Innocent MurmursDiastolic murmurs are never innocentInnocent murmurs are present in at least 50% of normal children– Still’s murmur : low pitched, vibratory,systolic ejection, increases with the supineposition.– Venous hum: continuous murmur insupraclavicular region, reduces on lying downor with pressure on neck.
1.Upon physical examination of a 3 year old girl who isnew to the practice, you note a continuous grade 2 tograde 3 murmur at the upper right sternal borderwhile she is sitting. In the supine position, you noteonly a grade 2 low pitched systolic murmur at theapex. Measurements of BP, pulses and precordialpalpations as well as the auscultation is normal. Ofthe following, the MOST appropriate next step is to: A. reassure the parents about the benign prognosis B. request a cardiology consultation C. request chest radiography D. request echocardiography E. request electrocardiography
Congenital Heart Disease-Congenital Heart Disease-StructuralStructuralPINKShunts ( L to R) : ASD VSD PDAStenosis: AS PS Coarctation HLHSBLUE TOF TGA Tricuspid atresia Truncus TAPVR Ebstein’s Single ventricle
Normal Cardiac PressuresNormal Cardiac Pressures120/<825/<5<5<8120/8025/15
ATRIAL SEPTAL DEFECTSATRIAL SEPTAL DEFECTS(ASD)(ASD)Three types exist : primum,secundum and sinus venosusThe most common is the secundumtypeSymptoms: None in childhood,arrhythmias in the 3 rd decade
ASD- cont...ASD- cont...Clinical signs include a 2-3/6 SEM atthe ULSB and a fixed wide split S2A large ASD causes right ventricularenlargementEKG: RAD and IRBBB
ASD - cont...ASD - cont...ECHO: DiagnosticNatural History: Arrhythmias andpulmonary obstructive vasculardisease in the 3rd and 4th decade.IE Prophylaxis ??Treatment : Surgical vs.transcatheter closure
VENTRICULAR SEPTALVENTRICULAR SEPTALDEFECTS ( VSD)DEFECTS ( VSD)This is the most common form of CHDThe VSDs are subdivided according to thepart of the septum they occur in :Muscular, perimembranous, inlet, outletA large VSD causes left ventricularenlargementWith a small VSD there is normal growthand development
VSD - cont…..VSD - cont…..With a large defect there may beCHF( usually at 6-8 weeks),pulmonary infections and delayedgrowthClinical signs : Loud 4-5/6 , harshholosystolic murmur, middiastolicrumble and a loud P2
VSD - cont…..VSD - cont…..EKG: LVH or BVHECHO: Diagnostic
VSD -cont...VSD -cont...Natural history : Small VSDs closespontaneously depending on the site.Unrepaired the large defects maylead to Eisenmenger’s syndrome.
VSD - cont…..VSD - cont…..Large VSDs are closed surgicallyusually at about 4- 6 months of age.Diuretics,digoxin and afterloadreducing agents are used prior tosurgery - if needed.
AVSD - cont...AVSD - cont...1/3rdof babies with this have DownsyndromeEKG : Characteristic with asuperior left axis.Echo : ConfirmatoryManagement : Anticongestivemedications and surgery at 4-8months of age.
PATENT DUCTUSPATENT DUCTUSARTERIOSUS ( PDA)ARTERIOSUS ( PDA)It is a connection between the aortaand the pulmonary artery.Very common in preterm babies.Usually closes in the first 2 weeks oflife.
PDA - cont…..PDA - cont…..Symptoms : a) None if small– b) If large can cause CHF at 6-8weeks in a term infant– c) In a preterm baby increasingrespiratory support usually occursafter day 3 of life.
PDAPDASigns: Systolic murmur in anewborn and a continuous “train ina tunnel” murmur in an older child.Best heard below the left clavicle.A large PDA causes LA and LVenlargement.Treatment : Preterm vs. term baby.
PDA - cont...PDA - cont...In a preterm it can be closedmedically using indomethacin.In a term baby if still open at 3months of age then coil closure bycardiac catherization is the methodof choice.
1. A 3 month old girl who has Down syndromeexhibits poor weight gain, tachypnea and a lowpitched grade 2 murmur. Chest radiographyreveals cardiomegaly and increased pulmonaryvascularity. EKG documents RVH and a superiorfrontal plane QRS. Of the following, the MOSTlikely diagnosis is:A. coarctation of the aortaB. complete atrioventricular septal defectC. patent ductus arteriosusD. Perimembranous VSDE. secundum ASD
2. A 5 day old infant born at 31 weeks gestation is onventilatory support. He has a grade 2 holosystolic murmurthat extends past the second heart sound. Pulses arebounding. Precordial palpation is hyperdynamic.Echocardiography reveals a large patent ductus arteriosus.Concentrations of hemoglobin, electrolytes and creatinineare normal.Of the following the most appropriate INITIALmanagement is to: A. administer furosemide intravenously B. administer indomethacin intravenously C. administer indomethacin via nasogastric tube. D. defer intervention because spontaneous closure islikely E. obtain a surgical consultation for ligation of theductus.
3. A term infant is born with a largeventricular septal defect. At what age isthis infant MOST likely to firstdemonstrate clinical findings ofcongestive cardiac failure?A. 2 daysB. 2 weeksC. 2 monthsD. 6 monthsE. 12 months
4. A term newborn has tachypnea, rales,tachycardia, audible gallop and diminishedarm and leg pulses. Echocardiography showsenlargement of both ventricular chambers withgood systolic function and no congenital heartdisease. Of the following, the MOST likelydiagnosis is:A. Carnitine deficiencyB. hyperthyroidismC. hypoglycemiaD. intracranial arteriovenous malformationE. pheochromocytoma
COARCTATION OF THECOARCTATION OF THEAORTAAORTA
Coarctation of the AortaCoarctation of the Aorta(CoA)(CoA)More common in malesAlmost always juxtaductal85% of children with CoA have abicuspid aortic valve.
CoA - cont….CoA - cont….Symptoms and Signs:SEVERE : ShockMODERATE : CHF,MILD : Headaches, leg claudicationDecreased femoral pulses are animportant sign esp. in neonates.BP lower in the lower limbs
CoA - cont….CoA - cont….ECHO : DiagnosticTreatment: For an infant in shock-PGE1 immediately.Surgical vs. transcatheter repair.
Hypoplastic Left HeartSyndrome Varying degrees of left heart hypoplasia atmultiple levels Babies present in cardiogenic SHOCK once theductus closes. Immediate treatment is PGE1 intravenously as aninfusion. Surgical treatment includes a Norwood procedureinitially, followed by a bidirectional Glenn at 6months and a Fontan procedure at about 2 years.
Hypoplastic Left HeartHypoplastic Left Heartsyndromesyndrome
PULMONIC STENOSIS ( PS)PULMONIC STENOSIS ( PS)This may be at the valve, subvalvaror supravalvar.Symptoms: None in mild ormoderate stenosis. Cyanosis is seenonly with critical PS.Signs: ejection click and a harshSEM , at the ULSB.ECHO : Diagnostic
PS - cont...PS - cont...Treatment : Balloon valvotomy if the RVpressure is over 50 mmHG
AORTIC STENOSIS ( AS)AORTIC STENOSIS ( AS)Stenosis possible at the valve,subvalvar or supravalvar.This is a more significant and adangerous lesion compared to PS.More common in males.Valvar AS is usually associated witha bicuspid aortic valve.
ASAS A type of subvalvar AS is also calledHCM which is the commonest causeof sudden death in childrenSymptoms:Mild : NoneModerate to severe: Chest pain,fatigability, syncope.
1. A 3 day old girl is found unconscious in her crib andis brought to the ED. Findings include: tachypnea,tachycardia, pallor; poor capillary refill;hepatomegaly; cardiomegaly with increasedpulmonary vascular markings; hemoglobinconcentration 17 gm/dl; and hematocrit, 51%. Of thefollowing, the cardiogenic shock in this girl MOSTlikely is due to: A. critical aortic stenosis B. erythroblastosis fetalis C. patent ductus arteriosus D. severe hypovolemia E. ventricular septal defect
2. A 6 hour-old infant has increasing pallor,tachypnea and respiratory distress. Physicalexamination reveals an enlarged liver, a galloprhythm, poor pulses in the upper extremitiesand absent pulses in the lower extremities. Inaddition to treating the infant for sepsis, themost appropriate INITIAL management is toadminister: A. a dopamine infusion B. a loading dose of digoxin C. a 25% glucose and water solution D. furosemide E. prostaglandin E1.
There has to be a RIGHT toThere has to be a RIGHT toLEFT shunt to causeLEFT shunt to causecyanosiscyanosis
Tetralogy of FallotTetralogy of FallotMost common cyanotic heart disease.The four abnormalities include:– Pulmonary stenosis– RVH– VSD– Overriding AortaSigns include cyanosis, murmur,squatting and spells.
TOF contTOF cont....A “tet” spell consists of rapid breathing andincreased cyanosis. Any event like crying orincreased physical activity can initiate thespell.Treatment includes:– holding the baby in a knee chest position– Morphine– Oxygen, beta blocker, general anesthesia,
TRANSPOSITION OF THETRANSPOSITION OF THEGREAT ARTERIESGREAT ARTERIES
Transposition of the greatTransposition of the greatArteriesArteries The aorta arises from the right ventricle and thepulmonary artery from the left. The mixing of the blood occurs at the PFO and thePDA. The signs include cyanosis and cardiomegaly.Reverse differential cyanosis! There may be no murmur. An echocardiogram is diagnostic.
1. The mother of a 5 month old girl reports thatfollowing a feeding, the child began to breathedeeply, became increasingly blue and then lostconsciousness. After being held briefly, the infantregained her usual color and became alert. Physicalexamination reveals a harsh murmur. Of thefollowing the MOST likely diagnosis is:A. aortic stenosisB. coarctation of the aortaC. myocarditisD. tetralogy of FallotE. ventricular septal defect
2. You are called at 3 AM from the nursery where 36 hour oldBB Bleu is noticed to be cyanotic. The nurses report that hehad been feeding well and appeared healthy with Apgarscores of 9/9. Until tonight he appeared pink. They report nosignificant tachypnea. You order a chest X-Ray and pulseoximetry to be done while you rush to the hospital. Onarrival the pulse oximetry indicated O2 saturation of 55%and the X-ray shows no increase in pulmonary vascularmarkings or infiltrate. The next MOST appropriateintervention is to: A. obtain a stat EKG to evaluate for SVT B. intubate the infant and place on 100% O2. C. start IV prostaglandin infusion at 0.05-0.2 mcg/kg/min D. start nitric oxide at 40ppm inspired to reduce pulmonaryvascular resistance E. arrange for transfer to a facility capable of ECMO.
3. Following an uncomplicated delivery, a 3.7 kg term infantdevelops cyanosis in the first hour of life. Findings at 3 hours ofage include: cyanosis;heart rate,140 beats/min;respiratoryrate, 56/min; no heart murmurs; pulse oximetery in room air,70% saturation in the right hand and 75% in the foot; in 100%FIO2 via head-hood oxygen, saturation increases to 90% in thefoot; chest radiography, normal. These findings are mostconsistent with: 1. Primary pulmonary hypertension of the newborn B. pulmonary valve atresia C. transient tachypnea of the newborn D. transposition of the great arteries E. truncus arteriosus
Congestive Cardiac FailureCongestive Cardiac Failure Tachycardia Tachypnea Hepatomegaly Cardiomegaly, murmur, HR too fast/slow FAILURE TO THRIVE CHDCHD 2 months-VSD, PDA Within 1stmonth- Coarctation, AS, HLHS Neonatal period:Truncus Arteriosus Normal heart Myocarditis
1.In addition to irritability,sweating anddifficulty breathing with feeding, thesymptom that is MOST indicative ofcongestive cardiac failure in a 3 week oldinfant is:A. ascitisB. coughC. cyanosisD. diminished feeding volumeE. pretibial edema
Rhythm AbnormalitiesRhythm AbnormalitiesEctopic beats: premature atrial ,ventricular– Benign if they disappear with exercise– Seen in the neonatal and adolescent age groupsAtrial Flutter,fibSVTVT– Electrolyte Imbalances– TOF– HCM, Long QT syndromeAV block
1. An 8 year old previously healthy boypresents for a school physical. He is activeand has no symptoms. On exam. He appearswell. His pulse noted by the nurse to be 80but with periods of bradycardia to 60 andthen followed by more rapid rates of 90/min.No other abnormalities are noted.His EKG :
Most common cause of irregular rhythm inMost common cause of irregular rhythm inchildren – SINUS ARRHYTHMIA – BENIGN!!!children – SINUS ARRHYTHMIA – BENIGN!!!
Irregular rhythm in a newborn baby- PrematureIrregular rhythm in a newborn baby- Prematureatrial contractions – BENIGN!!!atrial contractions – BENIGN!!!
Irregular rhythm incidentally noted in anIrregular rhythm incidentally noted in anadolescent- Ventricular Premature beats whichadolescent- Ventricular Premature beats whichdecrease with exercise – BENIGN!!!decrease with exercise – BENIGN!!!
SVTSVTRate above 230/min .Tachycardia – most likely SVT– Narrow complex tachycardia– WPW is the most common cause of reentrytachycardia in children
Treatment of SVTTreatment of SVT Hemodynamically stable:– Vagal maneuvers– Adenosine– Verapamil in children over 1 year Hemodynamically unstable :– DC cardioversion Chronic M/t:– Drugs: Beta blockers, digoxin– Radiofrequency ablation
Radiofrequency CatheterRadiofrequency CatheterAblationAblationUpdated mortality for pediatric RF:10/4651 patients:Increased with left sided procedure, lowweight, underlying heart disease, greater #of RF applicationsSchaffer MS et al , Am J Cardiol 2000
1. A 1 year old child is brought to the ER because hisparents thought his heart was pounding as they wereputting him to bed. EKG reveals a HR of 300/minthat spontaneously converts to a sinus rate of100/min. The parents estimate that the tachycardialasted 20 minutes; the child was asymptomaticthroughout. Of the following the MOST appropriatemanagement of this child is; A. administration of a beta blocker B. adminstration of digoxin C. administration of procainamide D. administration of verapamil E. observation without drug therapy
2. A 4 week old infant appears in your ED with ahistory of irritability, increased respiratory rateand poor feeding. On physical examination thechild is diaphoretic with decreased perfusion andtachypneic but still alert. You notice no murmurbut the monitor indicates a HR of 280 bpm. Allbut one of the following are appropriate;A. obtain a 12 lead EKGB. give verapamil 0.1 mg/kg push slowlyC. give adenosine 100 mcg/kg rapid pushD. fill a bag with ice and apply to infants faceE. pass an esophageal probe and pace theheart 20 bpm faster than the tachycardia
Atrial Flutter/ FibrillationAtrial Flutter/ Fibrillation Seen in two groups– Newborns: After t/tBENIGN!!– After extensive atrialsurgery such as Fontanop, atrial switch for TGAetc.– Treatment: DCCardioversion, AVblocking meds
AV BLOCKAV BLOCKFirst Degree – Prolonged PR interval– Rheumatic fever, ASD, PDASecond Degree– Type I: Varying PR intervals and dropped beat,Wenkebach– Type II: 2 or more than 2 :1 blockThird Degree:– Surgical, Lyme Disease– Mom with SLE
Second Degree AV Block –Second Degree AV Block –Type I and IIType I and II
4. SYNCOPE4. SYNCOPE Brief loss of consciousness with rapid recovery Seen in adolescents and in toddlers 20-50% of adolescents experience at least oneepisode of syncope– most cases benign– Vasovagal syncope is the most common type inadolescents Typical history , normal EKG
DANGER SYMPTOMSDANGER SYMPTOMSSyncope especially withEXERTION or EXCITEMENT-anger, fear, startleCardiac arrest with exercise orexcitement
Sudden Death in YoungSudden Death in YoungAthletesAthletes3619455333226HCMCoronaryAnomaliesASRuptured AoTunnelled LADMyocarditisDilated CMARVDMVP
Commotio CordisCommotio CordisYoung childrenBaseball, football, ice hockeyForce of blow is not unusually hardR on T phenomenonPrevention : ? softer balls, ? protectiveclothing,Role of automated External defibrillator
Long QT SyndromeLong QT Syndrome¶ Disorder of the electrical activity of the heart¶ Involves repolarization¶ Characterized by QT prolongation¶ Pts. are susceptible to sudden death due toTorsade de pointes¶ Syncope typically occurs with a startle orexertion¶ can be inherited or acquired
Special situations where theSpecial situations where theQT should ALWAYS beQT should ALWAYS bemeasuredmeasuredSyncopeSeizurescongenital Deafnessnear SIDS
1. A 5 year old girl is very excited following a ride onthe ferris wheel. In the midst of her excitement shesuddenly loses consciousness and falls to the ground.Paramedics on the scene document ventriculartachycardia. Family history reveals a maternal unclewho died suddenly at 16 years of age. Following treatment of the ventricular tachycardia, anelectrocardiogram most likely will demonstrate A. corrected QT interval of 0.52 sec B. P wave axis of –30 degrees C. PR interval of 0.81 sec D. QRS axis of –15 degrees E. QRS interval of 0.12 seconds
2. A 12 year old boy underwent repair for tetralogy ofFallot at 9 months of age. Last month, routine followup echocardiography revealed no residualshunts;moderate right ventricle enlargement; a 60mm Hg gradient from the right ventricle to the mainpulmonary artery;and normal LV size and function.Today he is dizzy and had a near syncopal episode ingym class.The MOST likely cause for his symptoms is A. left ventricular failure B. physical deconditioning C. pulmonary hypertension D. right ventricular failure E. ventricular arrhythmia
3. Julie, an otherwise healthy 9 year old comes tothe ED because she “passed out”. After askingquestions and examining the patient all but oneof the following reassures you that she hasvasovagal syncope which is a relatively benigncause of syncope in children. A. Julie was standing in line waiting to see “Harry Potterand the Goblet of fire” when she passed out. B. she fainted once before when she had a blood test C. after falling to the ground she came to quickly andremembered feeling warm and dizzy D. Julie was lying on a sofa watching TV when a doorslammed and she suddenly became unresponsive E. S1 and S2 were normal and no murmurs were noted
4. A 14 year old girl falls during a race. She isunconscious, cyanotic and has no pulse butspontaneously revives within seconds. Both patientand family histories are benign. Results of thephysical examination, chest radiography, EKG,echocardiography, EEG and an exercise ECG duringa treadmill stress test are normal. The mostappropriate NEXT step in management is to A. order a 30 day looping event recorder B. perform cardiac catheterization studies C. Perform 24 hour ambulatory ECG monitoring D. perform tilt table testing E. reassure the family that cardiac etiologies have beenexcluded
5.A 13 year old boy wishes to participate incompetitive sports. His father died suddenlyat age 28 years, and hypertrophiccardiomyopathy was found on autopsy. Of thefollowing , the MOST helpful test forassessing the boy’s risk is:A. echocardiographyB. electrocardiographyC. exercise myocardial perfusion scintigraphyD. Genetic testing for myosin chain mutationsE. Genetic testing for troponin mutations
PERICARDITISPERICARDITISFollows a viral URISharp chest pain, retrosternal, difficultyin deep inspirationPt. Resists lying downPain worsened by pressure over thesternumFriction rub, pulsus paradoxusEKG is diagnostic
TREATMENTTREATMENTReassuranceNSAIDSOccasional pericardial tap , windowPostpericardiotomy Syndrome: 2 weeksafter surgery
Infective EndocarditisInfective EndocarditisThe endocardium is a deterrant to adhesionby platelets and organisms.The denuded endothelium is a site forplatelet adhesion and subsequent vegetationgrowth The “Low pressure sink” is the site forvegetations.Polycythemia
Prevention of Infective EndocarditisPrevention of Infective EndocarditisGuidelines From the American Heart AssociationGuidelines From the American Heart AssociationA Guideline From the American Heart AssociationA Guideline From the American Heart AssociationRheumatic Fever, Endocarditis, and Kawasaki DiseaseRheumatic Fever, Endocarditis, and Kawasaki DiseaseCommittee, Council on CardiovascularCommittee, Council on CardiovascularDisease in the Young, and the Council on ClinicalDisease in the Young, and the Council on ClinicalCardiology, Council onCardiology, Council onCardiovascular Surgery and Anesthesia, and the QualityCardiovascular Surgery and Anesthesia, and the Qualityof Care andof Care andOutcomes Research Interdisciplinary Working GroupOutcomes Research Interdisciplinary Working GroupCirculation 2007;116;1736-1754;
ConclusionsConclusions(1) Only an extremely small number of cases of infectiveendocarditis might be prevented by antibiotic prophylaxisfor dental procedures even if such prophylactic therapywere 100% effective. (2) IE prophylaxis for dental procedures is reasonableonly for patients with underlying cardiac conditionsassociated with the highest risk of adverse outcome frominfective endocarditis.
(3) Administration of antibiotics solely toprevent endocarditis is not recommendedfor patients who undergo a genitourinary orgastrointestinal tract procedure.ConclusionsConclusions
Prosthetic cardiac valves or prosthetic material used for cardiac valve repair Previous IE Congenital heart disease (CHD)* -Unrepaired cyanotic CHD, including palliative shunts and conduits -Completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure† -Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization) Cardiac transplantation recipients who develop cardiac valvulopathy
Rheumatic CarditisRheumatic CarditisPresent in 50% cases“Sleeping” tachycardia is an early signMitral and aortic valves most commonlyinvolvedRheumatic ArthritisRheumatic ArthritisMost common manifestationPain, swelling and erythemaResolves within 1 week
RF-Treatment andRF-Treatment andPreventionPreventionBenzathine penicillin 1.2 mega units IMAspirin 75-100 mg/kg for 6-8 weeksSteroids for severe carditisDigoxin , diureticsPrevention with BP q 4 weeks.
1. Two weeks after a nonspecific upperrespiratory infection, a previously healthy , 3year-old boy is noted to have a resp. rate of 40breaths/min, a HR of 140 beats/min,hepatomegaly and a gallop rhythm. No heartmurmurs are detected.Of the following, theMOST likely diagnosis is:A. acute rheumatic feverB. infective endocarditisC. myocarditisD. paroxysmal atrial tachycardiaE. pericarditis
2. A 13 year old boy who has a bicuspid aortic valveand who received treatment for dental caries about3 weeks ago now complains of lethargy, decreasedenergy, and reduced appetite. Findings on physicalexamination include low grade fever, splinterhemorrhages, splenomegaly and a new murmurconsistent with aortic insufficiency. Among the following, the BEST study to confirm the diagnosisin this patient would be: A. blood culture B. chest radiograph C. complete blood count D. echocardiogram E. erythrocyte sedimentation rate
3. A 14 year old boy complains of dull chest pain overthe precordium. It began 4 days ago and occursintermittently. It is not associated with activity, butit does increase when he is in a supine position anddecreases when he is leaning forward. Thefrequency, duration, and the intensity of the painhas been increasing. Among the following,the MOSTlikely explanation for these findings is: A. acute rheumatic fever B. arrhythmia C. costochondritis D. myocardial ischemia E. pericarditis
4.Of the following, the procedure that doesnot require antibiotic prophylaxis in achild who has a congenital heart lesion is:A. cystoscopyB. dental treatment for cariesC. myringotomy with tube placementD. rigid bronchoscopyE. sigmoidoscopy
5. An 8 year old girl’s parents complain that shehas been hyperactive and somewhat labile for2 weeks. She has jerky sudden movements ofthe shoulders and seems to have greatdifficulty sitting still. On physical examinationthe MOST likely additional finding in thischild is :A. icteric scleraeB. mitral regurgitation murmurC. Osler nodesD. severe hypertensionE. splenomegaly
KAWASAKI DISEASE Fever of 5 days duration, enlargement oflymph nodes, mucositis, non purulentconjunctivitis, rash Thrombocytosis and elevated ESR seen in 2ndweek Coronary aneurysms are the most commoncardiac manifestation and occur during week 2. Treatment is IVIG 2gm/kg as a single dose andhigh dose aspirin. Steroids occasionally needed for casesunresponsive to IVIG.
1. A 9 week old infant has had irritability and fever to104 F for 8 days. Cultures of blood,urine andcerebrospinal fluid are negative. A coalescing redmaculopapular rash has been present on the trunkand extremities since the second day of the illness. Redscleral conjunctiva without exudate are noted. Of thefollowing, the MOST likely complication to develop is:A. aortic thrombosisB. cerebral infarctionC. coronary artery aneurysmsD. renal vein thrombosisE. splenic infarction
Some Tips…Some Tips…1. Read the question carefully. Is it MOSTlikely or LEAST likely.2. Look for the “code words”.3. Reason it out and exclude as manypossibilities as you can.