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Congenital Heart Defects

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... cardiomegally and increased pulm vascularity 50% R aortic arch Echo: ... can see narrowing Bicuspid aortic valve ... PA from LV associated anomalies: ... – PowerPoint PPT presentation

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Title: Congenital Heart Defects


1
Congenital Heart Defects
  • Craig T. Carter, D.O.
  • Assistant Professor EM/Peds
  • University of Kentucky

2
  • A nurse approaches you -
  • Doctor, Doctor - a kid just checked in with a
    history of Hypoplastic Left Heart syndrome What
    is that again??
  • You reply ..

3
  • I need to go to the bathroom, as soon as I get
    back, I will tell you all about it....

4
So you sit and think..
5
And think some more.
6
Back to Basics-Fetal Circulation
  • Four shunts of blood flow placenta, ductus
    venosus, foramen ovale and ductus arteriosus

7
Back to Basics-Fetal Circulation
8
Changes after birth
  • Shift of blood flow for gas exchange, from the
    placenta to the lungs.
  • Closure of ductus venosus no placenta
  • increase in pulmonary blood flow
  • functional closure of foramen ovale (increased LA
    pressure)
  • Closure of ductus arteriosus (O2)

9
Newborn Cardiac Exam
  • Vital signs RR, HR, BP
  • respiratory effort
  • color
  • palpate point of maximal impulse (PMI) of heart
  • palpate pulses of all extremities
  • auscultate

10
Case 1 2 day old infant
  • 2 day old baby girl is being examined for
    discharge physical.
  • Vitals HR120 RR40 BP r arm 75/40
  • Gen low hairline, webbed neck, edemetous dorsum
    of feet

11
Case 1 cardiac exam
  • Normal PMI, no thrill
  • No hepatosplenomegally
  • unable to palpate pulses in lower extremities
  • BPs all four extremities both legs systolic BP
    lower 40/30

12
Case 1 Auscultation
  • S2 splits normally
  • ejection click
  • systolic ejection murmur III/VI at the URSB and
    LLSB
  • systolic murmur radiates to the back
  • early diastolic decrescendo murmur at 3LICS

13
Case 1
  • What do you think is the congenital defect?

14
Case 1
  • What further evaluation do you need?

15
Coarctation of Aorta (COA)
16
Coarctation of Aorta (COA)
  • Incidence 8 of all congenital heart defects
  • Turners syndrome 30 have COA
  • Preductal 40 associated with other cardiac
    defects,symptomatic early in life
  • Postductal less likely to have symptoms early

17
COA
  • EKG LVH (but may be normal in 20)
  • X-rays heart size may be normal or slightly
    enlarged
  • Rib notching in older children
  • Echo can see narrowing
  • Bicuspid aortic valve
  • Dopplerdisturbed flow

18
COA complications
  • COA can cause CHF, HTN (intracranial
    bleeding,hypertensive encephalopathy)
  • Bicuspid aortic valve stenosis or regurg
  • LV failure
  • infective endocarditis

19
Preductal COA in Newborn
  • 80 of infants with preductal COA develop CHF by
    3 months of age!
  • Symptoms of CHF poor feeding, poor weight gain,
    dyspnea in first 2-6 weeks of life.

20
Case 2 Two week old with murmur
  • Two week old infant, who you saw as a newborn
    with normal exam, now is noted to have a heart
    murmur.
  • PMI LSB, not hyperdynamic
  • pulses equal all extremities
  • No HSM

21
Case 2 Cardiac exam
  • Grade III/VI holosystolic murmur at LLSB

22
Case 2 What would you like to do next?
  • EKG LVH,LAH
  • Blood pressures all four extremities
  • Xray look for cardiomegally and increase in
    pulmonary vascularity
  • oxygenation
  • Echo VSD

23
Ventricular Septal Defect (VSD)
  • The most common form of congenital heart disease
    20-25
  • may be located in different anatomical locations
  • may be associated with many other cardiac defects
    ( in many cases essential for survival)
  • may be small or large (can cause CHF)

24
Case 3 One day old infant
  • One day old infant with tachypnea and cyanosis
  • Gen cyanotic
  • RR65, HR 140, BP 40/20
  • pulse ox on RA less than 80

25
Case 3 Respiratory and Cardiac
  • Lungs clear, no retractions, RR rapid
  • Cardiac PMI at LSB
  • pulses palpable all extremities
  • S2 single and loud. No heart murmur.

26
What would you like to do?
  • ABG before oxygenation
  • Give 100 O2 and then repeat ABG
  • EKG, 4 extremity BP
  • CXR

27
Results of ABG
  • Before O2 PH 7.15,PaCO2 30 and Pa O2 40
  • After 100 O2 PH 7.12, PaCO2 25 and PaO2 50

28
Transposition of Great Arteries
29
Transposition of Great Arteries
  • 5 of all congenital heart defects
  • Aorta arises anteriorly from RV, PA arises
    posteriorly from LV
  • defects (VSD,ASD,PDA) that permit mixing of the
    two circulations are necessary for survival

30
TGA
  • ABGhypoxemia is unresponsive to O2
  • EKG RVH
  • X-rays egg-on-a-string silhouette
  • cardiomegally with increased pulmonary
    vascularity
  • Echo PA from LV
  • associated anomaliesVSD,ASD,PDA

31
Immediate Treatment
  • Prostaglandin E1 to reopen PDA
  • Oxygen
  • cardiology/surgery referral

32
DDX of Cyanotic Heart Dz
  • Transposition of the Great Arteries
  • Tetralogy of Fallot
  • Total Anomalous Pulmonary Venous Return
  • Tricuspid Atresia
  • Pulmonary Atresia
  • Truncus Arteriosus

33
Other congenital cyanotic defects
  • Ebsteins anomaly
  • single ventricle
  • Double-outlet right ventricle (depends on
    associated defectsif cyanotic or not)

34
Tetralogy of Fallot
35
Tetralogy of Fallot (TOF)
  • Large VSD
  • RV outflow obstruction
  • right ventricular hypertrophy
  • overriding of the aorta

36
Tetralogy of Fallot
  • 10 of all congenital heart defects
  • The MOST COMMON CYANOTIC cardiac defect beyond
    infancy

37
TOF physical exam
  • Varying degrees of cyanosis and clubbing
  • systolic thrill LSB
  • S2 single with ejection click
  • loud III-V/VI SEM LSB
  • continuous murmur of PDA

38
TOF
  • EKG RAD, RVH
  • Xray boot-shaped heart (hypoplastic MPA)
  • Echo image of four defects associated

39
Tetralogy of Fallot
40
TOF complications
  • Hypoxic spells
  • growth retardation with severe cyanosis
  • brain abscess and CVA
  • infective endocarditis
  • polycythemia

41
Hypoxic Spell ( TET Spell
  • Paroxysm of hyperpnea (rapid and deep)
  • irritability and prolonged cry
  • increasing cyanosis
  • decreased intensity of heart murmur
  • (may lead to limpness, convulsion,CVA or death)

42
Treatment of TET Spell
  • Knee-chest (squat )position
  • morphine sulfate
  • treat acidosis
  • oxygenation

43
Total Anomalous Pulmonary Venous Return
  • One percent of all congenital heart defects
  • Defect no direct communication between the
    pulmonary veins and the left atrium (they can
    drain supracardiac,cardiac,infracardiac or mixed)

44
Total Anomalous Pulmonary Venous Return
45
TAPVR findings
  • S2 widely split and fixed
  • S3 or S4 gallop
  • SEM III-IV/VI
  • middiastolic rumble at LLSB
  • Xrays cardiomegally
  • Snowman
  • Echo can define anatomy
  • EKGRAD

46
Tricuspid Atresia
  • 1-2 of all congenital heart disease in infancy
  • tricuspid valve is absent and RV is hypoplastic
  • associated defects of VSD,ASD or PDA are
    necessary for survival

47
Tricuspid Atresia
48
Tricuspid Atresia findings
  • Exam cyanosis
  • S2 single, often syst murmur of VSD, and occ of
    PDA present
  • early CHF

49
Tricuspid Atresia
  • EKG superior QRS between O and -90
  • LVH
  • Pulmonary vascularity is decreased
  • Echo defines minimal RV, and large LV

50
Pulmonary Atresia
  • Less than 1 of congenital heart diseases
  • valve is atretic, RV cavity is hypoplastic
  • need other defects ASD,PDA for survival

51
Pulmonary Atresia
52
Pulmonary Atresia findings
  • PE S2 is single
  • murmur of PDA
  • EKG normal axis,LVH
  • Xray decreased pulmonary vascularity
  • Echoatretic pulmonary valve and hypoplastic RV

53
Pulmonary Atresia
  • Prostaglandin E1
  • cardiac surgery

54
Truncus Arteriosus
  • Less than 1 of all congenital heart Dz
  • Only a single arterial trunk leaves the heart and
    gives rise to the pulmonary, systemic and
    coronary circulations
  • large VSD is always present

55
Truncus Arteriosus
  • PE cyanosis
  • wide pulse pressure and bounding pulses
  • harsh VSD murmur LSB
  • EKGnormal axis,LAH
  • Xrays cardiomegally and increased pulm
    vascularity
  • 50 R aortic arch
  • Echosingle great artery,VSD

56
Hypoplastic Left Heart
57
Hypoplastic Left Heart
  • Hypoplastic left heart syndrome refers to
    underdevelopment of the left side of the heart.
  • This syndrome may include
  • Small aorta This is the major blood vessel from
    the left ventricle to the body.

58
Hypoplastic Left Heart
  • May Include
  • Aortic valve atresia (absence) This valve
    normally opens and closes to let blood flow from
    the left ventricle to the aorta. When atresia is
    present, there is no connection between the left
    ventricle and aorta, and no forward blood flow.
  • Mitral valve stenosis or atresia This valve
    normally opens and closes to let blood flow
    between the left atrium and left ventricle.
    Stenosis causes little blood flow atresia causes
    no blood flow. Either atresia or stenosis may be
    present.
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