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Daniela Rafii, M.D. Associate Director, Pediatric Cardiology Maimonides Infants and Children’s Hospital of Brooklyn Over 50% of children have an innocent heart murmur no intervention, require reassurance Most common innocent heart murmurs: Still’s murmur AKA function heart: Musical/twangy/vibratory systolic ejection murmur Louder while supine Usually located over the LLSB Venous hum: Continuous murmur Softer/resolve while supine or w/ pressure to jugular vein or w/ turning the head Usually RUSB or LUSB AS Harsh crescendo-decrescendo SEM RUSB Radiates to the neck PS Harsh crescendo-decrescendo SEM LUSB Radiates to the back PDA continuous machinery murmur Diastolic murmurs are never innocent an cic qu es t Re or a 0% EK G 0% ... ra tX an st h ch es Re qu es ta tr de r a lo gy Or rd io ca 0% y 0% co n. .. ab o. . nt s re pa ta E. 0% th e D. qu es C. Re B. Reassure the parents about the benign prognosis Request a cardiology consult Order a chest X ray Request a transthoracic echocardiogram Request an EKG Re as su re A. Acyanotic (pink) CHD ASD (left to right shunt) VSD (left to right shunt) PDA (left to right shunt) AS PS Coarc Acyanotic CHD 3 types Primum Secundum (most common) Sinus Venosus Asymptomatic in childhood SEM loudest at LUSB relative PS murmur w/ fixed widely split S2 EKG w/ right ventricular conduction delay Dx echo Treatment surgical vs percutaneous closure Natural history in the 3rd-4th decade of life: Arrhythmias Pulmonary HTN Paradoxical emboli Acyanotic CHD Most common CHD 4 main types of VSD Perimembranous Muscular Inlet Outlet Small VSDs no sx only loud harsh 3/6 holosystolic murmur loudest at LLSB Large VSDs congestive heart failure (CHF) CHF x typically start at 6-8 weeks old from a VSD Babies present with: Some degree of resp distress: tachypnea, retractions, abd breathing, etc Irritability Hepatomegaly Cardiomegaly NO peripheral edema NO cyanosis Poor po intake, easy fatiguing w/ feeds, prolonged feeding times Poor weight gain: weight drops off before height Excessive sweating Sinus tachycardia Loud harsh holosystolic murmur loudest at the LLSB Cardiomegaly Inc lung markings Pulm edema EKG: Normal Sinus tachy LVH Echo: diagnostic If CHF treat with: Digoxin 5mcg/kg/dose bid Lasix 1mg/kg/dose bid Enalapril afterload reduction Increase caloric intake 24kcal/oz formula Surgical closure of large VSDs ~ 6 mo old sooner if FTT despite medical trt CHF Small VSDs: 80% close within the 1st year of life Large VSDs: CHF 40% of babies will die w/o treatment in 1st year of life Unrepaired large VSDs past 1yo develop Eisenmenger’s syndrome in teen years Progressive pulmonary HTN w/ progressive cyanosis Right to left shunt Mean survival mid-20’s AKA: AV Canal endocardial cushion defect AV septal defect ~50% of children w/ T21 have CHD ~40% of those with T21 and CHD have AVC EKG: often has left superior axis Echo: diagnostic Treatment: Medically manage CHF (same as w/ large VSD) Surgical repair 4-6 months old All babies are born with a PDA Usually closes within 1st 2 wks of life in full term babies Persists longer in preterm babies Small PDAs no symptoms Large PDAs CHF sx similar to large VSDs In preemies resp distress can be seen in the 1st few days of life Classic murmur continuous machinery murmur secondary to continuous shunt in systole and diastole loudest under L clavicle Murmur is not heard in newborns secondary to elevated pulm vascular resistance (PVR) Murmur develops only after PVR srops If significant sx in preterm babies Indomethacin May try CHF rx Surgical PDA ligation If CHF sx in term babies Medically treat CHF If babies can reach 10kg percutaneous PDA closure If cannot gain weight surgical PDA ligation du c nt tri c .. a rte ve nt r io r ic su Se u s la cu rs nd e um pt al at . .. ria ls ep ta ld . .. de . tu s rc ul a Pa te rg e La or ta an al ea th of n tio E. ve n D. 20% 20% 20% 20% 20% rc ta C. At r io B. Coarctation of the aorta Atrioventricular canal defect Patent ductus arteriosus Large ventricular septal defect Secundum atrial septal defect Co a A. ... in ce as ur g ica lc on ns s nt io in Ob ta su lt f.. m gr a ... in te rv e De fe r an ec ho ca et ha Pe rfo r m ist in rd io cin i.. . e id nd om er i fu ro se m er E. ist D. in C. 20% 20% 20% 20% 20% Ad m B. Administer furosemide intravenously Administer indomethacin intravenously Perform an echocardiogram Defer interventions since spontaneous closure is likely Obtain a surgical consult for PDA ligation Ad m A. tra d ... ft he ul ab d so un d ra m di og hy o gr ap gr a ho ca r Ec di o ra ne He a Ra di o ph y lo w al sw m sp i E. riu D. ca l C. 20% 20% 20% 20% 20% Ba B. Barium swallow Cervical spine radiography Echocardiogram Head ultrasound Radiography of the abdomen Ce rv i A. More common in males Almost always juxtaductal [A] Preductal [B] present earlier Postductal [C] present later 85% of children with coarc have a BAV Frequently seen in Turners syndrome {45, XO} 10% have severe coarc 30% have bicuspid aortic valve (BAV) Variable presentation Infant with cardiogenic shock Child or adolescent with systemic hypertension Child with a heart murmur Severe coarc in a neonate Often present before 2 weeks old Cardiogenic shock: resp distress/failure, poor perfusion, altered metal status Multisystem organ failure: NEC, renal failure, intracranial bleed **Diminished lower extremity pulses Moderate coarc in a neonate CHF: resp distress, poor feeding, poor growth Mild coarc in a neonate No symptoms +/- murmur Older children and adolescents do NOT present with heart failure sx Can have upper extremity hypertension refractory to antihypertensive rx Can have diminished lower extremity pulses Can have claudication Can just have a murmur EKG: Often normal Can have LVH with strain pattern (ST elevation +/- T wave inversion in precordial leads) CXR: often nonspecific findings, normal or: Cardiomegaly Increase pulm vascular markings Rib notching (not seen in infants, uncommon prior to 5 years old) Echo: diagnostic Treatment: Infant in shock immediate PGE + aggressively trt shock Surgical repair for children or for complex coarc Percutaneous stent placement in adult sized patients Varying degrees of left heart hypoplasia Babies present in cardiogenic shock when PDA closes Immediate treatment with PGE IV infusion Surgical treatment: Norwood palliation 1st week of life Bidirectional Glenn 4-6 months old Fontan palliation 2-4 years old Th or a nj ur y cic du ct i sy n. .. av a ro p rv en ac nt e rio n- lo s in ge Su pe to m y sy n. .. at h y ia m cy th e rd io E. Pr ot ei D. Po ly C. 20% 20% 20% 20% 20% ric a B. Polycythemia Postpericardiotomy syndrome Protein-losing enteropathy Superior vena cava syndrome Thoracic duct injury Po st pe A. Stenosis may be valvular, subvalvar, or supravalvar Mild-mod PS no sx Severe or critical PS cyanosis Murmur harsh SEM at LUSB Echo –> diagnostic Treatment: ballooning or surgical Stenosis may valvular, subvalvar, or supravalvar More common in males More significant lesion compared to PS, no cyanosis, (+) heart failure/cardiogenic shock Valvar AS is often associated w/ BAV Murmur harsh SEM at RUSB Echo –> diagnostic Treatment: ballooning or surgical Mild to severe usually asymmetric thickening of the myocardium Often autosomal dominant Incidence 1:500 Symptoms: Sudden death (on exertion) Arrhythmias Syncope Chest pain de fe c t ia ol e r io ta l se p lar cu Ve nt ri Se ve re hy po v rte ts a du c nt Pa te m su s is fe ta l no sis bl a st os is cs te or ti yt hr o E. la D. Er C. ica B. Critical aortic stenosis Erythroblastosis fetalis Patent ducts arteriosus Severe hypovolemia Ventricular septal defect Cr it A. 20% 20% 20% 20% 20% A. B. C. D. E. Dopamine infusion Loading dose of digoxin 25% glucose and water solution Furosemide Prostaglandin E1 Do pa m in Lo ei ad nf in us g io do 25 n % se glu of di co go se xin an d w at er s.. . Fu ro se m Pr id os e ta gla nd in E1 20% 20% 20% 20% 20% Cyanotic (blue) CHD Often have cyanosis with NO resp distress Need some sort of L to Right shunt to have cyanosis TOF (right to left shunt) TGA Tricuspid atresia (right to left shunt) Truncus arteriosus TAPVR Ebstein’s Anomaly (right to left shunt) Single Ventricles PS VSD Overriding aorta Right ventricular hypertrophy (RVH) Most common cyanotic heart lesion Signs/symptoms: Cyanosis Loud harsh SEM at the LUSB (PS murmur) Squatting in older kids Tet spells cyanosis often worsened or caused by crying Lose PS murmur b/c less blood across pulm valve Increase right to left shunt across VSD Trt calm kid down, knees to chest, morphine, oxygen, general anesthesia Aorta arises from the RV and the pulmonary artery arises from the LV Mixing of blood occurs at the PFO/ASD, PDA, +/-VSD Sx: Cyanosis +/- murmur May have higher sats in the lower extremities vs the upper extremities Echo: diagnostic TA left superior axis on EKG (like AVC) Sx occur when pulm veins are obstructed Can occur soon after birth Babies present with severe cyanosis (unresponsive to O2) and resp distress No murmur Treatment emergent surgery Apical displacement of the tricuspid valve Can present with: Severe TR R to L shunting neonatal cyanosis 25% can have SVT or WPW Neonatal treatment decrease PVR iNO Oxygen CXR severe cardiomegaly fF al de fe ct lo t tis ta l se p lar cu Ve nt ri Te tra lo M gy o yo ca rd i or ta ea th of n tio E. ic D. rc ta C. Ao rt B. Aortic stenosis Coarctation of the aorta Myocarditis Tetralogy of Fallot Ventricular septal defect Co a A. st en os is 20% 20% 20% 20% 20% tr a fo r ng e rr a A ... a o ns fe rt in sp i. . . 0. .. 40 pp m N O ti PG E St ar tI V St ar at in in th e at e fu si fa nt on an d at p. .. ... ev al to G EK st at a E. In tu b D. in C. 20% 20% 20% 20% 20% bt a B. Obtain a stat EKG to evaluate for SVT Intubate the infant and place on 100% O2 Start IV PGE infusion at 0.050.2mcg/kg/min Start iNO at 40ppm inspired to reduce PVR Arrange for transfer to a facility capable of ECMO O A. io su s te r ar gr un cu s Tr th e of sit io n an sp o ta Tr ea ... ft ea o e ch yp n alv yv an sie nt Tr .. es ia at r e. .. yh yp on ar on ar m E. Pu lm D. yp ul C. ar B. 20% 20% 20% 20% 20% Primary pulmonary hypertension of the newborn Pulmonary valve atresia Transient tachypnea of the newborn Transposition of the great arteries Truncus arteriosus Pr im A. lu ng eo na Tr d in e ta ac he fe t al N Re liq oe ui so d. .. ph ag ea lf is tu la ta l se p sy ... rt he a ft 20% 20% 20% si s ia ne m E. A D. le C. 20% 20% st ic B. Anemia Hypoplastic left heart syndrome Neonatal sepsis Retained fetal lung liquid syndrome Tracheoesophageal fistula H yp op la A. Most common arrhythmia in childhood Babies present with poor feeding, pallor, irritability Older kids present w/ palp, dizziness, fatigue EKG diagnostic narrow complex regular tachy >220bpm Can be associated with WPW If patient hemodynamically stable vagal maneuvers: Ice to face Valsalva If doesn’t work adenosine If patient hemodynamically unstable synchronized cardioversion Ice to face “Bearing down” Blowing against and occluded straw Gag Cough Atrial flutter: saw tooth p waves Atrial fib: irregularly irregular Adenosine is diagnostic not therapeutic Treat both with synchronized cardioversion 1st degree HB: prolonged PR interval 2nd degree HB: Rheumatic fever, myocarditis, KD, congenital Type I: Wenkebach, progressive prolongation of PR interval then dropped beat high vagal tone, benign Type II: dropped (nonconducted beats) can progress to CHB pacemaker 3rd degree: complete HB Postop, lyme disease, myocarditis Congenital CHB maternal SLE QTc >0.45 sec Genetic often family hx sudden death Arrhythmia torsades de pointes (type of VT) Be suspicious if: Family hx SCD Hx seizure d/o Congenital deafness Syncope following loud noises, being startled Acute treatment: magnesium Long term treatment: beta blockers Sudden, blunt, non-penetrating trauma to the chest V fib sudden death Typical story: healthy kid gets hit with a baseball to the chest and drops Treatment stat defib Benign causes of syncope: Vasovagal: during blood draws, site of blood Orthostatic hypotension: standing up quickly, standing up for too long, especially if hot Hyperventilation Breath holding spells: 6-18mo, associated w crying, +/- cyanosis Concerning, potentially life-threatening syncope: Associated w/ exertion (VT, LQTS, HCM, other cardiomyopathy) Associated w/ excitement/startle (LQTS) Family hx sudden death HCM 3 22 Coronary Anomalies AS 6 3 3 36 Ruptured Ao 5 Tunnelled LAD 5 Myocarditis 4 19 Dilated CM ARVD Carditis refers to inflammation of any of the 3 layers of the heart, occur in isolation or conjunction with one another Endocarditis inflammation of the cardiac valves valvar dysfunction Myocarditis inflammation of the muscular walls of the heart myocardial dysfunction, conduction abnormalities (heart block, arrhythmias) Pericarditis inflammation of the pericardium pericardial effusion Diagnosis Modified Duke Criteria 2 major 1 major + 3 minor 5 minor Treatment IV abx, type and length of treatment vary Positive Bcx w/ typical IE microorganism: Typical microorganism consistent w/ IE from 2 separate Bcx: Viridans-grp strep, or Strept bovis including nutritional variant strains, or HACEK group (Haemophilus spp, Actinobacillus, Cardiobacteriom hominis, Eikenella spp, Kingella), or Staph aureus, or Community-acquired Enterococci, in the absence of a primary focus Microorganisms consistent w/ IE from persistently (+) Bcx: 2 positive Bcx drawn >12 hours apart, or All of 3 or a majority of 4 or more separate Bcx (w/ first and last sample drawn at least 1 hour apart) Coxiella burnetii on at least 1 (+) Bcx or antiphase I IgG antibody titer >1:800 Evidence of endocardial involvement w/ (+) echo: Oscillating intracardiac mass on valve or supporting structure, or in the path of regurgitant jet, or on implanted material, or Abscess, or New partial dehiscence of prosthetic valve or new valve regurgitation Predisposing factor: CHD, recreational IV drug use Fever >38°C Evidence of embolism: arterial emboli, pulmonary infarct, Janeway lesions, conjunctival hemorrhage, mycotic aneurysm, intracarnial hemorrhage Immunological problems: glomerulonephritis, Osler’s nodes, Roth spots, rheumatoid factor Positive Bcx (that doesn't meet a major criterion) or serologic evidence of infection w/ organism consistent with IE but not satisfying major criterion Janeway lesions: small, erythematous, non-tender, macular or nodular lesion on soles/palms septic emboli microbscesses Osler’s nodes: painful, red, raised lesions on hands/feet immune complex deposition Roth spots: retinal hemorrhages w/ white or pale centers composed of coagulated fibrin immune complex mediated vasculitis 2007 AHA guidelines: Prosthetic valves Previous IE Unrepaired cyanotic heart disease Repaired CHD <6 months after surgery Repaired CHD >6mo if residual lesion near prosthetic material Cardiac transplant with valvulopathy Myocarditis inflammation of the muscular walls of the heart myocardial dysfunction/failure, heart block, arrhythmias Presentation in babies irritability, poor feeding, pallor, shock, cardiomegaly, hepatomegaly, pulm edema Presentation in older kids fatigue, dyspnea, chest pain, palpitations, pallor, hypotension, cardiomegaly, hepatomegaly, pulm edema Infectious vs noninfectious vs idiopathic Viral most common cause in US: coxsackie, adenovirus, parvovirus B19, enterovirus, EBV, CMV, HSV 6 Other infectious Lyme dz and other spirochetes, fungal, bacterial Toxic/hypersenstivity reactions chemotherapeutic agents, abx, amphetamines Systemic dz Giant-cell myocarditis, sarcoidosis, KD, Crohn’s, UC, SLE, thyrotoxicosis No single clinical or imaging test to confirm dx Dx made by: Clinical sx Serologic: CKMB, troponin, BNP, CRP Noninvasive: EKG, echo, MRI Invasive: myocardial biopsy Treatment: depends on severity of sx, mostly supportive: ionotropic support, mechanical vent, ECMO, trt CHF, antiarrhythmics, temporary pacing, IVIG or steroids Long term: fully recover (50%), chronic heart failure, need for transplant Viral pericarditis most common Preceded by a viral URI Sx: sharp chest pain, improves with leaning forward PE: friction rub, pulses paradoxus (exaggeration of normal finding of dec in systolic BP w/ inspiration) EKG diagnostic: diffuse ST segment elevation Echo: pericardial effusion Risk cardiac tamponade Treatment NSAID, pericardiocentesis if severe 20% 20% 20% 20% 20% m a ia le de e m ol u gv no sis E. Cy a D. gh C. Co u B. Ascites Cough Cyanosis Diminished feeding volume Pretibial edema As c it es A. 20% 20% 20% m on th s 12 hs 6 m on t hs m on t 2 ee ks w E. 2 D. s C. da y B. 20% 2 days 2 weeks 2 months 6 months 12 months 2 A. 20% ARF results from a complex interaction btw GAS and a susceptible host Abnormal immune response leads to acute inflammation of joints, brain, heart, and or skin All organ systems recover w/o sequelae except the heart ARF can lead to chronic RHD Diagnosis modified Jones Criteria 2 major or 1 major + 2 minor And evidence of a preceding streptococcal infection Major criteria: Except w/ presence of chorea or indolent carditis Polyarthritis Carditis Sydenham’s chorea Erythema marginatum Subcutaneous nodules—extensor surf Minor criteria Fever Polyarthralgia Elevated acute phase reactants (ESR, CRP) Prolonged PR interval Initial cardiac involvement is a pancarditis Most prominent feature is valve involvement #1 mitral (MR) #2 mitral + aortic (MR + AI) #3 aortic (AI) Treatment: Primary ppx: Treat strep infection High dose aspirin or NSAIDs or steroids (4-6 weeks) Secondary ppx: monthly IM PCN Long term RHD: MR + AI MR/MS + AI/AS Arteritis of medium size arteries AKA mucocutaneous lymph node syndrome Diagnosis 5 days of fever + 4/5 of the following: B/l nonexudative conjunctivitis Erythema of the lips or oral mucosa Changes in the extremities: swelling, erythema Rash Cervical LAN, >1.5cm Supplemental laboratory criteria: Albumin </=3.0 g/dL Anemia for age Elevation of ALT Plt after 7d >/=450 WBC >/= 15 Urine 10 WBC/highpower field Newburger, J. W. et al. Circulation 2004;110:2747-2771 Treatment for typical and atypical KD is the same IVIG w/i 10 days of fever, may repeat if still febrile High dose aspirin W/o treatment 20% of children will develop coronary artery aneurysms W/ treatment w/ IVIG and ASA5% of pts will develop aneurysms Aneurysms develop usually w/i 2 weeks Can have an associated mild myocarditis T21: CHD incidence 50%, think AV canal defects Turner’s: 10% coarc, 30% BAV Pompe’s: Hypertrophic cardiomyopathy (HCM) Alagille : Peripheral pulmonic stenosis (PPS) Williams’s: Supravalvar aortic stenosis, PPS Noonan: PPS and (HCM) Marfan’s: Aortic root dilatation, MVP DiGeorge (22q11 del): TOF, truncus arteriosus, interrupted Ao arch, right aortic arch Kartagener: dextrocardia, situs inversus totalis, immotile cilia Holt-Oram: Limb abnormalities, ASD Ellis-van Creveld: ASD Lithium: Ebstein’s anomaly Ethanol: ASD,VSD (fetal alcohol syndrome) Anticonvulsants: PS, AS, TOF Retinoic Acid: TGA Rubella: PDA, PPS Coxsachie B: Neonatal myocarditis Maternal Diabetes: HCM, TGA Maternal Lupus: Complete heart block PKU: VSD, ASD, complex CHD de se c va lo -1 5 f0 .1 2 gr ee s se c Q RS te r in ax is of va lo te r in PR Q RS de -3 0 of ax is e av w f0 .8 1 gr ee s f. .. lo rv a in te T Q E. P D. d C. 20% 20% 20% 20% 20% re ct e B. Corrected QT interval of 0.52 sec P wave axis of -30 degrees PR interval of 0.81 sec QRS axis of -15 degrees QRS interval of 0.12 sec Co r A. e er w S2 S1 an d as w Sh e .. al a m no r th e on ng lyi gt lin fa l so f .. . un ... gr o e. .. th e o be fo r ... e lin nt e d on ce n in gi st an d fa i Af te r E. Sh e D. as C. 20% 20% 20% 20% 20% w B. She was standing in line waiting to see “The Hunger Games: Mockingjay part 2” when she passed out She fainted once before when she had a blood test After falling to the ground she came to quickly and remembered feeling warm and dizzy She was lying on the sofa watching TV when a door slammed and she suddenly became unresponsive S1 and S2 were normal and no murmurs were noted Sh e A. fo r g te st in et ic tr op ... yo s. . m fo r g tin Ge n et ic Ge n Ex er ci se m te s yo c ar d ia lp gr a rd io tr oc a er f.. . ph y hy ra p di og E. ec D. El C. 20% 20% 20% 20% 20% ho ca r B. Echocardiography Electrocardiography Exercise myocardial perfusion scintigraphy Genetic testing for myosin chain mutations Genetic testing for troponin mutations Ec A. al xy sm Pa ro tis Pe ric ar di yc ... tis ac h at r ia lt yo ca rd i tis M rd i e en do ca fe ve r ic at tiv In fe c E. rh eu m D. e C. 20% 20% 20% 20% 20% cu t B. Acute rheumatic fever Infective endocarditis Myocarditis Paroxysmal atrial tachycardia Pericarditis A A. tis Pe ric a rd i m ia s M yo ca rd i al is c he nd rit i ia ch o yt hm Co st o Ar rh ic fe ve r E. at D. he um C. 20% 20% 20% 20% 20% er B. Acute rheumatic fever Arrhythmia Costochondritis Myocardial ischemia Pericarditis Ac ut A.