ECG in Congenital Heart Disease

Congenital Heart Disease can result in ECG changes, often related to atrial or ventricular overload and enlargement. Below a list of relatively common forms of congenital heart disease and their potential ECG changes. Adapted from Khairy et al.[1]

Secundum atrial septal defect

Information about Secundum atrial septal defec on Wikipedia (external link)

Rhythm: normal sinus rhythm, increased risk of AF with age
PR interval: first degree AV block in 6-19%
QRS axis: 0° to 180°; RAD; LAD in Holt-Oram or LAHB
QRS Configuration: rSr´ or rsR´ with RBBBi>RBBBc
Atrial Enlargement: RAE 35%
Ventricular hypertrophy: Uncommon
Particularities: "Crochetage" pattern

Ventricular Septal Defect

Information about Ventricular Septal Defect on Wikipedia (external link)

Rhythm: normal sinus rhythm, PVCs
PR interval: Normal or mild ↑; 1° AVB 10%
QRS axis: RAD with BVH; LAD 3% to 15%
QRS Configuration: Normal or rsr´; possible RBBB
Atrial Enlargement: Possible RAE±LAE
Ventricular hypertrophy: BVH 23% to 61%; RVH with Eisenmenger
Particularities: Katz-Wachtel phenomenon

AV canal defect

Information about AV canal defect on Wikipedia (external link)

Rhythm: normal sinus rhythm, PVCs 30%
PR interval: 1° AVB >50%
QRS axis: Moderate to extreme LAD; normal with atypical
QRS Configuration: rSr´ or rsR´
Atrial Enlargement: Possible LAE
Ventricular hypertrophy: Uncommon in partial; BVH in complete; RVH with Eisenmenger
Particularities: Inferoposteriorly displaced AVN

Patent ductus arteriosus

Information about Patent ductus arteriosus on Wikipedia (external link)

Rhythm: normal sinus rhythm, ↑ IART/AF with age
PR interval: ↑ PR 10% to 20%
QRS axis: Normal
QRS Configuration: Deep S V₁, tall R V₅ and V₆
Atrial Enlargement: LAE with moderate PDA
Ventricular hypertrophy: Uncommon
Particularities: Often either clinically silent or Eisenmenger

Pulmonary stenosis

Information about Pulmonary stenosis on Wikipedia (external link)

Rhythm: normal sinus rhythm
PR interval: Normal
QRS axis: Normal if mild; RAD with moderate/severe
QRS Configuration: Normal; or rSr´; R´ increases with severity
Atrial Enlargement: Possible RAE
Ventricular hypertrophy: RVH; severity correlates with R:S in V₁ and V₆
Particularities: Axis deviation correlates with RVP

Aortic coarctation

Rhythm: normal sinus rhythm
PR interval: Normal
QRS axis: Normal or LAD
QRS Configuration: Normal
Atrial Enlargement: Possible LAE
Ventricular hypertrophy: LVH, especially by voltage criteria
Particularities: Persistent RVH rare beyond infancy

Ebstein’s anomaly

ECG from a patient with Ebstein's anomaly showing huge P waves and low amplitude QRS waves. RBBB and T wave inversion are not present on this ECG.

ECG from a patient with Ebstein's anomaly

Information about Ebstein's anomaly on Wikipedia (external link)

Rhythm: normal sinus rhythm, possible EAR, SVT; AF/IART 40%
PR interval: 1° AVB common; short if WPW
QRS axis: Normal or LAD
QRS Configuration: Low-amplitude multiphasic atypical RBBB
Atrial Enlargement: RAE with Himalayan P waves
Ventricular hypertrophy: Diminutive RV
Particularities: Accessory pathway common; Q II, III, aVF, and V₁–V₄

Surgically repaired TOF

Rhythm: normal sinus rhythm, PVCs; IART 10%; VT 12%
PR interval: Normal or mild ↑
QRS axis: Normal or RAD; LAD 5% to 10%
QRS Configuration: RBBB 90%
Atrial Enlargement: Peaked P waves; RAE possible
Ventricular hypertrophy: RVH possible if RVOT obstruction or PHT
Particularities: QRS duration±QTd predictive of VT/SCD

Congenitally corrected TGA

Rhythm: normal sinus rhythm
PR interval: 1° AVB >50%; AVB 2%/year
QRS axis: LAD
QRS Configuration: Absence septal q; Q in III, aVF, and right precordium
Atrial Enlargement: Not if no associated defects
Ventricular hypertrophy: Not if no associated defects
Particularities: Anterior AVN; positive T precordial; WPW with Ebstein’s

Complete TGA/intra-atrial baffle

Rhythm: Sinus brady 60%; EAR; junctional; IART 25%
PR interval: Normal
QRS axis: RAD
QRS Configuration: Absence of q, small r, deep S in left precordium
Atrial Enlargement: Possible RAE
Ventricular hypertrophy: RVH; diminutive LV
Particularities: Possible AVB if VSD or TV surgery

UVH with Fontan

Rhythm: Sinus brady 15%; EAR; junctional; IART >50%
PR interval: Normal in TA; 1° AVB in DILV
QRS axis: LAD in single RV, TA, single LV with noninverted outlet
QRS Configuration: Variable; ↑R and S amplitudes in limb and precordial leads
Atrial Enlargement: RAE in TA
Ventricular hypertrophy: RVH with single RV; possible LVH with single LV
Particularities: Absent sinus node in LAI; AV block with L-loop or AVCD

Dextrocardia

An example of dextrocardia

Rhythm: normal sinus rhythm, P-wave axis 105° to 165° with situs inversus
PR interval: Normal
QRS axis: RAD
QRS Configuration: Inverse depolarization and repolarization
Atrial Enlargement: Not with situs inversus
Ventricular hypertrophy: LVH: tall R V₁–V₂; RVH: deep Q, small R V₁ and tall R right lateral
Particularities: Situs solitus: normal P-wave axis and severe CHD

ALCAPA

Rhythm: normal sinus rhythm
PR interval: Normal
QRS axis: Possible LAD
QRS Configuration: Ant-lat Q waves; possible ant-sept Q waves
Atrial Enlargement: Possible LAE
Ventricular hypertrophy: Selective hypertrophy of posterobasal LV
Particularities: Possible ischemia

References

Khairy P and Marelli AJ. Clinical use of electrocardiography in adults with congenital heart disease. Circulation. 2007 Dec 4;116(23):2734-46. DOI:10.1161/CIRCULATIONAHA.107.691568 | PubMed ID:18056539 | HubMed [khairy]