Pacing in Patients With Congenital Heart Disease
Many congenital cardiac defects are accompanied by abnormalities of the conduction system, either directly related to developmental abnormalities or as a consequence of the unique myocardial substrate created by large septal patches, extensive suture lines, long-standing cyanosis or abnormal pressure/volume status. Abnormalities vary from sick sinus syndrome, sino-atrial block, sinus arrest to atrio-ventricular (AV) disturbances, such as second- or third-degree AV block. Symptoms may include dizziness, syncope, heart failure or, simply, effort intolerance, lassitude or fatigue. Complete AV block carries a prognostic disadvantage and such patients require pacing whether they are symptomatic or not. Moreover, ventricular tachyarrhythmias may occur in patients with severe and complex congenital heart disease, particularly in those with complex cyanotic heart disease, Eisenmenger's syndrome, after corrective surgical procedures, and in those with impaired left ventricular function. Such patients may require an implantable cardiac defibrillator (ICD), which may present a challenge to the implanting cardiologist.
Indications for Device Implantation
Many congenital cardiac defects are accompanied by abnormalities of the conduction system, either directly related to developmental abnormalities or as a consequence of the unique myocardial substrate created by large septal patches, extensive suture lines, long-standing cyanosis or abnormal pressure/volume status. Abnormalities vary from sick sinus syndrome, sino-atrial block, sinus arrest to atrio-ventricular (AV) disturbances, such as second- or third-degree AV block. Symptoms may include dizziness, syncope, heart failure or, simply, effort intolerance, lassitude or fatigue. Complete AV block carries a prognostic disadvantage and such patients require pacing whether they are symptomatic or not. Moreover, ventricular tachyarrhythmias may occur in patients with severe and complex congenital heart disease, particularly in those with complex cyanotic heart disease, Eisenmenger's syndrome, after corrective surgical procedures, and in those with impaired left ventricular function. Such patients may require an implantable cardiac defibrillator (ICD), which may present a challenge to the implanting cardiologist.
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