Related Experiment Video
Updated: Jun 14, 2025

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Arrhythmias in congenital heart disease: A nationwide cohort study
Eva Havers-Borgersen1, Christian Jøns1, Jawad H Butt1
1Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
Insights
Adults with congenital heart disease (CHD) face a significantly higher risk of developing arrhythmias, such as advanced atrioventricular block and atrial flutter/fibrillation. This increased risk is associated with a greater likelihood of mortality.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease
- Electrophysiology
Background:
- The population of adults with congenital heart disease (CHD) is growing due to increased survival rates.
- This demographic shift is associated with a rising incidence of cardiac complications, particularly arrhythmias.
- Long-term risks of arrhythmias in adults with CHD remain under-researched.
Purpose of the Study:
- To investigate the long-term risk of arrhythmias in adults with congenital heart disease (CHD).
- To compare arrhythmia incidence in CHD patients versus the general population.
- To identify specific CHD subtypes associated with elevated arrhythmia risk.
Main Methods:
- Observational cohort study utilizing Danish national registries.
- Inclusion of all Danish patients born with CHD from 1977 to 2024.
- Follow-up until arrhythmia diagnosis, death, emigration, or March 2024, with age- and sex-matched controls.
Main Results:
- 45,820 CHD patients were compared to 183,280 controls.
- CHD patients had a 16.4-fold increased hazard ratio for arrhythmias (1.2 vs 0.1 per 1,000 PY).
- Advanced atrioventricular block and atrial flutter/fibrillation were most common; complex CHD conferred highest risk. Arrhythmia increased mortality risk by 6.9-fold.
Conclusions:
- Adults with CHD exhibit a substantially elevated risk of arrhythmias compared to the general population.
- Individuals with complex forms of CHD face a particularly high risk of developing arrhythmias.
- Arrhythmia in CHD patients is linked to a significantly increased risk of death, underscoring the need for improved management strategies.
Background:
As more patients with congenital heart disease (CHD) survive into adulthood, the population of adults with CHD is expanding. This trend is accompanied by an increasing incidence of complications, including arrhythmias. However, the long-term risk of arrhythmias remains sparsely investigated.
Methods:
In this observational cohort study, all Danish patients with CHD born from 1977 to 2024 were identified using registries and followed from date of birth until the occurrence of arrhythmia, emigration, death, or end of follow-up (March 2024). The risk of arrhythmias was assessed among patients with CHD and compared to age- and sex-matched controls from the background population.
Results:
A total of 45,820 patients with CHD (50.9% men) were identified and matched with 183,280 controls from the background population. During a median follow-up of 21.5 years, 2.6% of patients with CHD and 0.2% of controls developed arrhythmias-corresponding to incidence rates (IR) of 1.2 (95% CI 1.2-1.3) and 0.1 (95% CI 0.1-0.1) per 1,000 PY, respectively, and a hazard ratio (HR) of 16.4 (95% CI 14.4-18.7). The most common arrhythmias in patients with CHD were advanced atrioventricular block (IR 0.4 [95% CI 0.4-0.4] per 1,000 PY) and atrial flutter/fibrillation (IR 0.5 [95% CI 0.5-0.6] per 1,000 PY). Patients with malformations of the heart chambers, transposition of the great arteries, tetralogy of Fallot, and atrioventricular septal defect were at the highest risk of arrhythmias. Moreover, the risk of arrhythmias among those with ASD was not negligible. In patients with CHD, arrhythmia was associated with a significantly higher risk of death (HR of 6.9 [95% CI 5.9-8.1]).
Conclusions:
Patients with CHD are at significantly higher risk of arrhythmias than the background population, and those with complex CHD are at particularly high risk. In patients with CHD, arrhythmia is associated with an increased risk of death. Additional studies are warranted to investigate how we can improve the diagnosis and management of arrhythmias in CHD.
Related Concept Videos
Disturbances in Heart Rhythm
Arrhythmias are categorized by their speed, rhythm, and origin. A slow...
Mechanism of Cardiac Arrhythmias
ECG Interpretation of Arrhythmias I: Sinus Arrhythmias
Types of Arrhythmias
Sinus Node Arrhythmias
Sinus Bradycardia: Originating from the sinoatrial (SA) node, sinus bradycardia involves slower impulses, resulting in a heart rate of less than 60 beats per minute (bpm). Causes include sleep, vagal stimulation, beta-blockers, hypothyroidism,...
Electrophysiology of Normal Cardiac Rhythm
Pulse rhythm
Conversely, an irregular pulse pattern is termed dysrhythmia, stemming from disruptions in cardiac...
Conduction System of the Heart
The pacemaker cells are located in two primary nodes: the sinoatrial (SA) node and the atrioventricular (AV) node. The SA node pacemaker cells can autonomously depolarize, triggering an action potential that leads to the...

