Ask for presenting symptoms in a lucid and chronological order. Identify the age at onset of congestive heart failure to decide whether patient is having congenital or acquired heart, disease. In infants rapid breathing, easy fatiguability while feeding, inability to suck vigorously and continuously, failure to thrive and puffiness are recognised clinical features of CHF.
In an older child, assess and grade the degree of dyspnea. Grade I dyspneic while climbing stairs or running for a short distance; Grade II dyspneic while performing daily routine activity; Grade III dyspneic following below average activity like going to bathroom or walking from one room to the other; and Grade IV when child is dyspneic at rest and is orthopneic. Older child may give history of palpitation (unpleasant sensation of beating of heart) while in younger children an observant mother may give history of visible precordial pulsations and bulging of precordium. Ask history of and age at onset of cyanosis, cyanotic spells and squatting. Chest pain due to myocardial ischemia is rare in children but can occur due to pericarditis, severe aortic stenosis or regurgitation, pulmonary vascular obstructive disease, mitral valve prolapse and Kawasaki’s disease. Ask for history of arthritis and migratory arthralgias, chorea and syncope (due to arrhythmia). Epistaxis (acute rheumatic fever, pulmonic stenosis) and hemoptysis (tight mitral stenosis, pulmonary infarction, pulmonary edema) may occur due to underlying cardiac conditions. Ask for history of medications and status of penicillin prophylaxis in a patient suspected to have rheumatic heart disease. History of recurrent chest infections is suggestive of left-to-right shunt. Patients with rheumatic fever may give history of recurrent streptococcal sore throats.
Ask family history of consanguinity, heart disease and hypertension among siblings and parents. There is increased risk of congenital and even rheumatic heart disease among relatives as compared to general population. Maternal diabetes mellitus is associated with transposition of great vessels and hypoplastic left heart syndrome in the offspring. Ask for maternal history of rubella, mumps and other viral infections and intake of drugs during first trimester of pregnancy which is characterized by organogenesis. Rubella syndrome is associated with patent ductus arteriosus while mumps during pregnancy may be associated with endocardial fibroelastosis in the fetus.
General Physical Examination
Assess whether child is comfortable or dyspneic, evaluate the severity of dyspnea, posture (orthopnea), physical growth and development. Look for peculiar phenotype (Down syndrome, Turner’s syndrome, Marfan’s syndrome, Hurler’s syndrome, glycogenosis etc), malar flush, puffiness and chorea.
|1. Alagille syndrome||Micrognathia, broad over-hanging forehead, deep set eyes, cholestasis, butterfly vertebral anomalies, short distal phalanges||Peripheral pulmonic artery stenosis is most common|
|2. Cornelia de Lange syndrome||Bushy eyebrows, long curly eyelashes, small nose with anteverted nostrils, long philtrum, downward curving of angle of mouth, mental retardation.||Ventricular septal defect|
|3. Di George syndrome||Fish mouth, hypertelorism, anti-mongoloid slant of eyes, hypoplasia or aplasia of thymus and parathyroids, tetany, cell-mediated immune deficiency||Interrupted aortic arch, truncus arteriosus, tetralogy of Fallot.|
|4. Down syndrome-(trisomy 21)||Brachycephaly, upward slant of eyes, Brushfield spots, small mouth with protruding fissured tongue, hypotonia, incurved little finger, developmental retardation.||Endocardial cushion defect, atrioventricular canal defects and ventricular septal defect in 50% cases, Fallot’s tetralogy.|
|5. Duchenne’s muscular dystrophy||Sex-linked disorder with progressive muscular weakness, hypertrophy of calves due to fatty infiltration, positive Gower’s sign, waddling gait.||Cardiomyopathy|
|6. Ellis-van Creveld syndrome||Long narrow dysplastic chest (short ribs) and abdomen, notching of upper lip, neonatal teeth, postaxial polydactyly of hands, acromesomelic shortening of limbs, hypoplastic nails, cone shaped epiphyses, small pelvic bones, epispadias||ASD (50%) and single atrium.|
|7. Glycogen storage disease (Pompe’s disease)||Marked hypotonia, macroglossia, hepatomegaly, normal mental development, early death.||Cardiomegaly with or without EKG changes (left axis deviation, short PR interval and large QRS complexes).|
|8. Holt-Oram syndrome||Deformities of forearm bones due to absence of radius, finger-like triphalangeal or absent thumbs, syndactyly, polydactyly||Familial ASD, VSD|
|9. Hurler’s syndrome||Coarse features, thick lips and broad nose, cloudy cornea, hepatos-plenomegaly or mental retardation.||Mitral or aortic regurgitation, coronary artery disease, mitral valve prolapse.|
|10. Marfan’s syndrome||Tall stature, long slender arms and fingers/toes, positive “thumb sign* ’’ (tip of the thumb protrudes beyond the ulnar border of the palm when fist is clenched), subluxated lens, lower segment of the body is longer than the upper segment, long span.||Dilatation of aortic root with aortic regurgitation, mitral valve prolapse.|
|11. Noonam syndrome||Turner-like phenotype, webbed-neck, hypertelorism with anti-mongoloid slant of pelpebral fissures, pectus excavatum, cryptorchidism and small testes.||Pulmonary valvular stenosis or ASD, hypertrophic cardiomyopathy.|
|12. Rubella syndrome||Microcephaly, deafness, cataracts,, chorioretinitis, metaphysitis.||Peripheral pulmonary artery stenosis, patent ductus arteriosus.|
|13. Shprintzen’s syndrome (Velocardio-facial syndrome)||Prominent nose with broad nasal root, cleft of soft palate, malar hypoplasia, long face, narrow palpebral fissures, retrognathia, speech and learning disorders||VSD, right aortic arch and tetralogy of Fallot.|
|14. TAR syndrome||Thrombocytopenia, absent or hypo-plastic radii||About one-third have cardiac defects, TOP, VSD|
|15. Trisomy 13-15 (Patau’s syndrome)||Microophthalmia, mid-facial hypoplasia, cleft lip/palate, microcephaly, broad flat nose, low set ears, capillary hemangiomas, simian crease, localized scalp defects, postaxial polydactyly of hands or feet.||VSD, ASD, PDA, dextrocardia in 90% cases.|
|16. Trisomy 17-18 (Edward’s syndrome)||Microcephaly, dolichocephaly, prominent occiput, small eyes and oral opening, low set ears, micrognathia, short sternum, clenched hands with overlapping fingers, simian crease, short dorsiflexed first toe, rocker bottom feet.||VSD (90%), PDA (70%), ASD (20%), valvular regurgitation may occur at multiple sites.|
|17. Tuberous sclerosis||Adenoma sebaceum, epilepsy, intracranial clarification, mental retardation.||Rhabdomyoma of heart|
|18. Turner’s syndrome||Lymphedema of dorsa of hands and feet during neonatal period, triangular face with antimogoloid slant of eyes, short stature, broad chest with widely spaced nipples, webbed neck, low hair line, cubitus valgus, amenorrhea.|| Coarctation of aorta (OX) and pulmonary
stenosis (XO/XY), bicuspid aortic valve.
|19. Williams syndrome||Elfin facies, short pelpebral fissure, flat nasal bridge, stellate iris, long philtrum, prominent lips, mental retardation, hypercalcemia during infancy.||Supravalvular aortic stenosis, pulmonary artery stenosis.|
Pulse. Rate, volume (thrust), tension (force required to obliterate the pulse), rhythm (volume and rate), character (normal, bounding, collapsing or water hammer type, plateau), pulsus alternans (alternate stronger and weaker beat), pulsus bigeminus (alternate normal and ectopic beat), pulsus paradoxus, vessel wall, pulse on the other side and femorals (volume and temporal relationship with radial pulse) should be assessed. Look for pulse deficit by simultaneously recording heart rate and pulse rate (ectopics and auricular fibrillation). Sinus arrhythmia in which pulse rate becomes fast during inspiration and slows during expiration is common and physiological in children.
Pulsus paradoxus is a misnomer because it is exaggeration of physiological finding wherein arterial pressure or pulse volume markedly diminishes or even disappears during deep inspiration. The systolic blood pressure is more than 10 mmHg lower during inspiration as compared to expiration. Pulsus paradoxus is classically seen in patients with cardiac tamponade (pericardial effusion, constrictive pericarditis), severe bronchial asthma and pneumothorax or pleural effusion.
Temperature. Fever may occur due to rheumatic activity, subacute bacterial endocarditis, pulmonary infarction, and chest infection. Fever may also occur due to a cause unrelated to the cardiac condition.
Respiratory rate. Tachypnea is seen in CHF, anoxic spells, pulmonary emboli and intercurrent respiratory infection.
Blood pressure. Proper cuff size (to cover two-third of upper arm or it should be 20% wider than the diameter of upper limb) is essential. The use of a narrow blood pressure cuff is associated with spuriously high systolic blood pressure reading and vice versa. Record blood pressure on both the sides and lower limbs. The point at which Kortokoff sounds become low pitched and muffled is regarded as the best index of diastolic blood pressure in children whereas in adults disappearance of sounds is considered as a better criterion. In infants and newborn babies it is difficult to record blood pressure by the conventional method. Blood pressure can be recorded by flush method. Cuff is wrapped around the upper arm, limb is raised vertically and held above the head till palm becomes pale. The pressure in the cuff is raised beyond the expected systolic blood pressure while maintaining the arm in vertical position. The arm is then brought down to the side of the cot and cuff is gradually deflated. The point at which the palm becomes flushed is indicative of systolic blood pressure of the infant. The diastolic blood pressure cannot be recorded by this method. In newborn babies and young infants, it is more convenient and reliable to use non-invasive Doppler system to record the blood pressure.
Anemia, cyanosis, jaundice (chronic liver congestion, pulmonary infarction, unrelated), lymphadenopathy and edema should be looked for. Differential cyanosis may occur due to right-to-left shunt through patent ductus arteriosus due to severe pulmonary arterial hypertension. Pink fingers with blue toes suggest that there is normal connection of great vessels. When there is complete transposition of great vessels, the fingers will be more cyanosed than the toes. In newborn babies and infants, sacral edema and puffiness may be seen while pedal edema is rare.
Nails. Marked clubbing is seen in children with cyanotic heart disease. Clubbing, splinter hemorrhages, Osler nodes, tender red nodules over the finger pulps due to deposition of immune complexes and palmar ecchymosis (Janeway’s lesions) are indicative of subacute bacterial endocarditis.
Skin and Joints. Look for subcutaneous nodules, erythema marginatum, chorea and swelling of joints suggestive of rheumatic activity. However, fortunately chorea and arthritis never coexist. Infants with CHF may have cold sweat on the forehead due to sympathetic overactivity as a consequence of decreased cardiac output. Examine joints and throat for inflammatory signs.
Fundus. Roth spots (oval-shaped hemorrhages with pale center) may be seen in subacute bacterial endocarditis. In aortic regurgitation and patent ductus arteriosus dancing retinal vessels are characteristic.
Neck. Internal jugular vein is best used for assessment of venous pressure and pulse wave forms. Look for jugular venous pressure (normal or raised), venous and arterial pulsations and thrill. JVP is difficult to evaluate in infants due to short neck. In infants with congestive heart failure, scalp veins may become prominent and engorged. Examine thyroid gland for enlargement and bruit to rule out thyrotoxicosis.
Venous pulsations. Venous pulsations in relation to various phases of cardiac cycle are depicated. As opposed to arterial pulsations, venous pulsations are not palpable and are less readily visible in an erect posture. Inspiration enhances venous pulsations by increasing venous return to the thorax. They are absent in children with auricular fibrillation, superior mediastinal obstruction and constrictive pericarditis (prominent Y descent). Giant ‘a’ waves or cannon waves are seen in tricuspid stenosis or atresia, pulmonary stenosis, pulmonary arterial hypertension, Ebstein’s disease, nodal rhythm, ectopic beats, and complete heart block. Giant ‘v’ waves are seen in children with tricspid regurgitation.
Liver. Examine for liver size, tenderness, pulsations, hepatojugular reflux (compression of hepatic area is followed by engorgement of neck veins). Hepatojugular reflux is a sign of right ventricular compromise. Heptomegaly is the most reliable sign of CHF in infants.
Spleen. Splenomegaly may occur due to subacute bacterial endocarditis or intercurrent infections due to malaria or septicemia (typhoid fever).
Signs of CHF. Dyspnea, tachycardia, raised JVP, enlarged tender liver, edema, cardiomegaly and basal crackles are recognized clinical signs of CHF. Jugular venous pressure is assessed by visualizing the engorgement of internal jugular veins in the neck. The patient lies supine with neck elevated by 45°. The vertical distance from the angle of Louis (sternal angle) to the imaginary line drawn from the upper end of jugular venous column gives JVP which is measured in centimeters. When JVP is raised 3 cm above the sternal angle or internal jugular vein is engorged above the level of clavicle it is abnormal and suggestive of CHF, pericardial effusion, constrictive pericarditis and mediastinal mass. In infants, evaluation of JVP is unreliable due to short neck and dependent edema is uncommon. Hepatomegaly is the most consistent and reliable sign of CHF in infants. The central venous pressure can be measured by introducing a needle in the antecubital vein and connecting it to a manometer filled with saline. The normal CVP ranges between 4 to 6 cm of water and it is raised in CHF and reduced in hypovolemic shock.
EXAMINATION OF HEART
Bulging procordium (long standing cardiomegaly), and bulging intercostal spaces (pericardial effusion) should be looked for. There are several thoracic lines of reference used in the examination of the heart to express degree of cardiac enlargement.
Precordial pulsations. Note whether procordium is quiet (pericardial effusion, endocardial fibroelastosis, Ebstein’s disease), hyperdynamic or hyperkinetic (anemia, thyrotoxicosis, left-to-right shunt, aortic and mitral regurgitation etc.) and look for suprasternal and epigastric pulsations, dilated veins over the chest, collateral arteries etc.
Apex beat. It is the outermost and lowermost palpable impact of the left ventricle. The apex beat is best palpated with the child sitting and leaning forward. The important landmark for counting the intercostal spaces is the angle of Louis or sternal angle. It is felt as a ridge connecting manubrium sterni with body of the sternum. The rib corresponding to the sternal angle is second rib and space below is the second intercostal space. In preschool children apex beat is located in the 4th intercostal space just lateral to the midclavicular line. Subsequently, it is located in the 5th intercostal space inside or over the midclavicular line. Assess whether it is visible or not (look on both sides), its site (up, down and out, outwards, inwards or right side), and character (normal, feeble, tapping, heaving and hyperkinetic). Tapping apex is suggestive of palpable S1 in the mitral area while heaving (forceful, broad and sustained) apex is indicative of left ventricular hypertrophy due to pressure overload. Hyperkinetic apex beat is characterized by exaggerated ill-sustained thrust of cardiac impulse and is seen in conditions associated with volume overload (anemia, aortic regurgitation, PDA, VSD, MR, thyrotoxicosis). Left parasternal heave (due to right ventricular hypertrophy or conducted impulse from left artrial enlargement) and diastolic shock (palpable S2) should be looked for.
The point of maximal impulse (PMI) is helpful in determining whether the right or left ventricle is dominant. In patients with left ventricular dominance, the impulse is maximal at the apex whereas in right ventricular dominance the cardiac impulse is maximal over the lower left stemal border.
Thrill. Vibratory sensations of heart musculature conducted through the chest, wall (like purring of cat) is called thrill. It is associated with a murmur of grade IV or higher. Note the site and timing of thrill. Functional murmurs are never associated with a thrill.
It has a limited clinical utility and is used to outline cardiac borders and to assess the heart size. In pericardial effusion the dullness extends beyond the apex, and there is dullness over the 2nd left intercostal space which disappears when patient sits up i.e. shifting dullness (c.f. pulmonary hypertension). Dullness over right second intercostal space and manubrium stemi may be seen in aortic aneurysm and mediastinal mass (thymus).
After inspection, it is desirable to auscultate the heart in a sleeping infant before he is frightened or awakened by palpation and percussion. Offer a toy, key ring or your finger to the infant while auscultating to prevent him fiddling with the tubing of the stethoscope. The bell type chest piece is better suited for detecting low-frequency events (mitral diastolic murmur and third heart sound) whereas the diaphragm selectively picks up high-frequency sounds and murmurs. Good quality stethoscope with short tubes and flexible rubber ear pieces, waxless ears and intact drums of the examiner, quiet infant and serene surroundings are crucial for optimal auscultatory yield. The availability of echocardiography has deflated the ego of many a cardiologists. The defeatist attitude, however, should be replaced by the desire to improve and sharpen clinical acumen with the help of available technology.
Mitral (apex), tricuspid (above xiphoid cartilage), 3rd and 4th left parasternal intercostal spaces, pulmonary (second intercostal space adjacent to left sternal border) and A1 (second intercostal space adjacent to right sternal border) areas, neck vessels, sides and back of the chest and thyroid gland should be auscultated. Auscultation should be done with patient in the supine, upright, and left lateral positions and leaning forward.
Heart sounds. The temporal relationship between events in the cardiac cycle and production of heart sounds and jugular venous pulsations are diagrammatically depicted. The normal first heart sound is low pitched, greater in intensity, longer in duration and the pause preceding it is longer than that following it. First heart sound (closure of mitral and tricuspid valves) is best studied in the mitral area while second heart sound (closure of aortic and pulmonary valves) is best heard in the pulmonary and aortic areas. P2 is normally as loud as A2 and split (especially during inspiration) in children. P2 is louder than A2 upto 3-6 months of age. Proper evaluation of S2 provides diagnostic clues in a number of clinical situations. Normally the split in S2 is discernible only during inspiration. When split is heard both during inspiration and expiration, it is called a wide split. It is designated as a wide and variable split when the degree of splitting varies in inspiration and expiration; and a wide fixed split when it does not vary during inspiration and expiration. The opening of normal cardiac valves does not produce any sound but when they are diseased they may produce ejection clicks.
Look for intensity of heart sounds, whether relative and absolute, splitting (fixed or variable), 3rd heart sound (triple or gallop rhythm due to rapid filling of ventricles during early diastole), opening snap (snappy sound inside the apex that closely follows the second heart sound due to opening of stiff mitral valve), and ejection or systolic click (opening of diseased pulmonary and aortic valves). Fourth heart sound which represents atrial contraction may be heard in patients with severe pulmonic stenosis, pulmonary hypertension and Ebstein’s disease. It is a low pitched sound occurring before S1 and rarely heard in children. Third and fourth heart sounds are best heard with the patient turned to the left side and auscultated with the bell of the stethoscope.
Cardiac murmurs. They are produced due to turbulence of the blood flow at or near a valve or because of an abnormal communication within the heart. Site of maximum intensity, grade (1-6/6), timing (systolic or diastolic or continuous), character and quality (ejection, holosystolic or pansystolic, decrescendo or crescendo and continuous type) and radiation or conduction should be carefully evaluated. The murmurs are timed by simultaneous palpation of apex beat or carotid artery. Some murmurs are best heard in certain postures and phases of breathing. Murmurs originating from the right side of the heart increase in intensity during inspiration owing to increase in stroke output of the right ventricle. Conversly murmurs arising from the left side are accentuated during expiration. All murmurs increase in intensity with forceful manouvers like hand grip except mitral valve prolapse and idiopathic hypertrophic subaortic stenosis.
Mitral stenosis. Left lateral position (bell of stethoscope)
Aortic regurgitation. Child sitting, leaning forward, and during expiration(diaphragm or chest piece of stethoscope).
Tricuspid regurgitation. End of deep inspiration.
The intensity of murmur is conventionally graded from 1 to 6. Grade 1: barely audible; Grade II: soft but easily audible; Grade III: loud but not accompanied by thrill. Grade IV louder and associated with thrill, Grade V: audible with stethoscope barely touching the chest: Grade VI audible with stethoscope off the chest.
The presence of a continuous murmur in an acyanotic patient is a recognised feature of patent ductus arteriosus, rupture of sinus of Valsalva fistula into the right atrium or ventricle, origin of coronary artery from pulmonary artery, mitral stenosis with small atrial septal defect and venous hum. The continuous murmur must be differentiated from pericardial friction rub.
Pericardial rub. It is leathery to-and-fro, superficial, variable in intensity, and heard in both phases of cardiac contraction and altered by pressure of chest piece. It is best heard at the base of the heart or inside the apex especially when the patient leans forwards.
|Abnormal splitting of S2|
| 1. Widely split and fixed S2
I. Volume overload (ASD. TAPVR)
II. Electrical delay (RBBB, WPW syndrome type A)
III. Early aortic closure (MR)
| 2. Wide and variable split S2
I. Pulmonary stenosis
II. Mitral regurgitation
IV. Partial anomalous pulmonary venous connection with intact atrial septum
| 1. Narrowly split S2
I. Pulmonary hypertension
II. Aortic stenosis
| 2. Single S2
I. Pulmonary hypertension
II. One semilunar valve (PA, A A, PTA)
III. P2 not audible (TGA, TOP, severe PS)
IV. Severe aortic stenosis
| 3. Paradoxically spilt S2
I. Severe aortic stenosis
II. Left bundle branch block (WPW syndrome)
IV. Severe hypertension
V. Aortic regurgitation
|Abnormal intensity of P2|
| 1. Increased P2 (pulmonary hypertension)
2. Decreased P2 (severe PS, TOF, tricuspid stenosis)
Diagnostic Clinical Signs of Common Cardiac Conditions
Feeble pulse, left parasternal heave, tapping apex, palpable S2, diastolic thrill, loud S1 in mitral area, opening snap (occurring shortly after the second heart sound), mid diastolic rough and rumbling murmur with presystolic accentuation are characteristic clinical findings. The presystolic component disappears during auricular fibrillation. A loud first heart sound and opening snap indicates presence of a relatively pliable non calcified valve. A short middiastolic rumble (Carey-Coomb’s murmur) due to mitral valvulitis may be heard in patients with acute rheumatic carditis without any established mitral stenosis.
Heaving apex, systolic thrill in mitral area, muffled or inaudible S1, with high-pitched pansystolic murmur which is conducted towards the axilla and back are suggestive of mitral regurgitation.
Mitral valve prolapse
It is usually asymptomatic in children and characterized by a mid-systolic click, a high frequency usually loud intensity sound with a scratchy quality which is best heard in a sitting or standing position. There may be a mid-late grade II systolic murmur with a whistling character. The prevalence of mitral valve prolapse is increased in patients with Duchenne muscular dystrophy, osteogenesis imperfecta, Ehlers-Danlos syndrome, Marfan syndrome, van Willebrand disease, fragile X syndrome and anorexia nervosa.
Prominent venous pulsations (giant ‘v’ waves) in neck, pulsatile liver, high-pitched pansystolic murmur at lower end of sternum accentuated by deep inspiration are characteristic. Functional tricuspid regurgitation due to right ventricular failure is associated with third heart sound.
Collapsing pulse, wide pulse pressure, prominent arterial pulsations, pistol sounds, forceful heaving apex, decrescendo high pitched, blowing, early diastolic murmur in the aortic area are recognized features. The diastolic murmur is best heard with diaphragm of the stethoscope over the left upper parasternal area when patient sits up, leans forwards and breathes out. The increased flow of blood across the aortic valve may produce an ejection systolic murmur. The first heart sound is soft and aortic component of the second sound is delayed and accentuated. In severe aortic regurgitation a low-pitched diastolic flow murmur called as Austin-flint murmur may be audible at the mitral area.
Plateau or feeble pulse, narrow pulse pressure, heaving apex, systolic thrill over the aortic area conducted to neck vessels, delayed aortic component of S2, ejection systolic murmur conducted to neck vessels are characteristic findings of aortic stenosis. Ejection click may be audible in valvular aortic stenosis. The S2 may be paradoxically split in severe aortic stenosis. Williams syndrome is characterized by peculiar “elfin facies”, hypercalcemia, mental retardation and supravalvular aortic stenosis.
Atrial septal defect (ASD secundum)
Parasternal heave, grade II to III ejection systolic murmur over 2nd and 3rd intercostal spaces, and fixed wide splitting of S2, are diagnostic features of ASD secundum. The murmur is produced by increased flow of blood through the pulmonary valve and not due to left-to-right shunt. Cardiomegaly is mild and CHF is rare. Marked cardiomegaly suggests existence of additional lesions like mitral valve obstruction (Lutembacher syndrome) or mitral regurgitation (ostium primum defect).
Endocardial cushion defect (ostium primum defect)
The defect in the atrial septum is situated below the fossa ovalis and is associated with a cleft in the anterior leaflet of the mitral valve. In addition to the findings of ostium secundum listed above, there are additional clinical features suggestive of left ventricular hypertrophy, pansystolic murmur of mitral regurgitation and left axis deviation of more than – 300 on EKG. Congestive heart failure is common in children with common atrioventricular canal.
Ventricular septal defect
Wide pulse pressure, hyperdynamic precordium with forceful apex beat, systolic thrill over left sternal border, pansystolic murmur over left sternal region (3rd to 5th interspace), masking of both heart sounds are usual clinical findings. S2 may be split with accentuation of P2. A third heart sound may be audible at the apex. A diastolic flow murmur in the mitral area may be heard in children with a large defect.
Prominent ‘a’ waves in jugulars, parasternal heave, S2 is wide and variably split, harsh grade 2-5/6 ejection systolic murmur, and systolic ejection click best heard during expiration, are recognized clinical features of pulmonary stenosis. The louder and longer the murmur, the more severe is the stenosis.
Patent ductus arteriosus (PDA)
Collapsing pulse, heaving apex, systolic thrill below the left clavicle, machinery or rolling thunder harsh continuous systolo-diastolic murmur best heard over 2nd left intercostal space, and multiple clicks are recognized clinical findings of PDA. Pulmonary arterio-venous fistulae, coronary arterio-venous anastomoses, aortico-pulmonary fenestration and venous hum may produce continuous murmurs. Diastolic murmur in the mitral area may appear due to large blood flow across the mitral valve.
Tetralogy of Fallot
Fallot’s tetralogy consists of pulmonary stenosis, ventricular septal defect, right ventricular hypertrophy and over-riding of aorta. Cyanosis, clubbing, anoxic spells, mild right ventricular hypertrophy, ejection systolic murmur over pulmonary area, single second heart sound (absent pulmonary component), and absence of congestive heart failure are suggestive of tetralogy of Fallot.
Cyanosis, anoxic spells, prominent ‘a’ waves in jugular veins, presystolic pulsations in the liver, apical impulse of left ventricular type, non significant or absent cardiac murmur are usual clinical findings. It may be difficult to clinically differentiate these patients from tetralogy of Fallot.
Transposition of great vessels
Cyanosis with CHF since early infancy, right ventricular hypertrophy, loud and single S2 with systolic ejection click, short systolic ejection murmur of pulmonary stenosis or systolic regurgitant murmur of VSD, or no murmur are recognized clinical features of transposition of great vessels.
Cyanosis, clubbing, dominant ‘v’ waves in the neck, quiet precordium, left ventricular type apical impulse, loud S1, triple or quadruple heart sounds (multiple sounds are audible), systolic thrill with mid or pansystolic murmur at the left sternal border are suggestive of Ebstein’s anomaly. There may be short tricuspid delayed diastolic murmur. Both systolic and disatolic murmurs produced at the tricuspid valve may have a scratchy character not unlike a pericardial friction rub.
Coarctation of aorta
The blood pressure proximal to the stricture i.e. upper limbs is increased so that blood pressure in the arms is higher than the lower limbs. Femorals are feeble and delayed as compared to simultaneously felt radial pulsations. Upper arms are stronger and span may be longer than the height. Palms may appear pinker as compared to soles. Collateral vessels linking the subclavian arteries and intercostal arteries may be seen or felt over the interscapular and suprascapular areas. Collaterals are palpable over the under surface of ribs on the back and along both the borders of scapulae. Precordial examination shows evidences of left ventricular hypertrophy, aortic ejection systolic murmur (due to associated congenital bicuspid aortic valve) and constant ejection click. Ejection systolic or continuous murmur may be audible over the back due to presence of collaterals or flow of blood through the narrow segment of aorta.
There is cardiomegaly, marked tachycardia, low-intensity heart sounds with gallop rhythm. At times, a “tic-tac” rhythm in which interval between the first and second heart sounds is equal to or even longer than diastole may be heard. Regurgitant murmur may be produced by gross enlargement of heart with dilatation of the valves. Dysrhythmias in the form of ventricular premature beats or conduction disturbances are common. Evidences of congestive heart failure are usually present.
Functional Cardiac and Extracardiac Murmurs
Transient, nonsignificant murmurs are common during newborn period due to post-natal delay in circulatory adaptation. The closure of ductus and foramen ovale may be delayed. Conversely infants with VSD may not have any murmur during neonatal period which may appear after 4 to 6 weeks of age when pulmonary vascular resistance falls leading to establishment of left-to-right shunt.
The “functional” or “innocent murmurs” are best heard at the base, are low-pitched and low in intensity, systolic in timing, unassociated with thrill, modified by posture of the patient and usually disappear following exercise. Anemic children with a cardiac murmur should be reexamined after correction of anemia before any pathologic significance is ascribed to the murmur. Functional murmurs may also occur in children with fever, scoliosis, kyphoscoliosis and pectus excavatum. Venous hum is best audible at the right infraclavicular and supraclavicular areas as a continuous murmur (louder diastolic component) due to turbulence of blood in jugular system especially in children between 3 to 6 years of age. The murmur is best heard in upright position and disappears when child lies supine or by rotation of head. The murmur also disappears if neck veins are occluded with a finger or by increasing pressure over the chest piece of stethoscope. There are no peripheral features of PDA. Carotid bruit may be heard as an early ejection systolic murmur over the carotid arteries. Unlike aortic stenosis, there is no ejection click and no thrill or murmur is heard over the aortic area. In children with coarctation of aorta, look for murmur over the back due to collaterals. Auscultation of skull, liver and lumbar regions is advocated in children with unexplained CHF and hypertension, A continuous or ejection systolic murmur may be audible due to arterio-venous fistula, angiomatous malformation and arterial stenosis.
|General Physical Examination||Comfortable, dyspneic (severity), orthopneic, physical growth and development, facial dysmorphism, skeletal deformities, and malar flush. Pulse (rate, volume, rhythm, character, vessel wall, other peripheral pulses including volume and timing of femorals, sleeping pulse rate), temperature, respiratory rate, and blood pressure in both upper and lower limbs. Anemia, cyanosis, jaundice, lymphadenopathy, edema (pedal, facial, sacral), clubbing, splinter hemorrhages, Osler, nodes, Janeway lesions, subcutaneous nodules, erythema marginatum, arthritis, chorea, evidences of congestive heart failure, subacute bacterial endocarditis and rheumatic activity.|
|Inspection||Precordial bulge (costal or intercostal), pulsations over the precordium (normal, hyperdynamic or quiet), neck vessels, suprasternal area and epigastrium, collateral arteries and dilated veins, apex beat visible or not.|
|Palpation||Site and character (normal, tapping, heaving) of apex beat, point of maximal impulse of cardiac contraction, palpable heart sounds, left parasternal heave, and thrills (site and liming in relation to cardiac cycle).|
|Percussion||Outline cardiac borders, dullness beyond apex beat, dullness or impaired percussion note over the aortic area, (aneurysm), manubrium sterni (mediastinal mass) and pulmonary area (pulmonary eonus and pericardial effusion), shifting dullness over pulmonary area on sitting up (pericardial effusion).|
|Auscultation||Describe the findings in a systematic manner over all the cardiac areas: mitral, tricuspid, 3rd and 4th left parasternal areas, pulmonary area, aortic area, neck vessels, sides and back of chest, and thyroid gland.Heart sounds, intensity, single or split, variable or fixed splitting of S2, third heart sound, gallop rhythm, and fourth heart sound. Heart sounds produced by opening of the diseased valves i.e. opening snap and ejection clicks.Cardiac murmurs: site of maximum intensity, grade, timing (systolic, diastolic or continuous), character (ejection, pansystolic, crescendo or decrescendo) and conduction. Describe any functional cardiac and extracardiac murmurs including pericardial and pleuro-pericardial rubs.|