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Evaluation for Congenital Heart Diseases

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Title: Evaluation for Congenital Heart Diseases


1
Evaluation for Congenital Heart Diseases
  • Seoul National University Hospital
  • Department of Thoracic Cardiovascular Surgery

2
Heart Diseases in Children
  • Congenital heart diseases
  • Rheumatic heart disease Rheumatic fever
  • Other acquired diseases Kawasaki
  • Cardiomyopathy
  • Arrhythmia

3
Effects of CHD
  • No effect on a child
  • Decreased function when stressed
  • Decreased cardiopulmonary function
  • Other organ/system manifestation

4
Presentation of CHD
  • Shock like symptoms
  • Cyanosis
  • Congestive symptoms
  • Exercise intolerance
  • Asymptomatic heart murmur
  • Abnormality in routine chest PA
  • Chest pain
  • Syncope/ seizure/ fainting
  • Airway obstruction/ dysphagia

5
Shock like Symptoms
  • Non-specific collapse, pallor, cold clammy skin,
    hypotension, oligouria, acidosis
  • HLHS, critical AS, IAA, COA? unable to maintain
    systemic output during transitional circulation
  • Most common in neonate / infancy
  • Very important to recognize

6
Cyanosis
  • Cyanosis more than 5.0g of reduced Hb.
  • Central versus Peripheral cyanosis
  • Central cyanosis CNS, heart, lung
  • Pathophysiology TGA, TOF, CML, DDPC

7
Congestion
  • Symptoms dyspnea on feeding, sweating, poor
    weight gain, frequent respiratory infection
  • Most common mode in infancy
  • Obligatory shunt lesions/ Left to right shunt
    lesions/ CML with no PS/ Myocarditis/ Arrhythmia

8
Asymptomatic Murmur
  • Heart murmur noted in routine examination
  • ASD, VSD, PS, AS, Bicuspid AV
  • Common mode in childhood
  • Murmur does not mean there is a CHD
  • No murmur does not mean there is no CHD

9
Abnormality in Chest PA
Corrected TGA/ Pericardial Defect/ Cardiac Tumor
10
Chest Pain
Anomalous origin of left coronary artery from
pulmonary artery Aortic stenosis(severe) /
Hypertrophic cardiomyopathy
11
Syncope/ Seizure/ Fainting
  • Anoxic spell of acyanotic TOF
  • Arrhythmia VT, SVT, long QT syndrome
  • Syncope on exercise AS, SAS, IHSS

12
Airway Obstruction/ Dysphagia
13
Airway Obstruction / Dysphagia
Abnormal PA Branching
14
Mode of / Age at Presentation
  • Collapse within 3-4 days
  • Cyanosis usually within a month
  • Congestion 1-5 months
  • Murmur any time

15
Diagnosis of Cardiac Diseases
  • Anatomic Diagnosis
  • Hemodynamic Evaluation
  • Total assessment / Prognosis
  • Etiologic Diagnosis

16
Diagnostic Tools
  • History and physical examination
  • Chest X ray / EKG / Blood study
  • Echocardiography/ Other imaging tools
  • Catheterization/ Other invasive tools

17
Accuracy of Tools
  • History/PE important, rarely specific
  • X-ray/EKG not-confirmative
  • Echocardiography
  • confirmative, but non-invasive
  • Cardiac catheterization
  • confirmative, but invasive

18
History Taking
  • Cyanosis onset, progression, anoxic spell
  • Congestion feeding, wt. gain, respiratory
    infection, sweating, tachy-dyspnea
  • Exercise tolerance easy fatigability, DOE
  • Possible Causes maternal diseases, drug,
    infection, family history

19
Physical Examination
  • Inspection general appearance, nutrition,
    syndrome?, facial morphology, jugular venous
    pulse, respiratory pattern, rate, chest
    retraction, alae nasi flaring, dyspnea,
    precordial bulging, cyanosis, clubbing
  • Palpation apical pulse, precordial activity,
    thrill, arterial pulse, location and size of
    liver and spleen
  • Auscultation S1, S2, abnormal sounds, murmur
  • Please do not pull out stethoscope before you
    observe patients carefully

20
Chest X-ray
Heart size, shape, pulmonary vascularity
21
Chest X-ray
22
Electrocardiography
23
Purposes of Imaging
  • Anatomic-pathologic diagnosis
  • Hemodynamic assessment
  • (velocity, flow, pressure, stress-strain)
  • Volume, function, wall motion, torsion
  • Coronary perfusion / Metabolism
  • Tissue characterization

24
Echocardiography
  • Easy, non-invasive, accurate, real-time
  • Anatomic and physiologic information
  • Changed practice of pediatric cardiology

25
Echocardiograhy - Modalities
  • M-mode / 2-D / 3-D
  • Doppler / color Doppler
  • Trans-thoracic, trans-esophageal,
  • trans-abdominal, trans-vaginal,
  • intra-cardiac, intra-vascular

26
Echocardiograhy
27
Echocardiograhy
28
M-mode Echocardiograhy
29
3-D Echocardiograhy
30
New Development in Echo
  • Imaging edge detection/auto-measurement
  • Doppler 3-D flow / stress-strain
  • Contrast echo coronary perfusion

31
Other Imaging Tools
  • Magnetic Resonance Imaging (MRI)
  • CT / Electron-beam CT (EBCT)
  • Radionuclide / SPECT
  • Positron Emission Tomography

32
Magnetic Resonance Imaging
Spin echo Gradient echo Velocity
encoded
33
Magnetic Resonance Imaging
  • Sectional still image/ cine image/ 3-D
  • Flow information / volume flow
  • Less window dependant / post-op study, older age
    / functional evaluation

34
Magnetic Resonance Imaging
35
Magnetic Resonance Imaging
36
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40
Computerized Tomography
41
Computerized Tomography
42
Radionuclide Study
43
Radionuclide Study
44
Positron Emission Tomography
45
Purposes of Catheterization
  • Anatomic diagnosis
  • Hemodynamic assessment
  • Interventional procedure

46
Equipment
  • Biplane monitor / Cine with digital subtraction
  • Patient monitoring EKG, BP, pulse oximeter
  • Physiologic signal amplifier and recording device
  • Blood gas, O2 consumption, Dye/ Thermodilution
  • Emergency treatment tools
  • Room for Others anesthesia, echo, exercise

47
Catheterization Room
48
Fluoroscopic Monitor
49
Physiologic Signal - Display Recording
50
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53
Catheterization vs Echocardiograpy
  • How accurate non-invasive tests are
  • Risk of cardiac catheterization
  • How to obtain necessary information
  • during catheterization or surgery
  • Nature of surgical correction
  • Risk of possible undiagnosed diseases

54
Electrophysiologic Study
55
How to Approach to CHD- as a Clinician -
  • Does this baby have a CHD?
  • Which baby should be referred to pediatric
    cardiologist
  • The urgency with which that referral should be
    made
  • If not, what tests and in what order to make
    diagnosis

56
Babies with Suspected CHD
  • Clinical assessment
  • Mode of / age at presentation
  • Physical examination
  • Laboratory test
  • CBC, ABGA, hyperoxic test
  • Chest PA, EKG
  • Echocardiography if available

57
Suspected CHD without Confirmation
  • Any magic bullet for all?
  • IV inotropics
  • PGE 1
  • Decrease oxygen consumption
  • General supportive care

58
Incidence of CHD
  • Incidence of total CHD among races
  • the same in all races about 5 - 8 /1,000
  • Ethnic difference in incidences of individual
    anomalies and subtypes
  • Associated non-cardiac malformation

59
Racial Difference of CHD
  • Left sided lesions seem to be lower in Asians
  • Right isomerism seems to be higher in Asians
  • Subpulmonic VSD is higher in Asians

60
Classification of CHD
  • Why classify the sameness, differences
  • How to classify view point, purpose

61
Classification of CHD
  • Clinical viewpoint
  • cyanotic versus acyanotic
  • shunts/obstruction/regurgitation
  • Pathology viewpoint
  • normal vs abnormal connection
  • segmental approach
  • Developmental viewpoint

62
Classification of CHD Developmental viewpoint
  • Ectomesenchymal tissue migration abnormalities
  • Abnormal intra-cardiac flow
  • Cell death abnormalities
  • Extra-cellular matrix abnormalities
  • Abnormal targeted growth
  • Abnormal situs and looping

63
Naming of CHD
  • Unambiguous, accurate, and succinct
  • Capable of describing any combination of defects
  • Allows for precise classification of
    malformations to be made during patients life
  • Useful both for anatomical, clinical, and
    etiologic studies

64
Naming Unambiguous?
  • Different names for the same thing
  • The same name for different things

65
Ambiguity
  • Different names for the same thing
  • L-transposition
  • ventricular inversion
  • S,L,L corrected transposition
  • (physiologically) corrected transposition
  • Atrial solitus, discordant AV/ VA connection

66
Ambiguity
  • The same names for different things
  • D- transposition
  • a term for GA relationship
  • a term for VA connection
  • a term for specific diagnosis

67
Naming Simple, Specific ?
  • In most, simple VSD, ASD, PDA, TOF
  • In a few, not simple or specific TA
  • Rarely, complex isomerism

68
Tricuspid Atresia
69
Right Isomerism
  • Dextrocardia
  • Bilateral SVC
  • Separate hepatic vein
  • TAPVR
  • Complete AVSD
  • LV hypoplasia
  • Transposition
  • PS or pul. Atresia

70
Fetal Circulation
  • Is adapted to a special situation
  • Depends on placenta for O2/nutrients
  • Is rarely overloaded,
  • but if overloaded little reserve

71
Flow Pathway Distribution ( indicates the
proportion of combined output )
SVC-RV-MPA-Duct-Des. Ao IVC-PFO-LA-LV-Asc. Ao
ductus arteriosus, ductus venosus, atrial
communication
72
Oxygen Saturation
73
Fetal Circulation
  • Parallel circulation (combined output)
  • Communications between R L heart
  • Pulmonary circulation is redundant

74
Congenital Heart Diseases in Fetus
  • Often silent
  • TGA has little effect
  • HLHS RV is slightly overloaded
  • PA IVS no effect at all
  • When CHD causes volume overload, heart fails and
    hydrops ensues

75
Transitional Circulation
  • Dramatic changes in circulation at the moment
    of birth and onwards
  • Air breadth - lung expansion - Rp ?
  • Qp ? - LA pressure ? - PFO ?
  • P O2 ? - ductus arteriosus and venosus ?
  • Obliteration of placental circulation - Rs ?
  • IVC pressure ? - PFO ?

76
Transitional Circulation CHD
  • As circulation separates, TGA can not
    supply enough oxygen to the body
  • Obstructed pathway in either side hardly tolerate
  • right PA or critical PS in any CHD
  • left Aortic atresia or critical AS, IAA, COA
  • mitral atresia small PFO obstructed TAPVR

77
Neonatal Circulation CHD
  • Neonatal circulation
  • potential of increased Rp
  • potential of atrial communication
  • compliance of two ventricles is nearly equal
  • CHD and neonatal circulation
  • VSD, PDA usually not symptomatic
  • ASD usually not symptomatic
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