Slide 2 Slide 3 The Pediatric Heart Slide 4 Congenital Heart
Disease CHD is a type of defect or malformation in one or more
structures of the heart or blood vessels that occur before birth
CHD affects 8-10 out of every 1,000 children Approximately 500,000
adults in the U.S. have congenital heart disease Slide 5 Congenital
Heart Disease Symptoms in Adults Shortness of breath Limited
ability to exercise Symptoms in Children Can be detected prenatally
Usually detected by the presence of a heart murmur, but not all CHD
has a murmur, and not all murmurs are pathological!!! Slide 6 The
Evaluation of the Heart History Physical Examination
Electrocardiogram Chest radiograph Echocardiogram and further
evaluation by a cardiologist (cardiopulmonary stress test, 24- hour
Holter monitor, ambulatory EKG, cardiac catherization, etc Slide 7
The History Ask about timing (at rest or activity-related), onset,
and termination (gradual versus sudden), precipitating and
relieving factors Infants: Cyanosis, squatting, loss of
consciousness, tachypnea, diaphoresis, poor weight gain Older
children: Dizziness, syncope, exercise intolerance, dyspnea on
exertion, diaphoresis Slide 8 Acrocyanosis vs. Cyanosis Slide 9 The
Physical Exam Visual assessment of activity (agitation or lethargy)
Skin perfusion and skin color Vitals: heart rate, respiratory rate,
blood pressure (in all four extremities), and oxygen saturation
Dysmorphic features Cardiovascular exam Slide 10 Cardiac Defects in
Syndromes Down syndrome: AVSD Turner syndrome:Bicupsid aortic
valve, coarctation Marfan syndrome:MVP, MR, dilated aortic root
Fetal alcohol syndrome:VSD, ASD Maternal rubella:PDA, PPS Slide 11
Down Syndrome Slide 12 Marfan Syndrome Slide 13 Cardiovascular Exam
Inspection & Palpation Chest conformation (left precordial
bulge from longstanding cardiomegaly) Increased precordial activity
A diffuse point of maximal impulse A precordial or suprasternal
notch thrill A palpable pulmonary (P2) at upper LSB Slide 14
Cardiovascular Exam: Points of Auscultation Slide 15 Cardiovascular
Exam Auscultation The first heart sound (S1) is the sound of
atrioventricular (AV) valve closure is best heard the lower left
sternal border The second heart sound (S2) is the sound of
semilunar valve closure is best heard at the upper left sternal
border. S2 has a variable split (A2 and P2), that varies with
respiration, non-variable can be cardiac disease Slide 16
Cardiovascular Exam Auscultation The third heart sound (S3) is the
sound of rapid filling of the left ventricle, it occurs in early
diastole, after S2, and is medium- to low-pitched, usually only
heard in the supine position. The fourth heart sound (S4) is
associated with atrial contraction and increased atrial pressure,
its low-pitched, prior to S1 and usually not audible Slide 17
Cardiovascular Exam Auscultation Ejection clicks are high-pitched,
related to dilated great vessels or valve abnormalities Occur
anytime during systole: early, mid, or late Early clicks at the mid
LSB are from the pulmonic valve Aortic clicks are best heard at the
apex Slide 18 Cardiovascular Exam Auscultation Murmurs should
described by the following characteristics Location and radiation
Relationship to cardiac cycle and duration Intensity Quality
Variation with position Slide 19 Cardiovascular Exam Location of
pathology RUSB Aortic valve stenosis LUSB ASD Pulmonary valve
stenosis LLSB VSD inc AVSD Apex MR/MS/MVPS Aortic stenosis Slide 20
Cardiovascular Exam Relationship to the cardiac cycle and duration
Systolic Crescendo/decrescendo Pansystolic Throughout systole or
the same intensity Diastolic Continuous Systolic Ejection ASD
Coarctation Pansystolic VSD MR/TR Diastolic MS/TS Continuous
PDS/AVM Slide 21 Cardiovascular Exam Intensity of murmur Grade 1:
soft, heard with difficulty Grade 2: soft, but easily heard Grade
3: loud without a thrill Grade 4: loud with a precordial thrill
Grade 5: loud with a thrill heard with edge of stethoscope Grade 6:
loud with thrill and stethoscope off chest Slide 22 Cardiovascular
Exam Quality of murmur: harsh, musical, or rough; high, medium, or
low in pitch Variation with position: audible when the patient is
supine, sitting, standing, or squatting Slide 23 Extracardiac Exam
Arterial Pulse: Rate and Rhythm Quality and Amplitude of Pulse
Arterial Blood Pressure Extremities Abdomen Slide 24 Extracardiac
Exam Rate and Rhythm Rhythm should be regular, or with a phasic
variation with respiration (sinus arrhythmia) is normal Resting
Heart Rates Kawasaki Disease Diagnostic criteria Fever for more
than 5 days and at least four of: Bilateral painless, nonexudative
conjunctivitis Lip or oral cavity changes (eg, lip cracking and
fissuring, strawberry tongue, and/or inflammation of the oral
mucosa Cervical lymphadenopathy (> 1.5 cm in diameter and
usually unilateral Polymorphous exanthem Extremity changes (redness
and swelling of hands and feet with subsequent desquamation) Slide
43 Kawasaki Disease Slide 44 Slide 45 Other non-diagnostic findings
GI Vomiting diarrhea, gallbladder hydrops, elevated transaminases
Blood Elevated ESR or CRP, leukocytosis, hypoalbuminemia, mild
anemia in acute phase and thrombocytosis in subacute phase (usually
second or third week of illness) Renal Sterile pyuria, proteinuria
Slide 46 Kawasaki Disease Other non-diagnostic findings, cont.
Respiratory Cough, rhinorrhea, infiltrate on chest radiograph Joint
Arthralgia and arthritis Neurologic Mononuclear pleocytosis of
cerebrospinal fluid, irritability, facial palsy Slide 47 Kawasaki
Disease Cardiovascular complications Myocarditis Pericarditis
Valvular heart disease (usually mitral or aortic regurgitation)
Coronary arteritis (range from mild transient dilation to large
aneurysm of coronary arteries) Slide 48 Kawasaki Disease Atypical
Kawasaki Patients with fever for at least 5 days, But fewer than
four of the diagnostic features, And coronary abnormalities by
echocardiography Slide 49 Kawasaki Disease Coronary artery lesions
Range from mild transient dilation to large aneurysms of coronary
arteries Aneurysms rarely occur before day 10 of illness Untreated
patients have a 15-25% risk of developing coronary aneurysms Those
at greatest risk are males, less than 6 months of age, and not
treated with IVIG Slide 50 Kawasaki Disease Coronary artery lesions
Detected by two-dimensional echocardiography Most resolve in 5
years As aneurysms resolve, stenosis or obstruction can occur, up
to 19 % Giant aneurysms (>8mm) are less likely to resolve, and
nearly 50% become stenotic Acute thrombosis can occur, resulting in
myocardial infarction, fatal in 20% of cases Slide 51 Kawasaki
Disease Immediate management Referral to Pediatric Emergency
Department IVIG and aspirin therapy Echocardiograms immediately, at
2 weeks, and 6-8 weeks Slide 52 Kawasaki Disease Long term
management Depends on risk stratification of cardiac disease
present No cardiac disease, no f/u needed after 1 year Transient
ectasia, no f/u needed after 3-5 years Single small-medium
aneurysm, aspirin (asa) therapy until abnormality resolves and f/u
for 10 years Giant aneurysm or multiple small-medium aneurysm,
long-term asa +/- warfarin and f/u for 20 years Coronary artery
obstruction, long-term asa +/- warfarin +/- calcium channel
blockers and f/u every 6 months Slide 53 Rheumatic Heart Disease
Group A B-hemolytic streptococcal infection of the upper
respiratory tract is the trigger in predisposed individuals 30-50
new cases are seen each year Host susceptibility via immune
response genes occurs in 15% of the population Peak age of risk in
the US is 5-15 years More common in girls and African Americans
Slide 54 Rheumatic Heart Disease The immune response Sensitization
of B lymphocytes by streptococcal antigens Formation of
antistreptococcal antibody Formation of immune complexes that
cross-react with cardiac sarcolemma antigens Myocardial and
valvular inflammatory response Slide 55 Rheumatic Heart Disease:
Modified Jones Criteria for Diagnosis Major manifestations (2 major
or-) Carditis Polyarthritis Sydenham chorea Erythema marginatum
Subcutaneous nodules Plus Supporting evidence of preceding
streptococcal infection must be present Minor Manifestatiions (1
major and 2 minor) Clinical Previous rheumatic fever or rheumatic
heart disease Polyarthralgia Fever Laboratory Acuter phase
reaction: elevated erythrocyte sedimentation rate, C-reative
protein, leukocytosis Prolonged PR interval Slide 56 Rheumatic
Heart Disease Syndenham chorea Emotional instablity and involuntary
movements, including ataxia, slurring speed, self-limiting
Polyarthritis Large joint swelling (knees, hips, wrists, elbow, and
shoulders) Erythema Marginatum Subcutaneous Nodules Slide 57
Rheumatic Heart Disease Erythema MarginatumSubcutaneous nodules
Slide 58 Rheumatic Heart Disease Treatment of acute episode
Anti-infective therapy Benzathine penicillin or Penicillin V
Erythromycin Anti-inflammatory therapy Asa and corticosteroids in
severe cases Therapy of CHF Bedrest If CHF is present Slide 59
Rheumatic Heart Disease Treatment after acute phase Prevention of
reccurence 3-5 years of therapy Penicillin G IM every 3-4 weeks or
daily antibiotic therapy Rheumatic Heart Disease Most common in the
US is mitral insufficiency Second common in the US is aortic
insufficiency Others Mitral valve stenosis seen 5-10 years after
episode Aortic stenosis seen in older adults Slide 60
Cardiomyopathy Dilated cardiomyopathy- most frequent caused by
myocarditis, long-standing untreated tachyarrhythmias, left heart
obstructive lesions, congenital abnormalities of the coronary
arteries, anthracycline toxicity, genetic and metabolic disease
Hypertrophic cardiomyopathy Most common cause is familial Leading
cause of sudden cardiac death!! Diffuse point of maximal impulse!!
Restrictive cardiomyopathy- Rare Slide 61 References The Harriet
Lane Handbook, A Manual for Pediatric House Officers, 18 th Edition
by The Harriet Lane Service Childrens Medical and Surgical Center
of The John Hopkins Hospital Current, Diagnosis and Treatment in
Pediatrics, 18 th Edition by Hay Jr, et al. Slide 62 References
Pictures The Pediatric Heart:
http://www.artandmedicine.com/rowley/mutter/Guide.html Cyanosis
Acrocyanois:
http://newborns.stanford.edu/PhotoGallery/PerioralCyanosis1.html
Cyanosis: http://en.wikipedia.org/wiki/Cyanosis Dysmorphic features
Downs syndrome:
http://blogs.reuters.com/uknews/2008/11/24/downs-syndrome-numbers-
dont-add-up/http://blogs.reuters.com/uknews/2008/11/24/downs-syndrome-numbers-
dont-add-up/ AVSD:
http://www.rch.org.au/cardiology/defects.cfm?doc_id=509http://www.rch.org.au/cardiology/defects.cfm?doc_id=509
Points of Auscultation:
http://sharinginhealth.ca/clinical_evaluation/heart_sounds.htmlhttp://sharinginhealth.ca/clinical_evaluation/heart_sounds.html
Slide 63 References Pictures Kawasaki Disease little boy:
http://ep.bmj.com/content/89/1/ep3.full strawberry tongue:
http://reviews.in.88db.com/index.php/healthbeauty/hospitals/8066-kawasaki-
syndrome-disease-diagnosis-treatment
http://reviews.in.88db.com/index.php/healthbeauty/hospitals/8066-kawasaki-
syndrome-disease-diagnosis-treatment syndrome picture:
http://syndromepictures.com/kawasaki-syndrome- pictures/ rash:
http://www.skinatlas.com/Kawasaki%20syndrome/Kawasaki1.htm Slide 64
References Erythema marginatum
http://health-pictures.com/erythema-marginatum-picture.htm
Subcutaneous nodules
http://www.google.com/imgres?q=subcutaneous+nodules+images
http://www.google.com/imgres?q=subcutaneous+nodules+images Marfan:
http://www.nlm.nih.gov/medlineplus/marfansyndrome.htmlhttp://www.nlm.nih.gov/medlineplus/marfansyndrome.html
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