INNOCENT HEART MURMURS Heart murmurs are a common finding on routine examination of infants and children.

50% of normal children have an innocent heart murmur. How to evaluate a murmur? Search for symptoms or signs of congenital heart disease. Patients with innocent heart murmurs should be free of such symptoms or signs. Therefore, when history is obtained and during physical examination symptoms and signs, which may point to heart disease, should be identified. The presence of symptoms and/or signs of heart disease will suggest the pathological nature of the heart murmur being investigated. History Abnormal findings indicating a pathological origin of a heart murmur include: Shortness of Breath (SOB): This results from pulmonary edema due to increased pulmonary blood flow. Fluid escaping the engorged pulmonary vasculature will interfere with normal gaseous exchange, resulting in hypoxia, which precipitates tachypnea and SOB, particularly with exertion such as feeding. Cardiac asthma or exercise inducible reactive airway disease may occur as a consequence of pulmonary congestion. Easy fatigue: This results from pulmonary edema and poor cardiac output. Failure to thrive (FTT): Poor feeding secondary to pulmonary edema and poor cardiac output as well as increased caloric expenditure due to tachycardia and tachypnea will eventually lead to FTT. This will initially manifest as poor weight gain, followed by poor length progression. Cyanosis: Cyanosis is bluish discoloration of the oral mucosa and nail beds. This is caused by higher than normal concentration of deoxygenated hemoglobin, which is blue in color. Table 1. Case Presentation in the Evaluation of Cyanosis Case 1 2 3 4 Hemoglobin 14 g/dl 14 g/dl 6 g/dl 28 g/dl O2 saturation 95% 85% 85% 95% % Deoxygenated Hemoglobin 5% 15% 15% 5% Deoxygenated hemoglobin 0.7 g/dl 2.1 g/dl 0.9 g/dl 1.4 g/dl Findings Pink mucosa Cyanosis Pink mucosa Cyanosis

The level of deoxygenated hemoglobin in the blood is more important in determining the change in mucosa color. A child with cyanotic congenital heart disease, with severe anemia may not be cyanotic, because the level of deoxygenated hemoglobin in the blood (case 3) is not high enough to give cyanosis. On the other hand a patient with polycythemia (case 4) may be normally saturated by pulse oxymeter, however the small percentage of deoxygenated hemoglobin may be significant enough to cause cyanosis.

Central cyanosis is distinguished from acrocyanosis or peripheral cyanosis by involvement of the mucous membranes, including the tongue and buccal mucosa. Central cyanosis is associated with intracardiac right to left shunting, as well as lesions resulting in mixing of systemic and pulmonary venous return. Acrocyanosis is generally confined to the perioral, perinasal skin and nail beds and occur in children who are cold or vasoconstricted.
Oxygen Content (CaO2). Tissues need a requisite amount of O2 molecules for metabolism. Neither the PaO2 nor the SaO2 provide information on the number of oxygen molecules, i.e., of how much oxygen is in the blood. Of the three values used for assessing blood oxygen levels, how much is provided only by the oxygen content, CaO2 (units ml O2/dl). This is because CaO2 is the only value that incorporates the hemoglobin content. Oxygen content can be measured directly or calculated by the oxygen content equation : CaO2 = Hb (gm/dl) x 1.34 ml O2/gm Hb x SaO2 + PaO2 x (.003 ml O2/mm Hg/dl). Normal CaO2 ranges from 16 to 22 ml O2/dl. Because PaO2 and/or SaO2 can be normal in certain conditions associated with hypoxemia, one should always make sure CaO2 is adequate when assessing oxygenation. Make sure your cyanotic patient;s hemoglobin concentration is adequate for the degree of desaturation. 1

SaO2 is determined mainly by PaO2. The relationship between the two variables is the familiar oxygen dissociation curve. At low oxygen pressures there is relatively little increase in SaO2 for a given change in PaO2. Above a PaO2 of 20 mm Hg, the rate of change of SaO2 increases markedly, then slows again beyond a PaO2 of 60 mm Hg. In a cyanotic patient small changes in PaO2 may result in a marked increase in the rate of SaO2 (steep portion of hemoglobin dissociation curve). For this reason when performing a hyperoxia test PaO2 and not saturation must be taken into account. Hyperoxia Test: Used to evaluate the cyanotic neonate with RDS to differentiate between pulmonary and cardiac origin. In the test the neonate is either placed in a hood or intubated and 100% oxygen is administered for 5 to 10 minutes. An arterial PO2 is determined at baseline and after 10 minutes of hyperoxia. An increase in arterial PO2 of 30 mm Hg excludes most congenital cyanotic lesions. Additional relevant pregnancy history, congenital infections, drug use or associated syndromes or syndrome complexes should be thought since they are commonly associated with structural congenital heart disease (Table 2) Physical examination Abnormal findings indicating a pathological origin of a heart murmur include: Inspection Cyanosis: This is best determined by examining the patient in sunlight. Artificial light may alter patient color. Clubbing: This is enlargement of the tips of digits caused by hypoxia to peripheral tissue due to poor cardiac output and/or cyanosis. Peripheral tissue compensate by forming more capillaries to improve oxygenation, this results in swelling of the peripheries of digits. Clubbing of digits at its mildest form is noted when the normally seen angle at the skin-nail junction is lost, and in its worse condition, when the digit assumes a drumstick appearance. Clubbing is also seen in other diseases associated with tissue hypoxia such as lung diseases and hepatic and intestinal diseases such as Crohn's disease and ulcerative colitis due to anemia. Familial clubbing is also known to occur. Palpation Pulses: Pulses are the result of the difference between systolic and diastolic pressure. Increase in the difference between systole and diastole results in a more pronounced pulse. Pulses should be easily palpable and equal in their intensity throughout the body. Generalized week pulses are suggestive of poor cardiac output either due to severe heart failure or severe aortic stenosis. Better pulses in the upper upper extremities than the lower extremities are suggestive of coarctation of the aorta. Bounding (also known as water hammer) pulses are seen in patients with a low diastolic pressure due to aortic regurgitation or presence of a systemic to pulmonary arterial connection such as patent ductus arteriosus, collaterals or a surgically placed shunt such as Blalock-Taussig shunt.

Liver edge: In infants and small children, the liver character and size offer a more reliable indicator of systemic congestion. In newborns the liver may be normally palpable at 2.0 cm below the right costal margin. This decreases to approximately 1 cm by one year of age and remains just palpable to school age. Hepatomegaly may reflect a high right atrial pressure associated with congestive heart failure. The enlarged spleen should always be thought and suggest endocarditis in patients heart murmurs. Precordium: The precordium should be normally quiet. Hyperdynamic circulation due to increase pulmonary blood flow or RVH or LVH will cause prominence of the RV and/or LV impulses. Palpable thrill is felt when the heart murmur is 4/6 or louder. Prominent cardiac impulses or a thrill indicate pathology. Rarely a palpable second heart sound (S2) indicative of severe pulmonary hypertension mat be detected as a sharp impulse in the pulmonary outflow. Auscultation Lungs Pulmonary edema = rales, crackles Heart Heart Sounds: The sudden cessation of movement of blood is responsible for heart sounds. The abrupt interruption of flow caused by closure of the valves of the heart generates the first and second heart sounds. First heart sound should be audible. Second heart sound should split in inspiration and become single in expiration. Single second heart sound, or variation in splitting such as fixed splitting or reverse splitting, i.e. split in expiration and single in inspiration indicates pathology. In addition the quality of the pulmonary component of the second heart sound (the second component) should be evaluated, a loud P2 indicate pulmonary hypertension. Added sounds such as S4 are pathological. S3 may be normal. First heart sound (S1): Closure of atrio-ventricular valves. Second heart sound (S2): A2: closure of aortic valve 2

P2: closure of pulmonary valve Single S2 = absent pulmonary or aortic component or delayed closure of P2 superimposing A2, inaudible P2 due to TGA Does the splitting of S2 vary with respiration? Added sounds: Gallop rhythm: S3, S4 Murmurs Murmurs are produced by turbulent blood flow that causes vibratory activity of cardiac and vascular structures with sufficient intensity to be transmitted to the chest wall. The intensity, frequency, quality, time course, location, radiation and occasionally other measures, describe murmurs. The grade of intensity of the murmur has six classifications (Levine 1933): Grade: 1 is barely audible and may require several cycles to detect. Grade 2 is a soft murmur that can be readily heard Grade 3 is a moderately loud murmur unaccompanied by a thrill Grade 4 is a loud murmur accompanied by a thrill Grade 5 is a loud murmur that can be heard with the edge of the stethoscope Grade 6 is a loud murmur that can be heard without the stethoscope touching the chest wall. By definition grade four murmur is associated with a palpable thrill. The time course of a murmur is the factor most closely related to its cause. Murmurs can be separated into three periods: systolic, diastolic and continuous. Systolic murmurs have two subgroups: ejection and regurgitant (Leatham 1955). Systolic ejection murmurs: Flow from the ventricles does not begin until pressure exceeds aortic or pulmonary pressure. Consequently there is an interval after the first sound, before the murmur begins ( isovolumic contraction silent). Systolic ejection murmurs can be either innocent or pathologic. Examples of ejection murmurs include aortic stenosis and pulmonary stenosis. Mid-systolic murmurs result from an increase volume of blood flowing through normal valves. Examples include: patients with an ASD or high output state (anemia). Systolic Regurgitant murmurs: Systolic regurgitant: caused by ventricular septal defects (VSDs) or atrio-ventricular valve incompetence. In a VSD, not a truly regurgitant situation, a large left to right ventricular pressure gradient exists throughout systole, beginning with atrio-ventricular valve closure and persisting after semilunar valve closure. Systolic regurgitant murmurs are not benign or innocent. Diastolic murmurs Are divided into three groups based on the hemodynamic event causing the turbulent flow: 1. 2. 3. Diastolic decrescendo murmurs representing semilunar valve incompetence: aortic insufficiency, pulmonary insufficiency Diastolic flow murmurs caused by increased flow across either the mitral or tricuspid valve Atrial contraction murmur caused by flow across the mitral or tricuspid valve resulting from atrial contraction i.e. mitral stenosis

Systolic and diastolic murmur (continuous): Pressure difference between two structures during systole and diastole. PDA Shunts and collaterals Maneuvers with auscultation Supine, sitting and standing: Increase pre-load in supine exaggerating flow murmurs Valsalva maneuver:Increase intensity of MVP, decreases intensity of innocent heart murmurs Respiratory cycle:Inspiration increases blood flow to right heart Expiration increases blood flow to left heart Innocent heart murmurs William Evans first used the term innocent, in 1943. Also known as normal, functional, inorganic, innocuous, dynamic or benign. More than 50% of all children have innocent heart murmurs. Innocent heart murmurs are due to turbulent blood flow at the origin of the great vessels. It is better heard in children than in adults due to: Thin chest wall in children More angulated great vessels in children 3

More dynamic circulation in children

High cardiac output associated with fever, anemia, thyrotoxicosis, fright, and other increase the intensity of these heart murmurs. Features of innocent murmurs: Innocent heart murmurs are typically 2/6 or 1/6 in intensity, rarely they are 3/6, but never louder. Innocent heart murmurs could be systolic and diastolic such as in venous hum, but never purely diastolic. A typical innocent heart murmur is vibratory (or musical) in quality. Harsh murmurs indicate pathology. Innocent heart murmurs are never associated with abnormal heart sounds or abnormal palpation such as presence of a heave or a thrill. Types of innocent murmurs Stills murmur: Systolic ejection murmur, vibratory, musical in quality. Seen in infancy to adolescence. This murmur has been attributed to increased velocity of ejected blood from the left ventricle. It is heard best over the left lower sternal border and apex with the patient in the supine position. Pulmonary flow murmur: Second most common. Systolic ejection murmur heard best at the left mid to upper sternal border and less frequently over the aortic area. This murmur is heard best with the diaphragm due to its high frequency. Is caused by increased velocity of blood flow. Supra-clavicular arterial bruit: High pitched, brief early systolic ejection murmur best heard in the supra-clavicular fossae. Louder on right due to brachiocephalic arteries branching at that level. Neonatal peripheral pulmonary stenosis (PPS): Birth to 3-6 months. Systolic ejection murmur heard best at base of the heart, both axillae and the back. Due to relative small size of branch pulmonary arteries and the angle of bifurcation of the PAs Cervical venous hum; Continuous hum. Heard over right upper sternal border. Better in sitting, disappear in supine and when pressure is applied to neck to reduce venous blood return. Mammary souffl: Noted in lactating mothers, due to increase mammary blood flow. Features of pathological murmurs: 1. 2. 3. 4. 5. 6. All diastolic murmurs All regurgitant murmurs Late systolic murmurs Loud murmurs > 3/6 Continuous murmurs Associated cardiac abnormalities

REFERENCES 1. How to distinguish between innocent and pathologic heart murmurs in children. Rosenthal, A. The pediatric Clinics of North America, Dec 1984, page 129. 2. Innocent Murmurs. Newburger, J W. Nadas Pediatric Cardiology, page 281.

Clinical Cases: Atrial Septal Defect: ASD accounts for 10% of congenital heart disease and is the most common type of CHD to go undetected until adult life. Females are affected more than males (2-4:1). Most small atrial septal defects will close spontaneously by age 3 years. The left to right shunt across the defect is due to the relative greater compliance of the right ventricle. With the resulting increased flow across the tricuspid valve, a diastolic flow murmur is detected at the right atrial area. The increased flow causes a systolic ejection murmur in the right ventricular outflow tract. (pulmonary auscultation area). There is fixed splitting of the second heart sound from delayed right ventricular emptying both in systole and diastole. In shunts, which are greater than 2:1, chronic pulmonary over-circulation may lead to pulmonary arterial hypertension. This results in increased right ventricular work. The right ventricle subsequently hypertrophies and becomes less compliant which leads to a shunt reversal across the atrial septal defect (right to left shunt), which can result in cyanosis (Eisenmenger's physiology). These patients are also at risk for paradoxical emboli. Treatment is surgical closure and there is generally improved survival, except in instances in which pulmonary hypertension (greater than 40 mm Hg) is present. Ventricular Septal Defect: Other than for a bicuspid aortic valve, VSD is the most common type of congenital heart disease accounting for about 25% of cases. The majority of patients are asymptomatic, but patients may present with CHF in infancy if there is a large defect, a systolic murmur, or bacterial endocarditis. Small ventricular septal defects may go undetected until adulthood. As with ASD's if long standing, VSD's can lead to 4

Eisenmenger's physiology. Eisenmenger's represents the development of high pulmonary vascular resistance after many years of increased pulmonary blood flow (ASD/VSD/PDA). The increased flow results in reactive muscular hypertrophy and endothelial thickening/sclerosis of microscopic pulmonary vessels which eventually leads to their obliteration. This results in elevated pulmonary arterial and right ventricular pressures. If the pressure becomes high enough, there will be reversal of the shunt (right to left shunt). Most small ventricular septal defects will close spontaneously by age 5 years (about 30-60%). Pulmonary artery banding may be performed if definitive repair is not possible. This is an attempt to decrease the risk of developing pulmonary arterial hypertension by decreasing the amount of systemic to pulmonic shunting. The murmurs that result from a ventricualr septal defect vary depending on the size of the defect and its location in the ventricular septum. Because of the pressure gradient between the two chambers, blood ejects through the defect starting with the isovolumic contraction of the left ventricle and therefore obscuring S1.The regurgitant or holosystolic murmur is best heard in the right ventriuclar area.(LLSB). When a small defect is located in the muscular septum, systolic contraction may close the defect. This causes a characteristic murmur that begins with the first heart sound and but ends abruptly in mid systole. A supracristal septal defect will cause vibratory activity of the infundibulum and pulmonary artery. With a large ventricular septal defect the resulting increased flow across the mitral valve, results in a diastolic flow murmur detected at the mitral area(apex). Patent Ductus Arteriosus: PDA accounts for about 10% of congenital heart disease and females are affected more than males (4:1). If the ductus arteriosus remains patent, blood will be shunted from the aorta to the left pulmonary artery. The left atrium & left ventricle thus handle an increased volume of blood. Right ventricle is not involved. If long standing, pulmonary arterial hypertension may develop and result in shunt reversal. On exam there is a widened pulse pressure (due to diastolic siphon of blood into the pulmonary circulation). The lesion may cause CHF in premature infants due to natural history- ductus will initially close shortly after birth, however due to poor oxygenation in the underdeveloped lungs, it will reopen about day 5-7 with rapid overload. The murmur generated by a patent ductus artriosus is caused by pressure gradients between the aorta and the pulmonary artery throughout the cardiac cycle. The murmur is best heard over the left upper sternal border. A large patent ductus may be silent if systemic pulmonary artery pressure exists with little or no left to right shunt. Pulmonic Stenosis (Isolated): Pulmonic stenosis may be valvular, subvalvular, or supravalvular. In most patients, pulmonic valvar stenosis (PVS) is an isolated anomaly that may not present until adulthood (due to right heart failure) and there is generally a normal pulmonary vascularity on CXR. Affected patients will be acyanotic unless there is an ASD or VSD, which allows shunting from right to left. Balloon dilatation is the treatment of choice for PVS. In valvular pulmonary stenosis, the obstructed pulmonary valve causes a jet to strike the pulmonary artery. The murmur is best heart along the upper left sternal edge with transmission to the left infra-clavicular area. An ejection click is present best heard over the LLSB. With inspiration the click will decrease in intensity or may even disappear. With forced expiration right ventricular filling is diminished, resulting in greater excursion of the stenotic valve and a louder ejection click. Bicuspid Aortic Valve: Bicuspid aortic valve is the most common type of congenital heart disease (0.5 - 2% of the population), but typically does not present until adulthood. Although the valve may function normally, about 35% of affected patients will develop an abnormally turbulent flow pattern, which will lead to valve deformity and calcification. These patients are also at a greatly increased risk for infective endocarditis. Slowly evolving aortic regurgitation may result if the valve leaflets fail to close normally. There is an association with coarctation of the aorta and bicuspid aortic valve (60-85% of patients with coarctation of the aorta have a BAV. In patients with a bicuspid aortic valve, cessation of blood flow caused by the incomplete valve excursion leads to an ejection click best heard over the RUSB, MSB and apex. Aortic Stenosis : Affected neonates/infants with critical AS present with severe CHF. There are 3 types of lesions: 1- Valvar is the most common and usually secondary to a bicuspid valve. This lesion is associated with aortic coarctation and endocardial fibroelastosis, 2- Sub-valvular (IHSS) and 3Supra-valvular (William Syndrome). Valvar Aortic Stenosis: The obstruction caused by valvular aortic stenosis produces a vascular jet that strikes the wall of the ascending aorta. The resultant vibratory activity of the aorta is best detected along the upper right sternal edge. The murmur is a systolic ejection murmur, which begins after the first sound and ends well before aortic closure. In childhood the stenotic valve, usually bicuspid is characteristically mobile. With contraction, the valve moves from a position below the annulus it occupied in diastole to a position above the annulus. As the valve domes, it quite abruptly becomes taut, stooping the blood flow, resulting in a sharp ejection sound or click. The click has much the same characteristics as the first sound and can easily be misinterpreted as a duplicate first heart sound. The click is detected in the apical area, since is the left ventricle, not 5

the aorta, that transmits the vibratory activity to the chest wall. A thrill is frequently palpable at the supra-aortic notch as a result of gradients in excess of 25 mm Hg. In severe aortic stenosis a thrill is usually palpable at the LUSB. Aortic Coarctation: Coarctation is defined as a narrowing of the aorta distal to the left subclavian artery. It may be characterized by a long segment of narrowing or may be discrete. The clinical presentation of coarctation ranges from profound heart failure and shock in the newborn to asymptomatic hypertension or a murmur in an older child. Associated congenital heart disease is common with a VSD found in 50% of cases and a patent ductus in one-third which supplies blood to the descending aorta beyond the area of narrowing. The hallmark physical finding in coarctation consists of discrepant arterial pulses and systolic blood pressure in the upper and lower extremities. Observations should be made from all four limbs. Arterial pulses below the coarctation are diminished in amplitude and delayed in timing compared to the proximal pulses. Systolic blood pressure is elevated in the extremities proximal to the coarctation. There are several clinical circumstances that make the detection of pulse and pressure differences difficult or impossible. These include the following: a diminished cardiac output in an infant with coarctation and CHF, blood blow in the descending aorta maintained by right to left ductal flow, an anomalous right subclavian artery, arising distal to the coarctation (3-4%), left subclavian artery arising next to the coarctation with a stenotic orifice. Left ventricular pressure and volume overload produce a prominent, heaving ventricular impulse at the low left sternal border. Typically a constant systolic ejection click is heard at the apex and RUSB, signaling the presence of a bicuspid aortic valve. A grade 2-3/6 SEM, arising from the coarctation is usually best heard at the upper LSB, at the base and radiating to the left inter-scapular area. Continuous murmurs may be prominent throughout the chest in-patients with a well-developed arterial collateral system. Clinical Case Diagnosis Pathophysiology Treatment Outcome The case of an infant with a congenital cardiac defect will be presented and discussed. CLINICAL CASE The patient is a 2-month-old Caucasian female infant brought to your office for a "well child" exam. This is the first child born to this young couple. Mother is 19 years of age; Father is 20 years old. Neither of the parents have other children. There is no significant family history of illness. The infant was the product of a term, uncomplicated gestation. Her mother had not been pregnant before. Labor was spontaneous and lasted 10 hours. Delivery was vaginal. The baby appeared healthy after birth. APGARS were 8 at 1 minute and 9 at 5 minutes. Birth weight was 3.2 kg (50%); length was 50 cm (50%). The infant was discharged home in two days. She was bottle fed with a cow's milk formula, feeding 3 to 5 oz. every 3 hours. She initially did well. At about three weeks of age her mother noticed that the infant appeared to have some trouble finishing a bottle. By the time the infant was brought to your office it would take about an hour to finish 1/2 to 2/3 of a bottle. The baby did have a 2-week check-up and was thought to be well although a heart murmur was heard. She has not been back to the doctors office until today. PHYSICAL EXAM

Age = 2 months Weight = 4.0 kg (25%) Height = 55 cm (25%) Heart Rate = 165 beats per minute Respiratory Rate = 60 breaths per minute Blood Pressure = 85/60 mm Hg The infant is thin, malnourished and fussy. She is not cyanotic but is tachypneic. Tissue perfusion (capillary refill) is diminished. Pulses are equal in all extremities. On auscultation, lungs demonstrate rales. Cardiovascular Exam: The precordium is active. A heave is felt below the xyphoid process. S1 is normal, S2 is split. There is a grade 3 over 6 (IV/VI) holosystolic (regurgitant) murmur heard loudest at the mid-left sternal border. Diastole is quiet.

Heart sounds of the infant: The abdomen is soft. The liver edge is firm and is felt 5 cm below the mid right rib cage. Laboratory Evaluation: Electrocardiogram Electrocardiogram: Left ventricular hypertrophy.

Chest Radiograph Chest Radiograph: cardiomegaly and increased pulmonary vascularity

CLINICAL CASE 2 The patient is a 16 year-old Caucasian male recently moved to the area, which comes to your office for a routine physical. The patient is asymptomatic. This is the second child born to this couple. The patient has a younger sibling who is healthy. Mother is 46 years of age; Father is 50 years old. Neither of the parents have other children. There is a significant family history of congenital heart disease with a maternal second cousin born with hypoplastic left heart syndrome. Past Medical History: The patient is product of a term, uncomplicated gestation. Delivery was vaginal. APGARS were 8 at 1 minute and 9 at 5 minutes. Birth weight was 3.5 kg (50%); length was 50 cm (50%). A heart murmur was first heard two years ago and felt to be innocent. Review of systems is only significant for frequent headaches. His current medications include Tetracycline for treatment of acne and antihistamiens on a prn. PHYSICAL EXAM Age = 16 years Weight =198 lb. (90%) Height = 6 3 (>95%) Heart Rate = 72 beats per minute Respiratory Rate = 18 breaths per minute Blood Pressure = 152/80 mm Hg, RA; 150/80 mm Hg, LA; 110/81 mm Hg RL Healthy-appearing adolescent in no acute distress. No central cyanosis is demonstrated. Pulses are 21+ in the upper extremities and 1+ in the lower extremities with brachial femoral delay. Lungs were clear on auscultation.

CARDIOVASCULAR EXAM: The precordium is active. A heave is felt over the apex. No thrill (vibration) is palpable. S1 is normal, S2 is split. A constant systolic ejection click is heard at the apex and RUSB. A grade 2-3/6 SEM, is best heard at the upper LSB, at the base and radiating to the left inter-scapular area. A faint continuous murmur is auscultated throughout the chest. There is a grade 2 over 6 (II/VI) diastolic murmur heard loudest at the mid-left sternal border with some radiation to the apex. The abdomen is soft. The liver edge is firm and is felt 5 cm below the mid right rib cage. CHEST X RAY: There is mediastinal widening with a double contour at the aortic knuckle. The upper portion of this density extends upwards towards the left axilla, an enlarged left subclavian artery. There is some irregularity of the inferior borders of the upper ribs. CLINICAL CASE 3: A 12 year old, previously healthy Native American girl was admitted to the University of Minnesota Hospital with a diagnosis of chorea. She was in her usual state of good health until 4 days prior to admission, when she developed right sided, upper and lower extremity involuntary movements. This initially manifested them as difficulty with writing and progressed to persistent, asynchronous movements of the right arm and leg. She also has althralgia of knees without redness or swelling. She complained of shortness of breath and dyspnea when going up a short flight 8

of stairs. In addition the referring physician noted that she had multiple, lacy, round serpiginous, superficial skin lesions on her back. Her medical history is unremarkable except for a sore throat with red tonsils 1-month prior to her current illness. PHYSICAL EXAMINATION Age = 12 years Weight =40 Kg. (50%) Height = 140 cm (50%) Heart Rate = 92 beats per minute Respiratory Rate = 28 breaths per minute Blood Pressure = 120/60 mm Hg, Constant choreiform movements. HEENT exam normal. Fine rales over the lungs bases. The abdomen is soft. The liver edge is firm and is felt 5 cm below the mid right rib cage.No splenomegaly is noted. A lacy, serpigenous, well demarcated rash extended over her abdomen and trunk. There was no evidence of arthritis. Pulses +2 equal in the upper and lower extremities. CARDIOVASCULAR EXAM: The precordium is active. A heave is felt over the apex. No thrill (vibration) is palpable. The point of maximal impulse is displaced to the left anterior axillary line. S1 is normal, S2 is split. A grade 3/6 regurgitant murmur, is best heard over the apex, radiating to the axillae. A faint diastolic rumble is auscultated over the apex.

LABORATORY STUDIES: Mild anemia with hemoglobin of 10.2 g/L and abnormal platelet and WBC count. The sedimentation rate was 49 mm Hg (normal <15 mm Hg), and the CRP was elevated 4.0. An ASO screening test was negative, but quantitative titration of serum was 240 Todd units. The anti-DNase B titer was elevated at dilution at 1:1360 ELECTROCARDIOGRAM TREATMENT:

ASA: 80-100 MG/KG Oral Benzodiazepines Single IM dose of benzathine penicillin (1.2 million) Discussion: 9

Patients meets the Jones criteria with 3 major manifestations: carditis, sydenham chorea, and classic erythema marginatum. Minor criteria of elevated sedimentation rate and CRP. Finally there is clear supportive evidence of anti-streptococcal antibodies to deoxyribonuclease B (antiDnase B titer.

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