This document describes the perioperative management of a 6-month-old boy undergoing permanent pacemaker implantation for congenital complete heart block. Key aspects of management included premedication with atropine and promethazine to prevent vagal stimulation, induction with ketamine to avoid negative chronotropic effects, and maintenance with non-depressant anesthetics like isoflurane. Intraoperative monitoring and defibrillator equipment were readily available due to the risk of arrhythmias. The pacemaker implantation procedure and postoperative course were uncomplicated with this careful anesthetic approach.

1. Perioperative Management of Pacemaker Implantation for Congenital
Complete Heart Block
Sambhu N. Das, MD, and Shailaja C. Kale, MD
C ONGENITAL COMPLETE HEART block (CCHB) is
seen in 1:22,000 live births.1 It is one of the reasons for
sudden fetal and infant death syndrome.2,3 After birth, the
area. A pocket was created in the left side rectus sheath for the
pacemaker. The electrode was fixed to the anterior surface of the right
ventricle and checked for perfect pacing with different program set-
children manifest with a slow heart rate, episodes of syncope tings. The unipolar pacemaker (Medtronics 4965, 35-cm cable; Min-
(Stokes Adams syndrome), ventricular arrhythmias, and heart neapolis, MN) was set on VVI mode, a lower pacing rate of 70 per
failure.3 CCHB may be managed by isoproterenol, epinephrine, minute, amplitude of 7.5 V, and sensitivity of 2.8 mV. The wounds
and a temporary pacemaker (TP), but permanent pacemaker were closed with proper hemostasis.
implantation (PPI) is the long-term treatment.1 Intraoperatively, no bradycardia or arrhythmia was seen. The effect
Anesthesiologists rarely come across patients with CCHB, of the muscle relaxant was not reversed, and he was ventilated for 4
but when they do, they have to handle these children with hours in the intensive care unit until vital signs stabilized. The subse-
utmost care and attention. Reports of anesthetic management of quent period was uncomplicated.
CCHB for PPI are very few in number.4 Thus, the perioperative
anesthetic management of a case of CCHB for PPI is presented.
DISCUSSION
CASE HISTORY Complete heart block was first described as “impaired
A 6-month-old boy weighing 5 kg presented with CCHB. He was atrioventricular syndrome” by Morquio in 1901.5 The first
first diagnosed in utero by echocardiography at 28 weeks during a case of CCHB was reported by Plant and Steven in 1945.6 It
routine antenatal check up for fetal bradycardia. This boy, first child of may be associated with L-transposition of the great arteries,
the family, was born at 32 weeks without any cardiac or systemic ventricular septal defect, and multiple congenital anoma-
anomaly. The mother was not suffering from rheumatic heart disease, lies.3 Mothers of these children usually have a connective
systemic lupus erythematosus, and Sjogren’s syndrome. tissue disorder (ie, rheumatic disease, systemic lupus ery-
At admission to the hospital, the child was asymptomatic. The thematosus, or Sjogren’s syndrome).7,8 The maternal immu-
electrocardiogram showed atrioventricular block, ventricular rate of 37 noglobulins cross the placenta and damage the fetal cardiac
beats/min, and atrial rate of 100 beats/min (Fig 1). CCHB was recon- conduction system.9 Isolated CCHB has resulted from inde-
firmed by M-mode echocardiography of the aortic root (for ventricular pendent developmental malformation of the conduction sys-
rate) and left atrial level (for atrial rate). He was managed with an tem and fetal myocarditis.2 Although some patients may
infusion of isoproterenol (0.05-0.2 g/kg/min) to increase the heart remain asymptomatic, other infants present with congestive
rate, but no improvement was found. Subsequently, he was scheduled heart failure.4 Older children present with ventricular ar-
for elective epicardial PPI. rhythmias, decreased cardiac output, syncope, diminished
Premedication with 5 mg of promethazine syrup and 0.15 mg exercise tolerance, and fatigue.9,10 Although the ventricular
atropine were given 1 hour before surgery. In the operating room, rate is slow, the atrial rate is faster and accompanied by
continuous electrocardiogram, pulse oximeter, and noninvasive blood interruption of the atrioventricular bundle.11 M-mode echo-
pressure monitoring were established. The heart rate was 42 beats/min, cardiography is diagnostic, studying the cardiac activity at
and blood pressure 100/56 mmHg. An external pacemaker, transvenous
the aortic root level (ventricular rate) and left atrial level
pacing equipment, and electric defibrillator were kept ready. With the
(atrial rate).2 During echocardiography, the atrioventricular
caution that no anesthetic should cause any negative chronotropic
valves open and close at twice the rate of the semilunar
effect, ketamine, 10 mg, was administered via the previously placed
valves.3
venous catheter to induce anesthesia. Rocuronium, 5 mg, was used to
In this patient, the diagnosis was known in fetal life, and the
facilitate tracheal intubation. The left radial artery and a femoral vein
child was asymptomatic with hospitalization. The heart rate
were cannulated for blood pressure and central venous pressure mon-
was very slow at 37 beats/min for which epicardial pacing was
itoring. Anesthesia was maintained with oxygen in air with isoflurane,
planned.3 The mother was negative for collagen vascular anti-
pancuronium, morphine, and midazolam.
bodies, and there was no associated structural heart disease.
An incision below the xiphisternum was made and dissected until
the heart was visible. A tunnel was made up to the subrectus muscle
In the preoperative preparation, CCHB patients are to be
observed carefully for syncope, arrhythmias, and cardiac fail-
ure. For anesthesia, the use of drugs like halothane, narcotics,
From the Department of Cardiac Anesthesia, Cardiothoracic Cen- and vecuronium in combination with fentanyl or etomidate, all
tre, All India Institute of Medical Sciences, New Delhi, India. which slow nodal pacemakers or myocardial conduction,
Address reprint requests to S. C. Kale, MD, Department of Cardiac should be avoided.9 Atropine premedication may mitigate the
Anesthesia, Cardiothoracic Centre, 7th Floor, All India Institute of effects of intraoperative vagal stimulation and also should
Medical Sciences, Ansari Nagar, New Delhi, 110029 India. E-mail: precede succinylcholine or neostigmine administration.11 Ide-
shakale@hotmail.com
ally, a TP should be in place during induction of anesthesia
© 2004 Elsevier Inc. All rights reserved.
1053-0770/04/1805-0015$30.00/0 because life-threatening arrhythmias may occur at induction of
doi:10.1053/j.jvca.2004.07.014 anesthesia.9,10 Because the cardiac output entirely depends on
Key words: congenital complete heart block, pacemaker implanta- stroke volume, intravascular volume must be adequate,9 if the
tion, anesthesiologist, congenital heart disease TP fails.
628 Journal of Cardiothoracic and Vascular Anesthesia, Vol 18, No 5 (October), 2004: pp 628-629

2. PACEMAKER IMPLANTATION 629
Fig 1. ECG of congenital
complete heart block (atrioven-
tricular interruption). Ventricu-
lar rate 37 beats/min, atrial rate
100 beats/min.
Atropine and promethazine were used for premedication to managing the patient with CCHB with a very slow heart rate
counter vagal stimulation and drug-induced bradycardia. With requiring an epicardial pacemaker implantation.
adequate intravascular volume and use of ketamine, pancuro- In summary, congenital complete heart block rarely presents to
nium, isoflurane, morphine, and midazolam anesthesia, the anesthesiologists either for PPI or surgery. Early diagnosis and
authors did not face any problem. Diaz and Friesen4 used understanding of the pathophysiology of CCHB may help in
succinylcholine, halothane, fentanyl, and neostigmine with preventing sudden deaths or death during incidental surgery for
prior atropine administration in a case of CCHB for large bowel congenital anomalies. The perioperative management of PPI in-
obstruction caused by megacolon. Anesthesiologists are in- cludes the prior use of isoproterenol, epinephrine, and TP before
creasingly involved in the care of patients undergoing proce- the PPI. Atropine premedication and avoidance of anesthetics that
dures in catheterization and electrophysiologic laboratories in- slow heart rate are of prime importance, along with a defibrillator
cluding pacemaker implantation. This is good experience for and external and transvenous pacemakers in the operating room.
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