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Perioperative Management of Pacemaker Implantation for CongenitalComplete Heart Block

Sambhu N. Das, MD, and Shailaja C. Kale, MD

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ONGENITAL COMPLETE HEART block (CCHB) isseen in 1:22,000 live births.1 It is one of the reasons for

udden fetal and infant death syndrome.2,3 After birth, thehildren manifest with a slow heart rate, episodes of syncopeStokes Adams syndrome), ventricular arrhythmias, and heartailure.3 CCHB may be managed by isoproterenol, epinephrine,nd a temporary pacemaker (TP), but permanent pacemakermplantation (PPI) is the long-term treatment.1

Anesthesiologists rarely come across patients with CCHB,ut when they do, they have to handle these children withtmost care and attention. Reports of anesthetic management ofCHB for PPI are very few in number.4 Thus, the perioperativenesthetic management of a case of CCHB for PPI is presented.

CASE HISTORY

A 6-month-old boy weighing 5 kg presented with CCHB. He wasrst diagnosed in utero by echocardiography at 28 weeks during aoutine antenatal check up for fetal bradycardia. This boy, first child ofhe family, was born at 32 weeks without any cardiac or systemicnomaly. The mother was not suffering from rheumatic heart disease,ystemic lupus erythematosus, and Sjogren’s syndrome.

At admission to the hospital, the child was asymptomatic. Thelectrocardiogram showed atrioventricular block, ventricular rate of 37eats/min, and atrial rate of 100 beats/min (Fig 1). CCHB was recon-rmed by M-mode echocardiography of the aortic root (for ventricularate) and left atrial level (for atrial rate). He was managed with annfusion of isoproterenol (0.05-0.2 �g/kg/min) to increase the heartate, but no improvement was found. Subsequently, he was scheduledor elective epicardial PPI.

Premedication with 5 mg of promethazine syrup and 0.15 mgtropine were given 1 hour before surgery. In the operating room,ontinuous electrocardiogram, pulse oximeter, and noninvasive bloodressure monitoring were established. The heart rate was 42 beats/min,nd blood pressure 100/56 mmHg. An external pacemaker, transvenousacing equipment, and electric defibrillator were kept ready. With theaution that no anesthetic should cause any negative chronotropicffect, ketamine, 10 mg, was administered via the previously placedenous catheter to induce anesthesia. Rocuronium, 5 mg, was used toacilitate tracheal intubation. The left radial artery and a femoral veinere cannulated for blood pressure and central venous pressure mon-

toring. Anesthesia was maintained with oxygen in air with isoflurane,ancuronium, morphine, and midazolam.

An incision below the xiphisternum was made and dissected untilhe heart was visible. A tunnel was made up to the subrectus muscle

From the Department of Cardiac Anesthesia, Cardiothoracic Cen-re, All India Institute of Medical Sciences, New Delhi, India.

Address reprint requests to S. C. Kale, MD, Department of Cardiacnesthesia, Cardiothoracic Centre, 7th Floor, All India Institute ofedical Sciences, Ansari Nagar, New Delhi, 110029 India. E-mail:

hakale@hotmail.com© 2004 Elsevier Inc. All rights reserved.1053-0770/04/1805-0015$30.00/0doi:10.1053/j.jvca.2004.07.014Key words: congenital complete heart block, pacemaker implanta-

Tion, anesthesiologist, congenital heart disease

28 Journal of Cardiothoraci

rea. A pocket was created in the left side rectus sheath for the

acemaker. The electrode was fixed to the anterior surface of the right

entricle and checked for perfect pacing with different program set-

ings. The unipolar pacemaker (Medtronics 4965, 35-cm cable; Min-

eapolis, MN) was set on VVI mode, a lower pacing rate of 70 per

inute, amplitude of 7.5 V, and sensitivity of 2.8 mV. The wounds

ere closed with proper hemostasis.

Intraoperatively, no bradycardia or arrhythmia was seen. The effect

f the muscle relaxant was not reversed, and he was ventilated for 4

ours in the intensive care unit until vital signs stabilized. The subse-

uent period was uncomplicated.

DISCUSSION

Complete heart block was first described as “impairedtrioventricular syndrome” by Morquio in 1901.5 The firstase of CCHB was reported by Plant and Steven in 1945.6 Itay be associated with L-transposition of the great arteries,

entricular septal defect, and multiple congenital anoma-ies.3 Mothers of these children usually have a connectiveissue disorder (ie, rheumatic disease, systemic lupus ery-hematosus, or Sjogren’s syndrome).7,8 The maternal immu-oglobulins cross the placenta and damage the fetal cardiaconduction system.9 Isolated CCHB has resulted from inde-endent developmental malformation of the conduction sys-em and fetal myocarditis.2 Although some patients mayemain asymptomatic, other infants present with congestiveeart failure.4 Older children present with ventricular ar-hythmias, decreased cardiac output, syncope, diminishedxercise tolerance, and fatigue.9,10 Although the ventricularate is slow, the atrial rate is faster and accompanied bynterruption of the atrioventricular bundle.11 M-mode echo-ardiography is diagnostic, studying the cardiac activity athe aortic root level (ventricular rate) and left atrial levelatrial rate).2 During echocardiography, the atrioventricularalves open and close at twice the rate of the semilunaralves.3

In this patient, the diagnosis was known in fetal life, and thehild was asymptomatic with hospitalization. The heart rateas very slow at 37 beats/min for which epicardial pacing waslanned.3 The mother was negative for collagen vascular anti-odies, and there was no associated structural heart disease.In the preoperative preparation, CCHB patients are to be

bserved carefully for syncope, arrhythmias, and cardiac fail-re. For anesthesia, the use of drugs like halothane, narcotics,nd vecuronium in combination with fentanyl or etomidate, allhich slow nodal pacemakers or myocardial conduction,

hould be avoided.9 Atropine premedication may mitigate theffects of intraoperative vagal stimulation and also shouldrecede succinylcholine or neostigmine administration.11 Ide-lly, a TP should be in place during induction of anesthesiaecause life-threatening arrhythmias may occur at induction ofnesthesia.9,10 Because the cardiac output entirely depends ontroke volume, intravascular volume must be adequate,9 if the

P fails.

c and Vascular Anesthesia, Vol 18, No 5 (October), 2004: pp 628-629

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629PACEMAKER IMPLANTATION

Atropine and promethazine were used for premedication toounter vagal stimulation and drug-induced bradycardia. Withdequate intravascular volume and use of ketamine, pancuro-ium, isoflurane, morphine, and midazolam anesthesia, theuthors did not face any problem. Diaz and Friesen4 useduccinylcholine, halothane, fentanyl, and neostigmine withrior atropine administration in a case of CCHB for large bowelbstruction caused by megacolon. Anesthesiologists are in-reasingly involved in the care of patients undergoing proce-ures in catheterization and electrophysiologic laboratories in-

Fig 1. ECG of congenital

omplete heart block (atrioven-

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ar rate 37 beats/min, atrial rate

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anaging the patient with CCHB with a very slow heart rateequiring an epicardial pacemaker implantation.

In summary, congenital complete heart block rarely presents tonesthesiologists either for PPI or surgery. Early diagnosis andnderstanding of the pathophysiology of CCHB may help inreventing sudden deaths or death during incidental surgery forongenital anomalies. The perioperative management of PPI in-ludes the prior use of isoproterenol, epinephrine, and TP beforehe PPI. Atropine premedication and avoidance of anesthetics thatlow heart rate are of prime importance, along with a defibrillator

nd external and transvenous pacemakers in the operating room.

REFE

1. Lee C, Mason LJ: Pediatric cardiac emergencies. Anesthesiollin North Am 19:287-308, 20012. Machado MV, Tynan MJ, Curry PVL, et al: Fetal complete heart

lock. Br Heart J 60:512-515, 19883. Pinsky WW, Gillethe PC, Garson A, et al: Diagnosis, manage-ent, and long-term results of patients with congenital complete atrio-

entricular block. Pediatrics 69:728-733, 19824. Diaz JH, Friesen RH: Anesthetic management of congenital com-

lete heart block in childhood. Anesth Analg 58:334-336, 19795. Morquio L: Sur une maladie infantil et familiale caracterisee par

es modifications permanetes du pouls, des attaques syncopales etpileptiformes et la morte subite. Archives Medicine des Enfants:467-475, 19016. Plant RK, Steven RA: Complete atrioventricular block in a fetus.

CES

7. McCue CM, Mantakas ME, Tingelstad JB, et al: Congenital heartlock in newborns of mothers with connective disease. Circulation6:82-90, 19778. Paredes RA, Morgan H, Lachelin GCL: Congenital heart block

ssociated with maternal primary Sjogren’s syndrome. Br J Obstetynaecol 90:870-871, 19839. Frankville D: Anesthesia for noncardiac surgery in children and

dults with congenital heart disease, In: Lake C (ed): Pediatric Cardiacnesthesia (ed 3). Stamford, CT, Appleton & Lange, 1998, pp 616-62110. Gewitz M, Vetter V: Cardiac emergencies, in Fleisher GR,

uduig S (eds): Textbook of Pediatric Emergency Medicine (ed 4).hiladelphia, Lippincott Williams and Wilkins, 2000, pp 659-70011. Lev M, Cuadros H, Paul MH: Interruption of atrioventricular

undle with congenital atrioventricular block. Circulation 43:703-710,

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