Evaluation for Congenital Heart Diseases

Seoul National University HospitalDepartment 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 s

kin, 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 PACorrected 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-rayHeart 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 viewpointcyanotic versus acyanotic

shunts/obstruction/regurgitation

• Pathology viewpointnormal 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

SVC-RV-MPA-Duct-Des. Ao

IVC-PFO-LA-LV-Asc. Ao

* ductus arteriosus, ductus venosus,

atrial communication

Flow Pathway & Distribution (% indicates the proportion of combined output )

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