Electrocardiography on

Congenital Heart Disease

Radityo Prakoso

Division of Pediatric Cardiology and Congenital Heart Disease

Department of Cardiology and Vascular Medicine
Faculty of Medicine Universitas Indonesia
National Cardiovascular Center Harapan Kita, Jakarta, Indonesia
Radityo Prakoso

Disclosure

Nothing to be disclosed
Radityo Prakoso

Congenital Heart
Disease 708 Circulation Research February 15, 2013

• the most common CoA (409)

PDA (799)

congenital birth defects ASD (941)

PS (728) ,TOF (421),
TGA (315), PTA (107)

• is abnormality in AVSD (348)

AS (401)
HLH (266)

cardiocirculatory Ebstein (61)

TA (79)
structure or function that
SV (106)
HLH (266)

is present on birth, even if TOF (421)

VSD (3570)

it is discovered much later

Figure 1. Locations of heart malformations that are usually
identified in infancy, and estimated prevalence based on the
CONCOR database.9 Numbers indicate the birth prevalence per
million live births. AS indicates aortic stenosis; ASD, atrial septal
Moss defect;
and Adam’s Heart
AVSD, Disease in Infants,
atrioventricular Children,
septal and Adolescents.
defect; ed 9. 2016.
CoA, coarctation of
Radityo Prakoso

Congenital Heart
Disease
• Reported birth prevalence of CHD varies widely
among studies worldwide.

• The estimate of 8 per 1,000 live births is generally

accepted as the best approximation

• A worldwide annual birth rate around 150 million

births 1.35 million live births with CHD every
year

Van der Linde,D. J Am Coll Cardiol 2011;58;2241-7

Radityo Prakoso

Classification of CHD

Acyanotic Cyanotic

Parallel Common
L-R shunt Obstructive Lesion R-L shunt Circulation Mixing
without shunt Physiology

adapted from: Rilantono, L R. 5 Rahasia Penyakit Kardiovaskular. FKUI. 2012

Radityo Prakoso Diagnosis Algorithm of Acyanotic CHD

Acyanotic CHD

Left to right shunt Obstruction without shunt

Plethora Normal Pulmonary Vascularization

Can be determined
LVH/BVH RVH by ECG
LVH RVH

VSD AS
AS
PDA ASD CoA (infants)
CoA
AVSD MS

adapted from: Rilantono, L R. 5 Rahasia Penyakit Kardiovaskular. FKUI. 2012

Radityo Prakoso Diagnosis Algorithm of Cyanotic CHD

Cyanotic CHD

Decreased pulmonary flow Increased pulmonary flow

Oligemia Plethora

Can be determined
RVH LVH by ECG
LVH/BVH RVH

ToF TA+VSD+PS TrA TGA+IVS

PA+VSD PA+IVS TGA+VSD TAPVD
DORV+VSD+PS DOLV+PS APW DORV+VSD

adapted from: Rilantono, L R. 5 Rahasia Penyakit Kardiovaskular. FKUI. 2012

Radityo Prakoso

Shunt Lesion
Radityo Prakoso
Normal Cardiac Circulations

Qs (Systemic
Blood Flow)
Qp (Pulmonary
Blood Flow)

“shunt” refers to an abnormal connection allowing

blood to flow directly from one side of the cardiac
Shunt Lesion circulation to the other

Left Right Right Left

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso Shunt Lesion Circulation
Right Left Right Left

Qp/Qs Ratio Means Shunt lession

1:1 Normal No shunting

>1 Pulmonary Flow > systemic flow L-R shunt

<1 Systemic flow > pulmonary flow R-L shunt

exactly 1:1 Pulmonary Flow = Systemic flow bidirectional shunt

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso

Atrial Septal
Defect
Radityo Prakoso Blood Circulation in ASD

direction of blood flow across ASD during diastole :

• Normal route

• passing through ASD —> opposite ventricle

Compliance and capacity of the 2 ventricles

Left Right Right Left

workload LV larger than RV —> LV hypertrophied,
Severe RV noncompliance or distenbility
decrease compliance —> Left to Right Shunt in ASD —
> increased RV after load —> Pulmonary parenchymal induced with exertion
disease (PH) —> RV hypertophied, less compliant

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso

ECG in ASD
Depend on the type and size of the ASD

Small left-to-right shunt

No right atrial or ventricular dilation Normal ECG

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

ASD primum
AV conduction
in ASD primum

His bundle is displaced inferiorly, AV node is displaced posteriorly,

along the inferior rim of the septal defect near the orifice of the coronary sinus

Left Axis Deviation

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso
ASD primum
ASD primum : 1st-degree AV block, LAD, RVH

• Webb G, Gatzoulis MA. Atrial Septal Defects in the Adult.

Circulation. 2006; 114(15): 1645-53.

• Sinus rhythm (most)

• Left axis deviation
• Prolonged PR interval

(increased conduction time high right to low septal right atrium)
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Webb G, Gatzoulis MA. Atrial Septal Defects in the Adult. Circulation. 2006; 114(15): 1645-53.
Radityo Prakoso

ASD secundum

Significant left-to-right shunt

Right atrial enlargement
Right ventricular volume overload
Right axis deviation rSR’ pattern
Tall p waves in the right precordial
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
 Radityo Prakoso

ASD-PH-Eisenmenger syndrome
Eisenmenger ASD : RAD, RVH with extensive repolarization abnormalities

Pulmonary Hypertension
Q waves
rSR’ pattern in the right precordial Tall monophasic R waves
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016. with deeply inverted T waves
Webb G, Gatzoulis MA. Atrial Septal Defects in the Adult. Circulation. 2006; 114(15): 1645-53.
Radityo Prakoso

Ventricular Septal Defect

• Most common form of

CHD, 20% human
cardiac malformation

• Perimembranous type,
80% of all VSDs

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso
Type of VSD

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso

Pathophysiology
• Systolic Pathways of Blood flow in VSD

• through the usual outflow tract of

that ventricle

• through VSD to outflow tract of the

other ventricle

L-R shunt as long as PVr < SVr, if

reverse shunt reverses
• L-R — reduces LV output — compensated by elevate LV filling pressure
(Pv congestion at rest/during exertion)

• Large defect VSD: LV and RV ~ common chamber — Pulmonary artery

pressure = aorta pressure — pulmonary vascular disease/ Eisenmenger
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso
ECG in VSD

Children Infant
Small VSD Large VSD

Right Ventricular Hypertrophy

Normal ECG
Biventricular Hypertrophy
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

Patent Ductus Arteriosus

• The Ductus Arteriousus
usually close
spontaneously (72 hours
of birth), through the
contraction of an
arteriolar smooth muscle
— signaled by the rise in
postnatal systemic
oxygen level

• Common in preterm
infants

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso
Pathophysiology

• most L-R shunt (aorta to MPA) — systole and diastole — diastolic

“runoff” —> impaired coronary and splanchnic perfusion

• Large PDA — LVEDP increase — pulmonary congestion

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso

ECG in PDA
Not sensitive or specific for diagnostic

Hemodynamically insignificant PDA No ECG changes

Widened P waves
Larger PDA
Left Atrium Enlargement
Tall R (I,II,III,AVL,V5,V6) or Tall S (V1, V2)
Significant, Chronic PDA
Left Ventricular Hypertrophy

Significant L-to-R Ischemic ST segment (rare)

diastolic runoff Coronary steal
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016
Schneider DJ, Moore JW. Patent Ductus Arteriosus. Circulation. 2006; 114(17):1873-82.
Radityo Prakoso

Coronary Fistulas
• communications between coronary arteries and
the cardiac chambers (coronary-cameral fistulas)
or low- pressure veins (coronary arteriovenous
malformations)

• secondary to trauma, invasive cardiac procedures

(pacemaker, endomyocardial biopsy, CABG,
coronary angiography)

• drainage most often to RV, RA or pulmonary

arteries
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso

Pathophysiology
depends in the resistance of the fistulous connection
and on the site of fistula termination.

size, tortuosity, length of pathway

Blood follows the lower resistance pathway through the

fistula rather than traversing the smaller arterioles and
capillaries of the myocardium

Larger fistulas — diastolic “runoff”— coronary steal

ECG finding : Ischemic ST segment
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part I: Shunt Lesions .Circulation. 2008;117:1090-1099
Radityo Prakoso

Obstructive Lesion
Radityo Prakoso

Congenital Obstructive Lesion

Ventricular
Semilunar valves Great arteries
outflow tracts

narrowing

ventricular afterload symptoms related to

the severity of
ventricular obstruction
hypertrophy

diastolic cardiac output ,

dysfunction stroke volume
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part II Simple Obstructive Lesions. Circulation. 2008;117:1228-1237
Radityo Prakoso
Obstruction at the Right Ventricle
Outflow Tract
Stenosis branch PA

Distal to Pulmonary valve

(MPA; supravalvar
stenosis)

Pulmonary valve (valvar

pulmonary stenosis)

Proximal to Pulmonary
valve (subvalvar
pulmonary stenosis)

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part II Simple Obstructive Lesions. Circulation. 2008;117:1228-1237
Radityo Prakoso

Valvar Pulmonary Stenosis

• isolated valvar PS present in
8-10% patients with CHD

• ECG can be used to assess

severity of obstruction

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part II Simple Obstructive Lesions. Circulation. 2008;117:1228-1237
Radityo Prakoso

Valvar Pulmonary Stenosis

Severity Normal ECG Abnormal ECG

Slight RAD

Mild 40-50% R wave (right precordial) <10-15 mm

RAD

R:S ratio in V1 > 4:1

Moderate 10% R wave <20 mm

In infant, RV may be hypoplastic T wave (right precordial) upright
Axis more leftward (+30 to +70 degrees)
RAD / Extreme RAD

as well as evidence of LVH RAE
Severe Rare R wave >20 mm
T wave (right precordial) upright/inverted

Pure R / RS / QR (right precordial)


Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

ECG

Severe 

valvar pulmonary stenosis

Estimated RV pressure (mmHg) = Height R wave (mm) x 5

(2-20 y.o patient with pure R wave in V1 / V4R
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

Obstruction at the LVOT

Coarc Aorta ~ Turner
syndomre

Supravalvar AS ~
williams’syndrome

Valvar AS ~ Shone’s
complex
Sub aortic stenosis ~
Noonan syndomre

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part II Simple Obstructive Lesions. Circulation. 2008;117:1228-1237
Radityo Prakoso

Pathophysiology LVOT
obstruction
LVOT obstruction

LV hypertrophy Arrhythmia

reduced LV
compliance Left-sided heart failure

Pulmonary venous coronary insufficiency

congestion

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part II Simple Obstructive Lesions. Circulation. 2008;117:1228-1237
Radityo Prakoso

ECG in Aorta Stenosis

Lack of sensitivity and specificity for
Pediatric
detecting severe disease

LVH
Adult
T-wave inversion (lateral precordial)

Independent predictor for developing HF

in asymptomatic patients

Associated with increased myocardial fibrosis on MRI and increased risk of

cardiovascular death

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

Coarc Aorta
Maldistribution flow

pre-ductal post-ductal

hypertension (upper body), diminished pulse volume in the

lower extremities, heart murmur, angina, Heart failure

Premature coronary artery disease,

berry aneurysm

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the Adult Part II Simple Obstructive Lesions. Circulation. 2008;117:1228-1237
Radityo Prakoso

ECG in CoA
Infant Generally normal ECG

Older children / Long-standing LVH

adolescent left ventricular pressure overload LAE

Associated intracardiac lession ECG features

AVSD

DORV
 LAD
Primary myocardial disease
Strain pattern of ST segment or
Severe valvar / subvalvar aortic stenosis
T wave depression
Pulmonary hypertension 

RVH
in VSD / mitral stenosis
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

Complex Congenital
Heart Disease
 Radityo Prakoso

Ebstein’s Anomaly
septal leaflet of TV conjoined the
septal surface below valve
annulus into RV

coaptation adequate ~moderate TV regurgitation

Most important!
RV hypoplastic, RA dilatation

Reduced RV filling capacity

Shunt : ASD; PFO —
R-L shunt
Venous congestion Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the
Adult Part III: Complex Congenital Heart Disease. Circulation. 2008;117:1340-1350.
Radityo Prakoso

ECG in Ebstein's Anomaly

Tall, wide P waves and Right Bundle Branch Block (RBBB)

RA Enlargement Its severity directly related

Long intra-atrial conduction to abnormal formation of
septal leaflet

Prolonged PR interval
Minimal degree
Ventricular pre-excitation Slow connection

Atrial tachycardia
Atrial flutter
Intra-atrial reentrant tachycardia There’s accessory pathway
Atrial fibrillation in tricuspid annulus
AV node reentrant tachycardia
Ventricular arrhythmias
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

ECG in Ebstein's Anomaly

WPW

Tall p wave

Ebstein anomaly with Wolff–Parkinson–White (pre-excitation)

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

L-Transposition of great
arteries (ccTGA)
AV discordance,
VA discordance

Normal cardiac physiology

Undiagnosed
until adulthood

Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the
Adult Part III: Complex Congenital Heart Disease. Circulation. 2008;117:1340-1350.
Radityo Prakoso

ECG in ccTGA
Electrical activation From interventricular septum

Normal hearts
of the ventricle Left to right, slightly anterior

25% normal neonates may not qR in V6

ccTGA demonstrate Q wave in V6 RS in V1

Ventricular inversion Less common in right sided heart

Surfaces and ventricular bundle or
branches are inverted There’re confounding associated lesions
producing pressure or volume overload

Right to left electrical sequence Q waves in right precordial

More superior and anterior direction Absent Q waves in left precordial

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

Tetralogy of Fallot
malalignment of septum
infundibulum (anterior-cephalad)
3 2
narrowing RV overriding aorta
outflow (PS)
1
VSD subaortic
4
RV hypertrophy as result of
complication of hemodynamic

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
 Radityo Prakoso

Natural history

• mild obstruction : Qp<Qs (RV-lungs) —>

acyanotic, heart murmur ejection systolic
Progressive
(+)

• Severe obstruction : Qp>Qs (R-L shunt)

—> cyanotic, exertional dyspnea, “spell”
Any patient with unprepared TOF should be considered for
intervention
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

ECG in ToF

Increased right ventricular pressure Right ventricular hypertrophy

Tall R wave in V1 RAD

Abrupt R wave
R wave in V1 with RS in V2 (sudden transition)
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
 AV concordance,
VA discordance
D-TGA
Systemic arterial desaturation

systemic arterial acidosis

death within hours of

life (postnatal)

Natural Indication of
Radityo Prakoso communication :PFO, intervention :
PDA, BAS
Robert JS, Ziyam MH, John FR. Pathophysiology of Congenital Heart Disease in the
Adult Part III: Complex Congenital Heart Disease. Circulation. 2008;117:1340-1350.
Radityo Prakoso

ECG in D-TGA
Right Atrial Enlargement
and
Right Ventricular Hypertrophy

Ventricular septum Biventricular hypertrophy

Left ventricular Significant left

outflow tract ventricular volume
Defect (VSD) obstruction overload
Intact (IVS) and/or
LVOT

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

Post-operative
Abnormalities
Radityo Prakoso

Postoperative Arrhytmia

Junctional Ectopic Tachycardia (JET) and Supraventricular Tachycardia (SVT)

Hemodynamically significant postoperative arrhythmias (15%)
Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. ed 9. 2016.
Radityo Prakoso

Summary
• Congenital Heart Diseases (CHDs) are the most
common congenital birth defects which needed
to be discovered as soon as possible.

• Well interpretation of the electrocardiography

may be benefit (around 60%) for diagnose some
CHDs.

• Understanding pathophysiology of the diseases

can help us to conclude our ECG findings.
Radityo Prakoso

Acknowledgement

Thank you to Vizzi Alvi Fitrah Nasution 


for contribution completing this presentation
 Thank you :)

[email protected]