Congenital Heart Disease and Atrial Fibrillation

Congenital heart disease (CHD) is present in 3–6/1 000 individuals. The most common CHDs include:

Atrial septal defect (30%)
Ventricular septal defect (15%)
Tetralogy of Fallot (5%)
Patent ductus arteriosus (5%)
Coarctation of the aorta (5%)
Transposition of the great arteries (3%)
Ebstein’s anomaly (1%)
Other (<1%)

Atrial fibrillation (AF) occurs in 20% of patients with CHD.

Due to advances in medicine, the number of patients with CHD is increasing and they survive to older age. However, given the low prevalence and wide spectrum of CHD, recommendations for CHD are based on less robust evidence compared with, for example, recommendations for AF or arterial hypertension.

Most CHDs represent a thrombogenic condition due to non-physiological and turbulent blood flow; therefore, in some CHDs and AF, anticoagulation therapy is initiated irrespective of the CHA₂DS₂-VA score.

Illustration of atrial fibrillation in a patient with congenital heart disease, showing a schematic depiction of structural cardiac abnormality and ECG documentation of arrhythmia.

Anticoagulation therapy in patients with AF is indicated irrespective of the CHA₂DS₂-VA score in the following cases:

After cardiac surgery for congenital heart disease
Fontan procedure
Systemic right ventricle
Cyanotic heart disease:

Tetralogy of Fallot
Transposition of the great arteries
Tricuspid atresia
Truncus arteriosus
Total anomalous pulmonary venous drainage

In other patients with CHD and AF, anticoagulation therapy is guided by the CHA₂DS₂-VA score.

In valvular AF, warfarin (not a NOAC) should always be administered, irrespective of the CHA₂DS₂-VA score and CHD.

Valvular AF means that the patient has AF and valvular heart disease, specifically:

moderate or severe mitral stenosis, or
a mechanical prosthetic valve

For rate control of AF, the following may be administered:

β₁-selective beta-blockers
Verapamil
Diltiazem
Digoxin

For rhythm control of AF, the following may be administered:

Amiodarone

Congenital heart disease and atrial fibrillation	Class
Anticoagulation therapy should be administered in every patient with AF or atrial flutter and the following congenital heart disease: After cardiac surgery for congenital heart disease Fontan palliation Systemic right ventricle Cyanotic heart disease: Tetralogy of Fallot Transposition of the great arteries Tricuspid atresia Truncus arteriosus Total anomalous pulmonary venous drainage	IIa