First Aid for the Pediatrics Clerkship, 3 Ed.

Cardiovascular Disease



Normal Heart Sounds

Image S1 may split.

Image S2 normally splits with respiration.

Image S3 can represent normal, rapid ventricular refilling.

Image P2 should be soft after infancy.


The difference between grade I and II murmur: A grade I can be heard only in a quiet room with a quiet child.


Image Up to 90% of children have a murmur at some point in their lives.

Image Two to seven percent of murmurs in children represent pathology.


Murmurs are graded for intensity on a six-point system:

Image Grade I: Very soft murmur detected only after very careful auscultation.

Image Grade II: Soft murmur that is readily heard but faint (equal to S1/S2).

Image Grade III: Moderately intense murmur not associated with a palpable precordial thrill (louder than S1 or S2).

Image Grade IV: Loud murmur; a palpable precordial thrill is not present or is intermittent.

Image Grade V: Loud murmur associated with a palpable precordial thrill; the murmur is not audible when the stethoscope is lifted from the chest.

Image Grade VI: Loud murmur associated with a palpable precordial thrill. It can be heard even when the stethoscope is lifted slightly from the chest.


Murmur grading is usually written as “Grade [#]/6.”


See Figure 13-1 to correlate the following points:

1. This site corresponds to the location of the carotid arteries. Common murmurs heard here: carotid bruit, aortic stenosis (AS). AS is usually louder at the right upper sternal border and often has an associated ejection click.

2. Aortic valve. Right upper sternal border. Common murmurs: aortic valve stenosis (supravalvular, valvular, and subvalvular). Valvular stenosis will often have an ejection click, whereas the others will not.


FIGURE 13-1. Sites of auscultation.


Murmurs of ASD and TAPVR are secondary pulmonary flow murmurs.

3. Pulmonic valve. Left upper sternal border. Common murmurs: pulmonary valve stenosis, atrial septal defect (ASD), pulmonary flow murmur, pulmonary artery stenosis, AS, coarctation of the aorta, patent ductus arteriosus (PDA), total anomalous pulmonary venous return (TAPVR).

4. Tricuspid valve. Left lower sternal border. Common murmurs: ventricular septal defect (VSD), Still’s murmur, hypertrophic obstructive cardiomyopathy (HOCM), tricuspid regurgitation, endocardial cushion defect.

5. Mitral valve. Apex. Common murmurs: mitral regurgitation, mitral valve prolapse, Still’s murmur, aortic stenosis, HOCM.

6. This site correlates with areas of venous confluence. Common murmurs: venous hum or subclavian bruit.

Accentuation Maneuvers

Various positions and activities can diminish and intensify a murmur (see Table 13-1). The following section also reiterates the positions that aid in diagnosing innocent murmurs.

TABLE 13-1. Accentuation Maneuvers for Pathologic Murmurs


TABLE 13-2. Innocent Murmurs




Reminders for a systematic cardiac exam:

1.     Assess the child’s appearance, color, etc.

2.     Palpate the precordium.

3.     Listen in a quiet room, during systole and diastole.

4.     Listen first for heart sounds, then repeat your “sweep” of the chest for murmurs.

5.     Don’t forget to listen to the back and in the axillae.

6.     Move the patient in different positions.

7.     Feel the pulses and assess capillary refill.

8.     Palpate the liver.


Image Pulmonary flow murmurs, physiologic pulmonary branch stenosis, and Still’s murmurs can all be heard best when the patient is supine versus upright.

Image A Still’s murmur may disappear with the Valsalva maneuver.

Image Pulmonary flow murmurs are augmented by full exhalation, diminished by inhalation.

Image Venous hum can be extinguished or accentuated with head and neck movement. It disappears in the supine position, and can also be eliminated with digital compression of the jugular vein.


See Table 13-1.

Innocent Murmurs

Image Common to all innocent murmurs are:

Image Absence of structural heart defects.

Image Normal heart sounds (S1, S2).

Image Normal peripheral pulses.

Image Normal chest radiographs and electrocardiogram (ECG).

Image Asymptomatic.

Image Usually systolic and graded less than III.

Image No association with cardiovascular disease.

Image Accentuate in high-output states (fever and anemia).

Image See Table 13-2.



Any murmur > grade III is likely pathologic.

Image Always approach an ECG systematically:

1.     Measure atrial and ventricular rates.

2.     Define the rhythm (sinus, or other).

3.     Measure the P-R interval, QRS duration, and Q-T interval.

4.     Measure the axes of the P waves, QRS complexes, and T waves.

5.     Look for abnormalities of wave patterns and voltages.

Image Pediatric considerations:

Image The interpretation is age dependent.

Image Heart rate varies with age—higher in infants and children.

Image The QRS duration is shorter in children.

Image PR interval is shorter.

Image QTc is longer in infants than in older children (QT interval varies with heart rate).

Image Right axis deviation alone not enough as a criterion for right venticular hypertrophy (RVH).


Cardiology consultation is indicated with any “noninnocent” murmur.

Expiration Rate

Age-dependent—see Table 13-3.

TABLE 13-3. Heart Rate by Age


ECG Paper

Image Speed = 25 mm/s.

Image Small box = 0.04 sec = 1 mm.

Image Large box = 0.20 sec = 5 mm.

Atrial Rate

Image Look for P wave count that exceeds QRS complex count.

Image If P wave number is greater than QRS complex number, an atrial dysrhythmia may be present.

Image Premature atrial contractions (PACs) are common in infancy.

Ventricular Rate

Image Count number of small boxes between 2 R waves, then divide into 1500.

Image Count R-R cycles in six large divisions, then multiply by 50 (use with irregular or fast rate).


Found in sleep, sedation, vagal stimulation (stooling, cough, or gag), hypothyroid, hyperkalemia, hypothermia, hypoxia, athletic heart, second- or third-degree atrioventricular (AV) block, junctional rhythm, ↑ intracranial pressure, medicine (ie, digitalis, β blockers). Hypoxemia is the most common cause of bradycardia.


Found in fever, anxiety, hypovolemia, sepsis, congestive heart failure (CHF), hyperthyroidism, supraventricular tachycardia (SVT), ventricular tachycardia, atrial flutter and fibrillation, medicine (ie, theophylline, stimulants).

Sinus Arrhythmia

Normal variation in heart rate, due to inspiration and expiration (commonly seen in childhood).


Check for sinus rhythm:

Image Verify a P wave before every QRS complex.

Image Verify a QRS complex after every P wave.

Image All P waves should look the same.

Image Normal P wave axis (0° to +90°).

Image Upright P waves in leads I and aVF.

Image Normal P wave:

Image < 0.10 sec in children.

Image < 0.08 sec in infant.


Image Beginning of P to beginning of QRS.

Image Prolonged P-R (first-degree AV block): Found in myocarditis, digitalis, hyperkalemia, ischemia, ↑ vagal tone, hyperthyroidism.

Image Short P-R: Found in ectopic atrial pacemaker, preexcitation syndromes (Wolff-Parkinson-White syndrome [WPW], Lown-Ganong-Levine syndrome), and glycogen storage disease. It may show patient is at risk for SVT.


Prolonged: Found in right bundle branch block (RBBB), left bundle branch block (LBBB), WPW, premature ventricular contractions (PVCs), mechanical pacemaker rhythms.


Image QTc—corrected QT.

Image Normal: < 0.45 (< 6 months), < 0.44 (> 6 months).

Image Beginning of Q to end of T.

Image QTc = QT interval (sec) divided by the square root of the R-R interval (sec).

Image Long QT predisposes to ventricular tachycardia and is associated with sudden death.


QT—Congenital Syndromes


Ischemic heart disease






Abnormal Rhythms


Image Preceded by a P wave, followed by a normal QRS.

Image The length of two cycles (R-R) including a PAC is usually shorter than the length of two normal cycles.

Image No hemodynamic significance.


Image Premature and wide QRS, no P wave, T wave opposite to QRS:

Image Multifocal PVCs: Different-shaped PVCs in same strip.

Image Bigeminy: Coupled beat (sinus, PVC, sinus, PVC).

Image Trigeminy (sinus, sinus, PVC, sinus, sinus, PVC).

Image Couplets (sinus, sinus, PVC, PVC, sinus, sinus).

Image May be normal if they are uniform and ↓ with exercise.


Image A rapid atrial rate (∼300 bpm) with a varying ventricular rate, depending on degree of block (ie, 2:1, 3:1).

Image Sawtooth pattern (II, III, aVF, V1).

Image Normal QRS.

Image Usually suggests significant pathology (atrial enlargement).


Image Very fast atrial rate (350–600 bpm).

Image Irregularly irregular ventricular response.

Image No P waves; normal QRS.

Image Usually suggests significant pathology.


Image Series of 3++ PVCs with a heart rate between 120 and 200 bpm.

Image Wide, unusually shaped QRS complexes.

Image T waves in opposite direction of QRS complex.

Image Usually suggests significant pathology.


Image Very irregular QRS complexes.

Image The rate is rapid and irregular.

Image This is a terminal arrhythmia because the heart cannot maintain effective circulation.


Image Typically narrow QRS complex, no variability in R-R internal.

Image Reentrant tachycardia that utilizes the AV node in reentrant circuit.

Image Sudden onset and resolution.

Image Absent P waves.

Image Usually associated with structurally normal hearts.

Image SVT with aberrant conduction producing wide QRS may look like V-tach.



Image Examine leads I and aVF.

Image In lead I, count all forces above the baseline by the number of boxes (mm) and subtract all forces below baseline. If the total is +[+], the axis range is between ++90° and –90°.

Image Do the same in aVF. If the total is +[+], the QRS is also between 0° and ++180°.

Image Superimpose the ranges. Region of overlap is quadrant QRS lies in.

Image Find lead with isoelectric QRS complex. The axis points perpendicular to that lead.

Image If all leads are equiphasic, the axis is perpendicular to all leads and perpendicular to that plane. It is directed anterior or posterior and called indeterminate.


Axis Summary

0 to ++90: I++/aVF ++

0 to –90: I+/aVF–

+90 to 180: I–/aVF +

90 to 180: I–/aVF–


Image Right axis deviation (RAD): Caused by severe pulmonary stenosis with RVH, pulmonary hypertension (HTN), conduction disturbances (RBBB). RAD is normal in a newborn because of right ventricular dominance.

Image Left axis deviation (LAD) with RVH is highly suggestive of AV canal. Consider especially with Down syndrome.

Image Mild LAD with left ventricular hypertrophy (LVH) in a cyanotic infant suggests tricuspid atresia.


Normal = [+] in lead I, [+] in aVF:

Image I–, aVF– = Extreme axis deviation (direction based on Q wave).

Image I+, aVF– = LAD.

Image I, aVF+ = RAD.


Image Normal defines sinus rhythm and normally related atria (atrial situs solitus).

Image A P axis between 0 and –90° may result from an ectopic low right atrial pacemaker (in absence of sinus node dysfunction, it is not significant).

Image A P axis > +90° suggests atrial inversion or misplaced leads.


Image If differs by > 60 to 90° from QRS axis in presence of ventricular hypertrophy, it is called a “strain pattern” and may be a sign of ischemia.

Image If strain is present, examine left precordial leads (V5, V6) for abnormal repolarization (indicated by T-wave inversion).

Image LVH with strain in patients with aortic stenosis or hypertrophic cardiomyopathy is an ominous finding indicating severe disease.

Image Varies depending on age.

Abnormal Wave Patterns and Voltages


Image If no narrow Q waves in inferior (II, III, aVF) and leftward leads (I, V5, V6), suspect congenital heart disease (CHD) with ventricular inversion.

Image Q waves of new onset or of ↑ duration of previous Q waves, with or without notching of Q, may represent myocardial infarction (MI). Uncommon.

Image ST elevation or prolonged QTc is also supportive of MI.

Image Causes of ischemia and infarction: Anomalous origin of left coronary artery from pulmonary artery, coronary artery aneurysm and thrombosis in Kawasaki disease, asphyxia, cardiomyopathy, severe aortic stenosis, myocarditis, cocaine use.

Image A deep, wide Q wave in aVL is a marker for LV infarction. Suspect anomalous origin of left coronary artery, particularly in a child < 2 months old.


Image End of S to beginning of T.

Image Causes of ST displacement: Pericarditis, cor pulmonale, pneumopericardium, head injury, pneumothorax, early ventricular repolarization, and normal atrial repolarization.

Image Elevation may result from ischemia or pericarditis; depression is consistent with subendocardial ischemia or effects of digoxin.


Image Peaked, pointed T waves occur with hyperkalemia, LVH, and head injury.

Image Flattened T waves are seen in hypokalemia and hypothyroidism.


Image Peaked P waves (leads II and V1): P = > 2.5 mm (> 6 months) or > 3 mm (< 6 months).

Image Causes include cor pulmonale (pulmonary hypertension, RVH), anomalous pulmonary venous connection, large ASD (uncommon), Ebstein’s anomaly.


Image Wide P wave (notched in II, deep terminal inversion in V1): P = > 0.08 sec (< 12 months old) or > 0.10 sec (> 12 months old).

Image Causes include VSD, PDA, mitral stenosis.

Image The wider and deeper the terminal component, the more severe the enlargement.


Image R wave > 98% in V1 or S wave > 98% in I or V6.

Image ↑ R/S ratio in V1 or ↓ R/S in V6.

Image RSR′ in V1 or V3R in the absence of complete RBBB. RSR′ with R > 15 mm (> 1 year) is characteristic of RVH secondary to right ventricular overload.

Image In newborns, a pure R wave in V1 > 10 mm = pressure-type RVH.

Image Upright T wave in V1 (> 3 days).

Image Presence of a Q wave in V1, V3R, V4R.

Image Adult pattern may occur as early as 6 years.

Image A qR pattern of Q wave in V1 suggests severe RVH.

Image Causes include ASD, TAPVR, pulmonary stenosis, tetralogy of Fallot (TOF), large VSD with pulmonary HTN, coarctation in the newborn.

Image Caution! The diagnosis of RVH via ECG should be made cautiously in newborns. Consider right-sided obstructive lesions (tetralogy of Fallot) in children > 6 months.


Image R > 98% in V6, S > 98% in V1.

Image ↑ R/S ratio in V6 or ↓ R/S in V1.

Image Q > 5 mm in V6 with peaked T (occurs with LV diastolic overload and denotes septal hypertrophy).

Image Flat or inverted T waves in lead I or V6, in presence of LVH, suggests severe LVH.

Image Excessive LAD supports LVH but is not sufficient to make the diagnosis.

Image Causes include VSD, PDA, anemia, complete AV block, aortic stenosis, systemic HTN, obstructive and nonobstructive hypertrophic cardiomyopathies.


Image If criteria for RVH exist and left ventricular forces exceed normal mean values for age, the patient has CVH.

Image If LVH present, similar reasoning may apply to the diagnosis of RVH.

Image In the presence of RVH, dominant RV forces diminish apparent LV forces, causing lower LV voltages (small R in V6 and small S in V1).

Image Large equiphasic voltages in limb leads and midprecordial leads are called Katz-Wachtel phenomenon and suggest biventricular hypertrophy.

Image Causes include left-to-right shunts with pulmonary HTN (large VSD) and complex structural heart disease.

Image Cannot diagnose ventricular hypertrophy in the absence of normal conduction (RBBB).


Causes of Sudden Cardiac Deaths in Young Athlete

Image Hypertrophic cardiomyopathy

Image Arrhythmogenic right ventricular cardiomyopathy

Image Congenital coronary artery anomalies

Image Aortic rupture with Marfan syndrome

Image Wolff-Parkinson-White syndrome

Image Congenital long QT syndrome


Image < 5 mm in limb leads.

Image Causes include pericardial effusion, pericarditis, hypothyroidism.

Image Sometimes normal newborns have ↓ voltages—not a concern.


Image Ventricular preexcitation via accessory conduction pathway through the Bundle of Kent.

Image Accessory pathway conducts more rapidly (than the normal AV node) but takes longer to recover.

Image Shortened PR interval, widened QRS caused by slurred upstroke of the delta wave.

Image Associated with Ebstein’s anomaly.

Image ↑ risk of SVT and sudden death.

Image Treatment: Surgical ablation of accessory pathway.


Image There are four basic cross-sectional views taken of the heart with transthoracic echocardiography (TTE):

Image Parasternal (long and short axis).

Image Apical.

Image Subcostal (taken in the midline below the xiphoid process).

Image Suprasternal.

Image Transesophageal echocardiography employs a transducer introduced down the esophagus for enhanced imaging during cardiac surgery or catheterization.


In infants and children, coronary arteries can be evaluated nicely using TTE.

2-D Echocardiography

Image Cross-sectional images of the heart are seen via this method.

Image Parasternal views:

Image Long axis: Left ventricular inflow and outflow tracts.

Image Short axis: Aortic valve, pulmonary valve, pulmonary artery and branches, right ventricular outflow tract, atrioventricular valves, right side of heart.

Image Apical views: Atrial and ventricular septa, atria and ventricles, atrioventricular valves, pulmonary veins.

Image Subcostal views: Atrial and ventricular septa, atrioventricular valves, atria and ventricles, and pulmonary venous drainage.

Image Suprasternal views: Ascending and descending aorta, pulmonary artery size, systemic and pulmonary veins.

Color-Flow Doppler Echocardiography

Image Blood flow and direction can be seen via this method.

Image Red indicates blood flowing toward the transducer.

Image Blue indicates blood flowing away from the transducer.

Image When blood flow velocity exceeds a certain limit (called the Nyquist limit), the color signal is often yellow. This is indicative of high velocities that may be seen in VSDs, ASDs, and valvar regurgitation and stenosis.


Image In this mode, the information from one scan point is measured over time.

Image Motion creates a graph of depth of structures (ie, valves, ventricular wall, etc.) versus time.

Image This modality is used to determine cardiac chamber dimension, valve annuli size, fractional shortening and ejection fraction, left ventricular mass.


Image For the prenatal diagnosis of congenital heart diseases.

Image Allows for improved counseling and better understanding of the postnatal prognosis.

Image Screen at > 16 weeks.

Image Indications:

Image Fetal:

Image Abnormal screening obstetric ultrasound.

Image Extracardiac anomalies.

Image Chromosomal abnormalities.

Image ↑ first-trimester nuchal translucency measurement (trisomy 21 and Turner syndrome).

Image Maternal (diabetes, phenylketonuria).


Heart Size

Cardiothoracic ratio:

Image Measure largest width of the heart and divide by the largest diameter of the chest. A normal ratio is < 0.5.

Image The CXR must have a good inspiratory effort. For this reason, newborns and infants are difficult to evaluate by this method.

Image Cardiomegaly on CXR is most suggestive of volume overload; ECG better reflects ↑ pressure.

Cardiac Chamber Enlargement

Image Left atrial enlargement (LAE):

Image May produce a “double density” on the PA CXR.

Image More severe LAE can elevate the left mainstem bronchus.

Image Right atrial enlargement (RAE): RAE is noted most at the right lower cardiac border; however, it is difficult to diagnose by CXR alone.

Image Left ventricular enlargement:

Image The apex is seen further to the left and downward.

Image On lateral CXR, the posterior cardiac border is further displaced posteriorly.

Image Right ventricular enlargement:

Image VH is not seen well on PA CXR because it does not make up the cardiac silhouette.

Image On lateral CXR, it is noted by filling the retrosternal space.


In newborns and small infants, the upper aspects of the heart are obscured by a large “boat sail–shaped” opacity—the thymus. This organ will involute after puberty. It is often not seen in premature newborns.

Pulmonary Vascular Markings


Image Noted by the visualization of pulmonary vasculature in the lateral one-third of the lung field.

Image In an acyanotic child this could be ASD, VSD, PDA, endocardial cushion defect, or partial anomalous pulmonary venous return.

Image In a cyanotic child this could be transposition of the great arteries, TAPVR, hypoplastic left heart syndrome, persistent truncus arteriosus, or single ventricle.


Image The lung fields are dark, with small vessels.

Image Seen in pulmonary stenosis and atresia, tricuspid stenosis and atresia, tetralogy of Fallot.


Image Manifested as hazy lung fields.

Image Kerley B lines are often present.

Image Caused by LV failure or obstruction of the pulmonary veins.

Image Seen in mitral stenosis, TAPVR, cor triatriatum, hypoplastic left heart syndrome, or any left-sided obstructive lesion with heart failure.

Abnormal Cardiac Silhouettes


Image A “boot-shaped” heart with ↓ pulmonary vascular markings is sometimes seen. The boot is due to the hypoplastic main pulmonary artery.

Image RVH is noted.

Image About 25% will have a right aortic arch.

Transposition of the Great Arteries

Image An “egg-shaped” heart is sometimes seen.

Image The narrow superior aspect of the cardiac silhouette is due to the absence of the thymus and the irregular relationship of the great arteries.


Image A “snowman” shape is sometimes seen.

Image The left vertical vein, left innominate vein, and dilated superior vena cava create the “snowman’s” head.



Image Rheumatic fever is a delayed immunologic sequela of a previous group A streptococcal infection of the pharynx (not of the skin).

Image Cutaneous streptococcal infection is a precursor of glomerulonephritis.

Image Affects the brain, heart, joints, and skin.


Image Although an uncommon disease in the United States, small outbreaks occur in various regions.

Image Peak age range: 6–15 years.

Image A positive family history of rheumatic fever ↑ risk.

Image Incidence: 0.3–3% in developed countries.

Image Risk of RF after untreated strep pharyngitis is 1–3%.

Image Patients with the infection < 3 weeks have a 0.3% risk.

Image Follows pharyngitis by 1–5 weeks (average: 3 weeks).

Image Rate of recurrent RF with subsequent strep infection may approach 65%.

Image Recurrence rate ↓ to < 10% over 10 years.


Image To diagnose acute rheumatic fever you must fulfill the following combination of the Jones criteria:

Image Two major manifestations or

Image One major and two minor manifestations

Image In addition to the major and minor manifestations, patients may appear pale and complain of abdominal pain and epistaxis.

Image Aschoff bodies (found in atrial myocardium) are diagnostic.


Jones Criteria (Modified)

2 major or 1 major + 2


Major—J ♡ NES:

Image Joints—polyarthritis

Image ♡—carditis

Image Nodules, subcutaneous

Image Erythema marginatum

Image Sydenham’s chorea


Image Arthralgia

Image Fever

Image Elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)

Image Prolonged P-R interval Plus

Image Laboratory evidence of antecedent group A strep infection (ASO titer)


Image Incidence: 50% of patients.

Image Clinical presentation:

Image Tachycardia is common.

Image Heart murmur, most commonly due to valvulitis of the following (in order of decreasing frequency):

Image Mitral valve regurgitation

Image Aortic valve regurgitation

Image Tricuspid valve regurgitation—less common.

Image Pericarditis (a friction rub may be heard).

Image Cardiomegaly.

Image CHF (a gallop may be heard).


Image Most common manifestation, affecting 70%.

Image Usually affects the large joints, but can affect the spine and cranial joints.

Image Migratory in nature, affecting a new joint as other affected joints resolve (can affect more than one joint at a time).

Image Joints are red, warm, swollen, and very tender, particularly if moved.

Image Responds well to aspirin therapy (give once diagnosis is confirmed).

Image Duration is usually < 1 month, even without treatment.


Absence of tachycardia or murmur usually excludes the diagnosis of myocarditis.


Image Incidence: 15% of patients; most commonly prepubertal girls.

Image Movements last on average 7 months before slowly diminishing (can last up to 17 months).

Image Characteristics:

Image Initial emotional lability: Behaviors characteristic of attention deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD) have been noted to precede the movement disorder.

Image Loss of motor coordination.

Image Spontaneous, purposeless movement.

Image Motor weakness.


Rheumatic fever can cause long-term valvular disease, both stenosis and insufficiency.


Image Incidence: < 10% of patients.

Image Pink, erythematous macular rash.

Image Often has a clear center and serpiginous outline.

Image Nonpruritic.

Image Evanescent and migratory.

Image Disappears when cold.

Image Reappears when warm.

Image Found primarily on the trunk and proximal extremities.


Image Incidence: 2–10% of patients.

Image Hard, painless, small (0.5–1 cm) swellings over bony prominences, primarily the extensor tendons of the hand.

Image Can also be found on the scalp and along the spine.

Image Not transient, lasting for weeks.


If a patient’s arthritis doesn’t improve within 48 hours of therapeutic aspirin therapy, he or she probably does not have rheumatic fever.


Image Streptococcal antibody tests are the most reliable evidence of preceding group A strep infection → acute rheumatic fever.

Image Antistreptolysin O (ASO) titer is the most commonly used. It is elevated in 80% of patients with acute rheumatic fever (ARF) and 20% of normal individuals.

Image Other antibody tests exist (antihyaluronidase, antistreptokinase, anti-deoxyribonuclease B) wherein at least one will be positive in 95% of patients with ARF.

Image Positive throat cultures and “rapid strep tests” are less reliable because they do not differentiate acute infection versus chronic carrier state.


The chorea of rheumatic fever is known as Syndenham’s chorea or St. Vitus’ dance.


Image Upon diagnosis, the patient should receive benzathine penicillin G 1.2 million units IM to eradicate the streptococci (if < 27 kg = 600,000 U).

Image Patients allergic to penicillin can receive 4 days of erythromycin 40 mg/kg/day.

Image Prophylaxis should be initiated:

Image Benzathine penicillin G 1.2 million units IM every 3–4 weeks or

Image Penicillin 200,000 units PO three times per day or

Image Sulfadiazine 1 g PO once per day

Image Length of prophylaxis is undetermined but often advocated at least throughout adolescence, if not indefinitely. Obviously, compliance becomes a difficult issue.

Image Seventy-five percent of patients recover within 6 weeks, and less than 5% are symptomatic beyond 6 months. Seventy percent of those with carditis recover without permanent cardiac damage.


Erythema marginatum is never found on the face.



A 6-year-old girl with PDA develops fever and anorexia. Her Hgb is 9; she has hematuria, ↑ ESR, positive rheumatoid factor (RF), and immune complexes are present. Think: Bacterial endocarditis.

Predisposition: Congenital heart disease. Greatest risk: Systemic-pulmonary arterial communications such as patent ductus arteriosus. Chronic anemia, microscopic hematuria, elevated ESR, positive rheumatoid factor, circulating immune complexes, and low complement levels are all may be present in infective endocarditis.


Image α -Hemolytic streptococci are most common (70%).

Image Streptococcus viridans.

Image Staphylococcus aureus is also common, accounting for 20% of cases.

Image If felt to be secondary to cardiac surgery complications, Staphylococcus epidermidis, gram-negative bacilli, and fungi should be considered.

Image Culture-negative endocarditis: Coxiella burnetii or Bartonella.

Image Most endocarditis is left-sided.

Image Right-sided endocarditis is associated with IV drug use.


Subcutaneous nodules are also found in connective tissue diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis.


Most likely to occur on congenitally abnormal valves, valves damaged by rheumatic fever, acquired valvular lesions, prosthetic replacement valves, and any cardiac defect → turbulent blood flow.


Image Fever is most common.

Image New or changing heart murmur.

Image Chest pain, dyspnea, arthralgia, myalgia, headache.

Image Embolic phenomena:

Image Hematuria with red cell casts.

Image Acute brain ischemia.

Image Roth spots, splinter hemorrhages, Osler’s nodes, Janeway lesions (less common in children).


Subcutaneous nodules in rheumatic fever have a significant association with carditis.


Image High risk:

Image Prosthetic cardiac valves.

Image Previous bacterial endocarditis (due to scar formation on valve).

Image Congenital heart disease—complex cyanotic types.

Image Surgical pulmonary-systemic shunts.

Image Moderate risk:

Image Congenital cardiac diseases not in high and low risk.

Image Acquired valvular dysfunction.

Image Rheumatic heart disease, Libman-Sacks valve, antiphospholipid syndrome–associated valve disease.

Image Hypertrophic cardiomyopathy.

Image Complicated mitral valve prolapse (valvular regurgitation, thickened valve leaflets).

Image Low risk:

Image Isolated ASD, secundum type.

Image Surgically repaired cardiac defects > 6 months postoperative (ASD, VSD, PDA).

Image Heart murmurs with normal echocardiogram (physiologic or functional, flow murmurs).

Image Systemic diseases without cardiac valve involvement:

Image Kawasaki disease: Normal echo only.

Image Rheumatic heart disease: Normal echo only.

Image Cardiac pacemakers and implantable defibrillators.


A history of sore throat or scarlet fever is insufficient evidence for rheumatic fever without a positive strep test.


Carditis is the only manifestation of rheumatic fever that can cause permanent cardiac damage. Therefore, once definitively diagnosed, anti-inflammatory therapy (with prednisone in extreme cases, or aspirin) should be started.


Image Four sets of blood cultures over 48 hours from different sites.

Image Most common findings:

Image Positive blood cultures

Image Elevated ESR

Image Hematuria

Image Anemia

Image Echocardiographic evidence of vegetations or thrombi is diagnostic.


Janeway lesions—painless Osler’s nodes—painful


Image Four to eight weeks of organism-specific antibiotic therapy.

Image Surgery is necessary when endocarditis is refractory to medical treatment. Also considered in cases of prosthetic valves, fungal endocarditis, and hemodynamic compromise.

Image Antibiotic prophylaxis is necessary for children with structural heart disease and other predisposing conditions.


Risk Factors for Endocarditis

Image Previous endocarditis

Image Dental procedures

Image Gastrointestinal and genitourinary procedures

Image IV drug use (usually affects the tricuspid valve)

Image Indwelling central venous catheters

Image Prior cardiac surgery


Image Prophylaxis is recommended with:

Image Most dental and periodontal procedures.

Image Tonsillectomy or adenoidectomy.

Image Rigid bronchoscopy or surgery involving gastrointestinal (GI) or upper respiratory mucosa.

Image Gallbladder surgery.

Image Catheterization in setting of urinary tract infection, cystoscopy, urethral dilation.

Image Urinary tract surgery.

Image Incision and drainage of infected tissues.

Image Prophylaxis is not usually recommended with:

Image Intraoral injection of local anesthetic.

Image Shedding of primary teeth.

Image Tympanostomy tube insertion.

Image Endotracheal tube insertion.

Image Bronchoscopy with flexible bronchoscope.

Image Transesophageal echo.

Image Cardiac catheterization.

Image Cesarean section (only when no infection present).

Image GI endoscopy, with or without biopsy (prophylaxis for high-risk patients).

Image Genitourinary (GU) procedures with no infection present (except those above).

Image Circumcision.



Image Most often caused by viruses. Coxsackieviruses and echoviruses are most common. Recent evidence suggests adenovirus as a common etiology.

Image Immune-mediated diseases (eg, acute rheumatic fever, Kawasaki disease).

Image Collagen vascular diseases.

Image Toxic ingestions.


Clinically recognizable myocarditis is rare in the United States.


Image Presentation depends on the degree of myocardial injury.

Image Ranges from asymptomatic to fulminant CHF.

Image Common symptoms are fever, dyspnea, upper respiratory symptoms, vomiting, and lethargy.

Image CHF should be considered if patient is tachycardic and tachypneic and has a gallop on auscultation.


Image ECG findings: Low voltages, S-T changes, prolonged QT interval, premature beats.

Image Radiology: Chest radiographs will show cardiomegaly.

Image Echocardiography: Chamber enlargement is present with impaired ventricular function.


Image First, treat the underlying cause (ie, antibiotics if bacterial).

Image Since it is most often viral, treatment is largely supportive. Rest and activity limitation is important.

Image Treatment of CHF may be necessary (ie, diuretics, inotropic agents if severely ill). Gamma globulin also has been effective.



Image Viral (most common).

Image Bacterial infection (also common): acute rheumatic fever, S aureus, Haemophilus influenzae, Neisseria meningitidis, streptococci, tuberculosis.

Image Complications from heart surgery.

Image Collagen vascular diseases.

Image Uremia.

Image Medications (ie, dantrolene, oncology agents).


Image Precordial pain with radiation to the shoulder and neck (often relieved by standing).

Image Pericardial friction rub on auscultation.

Image Signs of cardiac tamponade:

Image Distant heart sounds.

Image Tachycardia.

Image Pulsus paradoxus.

Image Hepatomegaly and venous distention.


Digitalis is typically not given in pericarditis, as this blocks the compensatory tachycardia the heart utilizes to overcome ↓ venous return.


Image CXR: A pear- or water bottle–shaped heart indicates a large effusion.

Image Echocardiography is diagnostic (can also detect tamponade).


Image Treat the underlying disease process.

Image Supportive treatment for viral etiologies.

Image Pericardiocentesis is indicated if effusion is present.

Image Urgent drainage is indicated when symptoms of tamponade are present.


Onset of CHF is dependent on the fall of pulmonary vascular resistance and the subsequent ↑ left-to-right shunting.



Image Caused from either congenital heart disease (CHD) or acquired heart disease.

Image CHD: Most common cause is from volume or pressure overload.

Image VSD, PDA, and endocardial cushion defects are the most common causes of CHF in the first 6 months of life.

Image ASD can cause CHF in adulthood if unrepaired.

Image Acquired heart disease: Potential causes of CHF are metabolic abnormalities (ie, hypoxia, acidosis, hypoglycemia, hypocalcemia), myocarditis, rheumatic fever with carditis, cardiomyopathy, and drug toxicity.


A left-to-right shunt usually takes about 6 weeks to become significant enough to stress the left ventricle.


Image Often similar symptoms to those found in respiratory illnesses: tachycardia, tachypnea, shortness of breath, rales and rhonchi, intercostal retractions.

Image Poor weight gain/poor feeding.

Image Cold sweat on forehead.

Image Older children develop peripheral edema.

Image Gallop on auscultation.

Image Hepatomegaly, jugular venous distention (JVD).


Use of diuretics in CHF is preferred to salt and fluid restriction.


Image CXR: Cardiomegaly, evidence of pulmonary edema.

Image Echo: Enlarged ventricular chamber, impaired ventricular function.


Watch out for hypokalemia, as ↑ potassium is lost with some diuretic use.


Image Treat the underlying cause (ie, surgical correction of CHD, correction of metabolic defects).

Image Oxygen can be used if patient is hypoxic or in respiratory distress.

Image Medication:

Image Digitalis is used to improve ventricular function. Contraindicated in complete heart block and hypertrophic cardiomyopathy.

Image Diuretics are used to ↓ volume overload and pulmonary edema. Most common are the “loop diuretics” (ie, furosemide).

Image Afterload-reducing agents (ie, angiotensin-converting enzyme [ACE] inhibitors, calcium channel blockers, nitroglycerin) are used to dilate peripheral vasculature and thus ↓ the work on the heart.


Hypokalemia can precipitate digitalis toxicity.


Henoch-Schönlein Purpura

Image Immune-mediated vasculitis that affects the GI tract, joints, and kidneys and causes a characteristic rash (see Chapter 20).

Image Palpable purpura.

Image Most often occurs in winter months, following a group A streptococcal upper respiratory infection (URI).

Image GI involvement is most significant, → vomiting and upper and lower GI bleeding.

Image Renal involvement, in the form of glomerulonephritis, with RBC casts, can progress to acute renal failure. More common is chronic proteinuria, which can be a late sequelae.

Image Treatment is supportive, with full recovery within 4–6 weeks.

Kawasaki Disease


A 2-year-old boy with fever for 7 days, often reaching 104°F (40°C), develops nonexudative conjunctival injection bilaterally; intensely erythematous lips, palms, and soles; generalized erythema multiforme; and an enlarged, tender anterior cervical lymph node. Blood cultures are sterile, and platelets are ↑. Think: Kawasaki disease.

Beware of his risk for coronary aneurysms and MI. Fever lasting for 5 or more days is the hallmark that must be present with at least four of the following features: conjunctival injection, oropharyngeal mucous membrane changes, extremity changes, polymorphous rash, and cervical lymphadenopathy.


Image Also known as mucocutaneous lymph node syndrome.

Image Most common acquired heart disease in children.


Acute vasculitis of unknown etiology.


Image Affects infants and young children (> 80% under age 4 years).

Image More common in Asians than other racial groups.

Image More common in males than females (ratio 1.5:1).

Image Most common in winter/spring months.


Image Sterile pyuria.

Image Aseptic meningitis.

Image Thrombocytosis.

Image Desquamation of fingers and toes.

Image Elevated ESR or CRP.

Image Most significant sequelae:

Image Coronary aneurysms (usually resolve within 12 months of adequate therapy).

Image Pericardial effusion.

Image CHF.


Image Diagnostic criteria: Fever for > 5 days plus ≥ 4 of the following:

1.     Bilateral conjunctivitis (without exudate).

2.     Mucocutaneous lesions (“strawberry” tongue; dry, red, cracked lips; diffuse erythema of oral cavity).

3.     Changes in upper and lower extremities (erythema and/or edema of hands/feet).

4.     Polymorphic rash (usually truncal).

5.     Cervical lymphadenopathy (> 1.5 cm in diameter), usually unilateral.

Image Echocardiogram: Initial study at diagnosis to establish baseline and to evaluate for early coronary aneurysms; follow-up echo to establish presence or absence.


Image Used to prevent cardiac sequelae.

Image Intravenous immune globulin (IVIG): Usually one dose of IVIG, 2 g/kg over 10–12 hr. Reduces incidence of coronary artery dilation (< 3%).

Image High-dose aspirin (80–100 mg/kg/day divided in four doses) until 48–72 hr after defervescence.

Image If no coronary artery abnormality, low-dose aspirin (3–5 mg/kg/day as a single daily dose) for 6–8 weeks or until platelet count and ESR are normal.

Image If coronary artery abnormality, continue indefinitely in consultation with a pediatric cardiologist. Aspirin is reduced after the patient is afebrile for 48 hours.

Image Use of steroids remains controversial and typically reserved for cases refractory to repeat doses of IVIG.

Polyarteritis Nodosa


A necrotizing inflammation of the small and medium-sized muscular arteries.


Image Prolonged fever, weight loss, malaise, subcutaneous nodules on extremities.

Image Various rashes can be associated with this condition.

Image Respiratory symptoms: Rhinorrhea, congestion.

Image Often waxes and wanes.

Image Gangrene of distal extremities is found in severe disease.


Hypertension and abdominal pain can be important clues in polyarteritis nodosa.


Image No diagnostic tests.

Image Associated with abnormal cell counts (thrombocytosis, leukocytosis), abnormal urine analysis, elevated acute-phase reactants, perinuclear antineutrophil cytoplasmic antibody (p-ANCA).

Image Conclusive with findings of medium-sized artery aneurysms.

Image Echocardiographic evidence of coronary artery aneurysms is diagnostic if other clinical evidence is present.


Image Corticosteroids suppress the clinical manifestations.

Image Cyclophosphamide or azathioprine may be required to induce remission.

Takayasu’s Arteritis


Image Also known as aortoarteritis.

Image Chronic inflammatory disease involving:

Image Aorta.

Image Arterial branches from the aorta.

Image Pulmonary vasculature.


Takayasu’s arteritis is also known as “pulseless disease.”


Image Lesions are segmental and often obliterative.

Image Aneurysmal and saccular dilation also occur.

Image Thoracoabdominal aorta is the predominantly affected site in the pediatric population.


Most patients are female, aged 4–45 years.


Image A significant number of patients experience LV dysfunction and CHF (even in the absence of coronary artery involvement, HTN, or valvular abnormalities).

Image A lymphocytic infiltration consistent with myocarditis is present in about 50% of patients.

Image Other symptoms include fever, polyarthralgias, polyarthritis, and loss of radial pulsations.


Essentially, Takayasu’s arteritis is giant cell arteritis of the aorta (and large branches).


Corticosteroids may induce remission.

Wegener’s Granulomatosis


A rare vasculitis of both arteries and veins → widespread necrotizing granumolas.


Most common in adults, although occurrence in children has been described.


Children with cyanotic heart disease are at ↑ risk for strokes and scoliosis.


Image Rhinorrhea, nasal mucosa ulcers, sinusitis.

Image Hematuria.

Image Cough, hemoptysis, pleuritis.

Image Heart involvement: Granulamatous inflammation of cardiac muscle causing arrhythmias.


Image Antineutrophil cytoplasmic antibodies (c-ANCA) are present.

Image ESR is greatly elevated.

Image Organ biopsy (kidney and/or lung) may be essential to establish early diagnosis.


Image Corticosteroids alone may be unsuccessful.

Image Cyclophosphamide or azathioprine is recommended (have changed a once uniformly fatal disease into an excellent prognosis).

Central Cyanosis versus Acrocyanosis

Image Central cyanosis:

Image Involves mucous membranes.

Image Always pathologic in a newborn.

Image Cyanosis in neonates is almost always due to either pulmonary or cardiac disease.

Image > 5 mg/dL of deoxyhemoglobin.

Image Acrocyanosis:

Image Involves distal extremities.

Image Normal in newborns.

Image Peripheral cyanosis is the result of acrocyanosis, exposure to cold, and ↓ peripheral perfusion.


Cyanotic Heart Defects

Five T’s and a P

Tetralogy of Fallot

Transposition of the great vessels

Truncus arteriosus

Tricuspid atresia

Total anomalous pulmonary venous return (obstructive)

Pulmonic atresia


See Figure 13-2.

Tetralogy of Fallot (TOF)

The most common form of cyanotic CHD in the postinfancy period.


Four anomalies constitute the tetralogy:

1.     Right ventricular outflow tract obstruction (RVOTO).

2.     VSD.

3.     Aortic override.

4.     RVH.


Two key features are required for diagnosis of TOF:

Image VSD (typically large enough to equalize pressures in right and left ventricles).

Image RVOTO (eg, pulmonary stenosis).

Image Aortic override is variable.

Image RVH is secondary to the RVOTO.


Prenatal factors associated include maternal rubella or viral illness.


RVOTO dictates degree of shunting:

Image Minimal obstruction: → ↑ pulmonary blood flow as pulmonary vascular resistance (PVR) ↓, → CHF.

Image Mild obstruction: Hemodynamic balance pressure between right and left ventricles is equal, thus no net shunting (“pink tet”).

Image Severe obstruction: ↓ pulmonary blood flow, → cyanosis.



FIGURE 13-2. Basic echocardiography views.

A. Top left: This frame, taken from a normal subject, is a parasternal long-axis (P L Ax) view taken slicing the heart from a parasternal location at the fourth left interspace, subtending a sector with the right ventricle (RV) anteriorly. The sound beams then pass through the ventricular septum and the aorta (AO), the left ventricular cavity (LV), and the left ventricular posterior wall. Behind the ascending aortic root is the left atrium (LA). Posterior to the heart behind the pericardium, the descending aorta (DAO) can be seen running in its cross-section, indicating how far from the sagittal body plane this image is as the descending aorta runs on the left of the spine. Bottom left: From the same normal subject in the parasternal short-axis (P S Ax) view from the third intercostal space. The sector subtends the right side of the heart as it winds around the aortic root (AO) in the center of image. The right atrium (RA) is separated from the right ventricle (RV) by the tricuspid valve. The pulmonary artery (PA) is separated from the right ventricle by the pulmonary valve. The cusps of the aortic valve can also be identified in this figure. The pulmonary artery bifurcates into its left (L) and right branches. Top right, bottom right: These parasternal short-axis (P S Ax) views taken from the same normal subject are sequential scans through the heart from the top to the bottom (from the cranial-to-caudal direction). The top frame represents a short-axis view of the entire right heart. In the center, the aortic valve (AO) cusps are seen in their open position, demonstrating a tri-leaflet aortic valve. Posteriorly, the left atrium (LA), with the left atrial appendage (LAA) extending to the left side of the heart, is observed. The interatrial septum separates the left atrium from the right atrium (RA). The tricuspid valve separates the right atrium from the right ventricle (RV). In the bottom frame with the caudal scan, the right (RV) and left (LV) ventricles are seen. The ventricular septum is seen between the two ventricles. The mitral valve (MV) is seen in its open position with the anterior cusp (arrow) at the top and the posterior cusp (arrow) at the bottom.

B. This is a series of apical four-chamber views (A 4 Ch) from a normal subject, demonstrating the scan from the anterior to the posterior aspect of the heart (from the apex to base). The electrocardiogram shown on the bottom indicates the timing within the cardiac cycle. The top frame is taken in systole. A left pulmonary vein (PV) can be seen entering the left atrium (LA). The right atrium (RA) is separated from the left atrium (LA) by the faint echo of the interatrial septum. The aortic root (AO) can be seen to be arising out of the heart, and the left and right ventricles (LV, RV) can be seen separated from their respective atria by the tricuspid and mitral valves in the closed position, and from each other by the ventricular septum. The second frame, taken with more caudal scanning, demonstrates the descending aorta in cross-section, the pulmonary veins (PV) from the left and the right (arrows), the atria, and the ventricles. This is an end-systolic frame, as seen from the electrocardiogram. The third frame, taken with most caudal scanning, demonstrates the descending aorta (DAO) posteriorly, and a small portion of the left atrium (LA) with a coronary sinus (CS) running inferiorly at the crux of the heart. The other labels are as for the previous panels. The fourth frame shows the aortic arch from the suprasternal notch sagittal view (SSN, SAG) in a normal infant. The scan comes from the suprasternal notch area, and the sector subtends the innominate vein (IV) superiorly, as it crosses in front of the ascending aorta (AAO). The whole arch is seen from the ascending aorta to the descending aorta (DAO). The brachiocephalic artery (BCA) and left carotid artery (LCA) can be seen arising from the aortic arch. The circular right pulmonary artery can be seen running under the arch (RPA). The label obscures the area of the right bronchus, lying between the aortic arch and the right pulmonary artery. Anteriorly, the thymus (Th) is identified.

C. These images from a normal subject are taken with the subcostal transducer position in coronal and sagittal (SAG) planes. The scans pass from the subcostal region through the liver and diaphragm, and into the heart. Right panels: In the top right panel with posterior angulation, the left ventricle (LV) and right ventricle (RV) are identified. The aorta (AO) arises from the left ventricle, separated from it by the aortic valve; the right atrium (RA) and right ventricle (RV) can be identified. The bottom right panel, taken in an orthogonal cut in the sagittal plane, demonstrates the left ventricle (LV) cut in cross-section with its papillary muscles; the right ventricle (RV) and pulmonary artery (PA) can be seen wrapping around the left ventricle. Left panels: These subcostal oblique cuts demonstrate the exit of the aorta (AO) and the pulmonary artery (PA) from their respective ventricles, the left ventricle (LV) and the right ventricle (RV). In the upper left panel, the aorta (AO) can be seen arising from the left ventricle (LV) and arching toward the left side over the main pulmonary artery (PA). In the bottom left panel, taken with an orthogonal view and more anterior angulation, the right-sided structures, right atrium (RA), right ventricle (RV), main (M) pulmonary artery, and right (R) pulmonary artery can be seen as they surround the aortic valve (AO). (Reproduced, with permission, from Rudolph’s Pediatrics, 20th ed. Appleton & Lange, 1996.)


Most common cyanotic heart defect in children who survive infancy.


Image Failure to thrive (FTT) (if diagnosed late).

Image “Conotruncal facies.”

Image Variable cyanosis (clubbing later if unrepaired).

Image RV impulse; occasional thrill; single S2, systolic ejection murmur at the upper left sternal border with or without ejection click.

Image Squatting is a common posture in older, unoperated children with TOF:

Image Often occurs after exercise.

Image Causes trapping of desaturated blood in the lower extremities and ↑ systemic vascular resistance (SVR) while the RVOTO remains fixed. Thus, it:

Image ↓ right-to-left shunting.

Image ↑ pulmonary blood flow.

Image ↑ arterial saturation.


Image Most common: 2–6 months of age.

Image Occur in the morning or after a nap when SVR is low.

Image Precipitating factors:

Image Stress

Image Drugs that ↓ SVR

Image Hot baths

Image Fever

Image Exercise

Image Mechanism: Unknown, but likely due to ↑ cardiac output with fixed RVOT, → ↑ right-to-left shunting, which ↑ cyanosis

Image If prolonged or severe: Syncope, seizures, cardiac arrest.


Image CXR (Figure 13-3).

Image “Boot-shaped heart.”

Image ↓ pulmonary vascular markings.

Image Right aortic arch (25%).


CXR with the boot shape, ↓ pulmonary vascular markings, and a right aortic arch. Think: Tetralogy of Fallot.


Image Patient’s clinical status may prevent definitive repair initially.

Image Shunting (ie, Blalock-Taussig shunt) is often used when pulmonary stenosis is severe and an alternative route for blood to reach the lungs is necessary.

Image Complete repair entails:

Image VSD closure.

Image Relief of RVOTO.

Image Ligation of shunts.

Image ASD/patent foramen ovale (PFO) closure.


Without repair of TOF, mortality is:

Image 50% by 3 years

Image 90% by 20 years

Image 95% by 30 years


FIGURE 13-3. Chest x-ray in tetralogy of Fallot.

Arrows indicate right-sided aortic arch and upper thoracic aorta. Dashed lines indicate right-sided aortic indentation on the air bronchogram. (Reproduced, with permission, from Rudolph CD, et al (eds). Rudolph’s Pediatrics, 21st ed. New York: McGraw-Hill, 2002: 1821.)

Transposition of the Great Vessels

The most common cyanotic heart lesion in the newborn period.


This lesion occurs when, in the development of the heart, the primitive heart loops to the left instead of the right and the following result (see Figure 13-4):

Image Aorta originates from the RV.

Image Pulmonary artery originates from LV.

Image Aorta is anterior; pulmonary trunk is posterior.

Image Right and left hearts are in parallel:

Image Pulmonary venous return goes to the pulmonary artery via left ventricle.

Image Systemic venous return goes to the aorta via the right ventricle.

Image The presence of a VSD, ASD, or PDA is essential to survival.

Image A PDA alone is usually not sufficient to allow adequate mixing in the extrauterine environment.

Image Cyanosis becomes more prevalent with the closure of the PDA.

Image Presentation: CHF in the first week of life.

Image CXR: Egg-shaped heart with a narrow mediastinum. Cardiomegaly with ↑ pulmonary vascular marking.


Most common cyanotic congenital heart defect presenting in the neonatal period.

Intact Ventricular Septum (with no valve abnormality)

With VSD (a large VSD allows adequate mixing)


Image Early cyanosis, a single S2, and no murmur.

Image An intact atrial septum or very restrictive PFO is a medical emergency.

Image Symptoms are related to ↑ pulmonary blood flow, with CHF sometimes occurring early.

Image May have little cyanosis.


FIGURE 13-4. Transposition of the great vessels.

“Egg on a string”—spinal column serving as the string and the globular presentation of the heart as the egg. (Reproduced, with permission, from Moller JH, Neal WA. Fetal, Neonatal, and Infant Cardiac Disease, 2nd ed. Appleton & Lange, 1992: 532.)


Image ECG will be normal initially, but will demonstrate right ventricular hypertrophy by 1 month.

Image CXR: “Egg on a string.”

Image ECG: Right or biventricular hypertrophy.

Image BAS if VSD does not allow adequate mixing.


Transposition of the great vessel: “Big blue baby” as intrauterine growth is normal.


Image Patient is “ductal dependent” and will require prostaglandin E1 (PGE1) to keep the PDA patent.

Image Early balloon atrial septostomy (BAS) is necessary to allow mixing of oxygenated and deoxygenated blood.

Image Arterial switch procedure is definitive.

Image PA band to control ↑ pulmonary blood flow.

Image Arterial switch with VSD closure is definitive.

Truncus Arteriosus


Image A persistent truncus is a single arterial trunk that emerges from the ventricles, supplying the coronary, pulmonary, and systemic circulations (see Figure 13-5).

Image Association: DiGeorge syndrome.


Image I: Short common pulmonary trunk arising from right side of common trunk, just above truncal valve.

Image II: Pulmonary arteries (PAs) arise directly from ascending aorta, from posterior surface.

Image III: Similar to type II, with PAs arising more laterally and more distant from semilunar valves.


FIGURE 13-5. Truncus arteriosus.


Image The valve has two, three, or four leaflets and is usually poorly functioning.

Image The truncus overrides a VSD.


Image Presentation: CHF and cyanosis in first week.

Image Initial left-to-right shunt symptoms:

Image Dyspnea.

Image Frequent respiratory infections.

Image FTT.

Image If pulmonary vascular resistance ↑, cyanosis ↑.

Image Second heart sound is prominent and single due to the single semilunar valve.

Image Peripheral pulses are strong, often bounding.

Image Often, a systolic ejection click can be appreciated.


CXR shows cardiomegaly and ↑ pulmonary vascular markings.


Image Surgery must occur before patient develops significant pulmonary vascular disease (usually 3–4 months of age).

Image VSD is surgically closed, leaving the valve on the LV side.

Image The pulmonary arteries are freed from the truncus and are connected to a valved conduit (Rastelli procedure), which will serve as the new pulmonary trunk.

Hypoplastic Left Heart Syndrome (HLHS)


The syndrome consists of the following (see Figure 13-6):

Image Aortic valve hypoplasia, stenosis, or atresia with or without mitral valve stenosis or atresia.


FIGURE 13-6. Hypoplastic left heart syndrome.

Note small size of left ventricle.

Image Hypoplasia of the ascending aorta.

Image LV hypoplasia or agenesis.

Image Mitral valve stenosis or atresia.

Image The result is a single (right) ventricle that provides blood to the pulmonary system, the systemic circulation via the PDA, and coronary system via retrograde flow after crossing the PDA.

Image In utero:

Image All systemic blood flow is ductus dependent.

Image Pulmonary resistance > systemic vascular resistance.

Image Normal perfusion pressure is maintained with right-to-left shunt through PDA and pulmonary resistance.

Image At birth:

Image PDA closes.

Image Systemic vascular resistance > pulmonary resistance.

Image PDA closure + hypoplastic LV = ↓ cardiac output and ↓ aortic pressure → circulatory death and metabolic acidosis.

Image PDA dependent until intervention is undertaken.


The second most common congenital heart defect, presenting in the first week of life (and the most common cause of death from CHD in the first month).


Image Pulses range from normal to absent (depending on ductal patency).

Image Hyperdynamic RV impulse.

Image Single S2 of ↑ intensity.

Image Gallop at apex due if there is heart failure.

Image Nonspecific systolic murmur at left sternal border (LSB).

Image Skin may have a characteristic grayish pallor.


Image CXR: Cardiomegaly with globular-shaped heart; ↑ pulmonary vascular markings, pulmonary edema.

Image Echocardiogram is diagnostic.


Image No intervention: Due to the high mortality and complicated surgical course of this disease, ethical dilemmas are frequent as to how far physicians should intervene.

Image Three-stage surgery:

Image Norwood procedure: The pulmonary trunk is used to reconstruct the hypoplastic aorta, and the right ventricle subsequently becomes the functional left ventricle. This leaves the pulmonary arteries connected but separated from the heart. The pulmonary blood flow is then reestablished via systemic to pulmonary conduits from the subclavian arteries to the pulmonary arteries.

Image Glenn procedure: The superior vena cava is connected to the right PA, restoring partial venous return to the lungs.

Image Fontan procedure: The inferior vena cava is anastamosed to the PAs, resulting in complete venous diversion from the systemic circulation to the lungs.

Image Heart transplant: This alternative occurs either as a primary intervention (if an organ is available) or after any of the previous palliative surgeries have provided maximal but ultimately insufficient benefit.


Left-to-right shunt (see Figure 13-7).

Subendocardial Cushion Defect


Related to the ostium primum ASD, this defect results from abnormal development of the AV canal (endocardial cushions) resulting in:

Image A VSD.

Image An ostium primum ASD.

Image Clefts in the mitral and tricuspid valves.


Image Association: Down syndrome (30% of patients with this defect have trisomy 21).

Image Also frequently found with asplenia and polysplenia syndromes.


Subendocardial cushion defects are associated with Down syndrome.


FIGURE 13-7. Acyanotic heart defects.


Image Often the result of the specific components of the accumulated defects:

Image Holosystolic murmur from the VSD, if restrictive.

Image Systolic murmur from mitral and tricuspid valve insufficiency.

Image High risk of developing Eisenmenger syndrome.

Image ECG: Superior QRS axis with RVH, right bundle branch block (RBBB), and LVH, along with a prolonged PR interval.


Image Surgical correction is sometimes the only option (despite high risk) when patient has an unbalanced AV canal.

Image Some benefit from PA banding if shunting is predominantly at the ventricular level (rare).


Children with trisomy 21 often have more favorable anatomy for surgical intervention.

Atrial Septal Defect (ASD)

Ten percent of all congenital heart disease.


Three types:

Image Secundum defect (most common—50–70%): Located in the central portion of the atrial septum.

Image Primum defect (about 30% of ASDs):

Image Located at the atrial lower margin.

Image Associated with abnormalities of the mitral and tricuspid valves.

Image Sinus venosus defect (about 10% of ASDs): Located at the upper portion of the atrial septum and often extends into the superior vena cava.


ASD is the most common congenital heart lesion recognized in adults.


Image A common “co-conspirator” in CHD.

Image As many as 50% of patients with congenital heart defects have an ASD as one of the defects.

Image More common in females (male-to-female ratio 1:2).


Image Children with ASDs are typically asymptomatic.

Image Widely split and fixed S2. Murmurs are uncommon, but may occur as patient gets older. Murmur is a secondary pulmonary flow murmur.

Image Symptoms of CHF and pulmonary HTN occur in adults (second and third decades).


Image ECG: The left-to-right shunt may produce right atrial enlargement and RVH.

Image CXR: Cardiomegaly with ↑ pulmonary vascular markings.


Image Nearly 90% will close spontaneously.

Image One hundred percent close if < 3 mm.

Image ASDs > 8 mm are unlikely to close spontaneously.

Image Surgical or catheter closure (via a “clamshell” or “umbrella” device) are used when indicated.

Patent Foramen Ovale (PFO)

Image The foramen ovale is used prenatally to provide oxygenated blood from the placenta to the left atrium.

Image It normally functionally closes when ↑ left atrial pressure causes the septa to press against each other (many remain “probe-patent” into adulthood).

Image In some children, the tissue of the foramen ovale is insufficient to cover the foramen (either from insufficient growth or becoming stretched from ↑ pressure or volume).

Image Some CHDs require a PFO for patient survival after birth (eg, tricuspid and mitral atresia, TAPVR).


VSD is the most common congenital heart disorder.

Ventricular Septal Defect (VSD)


A 2-month-old male born at term appeared well until 3 weeks ago, when he became dyspneic and had difficulty feeding. A loud pansystolic murmur is heard at the left lower sternal border, and ECG shows LVH and RVH.

Think: VSD.

Small VSD causes no symptoms. Large VSD may result in left-to-right shunt and pulmonary HTN. Left-sided volume overload and RVH are suggestive of a large VSD.


Image The most common form of recognized congenital heart disease (30–60% of all patients with CHDs).

Image Usually membranous, as opposed to in the muscular septum.

Image Occurs in 2 per 1000 live births.


Dependent on defect size:

Image Small VSDs:

Image Usually asymptomatic.

Image Normal growth and development.

Image High-pitched, holosystolic murmur.

Image No ECG or CXR changes.

Image Large VSDs:

Image Can → CHF and pulmonary HTN.

Image May have FTT.

Image Lower-pitched murmur; intensity dependent on the degree of shunting.


Image ECG: LVH.

Image CXR: Cardiomegaly with ↑ pulmonary vascular markings.


Spontaneous closure occurs in 30–50% of VSDs.


Image Spontaneous closure:

Image Muscular defects are most likely to close (up to 50%), with closure occurring during the first year of life.

Image Inlet and infundibular defects do not reduce in size or close.

Image Intervention is based on the development of CHF, pulmonary HTN, and growth failure.

Image Initial management with diuretics and digitalis.

Image Surgical closure is indicated when therapy fails.

Image Catheter-induced closure devices are less commonly used with VSDs than ASDs.

Image Endocarditis prophylaxis.


A PDA murmur is common (and normal) in newborn infants. It will usually disappear within the first 12 hours of life.



Image Most often a problem in premature neonates:

Image Left-to-right shunts are handled poorly by premature infants.

Image Many develop idiopathic respiratory distress syndrome.

Image Some progress to develop left ventricular failure.

Image Failure of spontaneous closure:

Image Premature infants: Due to ineffective response to oxygen tension.

Image Mature infants: Due to structural abnormality of ductal smooth muscle.


Image PDA is more common in females (male-to-female ratio 1:3).

Image Incidence is higher at higher altitudes due to lower atmospheric oxygen tension.

Image Maternal rubella in the first trimester has also been implicated in PDA.


Image In the normal neonate, the ductus arteriosus closes primarily in response to a ductal PO2 > 50 mm Hg.

Image PDA closes within 15 hours after birth.

Image Complete closure by 3 weeks to become the ligamentum arteriosum.

Image Hypoxia and prematurity have a tendency to keep the ductus arteriosus patent.


Image Small PDAs usually are asymptomatic.

Image Large PDAs ↑ incidence of lower respiratory tract infections and CHF.

Image Machinery-like murmur.

Image Bounding peripheral pulses and wide pulse pressure.

Image If Eisenmenger syndrome results, patient may have cyanosis restricted to the lower extremities.


Image Indomethacin: Used in premature infants. Inhibits prostaglandin synthesis, → closure.

Image Catheter closure via devices such as double-umbrella devices and coils in older children.

Image Surgical ligation and division via a left lateral thoracotomy.

Image An occasional complication is recurrent laryngeal nerve injury → hoarseness.

Image Eisenmenger syndrome is a contraindication to surgery.


Subacute bacterial endocarditis (SBE) is more common in small PDAs than large ones.


Image PDA-dependent congenital heart abnormalities include:

Image Tetralogy of Fallot

Image Tricuspid atresia

Image TAPVR with obstruction

Image Aortic coarctation (severe)


Infective endocarditis is the most common complication of PDA in late childhood.

Image Pulmonic atresia

Image Hypoplastic left heart

Image Prostaglandin E1 (PGE1) can be potentially lifesaving in a cyanotic newborn with PDA-dependent congenital heart abnormalities.

Image CHD presenting in first 23 weeks of life are usually due to ductal-dependent lesions.


Image Critically ill newborn with:

Image Suspected ductal-dependent lesion.

Image Suspected LV outflow tract obstruction.

Image Dose:

Image 0.05 to 0.1 μg/kg/min (to reopen the ductus).

Image 0.01 μg/kg/min (to maintain ductal patency).

Image Side effects:

Image Apnea: Endotracheal intubation prior to transport.

Image Fever, hypotension, and seizures.

Image Do not delay PGE1 administration in critically ill neonates with suspected ductal-dependent lesion pending definitive cardiac diagnosis.

Eisenmenger Syndrome

Image Can occur in unrepaired left-to-right shunts (ie, VSD) that cause an ↑ pressure load on the pulmonary vasculature.

Image Pressure overload on the pulmonary vasculature can result in irreversible changes in the arterioles.

Image This develops into pulmonary vascular obstructive disease, usually over several years.

Image The pulmonary HTN reduces the left-to-right shunt and previous LVH often resolves.

Image Persistent HTN maintains an enlarged right ventricle and can dilate the main pulmonary segment (this becomes evident on CXR).

Image Avoidance of this condition via surgical correction of CHD is essential, as it causes irreversible changes.


See Figure 13-8.

Tricuspid Atresia


RV inlet is absent or nearly absent:

Image Eighty-nine percent have no evidence of tricuspid valve tissue, only dimple.

Image Seven percent have a membranous septum forming part of the right atrial floor.

Image Three percent are Ebstein’s.

Image One percent have a tiny, imperforate valvelike structure.


FIGURE 13-8. Congenital valvular defects.


Bicuspid aortic valve is the most common congenital heart defect, occurring in 1–2% of the population. This defect goes largely unrecognized.


Image ASD and/or VSD is usually present.

Image Seventy-five percent will present with cyanosis within the first week.


LV impulse displaced laterally.


ECG: LVH, prominent LV forces (due to ↓ RV voltages).


Image PGE1 to maintain ductal patency.

Image Surgical intervention.

Image Modified Blalock-Taussig (BT) shunt.

Image Glenn procedure, followed by Fontan procedure.

Pulmonary Atresia (with Intact Ventricular Septum)


Image Cyanosis within hours of birth (PDA closing).

Image Hypotension, tachypnea, acidosis.

Image Single S2, with a holosystolic murmur (tricuspid regurgitation).


Image ECG: ↓ RV forces and occasionally RVH.

Image CXR: Normal to enlarged RV with ↓ pulmonary vascular markings.


Image PGE1 to maintain ductal patency.

Image Balloon atrial septostomy (sometimes).

Image Reconstruction of RVOT with transannular patch or pulmonary valvotomy.

Image ASD left open to prevent systemic venous HTN.

Aortic Stenosis


A 4-year-old boy with recurrent episodes of syncope while playing has a harsh systolic murmur radiating to the carotids, diminished cardiac pulses, and severe LVH. Think: Congenital aortic stenosis.

Angina, syncope, and congestive heart failure are the presentation of aortic stenosis. Syncope during exertion occurs due to the reduced cerebral perfusion when arterial pressure declines. However, many patients now are diagnosed before the development of these symptoms on the basis of the finding of a systolic murmur followed by echocardiography.


Eighty-five percent of congenitally stenotic aortic valves are bicuspid.


Image Severe stenosis generally presents shortly after birth.

Image Older children may complain of chest or stomach pain (epigastric).

Image Patients with untreated severe aortic stenosis are at risk for syncope and sudden death.

Image The characteristic murmur is a crescendo-decrescendo systolic murmur.

Image A systolic ejection click is also common (particularly if bicuspid aortic valve).

Image In severe disease, paradoxical splitting of S2 occurs (split narrows with inspiration).


Supravalvular aortic stenosis is associated with idiopathic hypercalcemia.


Image Clinical findings, including ECG findings, and symptoms can be deceiving.

Image Echo or catheterization to evaluate pressure differences between the aorta and left ventricle is essential.


Image Surgical or interventional balloon.

Image Valvotomy is most common intervention:

Image Indication is usually if the measured catheterization gradient is > 50 mm Hg.

Image High incidence of recurrent stenosis.

Image Valve replacement: Deferred, when possible, until patient completes growth.

Aortic Insufficiency


Uncommon and usually associated with mitral valve disease or aortic stenosis.


Image A diastolic, decrescendo murmur is present at the left upper sternal border.

Image Presentation with symptoms indicates advanced disease.

Image Chest pain and CHF are ominous signs.


CXR: LV enlargement, dilated ascending aorta.


People with Marfan syndrome frequently have aortic insufficiency as well.


Image Surgery or balloon valvuloplasty to treat aortic stenosis may worsen the insufficiency.

Image Aortic valve replacement is the only definitive therapy.

Mitral Stenosis


Image Rare in children; usually a sequela of acute rheumatic fever.

Image Congenital forms are generally severe.


Image When symptomatic, dyspnea is the most common symptom.

Image Weak peripheral pulses with narrow pulse pressure.

Image An opening snap is heard on auscultation; also, a presystolic murmur may be heard.

Image Pulmonary venous congestion occurs, →:

Image CXR evidence of interstitial edema.

Image Hemoptysis from small bronchial vessel rupture.


Image Balloon valvuloplasty

Image Surgical:

Image Commissurotomy

Image Valve replacement

Mitral Valve Prolapse


Caused by thick and redundant valve leaflets that bulge into the mitral annulus.


Image Usually occurs in older children and adolescents.

Image Has a familial component (autosomal dominant).

Image Nearly all patients with Marfan syndrome have it.


Image Auscultation: Midsystolic click and late systolic murmur.

Image Often asymptomatic with some history of palpitations and chest pain.


Coarctation of the aorta is associated with Turner syndrome.


Management is symptomatic (eg, β blocker for chest pain).


Coarctation of the Aorta


Image Most commonly found in the juxtaductal position (where the ductus arteriosus joins the aorta).

Image Development of symptoms may correspond to the closure of the ductus arteriosus (the patent ductus provides additional room for blood to reach the postductal aorta).


The presence of ↓ pulses in the lower extremities is the clue for diagnosis of coarctation.


Image More common in males than females (male-to-female ratio 2:1).

Image Association: Seen in one-third of patients with Turner syndrome.


Comparison of the right upper extremity blood pressures and pulse oximeter readings with the lower extremity should be performed with a possible diagnosis of coarctation.


Clinical Presentation of Symptomatic Infants

Image FTT, respiratory distress, and CHF develop in the first 2–3 months of life.

Image Lower extremity changes: ↓ pulses in the lower extremities.

Image Acidosis may develop as the lower body receives insufficient blood.

Image Usually, a murmur is heard over the left back.

Clinical Presentation of Asymptomatic Infants or Children

Image Normal growth and development.

Image Occasional complaint of leg weakness or pain after exertion.

Image ↓ pulses in the lower extremities.

Image Upper-extremity HTN (or at least greater than in the lower extremities).


CXR: “3 sign,” dilated ascending aorta that displaces the superior vena cava to the right (see Figure 13-9).


Image Resection of the coarctation segment with end-to-end anastomosis is the intervention of choice for initial treatment.

Image Allograft patch augmentation can also be used.

Image Catheter balloon dilation can be used:

Image Has a higher restenosis rate than surgery.

Image Has an ↑ risk of producing aortic aneurysms.

Image Balloon dilation is more frequently used when stenosis occurs at the surgical site of a primary reanastamosis.

Ebstein’s Anomaly


Components of the defect (see Figure 13-10):

Image The tricuspid valve is displaced apically in the right ventricle.

Image The valve leaflets are redundant and plastered against the ventricular wall, often causing functional tricuspid atresia.

Image The right atrium is frequently the largest structure.


FIGURE 13-9. Coarctation of the aorta.


FIGURE 13-10. Ebstein’s anomaly.


Without intervention:

Image CHF in first 6 months.

Image Nearly 50% mortality.


Image Growth and development can be normal depending on severity of the lesion.

Image Older patients usually complain of dyspnea, cyanosis, and palpitations.

Image Widely split S1, fixed split S2, variable S3 and S4 (characteristic triple or quadruple rhythm).

Image Holosystolic murmur at left lower sternal border.

Image Opening snap.

Image Cyanosis from atrial right-to-left shunt.


Image ECG: Right axis deviation, right atrial enlargement, RBBB; WPW is present in 20%.

Image CXR: Cardiomegaly (“balloon-shaped”) “wall-to-wall heart” in severely affected infants.

Image Echocardiogram is diagnostic.


Intervention (87% do well):

Image Glenn procedure to ↑ pulmonary blood flow.

Image Severely affected infants may require aortopulmonary shunt.

Image Tricuspid valve replacement or reconstruction.

Image Right atrial reduction surgery.

Image Ablation of accessory conduction pathways.

Total Anomalous Pulmonary Venous Return (TAPVR)

The pulmonary veins bring the blood from the lungs to the right atrium (instead of the left atrium).


Image See Figure 13-11A and B.

Image No communication exists between the pulmonary veins and the left atrium.

Image All pulmonary veins drain to a common vein.

Image The common vein drains into the:

Image Right superior vena cava (50%).

Image Coronary sinus or right atrium (20%).

Image Portal vein or inferior vena cava (20%).

Image Combination of the above types (10%).

Image An ASD is needed for survival.


Dramatically more common in males (male-to-female ratio 4:1).


Presence or absence of obstruction of pulmonary venous return changes the clinical presentation.

TAPVR with Obstruction

Image Obstruction → ↑ pulmonary artery pressure (and subsequent pulmonary edema) that ↑ pulmonary then right atrial and ventricular pressures. This causes a right-to-left shunt and resultant cyanosis.

Image Presents with early, severe respiratory distress and cyanosis, no murmur, and hepatomegaly.

Image CXR: Normal-size heart, pulmonary edema.

Image Echocardiogram is diagnostic.

Image Management: Baloon atrial septostomy or immediate corrective surgery.

TAPVR Without Obstruction

Image Free communication between right atrium and left atrium.

Image Large right-to-left shunt (“large ASD”).

Image Presents later during first year of life, with mild FTT, recurrent pulmonary infections, tachypnea, right heart failure, and rarely cyanosis.

Image CXR: Cardiomegaly, large PAs; ↑ pulmonary vascular markings (“snowman” or “figure eight” sign) is found in infants > 4 months old.

Image Management: Surgical movement of pulmonary veins to the left atrium.

Hypertrophic Obstructive Cardiomyopathy

Image Autosomal dominant 60%, sporadic 40%.

Image Sudden death: 4% to 6% incidence.

Image Asymmetrical septal hypertrophy or idiopathic hypertrophic subaortic stenosis (IHSS) is the most common form.

Image Outflow obstruction potentially caused by leaflet of mitral valve.

Image ECG: LVH and left atrial enlargement, large Q wave (indicates septal hypertrophy).

Image Echo: Asymmetrical septal hypertrophy, outflow obstruction (which predict the severity of disease).


FIGURE 13-11. Total anomalous pulmonary venous return.

A. Supracardiac view. B. Infracardiac view.


Image Moderate restriction of physical activity.

Image β blocker or calcium channel blocker to improve filling.

Image Endocarditis prophylaxis.


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