Management of Cardiac Rhythm Disorders in Congenital Heart Disease

Barbara J Deal, MD, MS, Constantine Mavroudis, MD


Surgical interventions provide unique opportunities for the management of arrhythmias associated with congenital heart disease. Supraventricular tachycardia develops in 5-60% of children and adults with congenital heart disease and this incidence increases significantly with longer postoperative intervals and with the complexity of heart disease. Arrhythmias are associated with hospitalizations, decreased ventricular function, increased risk of stroke, and decreased long-term survival. Reoperations are frequently performed in patients with the most complex forms of heart disease and arrhythmias, such that a combined approach to treat hemodynamic sequelae and arrhythmias surgically may significantly improve quality of life and survival for these patients. With increasing awareness of the important morbidity associated with arrhythmias in patients with structural heart disease, there are now published clinical guidelines for performing surgical ablation during reoperations.

Understanding the mechanisms of various arrhythmias is essential to determine appropriate medical, catheter, or surgical intervention. Arrhythmia surgery requires a thorough knowledge of anatomic variants, arrhythmia types, and intramyocardial scar location. Successful performance of arrhythmia surgery requires awareness of the guidelines, the arrhythmia knowledge mentioned above, as well as a collaborative approach with the electrophysiology team. Appropriate device therapy at the time of reoperation may avoid repeat interventions and provide important adjunct therapies. Arrhythmia surgery provides definitive and durable treatment for many subsets of arrhythmias, and thus every reoperation for congenital heart disease can be viewed as an opportunity to assess electrical and arrhythmia substrates and to intervene to improve the hemodynamic condition as well as the rhythm status of the patient.

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Last updated: June 22, 2021