CARDIAC EVALUATION AND INTRODUCTION TO CONGENITAL HEART LESIONS
History
Children do not present with the typical features of congestive heart failure as seen in adults.
Age is very important when assessing child.
Infants:
} Feeding difficulties
} Easily fatigued
} Sweating while feeding
} Rapid respirations
Older children:
} Shortness of breath
} Dyspnea on exertion
Physical examination
Need to refer to normal heart and respiratory rates for ages to determine tachycardia and tachypnea.
Height and weight should be assessed to determine proper growth.
Always get upper and lower extremity blood pressures and pulses.
Hepatosplenomegaly suggests right-sided heart failure.
Rales on auscultation may indicate pulmonary edema and left-sided heart failure.
Cyanosis and clubbing result from hypoxia.
Table 13-1. Heart Murmur Gradation
| Grade | Quality |
| 1 | Soft, difficult to hear |
| 2 | Easily heard |
| 3 | Louder but no thrill |
| 4 | Associated with thrill |
| 5 | Thrill; audible with edge of stethoscope |
| 6 | Thrill; audible with stethoscope just off chest |
Diagnostic tests—chest radiograph
Evaluate:
Heart size
Lung fields
Ribs for notching
Position of great vessels
Electrocardiogram
Echocardiography—definitive diagnosis
Other—MRI, cardiac catheterization, angiography, exercise testing
Embryology—knowledge of cardiac embryology is helpful for understanding congenital cardiac lesions, their presentations, symptoms, and treatment.
Innocent Murmurs
A 5-year-old boy is seen for routine physical examination. Parents voice no concerns. Weight and height are at the 75th percentile. Vital signs are normal. Physical examination is remarkable for a soft musical 2/6 murmur best heard at the left lower sternal border.
Are also known as functional, normal, insignificant, or flow murmurs. These result from flow through a normal heart, vessels, and valves.
More than 30% of children ages 3−7 may have an innocent murmur heard at some time in their lives.
Presentation
Usually heard on routine physical examination; also easily heard during fever, infection, or anxiety
Never diastolic
Never greater than grade 2/6
Soft, vibratory, and musical at the left lower midsternum
Diagnostic tests—none
Treatment—reassurance of parents and patient recommended
Congenital Heart Disease
Introduction
Diagnosis usually made by age 1 month in most
Murmurs may not be heard in early life because of increased pulmonary vascular resistance (from fetal to neonatal transition physiology).
Etiology
Most are unknown
Associated with teratogens, such as alcohol and rubella
Genetic predisposition—trisomies; Marfan, Noonan, DiGeorge syndromes
Classification
Table 13-2. Congenital Heart Disease
| Shunting | ||||
| Regurgitant | Stenotic | Right → Left | Left → Right | Mixing |
| MVP | Aortic stenosis | Tetralogy of Fallot | Patent ductus | Truncus |
| PI, AI | Pulmonic stenosis | Ebstein anomaly | Ventricular septal defect | TAPVR |
| MI, TI | Coarctation | Tricuspid atresia | Atrial septal defect, endocardiac cushion defect | HLH,
Transposition |
Definition of abbreviations: TAPVR total anomalous pulmonary venous return; HLH hypoplastic left heart; MVP mitral valve prolapse; PI pulmonic insufficiency; AI aortic insufficiency; MI myocardial infarction; TI tricuspid insufficiency
LEFT TO RIGHT SHUNTS
Ventricular Septal Defect (VSD)
A 3-month-old child presents with poor feeding, poor weight gain, and tachypnea. Physical examination reveals a harsh, pansystolic 3/6 murmur at the left lower sternal border, and hepatomegaly
Most common congenital heart lesion
Most are membranous
Shunt determined by ratio of PVR to SVR
As PVR falls in first few weeks of life, shunt increases
When PVR>SVR, Eisenmenger syndrome (must not be allowed to happen)
Clinical findings
Asymptomatic if small defect with normal pulmonary artery pressure (most); large defect—dyspnea, feeding difficulties, poor growth, sweating, pulmonary infection, heart failure
Harsh holosystolic murmur over lower left sternal border ± thrill; S2 widely split
– With hemodynamically significant lesions, also a low-pitched diastolic rumble across the mitral valve heard best at the apex
Diagnosis—chest-ray (large heart, pulmonary edema), ECG (LVH), echocardiogram is definitive
Treatment
Small muscular VSD more likely to close in first 1–2 years than membranous
Less common for moderate to large to close → medical treatment for heart failure
(control failure and prevent pulmonary vascular disease)
Surgery in first year; indications:
Failure to thrive or unable to be corrected medically
Infants at 6–12 months with large defects and pulmonary artery hypertension
More than 24 months of age with Qp:Qs >2:1 (shunt fraction)
Complications
Large defects lead to heart failure, failure to thrive
Endocarditis
Pulmonary hypertension
Atrial Septal Defect (ASD)
Ostium secundum defect most common (in region of fossa ovalis)
Clinical
Few symptoms early in life because of structure of low-flow, left-to-right shunt
In older children, often with large defects; varying degrees of exercise intolerance
– With hemodynamically significant lesions, also a low-pitched diastolic rumble across the tricuspid valve heard best at the lower sternum
Physical examination
Wide fixed splitting of S2
Systolic ejection murmur along left mid to upper sternal border (from increased pulmonary flow)
Diagnosis
Chest x-ray—varying heart enlargement (right ventricular and right atrial); increased pulmonary vessel markings, edema
ECG—right-axis deviation and RVH
Echocardiogram definitive
Treatment
Most in term infants close spontaneously; symptoms often do not appear until third decade
− Surgery or transcatheter device closure for all symptomatic patients or 2:1 shunt
Complications
Dysrhythmia
Low-flow lesion; does not require endocarditis prophylaxis
Endocardial Cushion Defect
Pathophysiology
When both ASDs and VSDs occur, which are contiguous, and the atrioventricular valves are abnormal
Left-to-right shunt at both atrial and ventricular levels; some right-to-left shunting with desaturation (mild, intermittent cyanosis)
Atrioventricular valve insufficiency → increase volume load on one or both ventricles;
early heart failure, infections, minimal cyanosis, hepatomegaly, and failure to thrive
Physical examination
Heart failure early in infancy (hepatomegaly, failure to thrive)
Eisenmenger physiology occurs earlier
Moderate-to-severe increase in heart size with hyperdynamic precordium (precordial bulge and lift)
Widely fixed split S2 (like an isolated ASD)
Pulmonary systolic ejection murmur, low-pitched diastolic rumble at left sternal
Diagnostic tests
Chest x-ray—significant cardiomegaly, increased pulmonary artery and pulmonary blood flow and edema
ECG—signs of biventricular hypertrophy, right atrial enlargement, superior QRS axis
Echocardiogram (gold standard)
Treatment—surgery more difficult with heart failure and pulmonary hypertension (increased pulmonary artery pressure by 6−12 months of age); must be performed in infancy
Complications
Without surgery—death from heart failure
With surgery—arrhythmias, congenital heart block
Patent Ductus Arteriosus (PDA)
Results when the ductus arteriosus fails to close; this leads to blood flow from the aorta to the pulmonary artery
Risk factors
More common in girls by 2:1
Associated with maternal rubella infection
Common in premature infants (developmental, not heart disease)
Presentation
If small—possibly no symptoms
If large—heart failure, a wide pulse pressure, bounding arterial pulses, characteristic sound of “machinery,” decreased blood pressure (primarily diastolic)
Diagnostic tests
Chest x-ray—increased pulmonary artery with increased pulmonary markings and edema; moderate-to-large heart size
ECG—left ventricular hypertrophy
Echocardiogram—increased left atrium to aortic root; ductal flow, especially in diastole
Treatment
May close spontaneously
Indomethacin (preterm infants)
Surgical closure
Complications
Congestive heart failure
Infective endocarditis
STENOTIC LESIONS
Pulmonic Stenosis
Pathophysiology
− Deformed cusps → opens incompletely during systole; obstruction to right ventricular outflow → increased systemic pressure and wall stress → right ventricular hypertrophy (depends on severity of pulmonary stenosis)
− Arterial saturation normal unless ASD or VSD is present with R → L shunt
− Neonate with severe pulmonary stenosis = critical pulmonary stenosis = R → L shunt via foramen ovale
Physical examination
Heart failure only in severe cases, most in first month of life
Mild cases—normal life, usually no progression
− Moderate to severe—increasing gradient with growth: signs of right ventricular failure (hepatomegaly, peripheral edema, exercise intolerance)
− Pulmonary ejection click after S1 in left upper sternal border and normal S2 (in mild); relatively short, low-to-medium−pitched SEM over pulmonic area radiating to both lung fields
Diagnosis
ECG—right ventricular hypertrophy in moderate to severe; tall, spiked P-waves; right atrial enlargement (RAE)
Chest x-ray—poststenotic dilatation of pulmonary artery; normal-to-increased heart size (right ventricle) and decreasing pulmonary vascularity
Echocardiogram (gold standard)
Complications
Heart failure
Endocarditis (lower risk)
Secondary subvalvular muscular and fibrous hypertrophy
Treatment
Moderate to severe—balloon valvuloplasty initially; may need surgery
Neonate with critical pulmonary stenosis—emergent surgery
Aortic Stenosis
Most are bicuspid aortic valve—usually asymptomatic in children
Supravalvular stenosis (least common form)—sporadic, familial, or with Williams syndrome (mental retardation, elfin facies, heart disease, idiopathic hypercalcemia; deletion of elastin gene 7q11.23)
Clinical presentation—symptoms depend on severity of obstruction
If severe early in infancy = critical aortic stenosis = left ventricular failure and decreased cardiac output
If significant decrease in cardiac output—intensity of murmur at right upper sternal border may be minimal
Mild to moderate—usually asymptomatic with normal growth and development
Often discovered with murmur on routine physical examination
Rare—older children present with syncope, fatigue, angina, dizziness
With increasing severity—decreased pulses, increased heart size, left ventricular apical thrust
Early systolic ejection click at apex of left sternal border (does not vary with respiration)
° Severe—no click and decreased S1 (decreased left ventricular compliance), decreased S2 (aortic component), and maybe an S4
SEM upper-right second intercostal space; the louder (harsher) and longer the murmur, the greater the degree of obstruction; radiates to neck and left midsternal border; positive thrill in suprasternal notch
Diagnosis
ECG—left ventricular hypertrophy and strain
Chest x-ray—prominent ascending aorta; may have valve calcification (older children and adults); if severe → increased heart size (left ventricular hypertrophy)
Echocardiogram (gold standard)
Treatment
Balloon valvuloplasty
Surgery on valves
Valve replacement
Coarctation of the Aorta
Definition—narrowing at any point from transverse arch to iliac bifurcation; 90% just below origin of left subclavian artery at origin of ductus arteriosus (juxtaductal coarctation)
Adult versus childhood
Discrete juxtaductal coarctation (adult type)
Ascending aortic blood flows normally through narrowed segment to reach descending aorta, but there is left ventricular hypertrophy and hypertension
If mild, not recognized until later in childhood
Increased blood pressure in vessels proximal to coarctation and decreased blood pressure and pulses below constriction
Femoral and other lower pulses weak or absent; bounding in arms and carotids; also delay in femoral pulse compared to radial (femoral normally occurs slightly before radial)
Normally, leg systolic pressure is 10–20 mm Hg higher than in arms; in coarctation, leg systolic pressure is decreased (>5%)
If pressure is greater in right arm than left arm, suggests coarctation involving left subclavian artery
Short systolic murmur along left sternal border at third-to-fourth intercostal space → left scapula and neck
Hypertension due not only to mechanical but also to neurohormonal reasons
Over time, patient develops an extensive collateral circulation (systolic or continuous murmurs over left and right sides of chest with thrills), rib notching (dilated intercostal arteries)
Tubular hypoplasia (preductal, infantile type)
Severe narrowing starting at one of the head or neck vessels and extending to the ductus
Right ventricular blood flows across the PDA to supply the descending aorta so the perfusion of the lower part of the body is dependent upon right ventricular output
Seen as differential cyanosis—upper body is pink, lower is cyanotic; prominent heart failure as ductus closes (if completely atretic = interrupted aortic arch)
Presents with lower body hypoperfusion, acidosis, and severe heart failure with ductal closure; large heart, systolic murmur along left sternal border
Diagnostic tests
Chest x-ray—depends on age and effects of hypertension and collaterals
} Severe (infantile)—increased heart size and pulmonary congestion
} Adult—findings usually occur after first decade:
Increased size of subclavian artery—prominent shadow in left superior mediastinum
Notching of inferior border of ribs from passive erosion of increased collaterals in late childhood
Poststenotic dilatation of ascending aorta
Diagnosis
ECG—left ventricular hypertrophy in older children; in neonates, biventricular hypertrophy
Echocardiogram (gold standard)
Treatment
Neonate—PGE1 infusion to maintain patent, ductus, which establishes adequate lower extremity blood flow; surgery after stabilization
Surgery soon after diagnosis of any significant coarctation
Adult—treat heart failure and hypertension, then follow with surgery
Complications
Associated cerebrovascular disease
Systemic hypertension
Endocarditis
Aortic aneurysms
CYANOTIC LESIONS (RIGHT TO LEFT SHUNTS)
Cyanotic Lesions Associated With Decreased Pulmonary Blood Flow
Tetralogy of Fallot (TOF)
A 6-month-old infant is prone to episodes of restlessness, cyanosis, and gasping respirations. Symptoms resolve when he is placed in the knee-chest position. Physical examination reveals an underweight infant, with a harsh long systolic ejection murmur and a single second heart sound.
Components
Pulmonary stenosis and infundibular stenosis (obstruction to right ventricular outflow)
VSD
Overriding aorta (overrides the VSD)
Right ventricular hypertrophy
Most common cyanotic lesion
Pulmonary stenosis plus hypertrophy of subpulmonic muscle (crista supraventricularis) → varying degrees of right ventricular outflow obstruction
Blood shunted right-to-left across the VSD with varying degrees of arterial desaturation and cyanosis
If mild, patient may not be visibly cyanotic (pink tetralogy of Fallot)
With growth and further hypertrophy of infundibulum, cyanosis may be seen later in first year of life
With severe obstruction, cyanosis in the immediate neonatal period (ductal dependent)
If not corrected, older children are blue, have marked clubbing, and have dyspnea on exertion (child will squat to increase systemic vascular resistance and to decrease right-to-left shunt)
Paroxysmal hypercyanotic attacks (tet spells)
Acute onset of hyperpnea and restlessness → increased cyanosis → gasping →
syncope (increased infundibular obstruction with further right-to-left shunting
Treatment—place in lateral knee-chest position, give oxygen, inject subcutaneous morphine, give beta-blockers
Physical examination—substernal right ventricular impulse, systolic thrill along thirdto-fourth intercostal space on left sternal border, loud and harsh systolic ejection murmur (upper sternal border), may be preceded by a click; either a single S2 or soft pulmonic component
Diagnosis
Chest x-ray—hypertrophied right ventricle causes the apex to be uplifted above the diaphragm → boot-shaped heart plus dark lung fields (decreased pulmonary blood flow)
ECG—right axis deviation plus right ventricular hypertrophy
Echocardiogram (gold standard)
Pre-correction complications—cerebral thromboses, brain abscess, bacterial endocarditis, heart failure, but not common because of early correction
Treatment
Depends on degree of obstruction
PGE1 infusion—prevent ductal closure; given if cyanotic at birth
Augment pulmonary blood flow with palliative systemic to pulmonary shunt
(modified Blalock-Taussig shunt)
Corrective surgery (electively at age 4–12 months)—remove obstructive muscle, valvulotomy, and patching of VSD
Tricuspid atresia
Pathophysiology—no outlet from the right atrium to the right ventricle; entire venous (systemic) return enters the left atrium from a foramen ovale or ASD (there must be an atrial communication); left ventricular blood to right ventricle (atretic) via a VSD and is augmented by PDA; therefore, pulmonary blood flow depends on presence (and size) of VSD
Clinical presentation
Will present at birth with severe cyanosis
Increased left ventricular impulse (contrast to most others with right ventricular impulse), holosystolic murmurs along left sternal border (most have a VSD; though right ventricle is small, it is still a conduit for pulmonary blood flow)
Diagnosis
Chest x-ray—pulmonary undercirculation
ECG—left axis deviation plus left ventricular hypertrophy (distinguishes from most other congenital heart disease)
Echocardiogram (gold standard)
Treatment
PGE1 until aortopulmonary shunt can be performed
May need an atrial balloon septostomy (to make larger ASD)
Later, staged surgical correction
Ebstein anomaly
Development associated with periconceptional maternal lithium use in some cases
Downward displacement of abnormal tricuspid valve into right ventricle; the right ventricle gets divided into two parts: an atrialized portion, which is thin-walled, and smaller normal ventricular myocardium
Right atrium is huge; tricuspid valve regurgitant
Right ventricular output is decreased because
Poorly functioning, small right ventricle
Tricuspid regurgitation
Variable right ventricular outflow obstruction—abnormal anterior tricuspid valve leaflet. Therefore, increased right atrial volume shunts blood through foramen ovale or ASD → cyanosis
Clinical presentation
Severity and presentation depend upon degree of displacement of valve and degree of right ventricular outflow obstruction
May not present until adolescence or adulthood
If severe in newborn → marked cyanosis, huge heart
Holosystolic murmur of tricuspid insufficiency over most of anterior left chest
(most characteristic finding)
Diagnosis
Chest x-ray—heart size varies from normal to massive (increased right atrium); if severe, decreased pulmonary blood flow
ECG—tall and broad P waves, right bundle branch block, and a normal or prolonged PR interval
Treatment
PGE1
Systemic-to-pulmonary shunt
Then staged surgery
Cyanotic Lesions Associated with Increased Pulmonary Blood Flow
Transposition of the great arteries (TGA)
Pathophysiology
Aorta arises from the right ventricle, and the pulmonary artery from the left ventricle; d = dextroposition of the aorta anterior and the right of the pulmonary artery (normal is posterior and to the right of the pulmonary artery)
Series circuit changed to 2 parallel circuits; need foramen ovale and PDA for some mixture of desaturated and oxygenated blood; better mixing in half of patients with a VSD
Clinical presentation
With intact septum (simple TGA)—as PDA starts to close, severe cyanosis and tachypnea ensue
S2 usually single and loud; murmurs absent, or a soft systolic ejection murmur at midleft sternal border
If VSD is present, there is a harsh murmur at the lower left sternal border. If large, then holosystolic murmur, significant mixing of blood lessens cyanosis, but presents as heart failure
Diagnosis
Chest x-ray:
Mild cardiomegaly, narrow mediastinum, and normal-to-increased pulmonary blood flow
“Egg on a string” appearance—narrow heart base plus absence of main segment of the pulmonary artery
ECG—normal neonatal right-sided dominance
Echocardiogram (gold standard)
Treatment
PGE1 (keeps PDA patent)
Balloon atrial septostomy
Arterial switch surgery in first 2 weeks
Truncus arteriosus
Pathophysiology
Single arterial trunk arises from the heart and supplies all circulations.
Truncus overlies a ventral septal defect (always present) and receives blood from both ventricles (total mixing).
Both ventricles are at systemic pressure.
Clinical presentation
With dropping pulmonary vascular resistance in first week of life, pulmonary blood flow is greatly increased and results in heart failure.
Large volume of pulmonary blood flow with total mixing, so minimal cyanosis
If uncorrected, Eisenmenger physiology
Single truncal valve, which may be incompetent (high-pitched, early diastolic decrescendo at mid-left sternal border)
Initially, SEM with loud thrill, single S2, and minimal cyanosis
With decreasing pulmonary vascular resistance (PVR) → torrential pulmonary blood flow with heart failure; runoff from truncus to pulmonary circulation → wide pulse pressure with bounding pulses and hyperdynamic precordium
Apical mid-diastolic rumble (increased flow across mitral valve)
Diagnosis
Chest x-ray—heart enlargement with increased pulmonary blood flow
ECG—biventricular hypertrophy
Echocardiogram (gold standard)
Treatment
Treat heart failure
− Then surgery in first few weeks of life
MIXED LESIONS
Total Anomalous Pulmonary Venous Return (TAPVR)
Pathophysiology
Complete anomalous drainage of the pulmonary veins into the systemic venous circulation; total mixing of systemic venous and pulmonary venous blood within the heart produces cyanosis
Right atrial blood → right ventricle and pulmonary artery or to left atrium via foramen ovale or ASD
Enlarged right atrium, right ventricle, and pulmonary artery; and small left atrium; and left ventricle normal or small
Clinical manifestations depend on presence or absence of obstruction.
Obstruction (of pulmonary veins, usually infracardiac):
Severe pulmonary venous congestion and pulmonary hypertension with decreasing cardiac output and shock
Cyanosis and severe tachypnea; may not respond to ventilation and PGE1 →
need emergent diagnosis and surgery for survival
Heart failure early with mild-to-moderate obstruction and a large left-to-right shunt; pulmonary hypertension and mild cyanosis
− No obstruction—total mixing with a large left-to-right shunt; mild cyanosis; less likely to be severely symptomatic early
Diagnosis
Chest x-ray—large supracardiac shadow with an enlarged cardiac shadow forms a
“snowman” appearance; pulmonary vascularity is increased
ECG—RVH and tall, spiked P waves (RAE)
Echocardiogram (gold standard)
Treatment
PGE1
Surgical correction
Hypoplastic Left Heart Syndrome
Pathophysiology
Atresia of mitral or aortic valves, left ventricle, and ascending aorta (or any combination)
Right ventricle maintains both pulmonary and systemic circulation.
Pulmonary venous blood passes through foramen ovale or ASD from left atrium
→ right atrium and mixes with systemic blood to produce total mixing
Usually, the ventricular septum is intact and all of the right ventricular blood enters the pulmonary artery.
Ductus arteriosus supplies the descending aorta, ascending aorta and coronary arteries from retrograde flow.
Systemic circulation cannot be maintained, and if there is a moderate-to-large ASD → pulmonary overcirculation
Clinical presentation
− Cyanosis may not be evident with ductus open, but then gray-blue skin color (combination of hypoperfusion and cyanosis as ductus closes)
Signs of heart failure, weak or absent pulses, and shock
Enlarged heart with right parasternal lift; nondescript systolic murmur
Diagnosis
Chest x-ray—heart enlargement with increased pulmonary blood flow
− ECG—right ventricular hypertrophy and right arial enlargement with decreased left-sided forces
− Echocardiogram (gold standard)
Treatment
May do nothing if malformations or genotype not compatible with life
The best treatment today is the three-stage Norwood procedure. (better results currently than cardiac transplantation)
Other—many have a significant abnormality of central nervous system (CNS) and/ or kidneys: need careful genetic, neurologic examination and screening tests on any child being considered for surgery
REGURGITANT LESIONS
Mitral Insufficiency
Usually with other lesions (atrioventricular septal defects, cardiomyopathy); if isolated, dilated annulus, abnormal chordae tendineae, and deformed leaflets
Increased left atrium, left ventricular hypertrophy with dilatation; increased pulmonary venous pressure → pulmonary hypertension
High-pitched, holosystolic murmur at apex
If severe—respiratory infections, fatigue, and pulmonary edema/congestive heart failure
Diagnosis
ECG—bifid P waves, left ventricular hypertrophy
Chest x-ray—increased left atrial size, prominent left ventricle
Echocardiogram (gold standard)
Treatment—mitral valvuloplasty
Mitral Valve Prolapse
Abnormal cusps—billowing of one or both leaflets into left atrium toward end of systole (congenital defect)
Usually not recognizable until adolescence or adulthood; girls > boys
May present with chest pain or palpitations
Arrhythmias, especially unior multifocal premature ventricular contractions
Apical late systolic murmur, preceded by a click—in abrupt standing or Valsalva, click may appear earlier in systole and murmur may be more prominent
Diagnosis
ECG—usually normal
Chest x-ray—normal
Echocardiogram (gold standard)
No therapy, not progressive; adults (more in men) at risk for cardiovascular complications if have thickened leaflets
Tricuspid Insufficiency
Usually associated with Ebstein anomaly
Often with right ventricular dysfunction—dilatation with enlargement of annulus (volume overload or intrinsic myocardial disease)
With perinatal asphyxia in neonate (ischemia of papillary muscles)
OTHER CARDIAC PATHOLOGY
Infective Endocarditis
A 6-year-old boy has had high intermittent fevers for 3 weeks, accompanied by chills. He has a past history of bicuspid aortic valves and recently had dental work.
Etiology/epidemiology
Most are Streptococcus viridans (alpha hemolytic) and Staphylococcus aureus
Organism associations
viridans—after dental procedures
Group D streptococci—large bowel or genitourinary manipulation
Pseudomonas aeruginosa and Serratia marcescens—intravenous drug users
Fungi—after open heart surgery
Coagulase-negative Staphylococcus—indwelling intravenous catheters
Highest risk with prosthetic valve and uncorrected cyanotic heart lesions
Most cases occur after surgical or dental procedures (high risk with poor dental hygiene) are performed.
Clinical presentation
Prolonged intermittent fever, weight loss, fatigue, myalgia, arthralgia, headache, nausea, vomiting
New or changing heart murmur
Splenomegaly, petechiae, embolic stroke, CNS abscess, CNS hemorrhage, mycotic aneurysm (all more with Staphylococcus)
Skin findings—rare; late findings (uncommon in treated patients); represent vasculitis from circulating Ag-Ab complexes; if present, are highly suggestive
Osler nodes—tender, pea-sized, intradermal nodules on pads of fingers and toes
Janeway lesions—painless, small erythematous or hemorrhagic lesions on palms and soles
Splinter hemorrhage—linear lesions beneath nail beds
º Roth spots—retinal exudates
Diagnosis
Duke criteria (2 major or 1 major + 3 minor or 5 minor)
Table 13-3. Duke Criteria
| Major Criteria | Minor Criteria |
| Positive blood culture (two separate for usual pathogens; at least two for less common)
Evidence on echocardiogram (intracardiac or valve lesion, prosthetic regurgitant flow, abscess, partial dehiscence of prosthetic valve, new valvular regurgitant flow) |
Predisposing conditions
Fever Emboli or vascular signs Immune complex disease (glomerulonephritis, arthritis, positive rheumatoid factor, Osler node, Roth spots [retinal hemorrhages with white centers]) Single positive blood culture Echocardiographic signs not meeting criteria |
Complications
− Most common—heart failure from aortic or mitral lesions
− Others—systemic or pulmonary emboli, myocardial abscess, myocarditis, valve obstruction, heart block, meningitis, osteomyelitis, arthritis, renal abscess, immune complex−mediated glomerulonephritis
Treatment
Organism specific for 4−6 weeks (S. viridans, Enterococci, S. aureus, MRSA,
epidermidis, HACEK)
Heart failure—digitalis, diuretic, salt restriction
Surgery—severe aortic or mitral involvement with intractable failure, failure of blood culture to clear, abscess, recurrent emboli, increasing size of vegetations with worsening regurgitation
Prophylaxis (AHA, 2007) for:
Artifical valves
Previous history of infective endocarditis
Unrepaired or incompletely repaired cyanotic disease, including those with palliative shunts and conduits
A completely repaired defect with prosthetic material or device for first 6 months
Any residual defect at site of any repair
Cardiac transplant which develops a problem in a valve
Given ONLY for dental procedures with manipulation of gingival tissue or periapical area or perforation of oral mucosa; incision or biopsy of respiratory tract mucosa and surgery on infected skin or musculoskeletal structures
Drug of choice is amoxicillin
Acute Rheumatic Fever
A 6-year-old girl complains of severe joint pain in her elbows and wrists. She has had fever for the past 4 days. Past history reveals a sore throat 1 month ago. Physical examination is remarkable for swollen, painful joints and a heart murmur. Laboratory tests show an elevated erythrocyte sedimentation rate and high antistreptolysin (ASO) titers.
Etiology/epidemiology
Related to group A Streptococcus infection within several weeks
Antibiotics that eliminate Streptococcus from pharynx prevent initial episode of acute rheumatic fever
Remains most common form of acquired heart disease worldwide (but Kawasaki in United States and Japan)
Initial attacks and recurrences with peak incidence Streptococcus pharyngitis: age 5–15
Immune-mediated—antigens shared between certain strep components and mammalian tissues (heart, brain, joint)
Clinical presentation and diagnosis—Jones criteria. Absolute requirement: evidence of recent Streptococcus infection (microbiological or serology); then two major or one major and two minor criteria
Jones Criteria
| Major Criteria | Minor Criteria |
| Carditis | Fever |
| Polyarthritis (migratory) | Arthralgia |
| Erythema marginatum | Elevated acute phase reactants (ESR, CRP) |
| Chorea | Prolonged PR interval on ECG |
| Subcutaneous nodules | Plus evidence of preceding streptococci infection |
Treatment
Bed rest and monitor closely
Oral penicillin or erythromycin (if allergic) for 10 days will eradicate group A strep; then need long-term prophylaxis
Anti-inflammatory
Hold if arthritis is only typical manifestation (may interfere with characteristic migratory progression)
Aspirin in patients with arthritis/carditis without CHF
If carditis with CHF, prednisone for 2–3 weeks, then taper; start aspirin for 6 weeks
Digoxin, salt restriction, diuretics as needed
If chorea is only isolated finding, do not need aspirin; drug of choice is phenobarbital (then haloperidol or chlorpromazine)
Complications
Most have no residual heart disease.
Valvular disease most important complication (mitral, aortic, tricuspid)
Prevention
Continuous antibiotic prophylaxis
If carditis—continue into adulthood, perhaps for life; without carditis—lower risk; can discontinue after patient is in their twenties and at least 5 years since last episode
Treatment of choice—single intramuscular benzathine penicillin G every 4 weeks
If compliant—penicillin V PO BID or sulfadiazine PO QD; if allergic to both: erythromycin PO BID
Hypertrophic Obstructive Cardiomyopathy (HOCM)
Pathophysiology
− Obstructive left-sided congenital heart disease
Decreased compliance, so increased resistance and decreased left ventricular filling,
mitral insufficiency
Clinical presentation—weakness, fatigue, dyspnea on exertion, palpitations, angina, dizziness, syncope; risk of sudden death
Cardiovascular examination—left ventricular lift, no systolic ejection click (differentiates from aortic stenosis), SEM at left sternal edge and apex (increased after exercise, during Valsalva, and standing)
Diagnosis
ECG—left ventricular hypertrophy ± ST depression and T-wave inversion; may have intracardiac conduction defect
Chest x-ray—mild cardiomegaly (prominent LV)
Echocardiogram—left ventricular hypertrophy, mostly septal; Doppler—left ventricular outflow gradient usually mid-to-late systole (maximal muscular outflow obstruction)
Treatment
No competitive sports or strenuous exercise (sudden death)
Digoxin and aggressive diuresis are contraindicated (and infusions of other inotropes)
Beta blockers (propranolol) and calcium channel blockers (verapamil)
Pericardial Disease
Pericarditis
Definition—inflammation leads to accumulation of fluid in pericardial space (serous, fibrinous, purulent, hemorrhagic)
Causes
Viral—most common (coxsackie B, adenovirus, influenza, echovirus)
Purulent—bacterial infection (e.g., pneumonia); treatment—open pericardial drainage and removal of adhesions, plus antibiotics
Acute rheumatic fever—part of pancarditis
Juvenile rheumatoid arthritis—common; treatment—steroids or ASA
– Systemic lupus—should always be considered in any patient with pericarditis
Uremia—prolonged, severe renal failure → chemical irritation
Neoplasm—Hodgkin, leukemia, lymphosarcoma
Constrictive—most often without preceding illness; treatment: pericardiectomy
Clinical presentation
Most common initial finding is precordial pain
Sharp, stabbing, over precordium and left shoulder and back; worse supine; relief with sitting and leaning forward (referred pain from pleural and diaphragmatic irritation)
Cough, dyspnea, abdominal pain, vomiting
Friction rub is variable (if small amount of fluid); if large amount—muffled heart sounds, narrow pulse pressure, tachycardia, neck vein distension, and pulsus paradoxus
Diagnosis
ECG—low-voltage QRS, mildly elevated ST, generalized T-wave inversion; electrical alternans (various QRS amplitude)
Chest x-ray—need large amount of fluid to cause cardiomegaly with typical water bottle appearance; if constrictive: small heart
Echocardiogram—most sensitive; clear, echo-free space between the epicardium and pericardium; with tamponade: flattening of septal motion and collapse of right ventricular outflow tract in systole
HYPERTENSION
A 5-year-old girl is noted to have blood pressure above the 95th percentile on routine physical examination. The rest of the examination is unremarkable. Her blood pressure remains elevated on repeat measurement over the next few weeks. Past history is remarkable for a treated urinary tract infection 1 year ago. Complete blood cell count is normal; urinalysis is normal. Blood urea nitrogen is 24 mg/dL and creatinine is 1.8 mg/dL.
Routine blood pressure check beginning at 3 years of age
If increased blood pressure, check all 4 extremities (coarctation)
Normal—blood pressure in legs should be 10–20 mm Hg higher than in arms
Blood pressure increases with age—need standard nomograms
If mild hypertension, repeat twice over next 6 weeks
If consistently >95% for age, need further evaluation
Etiology—essential (primary) or secondary
Secondary—most common in infants and younger children
Newborn—umbilical artery catheters → renal artery thrombosis
Early childhood—renal disease, coarctation, endocrine, medications
Adolescent—essential hypertension
Renal and renovascular hypertension—majority of causes may be due to urinary tract infection (secondary to an obstructive lesion), acute glomerulonephritis, Henoch-Schönlein purpura with nephritis, hemolytic uremic syndrome, acute tubular necrosis, renal trauma, leukemic infiltrates, mass lesions, renal artery stenosis
Essential hypertension—more common in adults and adolescents
Positive family history
Multifactorial—obesity, genetic, and physiologic changes
Diagnosis
− CBC, blood chemistries, UA, ECG, echo, renal ultrasound, angiogram (less common)
Treatment
If obese—weight control, aerobic exercise, no-added-salt diet, monitor blood pressure
Pharmacologic treatment (secondary hypertension and selective primary)—similar use of drugs as in adults