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J Thorac Cardiovasc Surg 2008;136:767-773
© 2008 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Outcome of high-risk neonates with congenital complete heart block paced in the first 24 hours after birth

^a The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, Pa
^b West Virginia University School of Medicine, Morgantown, WVa
^c Children's National Medical Center, Washington, DC

Received for publication October 2, 2007; revisions received February 5, 2008; accepted for publication April 11, 2008.

^_* Address for reprints: Andrew C. Glatz, MD, The Children's Hospital of Philadelphia, 2nd Floor, Main Building, 34th St and Civic Center Blvd, Philadelphia, PA 19104-4399. (Email: glatz{at}email.chop.edu).

Objective: Risk factors for poor outcome with congenital complete heart block include prematurity, low birth weight, hydrops, low ventricular rates, and congenital heart disease. In this group, medical therapy is often ineffective, pacing is technically challenging, and mortality exceeds 80%. The purpose of this study is to assess outcomes of patients with congenital complete heart block who were paced in the first 24 hours after birth owing to the presence of known risk factors.

Methods: We performed a retrospective review of patients with congenital complete heart block paced in the first 24 hours after birth at our institution between November 1, 1995, and July 31, 2007.

Results: Thirteen patients were identified, 4 of whom had heterotaxy syndrome. Eleven patients had temporary epicardial pacing wires placed; 2 received permanent pacemakers as the initial mode of pacing. There were 7 deaths (54% mortality) at a mean age of 19.9 ± 19 days. Among 7 patients with structural heart disease, there was 1 survivor. Among 6 patients with structurally normal hearts, there were 5 survivors (P = .025). Patients with temporary wires who survived to permanent pacemaker implantation (6/11) used their temporary leads for 33.8 ± 18.3 days.

Conclusions: In the severely affected fetus with congenital complete heart block and significant structural heart disease, outcomes remain poor; however, neonates with congenital complete heart block and structurally normal hearts who are monitored antenatally and delivered in a planned fashion at an institution capable of early pacing can have favorable outcomes. The use of temporary pacing wires is an option in the management of these patients.