
malnourished child.
24
1. ETAT
monitored regularly. In young infants (less than 1 week old), note the time
between birth and the onset of unconsciousness.
Other causes of lethargy, unconsciousness or convulsions in some regions of
the world include Japanese encephalitis, dengue haemorrhagic fever, typhoid,
and relapsing fever.
Table 4. Differential diagnosis of the young infant (less than 2 months)
presenting with lethargy, unconsciousness or convulsions
Diagnosis or underlying cause In favour
Birth asphyxia —Onset in first 3 days of life
Hypoxic ischaemic encephalopathy —History of difficult delivery
Birth trauma
Intracranial haemorrhage —Onset in first 3 days of life in a low-birth-weight
or preterm Infant
Haemolytic disease of the —Onset in first 3 days of life
newborn, kernicterus —Jaundice
—Pallor
—Serious bacterial infection
Neonatal tetanus —Onset at age 3–14 days
—Irritability
—Difficulty in breastfeeding
—Trismus
—Muscle spasms
—Convulsions
Meningitis —Lethargy
—Apnoeic episodes
—Convulsions
—High-pitched cry
—Tense/bulging fontanelle
Sepsis —Fever or hypothermia
—Shock
—Seriously ill with no apparent cause