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Congenital Heart Diseases

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					Evaluation for Congenital Heart
            Diseases

           Seoul National University Hospital
    Department of Thoracic & Cardiovascular Surgery
  Heart Diseases in Children

      Congenital heart diseases
Rheumatic heart disease : Rheumatic fever
   Other acquired diseases: Kawasaki
            Cardiomyopathy
             Arrhythmia
         Effects of CHD

• No effect on a child
• Decreased function when stressed
• Decreased cardiopulmonary function
• Other organ/system manifestation
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
      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
               Cyanosis

• Cyanosis: more than 5.0g% of reduced Hb.
• Central versus Peripheral cyanosis
• Central cyanosis: CNS, heart, lung
• Pathophysiology: TGA, TOF, CML, DDPC
                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
    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
     Abnormality in Chest PA
Corrected TGA/ Pericardial Defect/ Cardiac Tumor
                     Chest Pain
* Anomalous origin of left coronary artery from pulmonary artery
* Aortic stenosis(severe) / Hypertrophic cardiomyopathy
  Syncope/ Seizure/ Fainting

• Anoxic spell of acyanotic TOF

• Arrhythmia : VT, SVT, long QT syndrome

• Syncope on exercise : AS, SAS, IHSS
Airway Obstruction/ Dysphagia
Airway Obstruction / Dysphagia
    Abnormal PA Branching
Mode of / Age at Presentation

• Collapse   : within 3-4 days
• Cyanosis   : usually within a month
• Congestion : 1-5 months
• Murmur      : any time
Diagnosis of Cardiac Diseases

   • Anatomic Diagnosis
   • Hemodynamic Evaluation
   • Total assessment / Prognosis
   • Etiologic Diagnosis
         Diagnostic Tools

• History and physical examination
• Chest X ray / EKG / Blood study
• Echocardiography/ Other imaging tools
• Catheterization/ Other invasive tools
      Accuracy of Tools
• History/PE : important, rarely specific
• X-ray/EKG : not-confirmative
• Echocardiography:
  confirmative, but non-invasive
• Cardiac catheterization :
  confirmative, but invasive
             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
      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
           Chest X-ray
Heart size, shape, pulmonary vascularity
Chest X-ray
Electrocardiography
     Purposes of Imaging
• Anatomic-pathologic diagnosis
• Hemodynamic assessment
  (velocity, flow, pressure, stress-strain)
• Volume, function, wall motion, torsion
• Coronary perfusion / Metabolism
• Tissue characterization
        Echocardiography

• Easy, non-invasive, accurate, real-time

• Anatomic and physiologic information

• Changed practice of pediatric cardiology
Echocardiograhy - Modalities

  • M-mode / 2-D / 3-D
  • Doppler / color Doppler
  • Trans-thoracic, trans-esophageal,
    trans-abdominal, trans-vaginal,
    intra-cardiac, intra-vascular
Echocardiograhy
Echocardiograhy
M-mode Echocardiograhy
3-D Echocardiograhy
  New Development in Echo


• Imaging: edge detection/auto-measurement

• Doppler: 3-D flow / stress-strain

• Contrast echo: coronary perfusion
    Other Imaging Tools


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




Spin echo   Gradient echo Velocity encoded
Magnetic Resonance Imaging

• Sectional still image/ cine image/ 3-D

• Flow information / volume flow

• Less window dependant / post-op
  study, older age / functional
  evaluation
Magnetic Resonance Imaging
Magnetic Resonance Imaging
Computerized Tomography
Computerized Tomography
Radionuclide Study
Radionuclide Study
Positron Emission Tomography
Purposes of Catheterization

   • Anatomic diagnosis

   • Hemodynamic assessment

   • Interventional procedure
                 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
Catheterization Room
Fluoroscopic Monitor
Physiologic Signal - Display & Recording
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
Electrophysiologic Study
   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
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
Suspected CHD without Confirmation


       Any magic bullet for all?
       – IV inotropics
       – PGE 1
       – Decrease oxygen consumption
       – General supportive care
       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
      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
    Classification of CHD

• Why classify : the sameness, differences

• How to classify : view point, purpose
Classification of CHD
• Clinical viewpoint
  cyanotic versus acyanotic
  shunts/obstruction/regurgitation
• Pathology viewpoint
  normal vs abnormal connection
  segmental approach
• Developmental viewpoint
    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
           Naming of CHD

• Unambiguous, accurate, and succinct
• Capable of describing any combination of
  defects
• Allows for precise classification of
  malformations to be made during patient’s life
• Useful both for anatomical, clinical, and
  etiologic studies
Naming : Unambiguous?


• Different names for the same thing

• The same name for different things
               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
            Ambiguity

The same names for different things
 D- transposition
 – a term for GA relationship
 – a term for VA connection
 – a term for specific diagnosis
Naming : Simple, Specific ?


• In most, simple : VSD, ASD, PDA, TOF

• In a few, not simple or specific : TA

• Rarely, complex : isomerism
Tricuspid Atresia
Right Isomerism
        • Dextrocardia
        • Bilateral SVC
        • Separate hepatic vein
        • TAPVR
        • Complete AVSD
        • LV hypoplasia
        • Transposition
        • PS or pul. Atresia
        Fetal Circulation

• Is adapted to a special situation

• Depends on placenta for O2/nutrients

• Is rarely overloaded,
  but if overloaded little reserve
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
Oxygen Saturation %
        Fetal Circulation

• Parallel circulation (combined output)

• Communications between R & L heart

• Pulmonary circulation is redundant
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
   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 ↓
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
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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posted:11/22/2011
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