1. Ryan Parnham, MSN, APN, CNP
Pediatric Cardiology
Originally Developed by:
Ryan Parnham, MSN, APN, CNP
OSF Critical Care Service
Revised Spring 2012
by Teresa D. Valerio, DNP, APN, FNP-BC

2. Today’s Topics Cardiac Physical Examination Congenital Heart Disease
Acquired Heart Disease

3. Purpose Enhance knowledge and confidence with pediatric cardiology
Emphasize the importance of recognizing significant pediatric cardiovascular abnormalities as a primary care provider

4. Readings from Textbooks
Burns, C.E. et al. (2009). Pediatric Primary Care.
Chapter 30, pgs
Chiocca, E.M. (2011). Advanced Pediatric Assessment.
Chapter 18, pgs
Hay, W.H. et al. (2011). Current Diagnosis & Treatment: Pediatrics.
Chapter 19, pgs , , ,

5. Other helpful resources
Westrol, M.S. & Raffi, K. (2012). Pediatric ECG Cases: Benign Variants or Life-Threatening Abnormalities? (PDF ed)
Wierwille, L. (2011). Pediatric heart murmurs: Evaluation and management in primary care. The nurse practitioner, 36(3), (PDF ed)
Rheumatic fever clinical guidelines at

6. Elements of the cardiac physical examination

7. History
Pregnancy/Birth History: prenatal care, complicated prenatal course, maternal risk factors (diabetes assoc. with hypertrophic cardiomyopathy), prematurity, method of delivery, prolonged hospital stay, birth weight, APGARs
Family History: CHD, sudden death, SIDS, cardiomyopathy (high incidence of inheritance, >20%), dysrhythmias, syndromes/genetic abnormalities

8. Past Medical History General Health Concerns from parents
Growth curve and weight gain (pay attention to stature of parents)
Chronic respiratory difficulties- asthma, frequent URIs/pneumonia
Other medical issues/surgeries
Down Syndrome, Marfan Syndrome, etc.

9. Important Questions Persistent tachypnea? Cyanosis? Failure to thrive?
Tiring and/or diaphoresis with feeding/minimal activity?
Palpitations?
Chest Pain with exertion?
Syncope/Near-syncope? Syncope with exertion? Palpitations with syncope?
Lethargy/Fatigue?
Inability to keep up with peers?
Worsening activity tolerance?

10. Vital Signs
Respiratory Rate- should be counted for a full minute in infants because of variability
Heart rate- resting if possible
Blood pressure- bilateral upper and lower extremities (at least once in their life, unless more often if clinically indicated)- use correct size cuff!
Pulse ox- check/recheck multiple extremities if abnormal reading

11. Overall Appearance
Anxious, distressed, diaphoretic, pale, tachypneic, retractions, cyanotic, difficult to console, dysmorphic, clubbing
A baby, child and adolescent can have a significant cardiac lesion and appear perfectly healthy with apparently normal vitals signs

12. Cardiovascular Assessment
Is chest symmetrical? Evidence of pectus excavatum, pectus carinatum.
Precordial activity quiet or hyperactive?
PMI displaced? (usually mid-clavicular line)
Palpable thrill over chest? Thrill at suprasternal notch?
JVD?

13. Auscultation
Listen at all landmarks (aortic, pulmonic, 2nd pulmonic, tricuspid, mitral) with bell and diaphragm
Listen to heart sounds
S1: closure of what valves?
S2: closure of what valves?

14. S1 Closure of the atrioventricular valves (mitral and tricuspid)
Normally heard as one sound, although occasionally splitting may be heard

15. S2 Closure of the semilunar valves (pulmonary and aortic)
Under normal circumstances, S2 is “split” during or near the end of inspiration (the right ventricle is filled more than the left with inspiration, causing a short delay in closure of the PV)
A fixed or widely split S2 could be pathologic, as can a single S2 that does not split.

16. Murmurs
What is a heart murmur?

17. Murmurs
Murmurs are audible sound waves caused by turbulent blood flow- a murmur is not a hole, or a defective valve, etc.
Most murmurs are “innocent,” or Still’s murmurs, 32-80% of all children will have a murmur at some point in their life, often with fever
Structural or physiologic cardiac problems can also cause murmurs; 1% of children have structural heart disease

18. Grading of Murmurs 1: Heard only with intense concentration
2: Faint, but heard immediately
3: Easily heard, of intermediate intensity
4: Easily heard and palpable thrill present
5: Very loud, thrill present, and audible with edge of stethoscope on chest wall
6: Audible with stethoscope off chest wall

19. Innocent Vibratory Murmur or Still’s Murmur
Most common during childhood
Low to medium pitch, early systole
Usually grade 2, but can range 1-3
Maximal at LLSB w/ radiation to apex
Usually loudest supine and fades with sitting and standing
Vibratory, harmonic, musical
Often louder during febrile illness or times of increased cardiac output
Usually disappear with time and can come and go

20. Innocent Pulmonary Flow Murmur
A relatively soft systolic murmur appreciated at the upper left sternal border
Often louder while lying supine and fades/disappears with sitting

21. Venous Hum
Continuous murmur usually heard at the infraclavicular area of the anterior chest, R>L
Loudest while sitting (venous return from the jugular veins and subclavian veins entering the SVC ) and disappears supine
Can be diminished or muted with gentle compression of jugular vein
Commonly associated with vibratory murmur

22. Pathological Systolic Murmurs
Upper Right Sternal Border: Aortic Stenosis, subaortic membrance, PPAS
Upper Left Sternal Border: Pulmonary Stenosis, PDA, PPAS
Mid to Lower Left Sternal Border: VSD, TR, LVOT narrowing (HCM)/Subaortic stenosis
Apex: MR, poss VSD

23. Pathological Systolic Murmurs
Usually don’t change with position, except HCM causing LVOT obstruction becomes louder with standing.
MR may be somewhat more pronounced while lying on the left side.
Often times “harsh”, usually quite different from an innocent murmur, but not always

24. Diastolic Murmurs
Always pathological- there are no “normal” or “innocent” diastolic murmurs
What are some diastolic murmurs?

25. “Early” Diastolic Murmurs
Decrescendo in nature
Commonly aortic regurgitation or pulmonary regurgitation (insufficiency)

26. Mid to Late Diastolic Murmurs
Tricuspid stenosis or mitral stenosis

27. Continuous Murmurs
Heard through systole and diastole- most common “pathological” continuous murmur is a Patent Ductus Arteriosus

28. Cardiovascular Exam (cont’d)
Resp- Lungs clear? Equal? Congested? Diminished? Tachypneic? Retractions?
Skin- Warm? Pink? Well-perfused?
Abdomen- soft? Liver enlarged? Ascites? Situs solititus or inversus?
Pulses- Weak? Bounding? Normal? Brachial/radial-femoral delay?
Extremities: Capillary Refill Brisk? Clubbing? Edema?

29. Exam (con’t)
If you want to truly perform a quality cardiovascular examination, auscultate the patient lying, sitting, standing and squatting, especially with athletes, patients with syncope with exertion, or family history of sudden death and/or hypertrophic cardiomyopathy!

30. When Do You Refer for a Murmur?
Is this a new murmur?
What are the characteristics of the murmur? Systolic vs. diastolic.
Is the child febrile?
Is the child “symptomatic”?
What testing has been done thus far- EKG, CXR, ECHO
Family history

31. When Do You Refer for a Murmur?
If no definite “red flags,” consider having the child RTC to re-listen in a week or two (especially if febrile!!!)
If some concern, order an EKG and CXR- relatively inexpensive and can provide useful info
If EKG and CXR normal, but still uncertain, ok to refer or consider ordering an echo (caution- expensive and may “over-diagnose”)
If child in obvious distress- refer immediately/admit for evaluation
If the patient is in competitive athletics and it is a new murmur, consider referral.
At least order an EKG and CXR prior to referral- appreciated by the cardiology group!

32. Congenital Heart Disease

33. Congenital Heart Disease
8-10/ 1,000 liveborn infants will have a congenital cardiac malformation (0.8-1%)
Risk of recurrence in families with one parent or one sibling with CHD is 1-4%
Some defects are associated with even higher recurrence rates in families, and geneticists are beginning to identify certain genes that may help explain this

34. What causes CHD? Genetic factors? Environmental factors?
Genetic and environmental factors?
Possible environmental factors: maternal infection/illness, medication use, substance abuse, chronic diseases such as diabetes, lupus, etc.

35. Review of Fetal Circulation

36. Congenital Cardiac Lesions
Acyanotic and Cyanotic

37. Acyanotic Lesions Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Atrioventricular Septal Defects/AV Canal
Patent Ductus Arteriosus (PDA)
Aortic Stenosis
Pulmonary Stenosis
Coarctation of the Aorta

38. Cyanotic Lesions d-transposition of the great arteries (d-TGA)
Tetralogy of Fallot
Hypoplastic right heart syndrome
Hypoplastic left heart syndrome
Double outlet right ventricle (DORV)
Truncus arteriosus
Total anomalous pulmonary venous return (TAPVR)

39. Commonly Seen Acyanotic Lesions

40. Atrial Septal Defects (ASD)
Common defect
May be a PFO/small secundum ASD % of the population has this
May be larger, causing significant L to R shunting
Can go undetected for years
Exam: wide, fixed splitting of S2, often a systolic murmur r/t increased pulm flow
“Easily” fixed (surgery vs. transcatheter)

42. Ventricular Septal Defect
Ventricular septum fails to “fill in” completely during embryonic development
Various degrees of VSDs from tiny to large
May be asymptomatic, mildly symptomatic, or in congestive heart failure
May not present clinically until 1-2 months of life
Often associated with other lesions
Isolated VSD’s typically have favorable surgical outcomes
Many small and even mod sized VSD’s can close spontaneously for up to 4 years of age

44. Mitral Valve Prolapse
The most common valvular abnormality (2-6% of the population)- more common in females than males (2:1)
Usually benign, but may also have mitral regurgitation
Can worsen with time, or improve
Exam: mid-late systolic click (often), blowing, holosystolic murmur if significant degree of MR
Diagnosis- echocardiogram (often wrongly and over-diagnosed by exam only)
May be associated with connective tissue disorders such as Marfan Syndrome

46. Coarctation of the Aorta
Narrowed area of the aorta (usually descending or transverse arch)
May cause hypertension
Often systolic murmur LUSB with radiation to left back
Discrepancy between upper and lower extremity blood pressure and pulses
Usually an “easy” repair

48. Common Cyanotic Lesions

49. Tetralogy of Fallot
4 defining characteristics- Pulmonary stenosis, RVH, VSD, and aortic override
May be associated with a genetic syndrome
Surgical repair in first year of life (often need a shunt placed soon after birth)
If PV is replaced, usually have some degree of dysfunction that needs to be monitored over time

51. D-transposition of the Great Arteries
Aorta rises from the RV and Pulmonary Artery rises from the LV- complete separation of pulmonary and systemic circulations
Arterial switch procedure

53. There are many advances underway in pediatric cardiac surgery and cardiac catheterization techniques that are not only improving surgical outcomes, but resulting in shorter hospital stays and a better quality of life.

54. Acquired Heart Disease

55. Kawasaki Disease (KD) An acute, self-limited vasculitis
Unknown cause, but an infectious cause/virus is suspected
Leading cause of acquired heart disease in US and Japan
Usually seen in children <5 y.o.
First described by Dr. Kawasaki in Japan, 1961

56. KD- Diagnostic Criteria
Fever >/= 5 days
Bilateral conjuctival injection
Changes of mucous membranes- injected pharynx, fissured lips, strawberry tongue
Changes of peripheral extremities- peripheral edema, peripheral erythema, desquamation of palms
Polymorphous rash
Cervical adenopathy
Diagnosis is presence of fever and 4 of 5 remaining criteria

57. KD- Cardiovascular concerns
Development of coronary artery aneurysms (usually around 2 wks after onset of symptoms)
Evaluated by echocardiogram
Half of patients with aneurysms will remodel vessel wall- never completely normal. Probably at higher risk for future coronary artery disease- long-term antiplatelet therapy generally recommended

58. Strawberry Tongue

61. Treatment
Standard treatment is high-dose IVIG (immunoglobulins) in combination with high doses of aspirin
Screening/repeat echocardiograms
Possible cardiac cath with intervention if coronary artery aneurysms causing myocardial dysfunction or for further evaluation

62. Long-term prognosis without coronary aneurysm
No significant risk of increased mortality
Possible increased risk of premature atherosclerosis r/t vasculitis?
Little long-term data, although there have been a couple of studies suggesting abnormal vasodilatory properties of coronary arteries in KD patients with seemingly “unaffected” coronaries

63. Rheumatic Fever
A systemic illness thought to occur following group A beta hemolytic streptococcal pharyngitis (GABHS) in children, median age 10 years (can happen in adults though)
Although uncommon in present day, accurate clinical diagnosis is vital for optimal long-term prognosis.
Was the leading cause of death in patients aged 5-20 in the US about 100 yrs ago.
Increased risk- untreated or delayed treatment of a strep infection

64. History Sore throat 1-5 wks prior to onset of RF symptoms
Fever, rash, headache, wt loss, fatigue, diaphoresis, chest pain/pounding, migratory joint pain, skin nodules, motor dysfunction, previous RF (higher risk of recurrence)
Can cause endocarditis, myocarditis, and pericarditis. Usually affecting the mitral and aortic valves resulting in regurgitation

65. Jones Criteria
Requires presence of 2 major, or 1 major and 2 minor criteria. Previous Group A strep is also necessary

66. Major Criteria
Carditis (EKG, CXR, ECHO, Exam)- new murmur and tachycardia. Wide pulse pressure if severe aortic regurgitation. May also have CHF- JVD, hepatomegaly, gallop rhythm, friction rub, peripheral edema
Polyarthritis
Chorea- brief, irregular, unpredictable, purposeless movements that flow from one body part to another without a rhythmic pattern
Erythema marginatum- 1 to 3 cm pink/red nonpruitic macules or papules on trunk and limbs (not on face)
Subcutaneous nodules- infrequent, but occur on surfaces of elbows, knees, ankles, knuckles, and other spinous processes- firm and nontender

67. Subcutaneous Nodules

68. Erythema Marginatum

69. Minor Criteria
Fever- usually greater than 39 deg C, although may be low-grade, may last 2-3 weeks
Arthralgia- cannot be considered minor if arthritis is present
Prolonged PR interval in EKG
Elevated erythrocyte sedimentation rate (ESR) and C-Reactive Protein (CRP)

70. Evidence of GAS pharyngitis – one of the following MUST be present
Positive throat culture or rapid strep antigen test
Elevated or rising streptococcal antibody titer (such as antistreptolysin O [ASO])

71. Lab Studies Throat culture Rapid antigen detection test
Antistreptococcal antibodies- usually using antistreptolysin O (ASO)
C-RP, ESR

72. Imaging CXR- pulm cong/CHF Echo- MR, AR, LV dilation
ECG- sinus tachy, 1st, 2nd or 3rd degree AV block (myocarditis), ST segment elevation, atrial arrhythmias

73. Treatment
Antibiotics- Oral penicillin remains drug of choice- alternatives: IM Pen G, E-mycin, 1st gen Cephalosporin (do not use tetracyclines or sulfonamides)
High dose aspirin and steroid to treat inflammation (cont to monitor C-RP and ESR for effectiveness)
Digoxin, diuretics, afterload reduction depending on cardiac manifestations
After acute phase symptoms have subsided, improved, may undergo cardiac surgery for valve repair/replacement- if AV and/or MV replaced with prosthetic, will require lifetime anticoagulation (Warfarin)

74. What does this mean as FNP’s
Recognizing and treating streptococcal infections in a timely manner
Awareness of potential systemic symptoms related to untreated/delayed treatment of a strep infection- these may not occur until up to several weeks after the sore throat has resolved
Only infections of the pharynx initiate RF (in developed countries)
Secondary prophylaxis of patients with history of RF- daily PCN or injections q 3-4 wks
SBE prophylaxis with antibiotics prior to dental and other procedures

75. Revised AHA guidelines for Bacterial Endocarditis Prevention
Previous guidelines recommended antibiotics prior to dental/surgical procedures for anyone with any type of intracardiac abnormality (MVP, bicuspid AV, etc)
Recent findings- bactremia is transient and can occur with routine activities (brushing teeth, flossing, chewing)
Only “high-risk” patients are recommended to have antibiotic SBE prophylaxis

76. “High risk” population includes:
Prosthetic heart valves
Previous endocarditis
Unrepaired cyanotic defect
Palliative shunt/conduit present
Complete repair with prosthetic material for first 6 months after procedure
Heart transplantation
Recommended only for procedures which manipulates the tissue or oral mucosa causing bleeding, etc. (not a routing cleaning)
Those not in “high risk” category are not at zero risk- still slightly elevated compared to those without intracardiac abnormalities

77. Other common complaints
Chest pain- rarely attributed to any underlying cardiovascular disease. Coronary artery abnormalities in children rarely present with chest pain
Syncope- very common in adolescents and teenagers. Usually vasovagally mediated and r/t hydration, eating habits, etc. “Red flags”- syncope during strenuous activity, accompanied with palpitations, family history of sudden death or arrhythmias