3Purpose Enhance knowledge and confidence with pediatric cardiology Emphasize the importance of recognizing significant pediatric cardiovascular abnormalities as a primary care provider
4Readings from Textbooks Burns, C.E. et al. (2009). Pediatric Primary Care.Chapter 30, pgsChiocca, E.M. (2011). Advanced Pediatric Assessment.Chapter 18, pgsHay, W.H. et al. (2011). Current Diagnosis & Treatment: Pediatrics.Chapter 19, pgs , , ,
5Other 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
7HistoryPregnancy/Birth History: prenatal care, complicated prenatal course, maternal risk factors (diabetes assoc. with hypertrophic cardiomyopathy), prematurity, method of delivery, prolonged hospital stay, birth weight, APGARsFamily History: CHD, sudden death, SIDS, cardiomyopathy (high incidence of inheritance, >20%), dysrhythmias, syndromes/genetic abnormalities
8Past Medical History General Health Concerns from parents Growth curve and weight gain (pay attention to stature of parents)Chronic respiratory difficulties- asthma, frequent URIs/pneumoniaOther medical issues/surgeriesDown Syndrome, Marfan Syndrome, etc.
9Important 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?
10Vital SignsRespiratory Rate- should be counted for a full minute in infants because of variabilityHeart rate- resting if possibleBlood 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
11Overall AppearanceAnxious, distressed, diaphoretic, pale, tachypneic, retractions, cyanotic, difficult to console, dysmorphic, clubbingA baby, child and adolescent can have a significant cardiac lesion and appear perfectly healthy with apparently normal vitals signs
12Cardiovascular 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?
13AuscultationListen at all landmarks (aortic, pulmonic, 2nd pulmonic, tricuspid, mitral) with bell and diaphragmListen to heart soundsS1: closure of what valves?S2: closure of what valves?
14S1 Closure of the atrioventricular valves (mitral and tricuspid) Normally heard as one sound, although occasionally splitting may be heard
15S2 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.
17MurmursMurmurs 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 feverStructural or physiologic cardiac problems can also cause murmurs; 1% of children have structural heart disease
18Grading of Murmurs 1: Heard only with intense concentration 2: Faint, but heard immediately3: Easily heard, of intermediate intensity4: Easily heard and palpable thrill present5: Very loud, thrill present, and audible with edge of stethoscope on chest wall6: Audible with stethoscope off chest wall
19Innocent Vibratory Murmur or Still’s Murmur Most common during childhoodLow to medium pitch, early systoleUsually grade 2, but can range 1-3Maximal at LLSB w/ radiation to apexUsually loudest supine and fades with sitting and standingVibratory, harmonic, musicalOften louder during febrile illness or times of increased cardiac outputUsually disappear with time and can come and go
20Innocent Pulmonary Flow Murmur A relatively soft systolic murmur appreciated at the upper left sternal borderOften louder while lying supine and fades/disappears with sitting
21Venous HumContinuous murmur usually heard at the infraclavicular area of the anterior chest, R>LLoudest while sitting (venous return from the jugular veins and subclavian veins entering the SVC ) and disappears supineCan be diminished or muted with gentle compression of jugular veinCommonly associated with vibratory murmur
22Pathological Systolic Murmurs Upper Right Sternal Border: Aortic Stenosis, subaortic membrance, PPASUpper Left Sternal Border: Pulmonary Stenosis, PDA, PPASMid to Lower Left Sternal Border: VSD, TR, LVOT narrowing (HCM)/Subaortic stenosisApex: MR, poss VSD
23Pathological 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
24Diastolic MurmursAlways pathological- there are no “normal” or “innocent” diastolic murmursWhat are some diastolic murmurs?
25“Early” Diastolic Murmurs Decrescendo in natureCommonly aortic regurgitation or pulmonary regurgitation (insufficiency)
26Mid to Late Diastolic Murmurs Tricuspid stenosis or mitral stenosis
27Continuous MurmursHeard through systole and diastole- most common “pathological” continuous murmur is a Patent Ductus Arteriosus
29Exam (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!
30When 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, ECHOFamily history
31When 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 infoIf 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 evaluationIf 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!
33Congenital 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
34What 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.
38Cyanotic Lesions d-transposition of the great arteries (d-TGA) Tetralogy of FallotHypoplastic right heart syndromeHypoplastic left heart syndromeDouble outlet right ventricle (DORV)Truncus arteriosusTotal anomalous pulmonary venous return (TAPVR)
40Atrial Septal Defects (ASD) Common defectMay be a PFO/small secundum ASD % of the population has thisMay be larger, causing significant L to R shuntingCan go undetected for yearsExam: wide, fixed splitting of S2, often a systolic murmur r/t increased pulm flow“Easily” fixed (surgery vs. transcatheter)
42Ventricular Septal Defect Ventricular septum fails to “fill in” completely during embryonic developmentVarious degrees of VSDs from tiny to largeMay be asymptomatic, mildly symptomatic, or in congestive heart failureMay not present clinically until 1-2 months of lifeOften associated with other lesionsIsolated VSD’s typically have favorable surgical outcomesMany small and even mod sized VSD’s can close spontaneously for up to 4 years of age
44Mitral Valve ProlapseThe most common valvular abnormality (2-6% of the population)- more common in females than males (2:1)Usually benign, but may also have mitral regurgitationCan worsen with time, or improveExam: mid-late systolic click (often), blowing, holosystolic murmur if significant degree of MRDiagnosis- echocardiogram (often wrongly and over-diagnosed by exam only)May be associated with connective tissue disorders such as Marfan Syndrome
46Coarctation of the Aorta Narrowed area of the aorta (usually descending or transverse arch)May cause hypertensionOften systolic murmur LUSB with radiation to left backDiscrepancy between upper and lower extremity blood pressure and pulsesUsually an “easy” repair
49Tetralogy of Fallot4 defining characteristics- Pulmonary stenosis, RVH, VSD, and aortic overrideMay be associated with a genetic syndromeSurgical 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
53There 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.
55Kawasaki Disease (KD) An acute, self-limited vasculitis Unknown cause, but an infectious cause/virus is suspectedLeading cause of acquired heart disease in US and JapanUsually seen in children <5 y.o.First described by Dr. Kawasaki in Japan, 1961
56KD- Diagnostic Criteria Fever >/= 5 daysBilateral conjuctival injectionChanges of mucous membranes- injected pharynx, fissured lips, strawberry tongueChanges of peripheral extremities- peripheral edema, peripheral erythema, desquamation of palmsPolymorphous rashCervical adenopathyDiagnosis is presence of fever and 4 of 5 remaining criteria
57KD- Cardiovascular concerns Development of coronary artery aneurysms (usually around 2 wks after onset of symptoms)Evaluated by echocardiogramHalf 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
61TreatmentStandard treatment is high-dose IVIG (immunoglobulins) in combination with high doses of aspirinScreening/repeat echocardiogramsPossible cardiac cath with intervention if coronary artery aneurysms causing myocardial dysfunction or for further evaluation
62Long-term prognosis without coronary aneurysm No significant risk of increased mortalityPossible 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
63Rheumatic FeverA 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
64History 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
65Jones CriteriaRequires presence of 2 major, or 1 major and 2 minor criteria. Previous Group A strep is also necessary
66Major CriteriaCarditis (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 edemaPolyarthritisChorea- brief, irregular, unpredictable, purposeless movements that flow from one body part to another without a rhythmic patternErythema 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
69Minor CriteriaFever- usually greater than 39 deg C, although may be low-grade, may last 2-3 weeksArthralgia- cannot be considered minor if arthritis is presentProlonged PR interval in EKGElevated erythrocyte sedimentation rate (ESR) and C-Reactive Protein (CRP)
70Evidence of GAS pharyngitis – one of the following MUST be present Positive throat culture or rapid strep antigen testElevated or rising streptococcal antibody titer (such as antistreptolysin O [ASO])
71Lab Studies Throat culture Rapid antigen detection test Antistreptococcal antibodies- usually using antistreptolysin O (ASO)C-RP, ESR
72Imaging 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
73TreatmentAntibiotics- 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 manifestationsAfter 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)
74What does this mean as FNP’s Recognizing and treating streptococcal infections in a timely mannerAwareness 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 resolvedOnly infections of the pharynx initiate RF (in developed countries)Secondary prophylaxis of patients with history of RF- daily PCN or injections q 3-4 wksSBE prophylaxis with antibiotics prior to dental and other procedures
75Revised 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 valvesPrevious endocarditisUnrepaired cyanotic defectPalliative shunt/conduit presentComplete repair with prosthetic material for first 6 months after procedureHeart transplantationRecommended 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
77Other common complaints Chest pain- rarely attributed to any underlying cardiovascular disease. Coronary artery abnormalities in children rarely present with chest painSyncope- 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