Pacemaker Therapy in Adults With Congenital Heart Defects
Pacemaker Therapy in Adults With Congenital Heart Defects
Review the feasibility, safety and outcomes in adults with congenital heart disease who
 undergo pacemaker implantation for bradycardia, tachycardia or heart failure indications
Survival to adulthood is now common for children born with congenital heart defects (CHD).
 This improved survival is in large part due to advances in cardiac surgery over the past 4
 decades. However, few cardiac surgical repairs are curative. Many 'repaired' children with
 congenital heart defects later develop problems as adults. Arrhythmias, need for additional
 surgery, and heart failure are the more common late sequelae of congenital heart disease (1).
 In addition, many adults with CHD will need a pacemaker for sick sinus syndrome (inability of
 the inherent pacemaker of the heart to function properly resulting in bradycardia sometimes
 alternating with tachycardia), tachyarrhythmias or heart block (2, 3). Certain group of
 patients, such as those with congenital corrected transposition of the great arteries
 (CCTGA), d-transposition s/p atrial switch procedures and those with single ventricle
 physiology are at particular risk of developing heart failure (4). Attached figures show the
 anatomy of these congenital heart defect. Cardiac resynchronization therapy improves exercise
 tolerance, symptoms and reduces mortality in adults with heart failure due to acquired left
 ventricular dysfunction.(5-8). In addition, automatic internal cardioverter - defibrillators
 (ICD) have now been shown to decrease mortality in patients with low ejection fraction due to
 ischemic or non-ischemic etiologies (9, 10).

 Patients with CHD pose a challenge to traditional transvenous pacemaker lead placement due to
 either complex venous anatomy that precludes conventional percutaneous approaches for
 pacemaker implantation or occlusions of central venous form multiple prior procedures (11,
 12). As evident in the study by Janousek et.al, 7 out of 8 patients enrolled in this study
 required a thoracotomy for lead placement (4). While thoracoscopic epicardial lead placement
 has been described for placement of pacemaker leads in adults without congenital heart
 defects (13, 14), it has not been described for adult patients with congenital heart disease.
Inclusion Criteria:

 -  Congenital heart disease diagnosis

 -  Pacemaker implantation completed during the period of Jan. 1, 1996 to Dec. 1, 2005

 -  Age 18 to 80, inclusive

 Exclusion Criteria:

 -  Those who do not meet inclusion criteria