Cardiology

Dr. Darawshe mohamad

 CARDIAC EVALUATION AND CONGENITAL HEART
LESIONS
• Features of congestive heart failure:
• 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.
 PEDIATRIC HEART SOUNDS
 First heart sound (S1)
– Closure of mitral and tricuspid valves (MV, TV)
– a split S1 represents asynchronous closure of the 2 valves (20−30 msec difference)
• Pathologic Split S1 (heared as a click):
– Apical mid systolic click >mitral valve prolapse
– At upper left sternal border > pulmonic valve stenosis
– Right upper sternal border > aortic stenosis

 Second heart sound (S2)

– Closure of pulmonary and aortic valves (PV, AV)
– close simultaneously, upper left sternal border
– Wider splitting of S2 on inspiration is related to increased venous return.
– Loud single S2: heard with PA hypertension

 Third heart sound (S3)

– Hear early in diastole; creates a gallop rhythm with S1 + S2
– heard normally in children with no pathology

 Fourth heart sound (S4)

– late diastole, just prior to S1
– produced by a decrease in compliance (increased stiffness) of the LV
– may be found with improving CHF, myocarditis, or a cardiomyopathy
Innocent Murmurs
 Congenital Heart Disease
• In most cases, diagnosis usually made by age 1 month.
• Etiology:
− Most are unknown
− Associated with teratogens, such as alcohol and rubella
− Genetic predisposition: trisomies; Marfan, Noonan, DiGeorge syndromes
 Ventricular Septal Defect (VSD)
• Most common congenital heart lesion 25-30 %
• 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
• Diagnosis
– Chest X-ray (large heart, pulmonary edema), ECG
(LVH), echocardiogram is definitive.
 VSD
• 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
• Physical examination:
– Wide fixed splitting of S2
– Systolic ejection murmur along left mid to upper sternal border (from increased
pulmonary flow)
– Large lesions with diastolic murmur.
• 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
– Transcatheter device or surgical closure is advised for all symptomatic
patients, as well as for asymptomatic patients with Qp:Qs ratio of at least
2:1 and those with RV enlargement. The timing for elective closure is
usually after the 1st yr of life and before entry into school.
– Complications
– Dysrhythmia
– Low-flow lesion; does not require endocarditis prophylaxis
 Endocardial Cushion Defect
• Pathophysiology:
– When both ASDs and VSDs occur and the atrioventricular valves are abnormal
– Left-to-right shunt; 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 occurs earlier
– Moderate-to-severe increase in heart size
– Widely fixed split S2 (like an isolated ASD)
– Pulmonary systolic ejection murmur, low-pitched diastolic rumble at left sternal border and apex; may also
have mitral insufficiency (apical harsh holosystolic murmur radiating to left axilla).
• Diagnostic tests:
– Chest x-ray: significant cardiomegaly, edema
– ECG: signs of biventricular hypertrophy, right atrial enlargement.
– Echocardiogram (gold standard).
• Treatment:
– Initial management includes diuretics (± digoxin) and afterload reduction for treatment of heart failure.
– Surgery more difficult with heart failure and pulmonary hypertension must be performed in infancy
• Complications
– Without surgery; death from heart failure. With surgery;arrhythmias, congenital heart block
 Endocardial Cushion Defect

Note: Patients with trisomy 21

are at a higher risk for
endocardial cushion defects.
Patent Ductus Arteriosus (PDA)
 Coarctation of the Aorta
• approximately 10% of all congenital heart defects.
• Tubular hypoplasia (preductal, infantile type)
• Right ventricular blood flows across the PDA to supply the descending aorta.
• Seen as differential cyanosis: Upper body is pink, lower is cyanotic; prominent heart
failure as ductus closes.
• 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
• 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 (opens ductus arteriosus), surgery after stabilization
– Surgery soon after diagnosis of any significant coarctation
• Complications: Associated cerebrovascular disease, Systemic hypertension,
Endocarditis, Aortic aneurysms
 Case 2
• 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.
 Tetralogy of Fallot (TOF)
• Tetralogy of Fallot is one of the conotruncal family of heart lesions
• 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)
 Tetralogy of Fallot (TOF)
• Physical examination:
– substernal right ventricular impulse, systolic thrill along 3-4 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.
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
• Complication-
• Cerebral thrombosis/ brain abscess- under 2 y.
• Endocarditis.
 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; left ventricular blood to right ventricle 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.

• 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 to maintain pulmonary outflow until surgery.
– Surgery is staged with an initial subclavian artery-to-pulmonary
shunt (Blalock-Taussig procedure)
– followed by a two-stage procedure: bidirectional cavopulmonary
shunt (bidirectional Glenn) and Fontan procedure.
 Transposition of the great arteries
(TGA)
• Cyanotic Lesion Associated with Increased Pulmonary Blood Flow.

• Most common cyanotic lesion presenting in the immediate newborn

period

• Pathophysiology:
– Aorta arises from the right ventricle, and the pulmonary artery
from the left ventricle;
– 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
 Transposition of the great arteries
(TGA)
• 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 (Rashkind procedure)
– 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
– Initially, SEM with loud thrill, single S2, and minimal cyanosis
• Diagnosis
• - pulmonary saturation = aortic saturation
– 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
Infective Endocarditis
 Diagnosis
• Duke criteria (Two major criteria, 1 major and 3 minor, or 5 minor criteria suggest
definite endocarditis).
• The critical information for appropriate treatment of infective endocarditis is
obtained from blood culture.
• Empirical therapy after appropriate blood cultures are drawn but before the identifiable
agent is recovered may be initiated with vancomycin plus gentamicin in patients without a
prosthetic valve and when there is a high risk of S. aureus, enterococcus, or viridans streptococci
(the 3 most common
• organisms)
Patients with permanently damaged valves from rheumatic heart disease should also be
considered for prophylaxis. prophylaxis for gastrointestinal or genitourinary procedures is
no longer recommended in the majority of cases.
 Case 4
• 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.
 Acute Rheumatic Fever
• Etiology/epidemiology
– Related to group A Streptococcus infection within several weeks
– Antibiotics that eliminate Streptococcus from pharynx prevent initial episode of acute rheumatic
fever
– 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.
• 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
## A major change in the 2015 revision of the Jones Criteria is the acceptance of subclinical
carditis (defined as without a murmur of valvulitis but with echocardiographic evidence of
valvulitis) or clinical carditis (with a valvulitis murmur) as fulfilling the major criterion of
carditis in all populations.
## Acute rheumatic carditis usually presents as tachycardia and cardiac murmurs, with or
without evidence of myocardial or pericardial involvement. Moderate to severe rheumatic
carditis can result in cardiomegaly and heart failure with hepatomegaly and peripheral and
pulmonary edema.
 Acute Rheumatic Fever
• 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 (NSAID or Aspirin)
• 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 in CHF.
– 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
• See table
– 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
• Treatment of LQTS includes the use of β-blocking
agents at doses that blunt the heart rate
response to exercise. Propranolol and nadolol
may be more effective than atenolol and
metoprolol. Some patients require a pacemaker
because of drug-induced bradycardia. An
implantable cardiac-defibrillator (ICD) is indicated
in patients with continued syncope despite
treatment with β-blockers, and those who have
experienced cardiac arrest.
 Myocarditis
• Acute or chronic inflammation of the myocardium is characterized by inflammatory cell
infiltrates, myocyte necrosis, or myocyte degeneration and may be caused by infectious,
connective tissue, granulomatous, toxic, or idiopathic processes. There may be associated
systemic manifestations of the disease, and occasionally the endocardium or pericardium is
involved, but coronary pathology is uniformly absent. Patients may be asymptomatic, have
nonspecific prodromal symptoms, or present with overt congestive heart failure,
compromising arrhythmias, or sudden death. It is thought that viral infections are the most
common etiology( enterovirus/coxackie) , although myocardial toxins, drug exposures,
hypersensitivity reactions, and immune disorder.
• Infants and young children more often have a fulminant presentation with fever, respiratory
distress, tachycardia, hypotension, gallop rhythm, and cardiac murmur. Associated findings
may include a rash or evidence of end organ involvement such as hepatitis or aseptic
meningitis.
• Electrocardiographic changes are nonspecific, often suggestive of acute ischemia.
• Chest radiographs in severe, symptomatic cases reveal cardiomegaly, pulmonary vascular
prominence, overt pulmonary edema, or pleural effusions.
• Echocardiography often shows diminished ventricular systolic function.
• Cardiac MRI is a standard imaging modality for the diagnosis of myocarditis.
• Endomyocardial biopsy may be useful in identifying inflammatory cell infiltrates or myocyte
damage and performing molecular viral analysis using polymerase chain reaction techniques.
• -
Tx Primary therapy for acute myocarditis is supportive, including β-blockers and ACE
inhibitors . Acutely, the use of inotropic agents, preferably milrinone, should be considered but
used with caution because of their proarrhythmic potential. Diuretics are often required as
well. In sever cases meccanical ventilation or ECHMO.
• Intravenous immune globulin (IVIG) may have a role in the treatment of acute or fulminant
myocarditis, and corticosteroids have been reported to improve cardiac function, but the data
are not convincing in children.

• PROGNOSIS -of symptomatic acute myocarditis in newborns is poor, and a 75% mortality has
been reported. The prognosis is better for children and adolescents, although patients who
have persistent evidence of DCM often progress to need for cardiac transplantation. Recovery
of ventricular function, however, has been reported in 10–50% of patients.