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Pediatric Board ReviewPediatric Board Review20082008Pediatric CardiologyPediatric CardiologyPrema Ramaswamy, M.D.Co-Direct...
PEDIATRIC CARDIOLOGYPEDIATRIC CARDIOLOGYInnocent MurmursCongenital structural heart diseaseRhythm problems , syncope et...
Innocent MurmursDiastolic murmurs are never innocentInnocent murmurs are present in at least 50% of normal children– Sti...
1.Upon physical examination of a 3 year old girl who isnew to the practice, you note a continuous grade 2 tograde 3 murmur...
Congenital Heart Disease-Congenital Heart Disease-StructuralStructuralPINKShunts ( L to R) : ASD VSD PDAStenosis: AS...
Normal Cardiac PressuresNormal Cardiac Pressures120/<825/<5<5<8120/8025/15
ATRIAL SEPTAL DEFECTATRIAL SEPTAL DEFECT<5<8
ATRIAL SEPTAL DEFECTSATRIAL SEPTAL DEFECTS(ASD)(ASD)Three types exist : primum,secundum and sinus venosusThe most common...
ASD- cont...ASD- cont...Clinical signs include a 2-3/6 SEM atthe ULSB and a fixed wide split S2A large ASD causes right ...
ASD - cont...ASD - cont...ECHO: DiagnosticNatural History: Arrhythmias andpulmonary obstructive vasculardisease in the 3...
VENTRICULAR SEPTALVENTRICULAR SEPTALDEFECTDEFECT120/<825/<5
VENTRICULAR SEPTALVENTRICULAR SEPTALDEFECTS ( VSD)DEFECTS ( VSD)This is the most common form of CHDThe VSDs are subdivid...
VSD - cont…..VSD - cont…..With a large defect there may beCHF( usually at 6-8 weeks),pulmonary infections and delayedgrow...
VSD - cont…..VSD - cont…..EKG: LVH or BVHECHO: Diagnostic
VSD -cont...VSD -cont...Natural history : Small VSDs closespontaneously depending on the site.Unrepaired the large defec...
VSD - cont…..VSD - cont…..Large VSDs are closed surgicallyusually at about 4- 6 months of age.Diuretics,digoxin and afte...
ENDOCARDIAL CUSHIONENDOCARDIAL CUSHIONDEFECTSDEFECTS
AVSD - cont...AVSD - cont...1/3rdof babies with this have DownsyndromeEKG : Characteristic with asuperior left axis.Ech...
PATENT DUCTUSPATENT DUCTUSARTERIOSUSARTERIOSUS25/15120/80
PATENT DUCTUSPATENT DUCTUSARTERIOSUS ( PDA)ARTERIOSUS ( PDA)It is a connection between the aortaand the pulmonary artery....
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 pr...
PDAPDASigns: Systolic murmur in anewborn and a continuous “train ina tunnel” murmur in an older child.Best heard below th...
PDA - cont...PDA - cont...In a preterm it can be closedmedically using indomethacin.In a term baby if still open at 3mon...
1. A 3 month old girl who has Down syndromeexhibits poor weight gain, tachypnea and a lowpitched grade 2 murmur. Chest rad...
2. A 5 day old infant born at 31 weeks gestation is onventilatory support. He has a grade 2 holosystolic murmurthat extend...
3. A term infant is born with a largeventricular septal defect. At what age isthis infant MOST likely to firstdemonstrate ...
4. A term newborn has tachypnea, rales,tachycardia, audible gallop and diminishedarm and leg pulses. Echocardiography show...
COARCTATION OF THECOARCTATION OF THEAORTAAORTA
Coarctation of the AortaCoarctation of the Aorta(CoA)(CoA)More common in malesAlmost always juxtaductal85% of children ...
CoA - cont….CoA - cont….Symptoms and Signs:SEVERE : ShockMODERATE : CHF,MILD : Headaches, leg claudicationDecreased femo...
CoA - cont….CoA - cont….ECHO : DiagnosticTreatment: For an infant in shock-PGE1 immediately.Surgical vs. transcatheter ...
Hypoplastic Left HeartSyndrome Varying degrees of left heart hypoplasia atmultiple levels Babies present in cardiogenic ...
Hypoplastic Left HeartHypoplastic Left Heartsyndromesyndrome
PULMONIC STENOSISPULMONIC STENOSIS
PULMONIC STENOSIS ( PS)PULMONIC STENOSIS ( PS)This may be at the valve, subvalvaror supravalvar.Symptoms: None in mild o...
PS - cont...PS - cont...Treatment : Balloon valvotomy if the RVpressure is over 50 mmHG
AORTIC STENOSISAORTIC STENOSIS
AORTIC STENOSIS ( AS)AORTIC STENOSIS ( AS)Stenosis possible at the valve,subvalvar or supravalvar.This is a more signifi...
ASAS A type of subvalvar AS is also calledHCM which is the commonest causeof sudden death in childrenSymptoms:Mild : Non...
HYPERTROPHICHYPERTROPHICCARDIOMYOPATHYCARDIOMYOPATHY
1. A 3 day old girl is found unconscious in her crib andis brought to the ED. Findings include: tachypnea,tachycardia, pal...
2. A 6 hour-old infant has increasing pallor,tachypnea and respiratory distress. Physicalexamination reveals an enlarged l...
BLUE LESIONSBLUE LESIONS
There has to be a RIGHT toThere has to be a RIGHT toLEFT shunt to causeLEFT shunt to causecyanosiscyanosis
Tetralogy of FallotTetralogy of FallotMost common cyanotic heart disease.The four abnormalities include:– Pulmonary sten...
TOF contTOF cont....A “tet” spell consists of rapid breathing andincreased cyanosis. Any event like crying orincreased ph...
TRANSPOSITION OF THETRANSPOSITION OF THEGREAT ARTERIESGREAT ARTERIES
Transposition of the greatTransposition of the greatArteriesArteries The aorta arises from the right ventricle and thepul...
1. The mother of a 5 month old girl reports thatfollowing a feeding, the child began to breathedeeply, became increasingly...
2. You are called at 3 AM from the nursery where 36 hour oldBB Bleu is noticed to be cyanotic. The nurses report that heha...
3. Following an uncomplicated delivery, a 3.7 kg term infantdevelops cyanosis in the first hour of life. Findings at 3 hou...
Congestive Cardiac FailureCongestive Cardiac Failure Tachycardia Tachypnea Hepatomegaly Cardiomegaly, murmur, HR too f...
1.In addition to irritability,sweating anddifficulty breathing with feeding, thesymptom that is MOST indicative ofcongesti...
Rhythm AbnormalitiesRhythm AbnormalitiesEctopic beats: premature atrial ,ventricular– Benign if they disappear with exerc...
1. An 8 year old previously healthy boypresents for a school physical. He is activeand has no symptoms. On exam. He appea...
Most common cause of irregular rhythm inMost common cause of irregular rhythm inchildren – SINUS ARRHYTHMIA – BENIGN!!!chi...
Irregular rhythm in a newborn baby- PrematureIrregular rhythm in a newborn baby- Prematureatrial contractions – BENIGN!!!a...
Irregular rhythm incidentally noted in anIrregular rhythm incidentally noted in anadolescent- Ventricular Premature beats ...
SVTSVTRate above 230/min .Tachycardia – most likely SVT– Narrow complex tachycardia– WPW is the most common cause of ree...
Treatment of SVTTreatment of SVT Hemodynamically stable:– Vagal maneuvers– Adenosine– Verapamil in children over 1 year ...
Radiofrequency CatheterRadiofrequency CatheterAblationAblationUpdated mortality for pediatric RF:10/4651 patients:Increase...
1. A 1 year old child is brought to the ER because hisparents thought his heart was pounding as they wereputting him to be...
2. A 4 week old infant appears in your ED with ahistory of irritability, increased respiratory rateand poor feeding. On ph...
Atrial Flutter/ FibrillationAtrial Flutter/ Fibrillation Seen in two groups– Newborns: After t/tBENIGN!!– After extensive...
AV BLOCKAV BLOCKFirst Degree – Prolonged PR interval– Rheumatic fever, ASD, PDASecond Degree– Type I: Varying PR interva...
Second Degree AV Block –Second Degree AV Block –Type I and IIType I and II
Third degree AV BlockThird degree AV Block
4. SYNCOPE4. SYNCOPE Brief loss of consciousness with rapid recovery Seen in adolescents and in toddlers 20-50% of adol...
BENIGN SYNCOPEBENIGN SYNCOPE Vasovagal Orthostatic hypotension Hyperventilation Breath holding spells
DANGER SYMPTOMSDANGER SYMPTOMSSyncope especially withEXERTION or EXCITEMENT-anger, fear, startleCardiac arrest with exer...
Sudden Death in YoungSudden Death in YoungAthletesAthletes3619455333226HCMCoronaryAnomaliesASRuptured AoTunnelled LADMyoca...
Commotio CordisCommotio CordisYoung childrenBaseball, football, ice hockeyForce of blow is not unusually hardR on T ph...
Long QT SyndromeLong QT Syndrome¶ Disorder of the electrical activity of the heart¶ Involves repolarization¶ Characterized...
QT IntervalQT Interval
Torsade de PointesTorsade de Pointes
Special situations where theSpecial situations where theQT should ALWAYS beQT should ALWAYS bemeasuredmeasuredSyncopeSei...
1. A 5 year old girl is very excited following a ride onthe ferris wheel. In the midst of her excitement shesuddenly loses...
2. A 12 year old boy underwent repair for tetralogy ofFallot at 9 months of age. Last month, routine followup echocardiogr...
3. Julie, an otherwise healthy 9 year old comes tothe ED because she “passed out”. After askingquestions and examining the...
4. A 14 year old girl falls during a race. She isunconscious, cyanotic and has no pulse butspontaneously revives within se...
5.A 13 year old boy wishes to participate incompetitive sports. His father died suddenlyat age 28 years, and hypertrophicc...
SYNDROMESSYNDROMES Down’s: Incidence 50% . AV canal defects. Turner’s : 10%. Coarctation , bicuspid aortic valve Willia...
Congenital Heart Disease-Etiology-Congenital Heart Disease-Etiology-Environmental Factors/ToxinsEnvironmental Factors/Toxi...
PERICARDITISPERICARDITISFollows a viral URISharp chest pain, retrosternal, difficultyin deep inspirationPt. Resists lyi...
PericarditisPericarditis
PERICARDITIS- EKGPERICARDITIS- EKG
TREATMENTTREATMENTReassuranceNSAIDSOccasional pericardial tap , windowPostpericardiotomy Syndrome: 2 weeksafter surgery
Infective EndocarditisInfective EndocarditisThe endocardium is a deterrant to adhesionby platelets and organisms.The den...
IE- Lab. TestsIE- Lab. TestsBLOOD CULTURESEcho
Prevention of Infective EndocarditisPrevention of Infective EndocarditisGuidelines From the American Heart AssociationGuid...
ConclusionsConclusions(1) Only an extremely small number of cases of infectiveendocarditis might be prevented by antibiot...
 (3) Administration of antibiotics solely toprevent endocarditis is not recommendedfor patients who undergo a genitourina...
 Prosthetic cardiac valves or prosthetic material used for cardiac valve repair Previous IE Congenital heart disease (C...
Myocarditis- EtiologyMyocarditis- Etiology
Myocarditis- signs andMyocarditis- signs andsymptomssymptoms DILATED heartSinusTACHYCARDIACHF InflamedMyocardium andcond...
PERIPERI MYOMYO ENDOENDOHeart Normal Normal PathologyCause Viral Viral BacterialSymptom Chest pain C.pain,irr.beats FeverS...
Jones’ Modified CriteriaJones’ Modified Criteria
Rheumatic CarditisRheumatic CarditisPresent in 50% cases“Sleeping” tachycardia is an early signMitral and aortic valves...
RF-Treatment andRF-Treatment andPreventionPreventionBenzathine penicillin 1.2 mega units IMAspirin 75-100 mg/kg for 6-8 ...
1. Two weeks after a nonspecific upperrespiratory infection, a previously healthy , 3year-old boy is noted to have a resp....
2. A 13 year old boy who has a bicuspid aortic valveand who received treatment for dental caries about3 weeks ago now comp...
3. A 14 year old boy complains of dull chest pain overthe precordium. It began 4 days ago and occursintermittently. It is ...
4.Of the following, the procedure that doesnot require antibiotic prophylaxis in achild who has a congenital heart lesion ...
5. An 8 year old girl’s parents complain that shehas been hyperactive and somewhat labile for2 weeks. She has jerky sudden...
KAWASAKI DISEASE Fever of 5 days duration, enlargement oflymph nodes, mucositis, non purulentconjunctivitis, rash Thromb...
1. A 9 week old infant has had irritability and fever to104 F for 8 days. Cultures of blood,urine andcerebrospinal fluid a...
Some Tips…Some Tips…1. Read the question carefully. Is it MOSTlikely or LEAST likely.2. Look for the “code words”.3. Re...
GOOD LUCK !!!!!!!GOOD LUCK !!!!!!!
Congenital heart-disease2787
Congenital heart-disease2787
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Congenital heart-disease2787
Congenital heart-disease2787
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Congenital heart-disease2787
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Congenital heart-disease2787

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Congenital heart-disease2787

  1. 1. Pediatric Board ReviewPediatric Board Review20082008Pediatric CardiologyPediatric CardiologyPrema Ramaswamy, M.D.Co-Director, Pediatric Cardiology,Maimonides Infants and ChildrensHospital of Brooklyn
  2. 2. PEDIATRIC CARDIOLOGYPEDIATRIC CARDIOLOGYInnocent MurmursCongenital structural heart diseaseRhythm problems , syncope etc.Peri, myo , endocarditis, Rheumatic feverSyndromesKawasaki Disease
  3. 3. Innocent MurmursDiastolic murmurs are never innocentInnocent 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.
  4. 4. 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
  5. 5. Congenital Heart Disease-Congenital Heart Disease-StructuralStructuralPINKShunts ( L to R) : ASD VSD PDAStenosis: AS PS Coarctation HLHSBLUE TOF TGA Tricuspid atresia Truncus TAPVR Ebstein’s Single ventricle
  6. 6. Normal Cardiac PressuresNormal Cardiac Pressures120/<825/<5<5<8120/8025/15
  7. 7. ATRIAL SEPTAL DEFECTATRIAL SEPTAL DEFECT<5<8
  8. 8. ATRIAL SEPTAL DEFECTSATRIAL SEPTAL DEFECTS(ASD)(ASD)Three types exist : primum,secundum and sinus venosusThe most common is the secundumtypeSymptoms: None in childhood,arrhythmias in the 3 rd decade
  9. 9. ASD- cont...ASD- cont...Clinical signs include a 2-3/6 SEM atthe ULSB and a fixed wide split S2A large ASD causes right ventricularenlargementEKG: RAD and IRBBB
  10. 10. ASD - cont...ASD - cont...ECHO: DiagnosticNatural History: Arrhythmias andpulmonary obstructive vasculardisease in the 3rd and 4th decade.IE Prophylaxis ??Treatment : Surgical vs.transcatheter closure
  11. 11. VENTRICULAR SEPTALVENTRICULAR SEPTALDEFECTDEFECT120/<825/<5
  12. 12. VENTRICULAR SEPTALVENTRICULAR SEPTALDEFECTS ( VSD)DEFECTS ( VSD)This is the most common form of CHDThe VSDs are subdivided according to thepart of the septum they occur in :Muscular, perimembranous, inlet, outletA large VSD causes left ventricularenlargementWith a small VSD there is normal growthand development
  13. 13. VSD - cont…..VSD - cont…..With a large defect there may beCHF( usually at 6-8 weeks),pulmonary infections and delayedgrowthClinical signs : Loud 4-5/6 , harshholosystolic murmur, middiastolicrumble and a loud P2
  14. 14. VSD - cont…..VSD - cont…..EKG: LVH or BVHECHO: Diagnostic
  15. 15. VSD -cont...VSD -cont...Natural history : Small VSDs closespontaneously depending on the site.Unrepaired the large defects maylead to Eisenmenger’s syndrome.
  16. 16. 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.
  17. 17. ENDOCARDIAL CUSHIONENDOCARDIAL CUSHIONDEFECTSDEFECTS
  18. 18. AVSD - cont...AVSD - cont...1/3rdof babies with this have DownsyndromeEKG : Characteristic with asuperior left axis.Echo : ConfirmatoryManagement : Anticongestivemedications and surgery at 4-8months of age.
  19. 19. PATENT DUCTUSPATENT DUCTUSARTERIOSUSARTERIOSUS25/15120/80
  20. 20. 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.
  21. 21. 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.
  22. 22. PDAPDASigns: 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.
  23. 23. 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.
  24. 24. 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 aortaB. complete atrioventricular septal defectC. patent ductus arteriosusD. Perimembranous VSDE. secundum ASD
  25. 25. 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.
  26. 26. 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 daysB. 2 weeksC. 2 monthsD. 6 monthsE. 12 months
  27. 27. 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 deficiencyB. hyperthyroidismC. hypoglycemiaD. intracranial arteriovenous malformationE. pheochromocytoma
  28. 28. COARCTATION OF THECOARCTATION OF THEAORTAAORTA
  29. 29. Coarctation of the AortaCoarctation of the Aorta(CoA)(CoA)More common in malesAlmost always juxtaductal85% of children with CoA have abicuspid aortic valve.
  30. 30. CoA - cont….CoA - cont….Symptoms and Signs:SEVERE : ShockMODERATE : CHF,MILD : Headaches, leg claudicationDecreased femoral pulses are animportant sign esp. in neonates.BP lower in the lower limbs
  31. 31. CoA - cont….CoA - cont….ECHO : DiagnosticTreatment: For an infant in shock-PGE1 immediately.Surgical vs. transcatheter repair.
  32. 32. 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.
  33. 33. Hypoplastic Left HeartHypoplastic Left Heartsyndromesyndrome
  34. 34. PULMONIC STENOSISPULMONIC STENOSIS
  35. 35. 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
  36. 36. PS - cont...PS - cont...Treatment : Balloon valvotomy if the RVpressure is over 50 mmHG
  37. 37. AORTIC STENOSISAORTIC STENOSIS
  38. 38. 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.
  39. 39. ASAS A type of subvalvar AS is also calledHCM which is the commonest causeof sudden death in childrenSymptoms:Mild : NoneModerate to severe: Chest pain,fatigability, syncope.
  40. 40. HYPERTROPHICHYPERTROPHICCARDIOMYOPATHYCARDIOMYOPATHY
  41. 41. 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
  42. 42. 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.
  43. 43. BLUE LESIONSBLUE LESIONS
  44. 44. There has to be a RIGHT toThere has to be a RIGHT toLEFT shunt to causeLEFT shunt to causecyanosiscyanosis
  45. 45. Tetralogy of FallotTetralogy of FallotMost common cyanotic heart disease.The four abnormalities include:– Pulmonary stenosis– RVH– VSD– Overriding AortaSigns include cyanosis, murmur,squatting and spells.
  46. 46. 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,
  47. 47. TRANSPOSITION OF THETRANSPOSITION OF THEGREAT ARTERIESGREAT ARTERIES
  48. 48. 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.
  49. 49. 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 stenosisB. coarctation of the aortaC. myocarditisD. tetralogy of FallotE. ventricular septal defect
  50. 50. 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.
  51. 51. 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
  52. 52. 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
  53. 53. 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. ascitisB. coughC. cyanosisD. diminished feeding volumeE. pretibial edema
  54. 54. Rhythm AbnormalitiesRhythm AbnormalitiesEctopic beats: premature atrial ,ventricular– Benign if they disappear with exercise– Seen in the neonatal and adolescent age groupsAtrial Flutter,fibSVTVT– Electrolyte Imbalances– TOF– HCM, Long QT syndromeAV block
  55. 55. 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 :
  56. 56. Most common cause of irregular rhythm inMost common cause of irregular rhythm inchildren – SINUS ARRHYTHMIA – BENIGN!!!children – SINUS ARRHYTHMIA – BENIGN!!!
  57. 57. Irregular rhythm in a newborn baby- PrematureIrregular rhythm in a newborn baby- Prematureatrial contractions – BENIGN!!!atrial contractions – BENIGN!!!
  58. 58. 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!!!
  59. 59. SVTSVTRate above 230/min .Tachycardia – most likely SVT– Narrow complex tachycardia– WPW is the most common cause of reentrytachycardia in children
  60. 60. 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
  61. 61. 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
  62. 62. 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
  63. 63. 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 EKGB. give verapamil 0.1 mg/kg push slowlyC. give adenosine 100 mcg/kg rapid pushD. fill a bag with ice and apply to infants faceE. pass an esophageal probe and pace theheart 20 bpm faster than the tachycardia
  64. 64. 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
  65. 65. AV BLOCKAV BLOCKFirst Degree – Prolonged PR interval– Rheumatic fever, ASD, PDASecond Degree– Type I: Varying PR intervals and dropped beat,Wenkebach– Type II: 2 or more than 2 :1 blockThird Degree:– Surgical, Lyme Disease– Mom with SLE
  66. 66. Second Degree AV Block –Second Degree AV Block –Type I and IIType I and II
  67. 67. Third degree AV BlockThird degree AV Block
  68. 68. 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
  69. 69. BENIGN SYNCOPEBENIGN SYNCOPE Vasovagal Orthostatic hypotension Hyperventilation Breath holding spells
  70. 70. DANGER SYMPTOMSDANGER SYMPTOMSSyncope especially withEXERTION or EXCITEMENT-anger, fear, startleCardiac arrest with exercise orexcitement
  71. 71. Sudden Death in YoungSudden Death in YoungAthletesAthletes3619455333226HCMCoronaryAnomaliesASRuptured AoTunnelled LADMyocarditisDilated CMARVDMVP
  72. 72. Commotio CordisCommotio CordisYoung childrenBaseball, football, ice hockeyForce of blow is not unusually hardR on T phenomenonPrevention : ? softer balls, ? protectiveclothing,Role of automated External defibrillator
  73. 73. 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
  74. 74. QT IntervalQT Interval
  75. 75. Torsade de PointesTorsade de Pointes
  76. 76. Special situations where theSpecial situations where theQT should ALWAYS beQT should ALWAYS bemeasuredmeasuredSyncopeSeizurescongenital Deafnessnear SIDS
  77. 77. 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
  78. 78. 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
  79. 79. 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
  80. 80. 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
  81. 81. 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. echocardiographyB. electrocardiographyC. exercise myocardial perfusion scintigraphyD. Genetic testing for myosin chain mutationsE. Genetic testing for troponin mutations
  82. 82. SYNDROMESSYNDROMES Down’s: Incidence 50% . AV canal defects. Turner’s : 10%. Coarctation , bicuspid aortic valve Williams’s : Supravalvar aortic stenosis, PPS Alagille : Peripheral pulmonic stenosis (PPS) Noonan : PPS and HCM Marfan’s : Aortic root dilatation, MVP DiGeorge: Truncus Arteriosus, Interrupted aortic arch. Catch 22 : conotruncal abn. such as VSD,TOF,collaterals, right aortic arch Kartagener : Dextrocardia, situs inversus, immotile cilia Holt-Oram: Limb abnormalities with ASD Ellis-van Creveld: ASD Pompe’s D: Hypertrophic cardiomyopathy
  83. 83. Congenital Heart Disease-Etiology-Congenital Heart Disease-Etiology-Environmental Factors/ToxinsEnvironmental Factors/Toxins Lithium: Ebstein’s anomaly Ethanol: ASD,VSD ( Fetal Alcohol Syndrome) Anticonvulsants: PS, AS, TOF Retinoic Acid: Transposition Rubella: PDA, PPS Coxsachie B : Neonatal myocarditis Maternal Diabetes: HCM, TGA Maternal Lupus: Complete heart block PKU: VSD, ASD, complex CHD
  84. 84. PERICARDITISPERICARDITISFollows a viral URISharp chest pain, retrosternal, difficultyin deep inspirationPt. Resists lying downPain worsened by pressure over thesternumFriction rub, pulsus paradoxusEKG is diagnostic
  85. 85. PericarditisPericarditis
  86. 86. PERICARDITIS- EKGPERICARDITIS- EKG
  87. 87. TREATMENTTREATMENTReassuranceNSAIDSOccasional pericardial tap , windowPostpericardiotomy Syndrome: 2 weeksafter surgery
  88. 88. Infective EndocarditisInfective EndocarditisThe 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
  89. 89. IE- Lab. TestsIE- Lab. TestsBLOOD CULTURESEcho
  90. 90. 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;
  91. 91. 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.
  92. 92.  (3) Administration of antibiotics solely toprevent endocarditis is not recommendedfor patients who undergo a genitourinary orgastrointestinal tract procedure.ConclusionsConclusions
  93. 93.  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
  94. 94. Myocarditis- EtiologyMyocarditis- Etiology
  95. 95. Myocarditis- signs andMyocarditis- signs andsymptomssymptoms DILATED heartSinusTACHYCARDIACHF InflamedMyocardium andconduction systemArrhythmias
  96. 96. PERIPERI MYOMYO ENDOENDOHeart Normal Normal PathologyCause Viral Viral BacterialSymptom Chest pain C.pain,irr.beats FeverSigns Rub Tachycardia FeverTest EKG,echo CXR, echo B. CultureTreatment NSAIDS ?IVIG AntibioticsCourse Benign Can be fatal insidious
  97. 97. Jones’ Modified CriteriaJones’ Modified Criteria
  98. 98. Rheumatic CarditisRheumatic CarditisPresent in 50% cases“Sleeping” tachycardia is an early signMitral and aortic valves most commonlyinvolvedRheumatic ArthritisRheumatic ArthritisMost common manifestationPain, swelling and erythemaResolves within 1 week
  99. 99. RF-Treatment andRF-Treatment andPreventionPreventionBenzathine penicillin 1.2 mega units IMAspirin 75-100 mg/kg for 6-8 weeksSteroids for severe carditisDigoxin , diureticsPrevention with BP q 4 weeks.
  100. 100. 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 feverB. infective endocarditisC. myocarditisD. paroxysmal atrial tachycardiaE. pericarditis
  101. 101. 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
  102. 102. 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
  103. 103. 4.Of the following, the procedure that doesnot require antibiotic prophylaxis in achild who has a congenital heart lesion is:A. cystoscopyB. dental treatment for cariesC. myringotomy with tube placementD. rigid bronchoscopyE. sigmoidoscopy
  104. 104. 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 scleraeB. mitral regurgitation murmurC. Osler nodesD. severe hypertensionE. splenomegaly
  105. 105. 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.
  106. 106. 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 thrombosisB. cerebral infarctionC. coronary artery aneurysmsD. renal vein thrombosisE. splenic infarction
  107. 107. 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.
  108. 108. GOOD LUCK !!!!!!!GOOD LUCK !!!!!!!

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