Permanent Pacemaker Implantation in Infants, Children, and Adolescents Long-term Follow-up JAIME BENREY, M.D., PAUL C. GILLETTE, M.D., ANTOINE T. NASRALLAH, M.D., AND GRADY L. HALLMAN, M.D. SUMMARY Twenty-four patients in the pediatric age range who underwent implantation of a cardiac pacemaker for treatment of complete atrioventricular (A-V) block were followed for: an average of five years (range 1-12 years). The etiology of the A-V block was surgical in 13 cases, congenital in nine, and acquired in two. Twenty patients had symptoms of cerebrovascular insufficiency and four had congestive heart failure. To date, 18 of the 24 patients studied are
alive and well. Death occurred in six patients, five of whom had complex congenital heart defects, and one of whom had Refsum's disease. Death probably was caused by complete heart block despite pacemaker treatment in four patients, and congestive heart failure in two. In 18 of the 24 children with disabling complete A-V block, pacemaker therapy provided relief of symptoms and prolonged life.
REMARKABLE PROGRESS has been made in the management of patients with complete atrioventricular (A-V) block by artificial cardiac pacing. After the experimental work of Callaghan and Bigelow' in 1951 and the first clinical application by Zoll' in 1952, Weirich et al.3 applied direct myocardial stimulation in patients with surgically induced heart block. Subsequent improvements in pacemakers have been contributed by Glenn,4 Chardack,5 Zoll,8 and Kantrowitz.7 The increasing use of cardiac pacemakers in the treatment of patients with complete A-V block is the result of several factors: 1) improved and simplified techniques of both temporary and permanent pacing;8 2) advances in technology leading to more dependable electrodes and generators;9 3) increasing clinical experience and follow-up data indicating a favorable effect on prognosis; and 4) better cardiovascular performance in patients with A-V block treated with pacemakers rather than with chronotropic agents.'0 Despite the extensive use of pacemakers in adults, experience with children is small. Seven series reported in the literature have listed the indications for, and short-term follow-up of pacemakers in children.'",' A review of our 12 years experience (average follow-up, five years; range, 1-12 years) with implantation of cardiac pacemakers in 24 children is presented.
Luke's Episcopal Hospital, and Texas Heart Institute. There were 14 males and ten females. Indications for pacemaker implantation included symptoms of cerebrovascular insufficiency ranging from dizziness to syncope and convulsions in 20 patients, and signs and symptoms of congestive heart failure in four patients. Thirteen patients had complete A-V block as a complication of surgical repair of a congenital heart defect (table 1). Nine patients had congenital complete A-V block (table 2) and in four of them this was an associated congenital cardiac defect. Complete A-V block was "acquired" spontaneously in two patients. One of these had onset of A-V block at age nine years associated with Refsum's disease'7 (chronic polyneuropathy associated with ichthyosis, deafness, and retinitis pigmentosa secondary to lipoid storage). Atrioventricular block had occurred in another patient at 20 years of age during her second trimester of pregnancy. The resting ventricular rate in the 24 patients ranged from 31 to 85, average 46 beats/min. The QRS duration and morphology was normal in seven patients; 11 had left bundle branch block and six had right bundle branch block. Four patients exhibited an unstable cardiac pacemaker, shifting from a junctional rhythm to an idioventricular rhythm.
Materials and Methods
The 11 pulse generators implanted from 1962 through 1967 were each of the fixed-rate type. Beginning in 1968 the demand type became available and was used in the other 13 patients. The pacemaker was implanted epicardially in all patients in this series.
Pacemakers
Patients During
'the I11-year period,
from
July
1962
through
September 1973, 24 patients ranging in age from eight days to 21 years (average 9.7 years), underwent implantation of a cardiac pacemaker at the Texas Children's Hospital, St.
Results Patients
From the Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas, and the Divisions of Cardiology and Surgery, Texas Heart Institute and St. Luke's Episcopal Hospital, Houston, Texas. Supported in part by Grant HL 5756 from the National Institutes of Health, USPHS, and by USPH Grant RR-00188 from the General Clinical Research Branch, National Institutes of Health. Address for reprints: Paul C. Gillette, M.D., Texas Children's Hospital, 6621 Fannin, Houston, Texas 77025. Received June 26, 1975; revision accepted for publication September 5, 1975.
The 24 patients were followed from one year to 12 years (average five years). Six patients died (25%), five of whom had associated complex congenital heart defects and one of whom had Refsum's disease (table 3). Death was sudden and unexpected in three patients. Two patients died from heart failure despite pacing and in one patient death resulted from intractable ventricular fibrillation during the replacement of a defective pulse generator. 245
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VOL. 53, No. 2, FEBRUARY 1976
CIRCULATION
246
TAB3LE 1. Feat-tres of 1 Patients who Experienced Postsorgical Heart Block as a Complication of Surgical Repair of a Congenital Heart Defect Pt no.
1 2 3 4 5 6 7 8 9 10 11 12 13
Sex
M M 1M M M F F M M M F M F
Age (yrs)
21 12
3 13 2 20 4 5 4 17 14 20
Ventricular rate
Congenital heart lesion
Ebstein's disease VSD, subaortic st. VSI) (type 2) VSI) (type 2) VSI) (type 2) VSD (type 2) Tetralogy A-V canal Tetralogy Ostium primum Common ventricle Aortic stenosis Ostium primtlm
Surgery
Symptoms
(beats/min)
TVIt VSD clos. res. memb. VSI) closure VS D closure VSD closure VSD closure Total repair
Syncope Syncope Syncope
42 50 48 85 32 46 33 56 46 36 60 60 45
MVIR Total repair Patch closure VSD closure AVR ASI) closure
Syllcope
SyIIcope CHF Syncope CHF Syncope
Syncope Syncope Syncope Syncope
Al)breviations: ASD = atrial septal defect; AVR = aortic valve replacement; CHF = congestive heart failure; MVR mitral valve replacement; res. meml). = resection of subaortic membrane; st. = stenosis; TVR = tricuspid valve replacement; VSD = ventricular septal defect.
The nine patients with congenital complete A-V block are alive and improved by the pacemaker, as are eight of 13 patients with surgical A-V block and one patient with idiopathic A-V block which developed during pregnancy. An example of the improvement possible is that of a 9year-old girl with isolated congenital A-V block who was considered by her parents to be asymptomatic. During a treadmill exercise test she became exhausted after only one minute. After the pacemaker was inserted the parents noted increased physical activity and the child was able to exercise for nine minutes on the treadmill. Pacemakers
The average duration of pacemaker function at the completion of the follow-up study was 703 days (range 43 to 1,725 days) (table 4). In 12 patients only one unit was implanted; five of them died but in seven the initial pacemaker was functioning at the end of the study. These seven are the most recent patients in the series. Pacemaker malfunction was observed 28 times in 12 patients. The type of malfunction included: 1) failure to capture the ventricles, 2) sudden spontaneous loss of synchronization, 3) progressive decay of pacemaker rate, and 4)
non-sensing by the pacemaker of the R waves falling during the alert period. Failure of the impulse generator occurred 23 times, 87.5% of the failures. The fault in the generator was usually depletion of the battery. Each of the deteriorating pacemakers demonstrated a change in rate as failure occurred. Electrodyne units showed an increase in interval from 10 to 150 msec at the time of failure. Three of the malfunctioning Medtronic units demonstrated repetitive firing; fortunately in these cases the impulse was below threshold for tachyarrhythmias. Other arrhythmias included premature ventricular contractions in five patients (escape bigeminy), paroxysmal ventricular tachycardia in three, and ventricular fibrillation in two. Wire fractures, loss of contact between wire and wire in splices and between wires and metal adaptors, and dislodgement of the electrode occurred five times, 12.5% of the late failure of pacemakers in this group of patients (table 4). Discussion Reports of permanent cardiac pacing in children have been infrequent. Several reasons have been offered to explain the paucity of such cases in the literature: 1) the in-
TABLE 2. Features of Nine Patients with Congenital Heart Block Pt no.
Sex
Age (yrs)
Congenital heart lesion
14
M
13
TGA
15
M
10
VSD,PS
16
M
1
CTGA
17
M
3
VSD, PS, ASD
18 19 20 21
F F F F F
22
10 4 8 days 9 10
None None None None None
Ventri cular rate
Surgery
Symptoms
(beats/min)
W/C shunt PM impl. VSD, PV PM impl.
Syncope
50
CHF
52
PA banding PM impl. Total correction PM impl. PM impl. PM impl. PM impl. PM impl. PM impl.
Syncope
52
Syncope
43
Syncope Syncope CHF Syncope Syncope
50 52 32 41 30
Abbreviations: ASD = atrial septal defect; CHF = congestive heart failure; CTGA = corrected transposition of the great arteries; = pulmonary artery; PM impl. = pacemaker implantation; PS = pulmonic stenosis; PV = pulmonary valvotomy; TGA = transposition of the great arteries; VSD = ventricular septal defect; WV/C = WVaterston-Cooley shunt.
PA
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PACEMAKERS IN INFANTS AND CHILDREN/Benrey et al.
247
TABLE 3. Features and Cause of Death in Six Patents Pt
Aae
no.
Sex
(yrs)
1 14 15 2
M M M M
21 13 10 12
16 23
M M
1 13
Congenital heart lesion
Ebsteiii's d. TGA Tetralogy VSI), subaortic stenosis CTGA Refsum's d.
Type of heart
Sturgery
block Surgical Congenital Surgical Surgical
TVIt W/C shunt Total repair VSI) closure, res. memb. PA banding None
Survival
Congenital Acquired
(days)
Cause of death
43 70 1342 466
CHF Sudden death CHF Sudden death
137
1533
Sudden death V. Fib.
Ahl)reviations: CHF = congestive lheart failure; CTGA = corrected transposition of the great arteries; d. = disease; PA = pulmonarv artery; res. meml). = resection of subaortic membrane; TGA = transposition of the great arteries: TVR = tricuspid valve replacement; V. Fil). = ventricular fibrillation; VSD = ventricular septa! defect; \V/C = Waterston-Cooley shunt.
cidence of complete heart block in children is lower than in adults,'4 2) most patients with congenital complete heart block tend to do well and do not experience symptoms or major difficulty,'18' and 3) long-term pacing in infants and children is difficult because of the complications that arise from the small size of the patient compared to the size of the pacemaker." The anticipated need for multiple operations to replace batteries or wires also discourages the use of pacemakers in children. We did not find that the pacemaker upset the family life. Our patients averaged one hospitalization for one week per two years. The prognosis in children with complete A-V block is related to the presence or absence of associated cardiovascular defects,'8 stability of the cardiac pacemaker,'8 and the etiology of the disease process. Although Stokes-Adams attacks are uncommon in children with congenital complete A-V block, in cases when they have occurred many have been fatal.20"28 According to Scarpelli and Rudolph,27 Stokes-Adams attacks are the most common mechanism of death in children with complete A-V block. In a collective series reported by Michaelsson and Engle,28
nine of 418 (2%) patients with congenital complete A-V block died suddenly. Five deaths occurred in patients who had experienced previous episodes of dizziness or syncope but did not have a pacemaker implanted. Since all of our symptomatic patients with congenital complete A-V block who required a pacemaker are alive and well, we believe pacemakers have improved their prognosis. Complete A-V block following surgical repair of intracardiac malformations is a serious complication. The prognosis of surgical complete A-V block is almost always poor. In a series reported by Squarcia et al.29 seven patients of 911 had persistent complete heart block after leaving the operating room, five of whom died within 24 hours after surgery and the remaining two died soon after discharge. Ten of our 13 patients with surgical complete A-V block are alive. Thus the prognosis of surgical complete A-V block can be significantly improved by the use of pacemakers. Acquired nonsurgical complete A-V block was observed in a patient with Refsum's disease in our series who exhibited syncope. Patients with Refsum's disease are prone to sudden death;'0 to our knowledge no case of complete A-V block in a patient with Refsum's disease has been documented previously. The newborn period is a vulnerable time for death, especially in children with congenital third degree A-V block. Reports of mortality occurring in the newborn period sug-
gest that such infants with ventricular rates of less than 50 per minute are the most likely to succumb.21-23 An example is illustrated by our youngest case, an eight-day-old newborn who was admitted to Texas Children's Hospital with a ventricular rate of 32 per minute and signs of severe congestive heart failure. After successful pacemaker implantation, the child's progress has been good. The congestive heart failure cleared promptly and subsequent development of the infant has been normal. Initial management of the infant or child with complete heart block, in whom pacing is indicated, usually involves insertion of a transvenous intracardiac electrode into the right ventricle and stimulation of the heart from an external battery-operated pulse generator. This technique protects the patient from serious arrhythmias occurring during the administration of anesthesia. In the management of a pacemaker which has failed, a temporary transvenous pacemaker should be placed before surgery. In a pacemaker which is threatening to fail, i.e., the TABLE 4. Battery Replacement in 12 Patients Pt
Battery change
no.
3
1 2 3 4
5 6 7 8 1 1 2 3 1
6 4
7 8
1
9
1
17
1 2 3 4 1 2 3 1 1
24
12 5
2 3
13 23
PM
1 1 =
Cause
PM failure PM failure Runaway PM Broken wire Infection PM failure PM failure PM failure PM failure PM failure PM failure PM failure PM failure PM failure Infection PM failure PM failure PM failure PM failure PM failure PM failure PM failure Runaway PM Broken wire PM failure Runaway PM Broken wire PM failure
pacemaker.
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Duration
(days)
320 452 538 712 243 464 148 790 760 435 769 1021 1155 1030 373 790 573 540 816 828 930 1020 970 122 1285 635 1138 1535
Type of pacemaker
Electrodyne Atricor
Electrodyne Atricor Atricor Atricor Medtronic Medtronic Gen. Electric Electrodyne Electrodyne Cordis Gen. Electric Medtronic Medtronic
Electrodyne Electrodyne Medtronic Medtronic Cordis Medtronic
Nuclear PM Medtronic Electrodyne Gen. Electric Medtronic Medtronic Medtronic
248
CIRCULATION
rate is decreasing, it is usually not necessary to use a temporary pacemaker. We have found that by using regular check-ups in a pacemaker clinic, and telephone checks, emergency pacemaker changes can be largely avoided. The results of our 12-year-experience with long-term pacing in 24 children with complete A-V block are good, not only in extending life but also in relieving symptoms. Because of the increasing reliability of the generator units, we believe a permanent cardiac pacemaker should be used in the young patient who has symptoms. Based on the above data and those from the literature, we have developed the following criteria for implantation of a permanent pacemaker in children, of which any one of the following is an indication for implantation: 1) Surgical A-V block after two weeks 2) Stokes-Adams attack in any patient with complete A-V block 3) Congestive heart failure in a patient with complete A-V block 4) Moderately severe exercise intolerance 5) A ventricular rate less than 50 beats/min in a newborn 6) An atrial rate greater than 150 beats/min in a newborn.
References 1. Callahan JC, Bigelow WG: An electrical artificial pacemaker for standstill of the heart. Am Surg 134:8, 1951 2. Zoll PM: Resuscitation of the heart in ventricular standstill by external electric stimulation. N Engl J Med 247:768, 1952 3. Weirich WL, Gott VL, Lillehei CW: The treatment of complete heart block by the continued use of a myocardial electrode and an artificial pacemaker. Surg Forum 8:360, 1957 4. Glenn WWL, Mauro A, Longo E, Lavretes PH, Mackay FJ: Remote stimulation of the heart by radio frequency transmission. N Engl J Med 216:948, 1959 5. Chardack WM, Gage AA, Greatbach W: Correction of complete heart block by a self-contained and subcutaneously implanted pacemaker: Clinical experience with 15 patients. J Thorac Cardiovasc Surg 42:814, 1961 6. Zoll PM, Frank HA, Zarsky LRN, Linenthal AJ, Belgard AH: Longterm electric stimulation of the heart for Stokes-Adams disease. Am J Surg 154:330, 1961 7. Kantrowitz A, Cohen R, Raillard H, Schmidt J, Feldman DS: The treatment of complete heart block with an implanted, controllable pacemaker. Surg Gynecol Obstet 115:415, 1962 8. Chardack WM, Gage A, Frederico AJ, Schmert GI, Greatbach W: The long-term treatment of heart block. Prog Cardiovasc Dis 9:105, 1966
VOL. 53, No. 2, FEBRUARY 1976
9. Gadboys HG, Lubkan S, Litwak RS: Long-term follow-up of patients with cardiac pacemaker. Am J Cardiol 21:55, 1968 10. Dack S, Donoso E: Heart block with Stokes-Adams syndrome: Predications and results of cardiac pacing. Ann N Y Acad Sci 167:519, 1 969 11. Liu L, Griffiths P, Gest PH: Implanted pacemaker in children: A report on their application in five patients. Am J Cardiol 20:639, 1967 12. Trusler GA, Mustard WT, Keith JD: The role of pacemaker therapy in congenital complete heart block: Report of three cases. J Thorac Cardiovasc Surg 55:105, 1968 13. Martin MV: Implantation of a Chardack-Greatbach adjustable rate and current pacemaker in a 4 month old infant. Pediatrics 37:323, 1966 14. Glenn WL, de Leuchlenberg N, Van Heeckeven DW, Sato G, Holcomb WA, Paisson K: Heart block in children. J Thorac Cardiovasc Surg 58: 361, 1969 15. Mouguin M, Maysse J, Duraund M, Lauvent D, Sprovior K: Implantation d'un stimulatent interne pour correction d'un bloc auriculoventriculare chiriogucal chez une enfant de 7 ans. Arch Neal Cocur 55: 241, 1962 16. Kangor JJ, Griffiths SP, Blumenthal S: Congenital complete heart block. Am J Cardiol 20:632, 1967 17. Steinberg D, Vroom FQ, Engel WK, Cammermeyer J, Mize CE, Avigan J: Refsum's disease: A recently characterized lipidosis involving the nervous system. Ann Intern Med 66:365, 1967 18. Ayers CR, Boineau JP, Spach MS: Congenital complete heart block in children. Am Heart J 72:381, 1966 19. Campbell M, Emanuel R: Six cases of congenital complete heart block followed for 34-40 years. Br Heart J 29:577, 1967 20. Nakamura FF, Nadas AS: Complete heart block in infants and children. N Engl J Med 270:1261, 1964 21. Moltham ME, Miller RM, Hastreiter AR, Paul MH: Congenital heart block with fatal Adams-Stokes attacks in childhood. Pediatrics 30:32, 1962 22. Wallgren A, Winlslad A: Congenital heart block. Acta Paediat 20:175, 1937 23. Crittenden IH, Latta H, Ticinovich DA: Familial congenital heart block. Am J Dis Child 108:104, 1964 24. Landtman B, Londer E, Hjelt L, Tuteri L: Congenital complete heart block. Ann Paediat Fenn 10:99, 1964 25. Brested JH: Edwin Smith surgical papyrus. In Facsimile and Hieroglyphic Transliteration with Translation and Commentary, 2 vols. Chicago, The University of Chicago Oriental Institute Publications, 1930, p 372 26. Katz LN, Shaffer AB: The effect of cardiac arrhythmias on the performance of the heart: In Mechanisms and Therapy of Cardiac Arrhythmias, edited by Dreyfus LS and Likoff W. New York, Grune and Stratton, l966, p 28 27. Scarpelli EM, Rudolph AM: The hemodynayiics of congenital heart block. Prog Cardiovasc Dis 6:327, 1964 28. Michaelsson M, Engle MA: Congenital Complete Heart Block, An International Study of the Natural History. In Pediatric Cardiology, vol. 4, no. 3, edited by Brest AN, Engle MA (guest ed). Philadelphia, F. A. Davis Company, 1972, p 86 29. Squarcia U, Merideth J, McGoon DC, Weidman WH: Prognosis of transient atrioventricular conduction disturbances complicating open heart surgery for congenital heart defects. Am J Cardiol 28:648, 1971
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Permanent pacemaker implantation in infants, children, and adolescents. Long-term follow-up. J Benrey, P C Gillette, A T Nasrallah and G L Hallman Circulation. 1976;53:245-248 doi: 10.1161/01.CIR.53.2.245 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1976 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539
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alive and well. Death occurred in six patients, five of whom had complex congenital heart defects, and one of whom had Refsum's disease. Death probably was caused by complete heart block despite pacemaker treatment in four patients, and congestive heart failure in two. In 18 of the 24 children with disabling complete A-V block, pacemaker therapy provided relief of symptoms and prolonged life.
REMARKABLE PROGRESS has been made in the management of patients with complete atrioventricular (A-V) block by artificial cardiac pacing. After the experimental work of Callaghan and Bigelow' in 1951 and the first clinical application by Zoll' in 1952, Weirich et al.3 applied direct myocardial stimulation in patients with surgically induced heart block. Subsequent improvements in pacemakers have been contributed by Glenn,4 Chardack,5 Zoll,8 and Kantrowitz.7 The increasing use of cardiac pacemakers in the treatment of patients with complete A-V block is the result of several factors: 1) improved and simplified techniques of both temporary and permanent pacing;8 2) advances in technology leading to more dependable electrodes and generators;9 3) increasing clinical experience and follow-up data indicating a favorable effect on prognosis; and 4) better cardiovascular performance in patients with A-V block treated with pacemakers rather than with chronotropic agents.'0 Despite the extensive use of pacemakers in adults, experience with children is small. Seven series reported in the literature have listed the indications for, and short-term follow-up of pacemakers in children.'",' A review of our 12 years experience (average follow-up, five years; range, 1-12 years) with implantation of cardiac pacemakers in 24 children is presented.
Luke's Episcopal Hospital, and Texas Heart Institute. There were 14 males and ten females. Indications for pacemaker implantation included symptoms of cerebrovascular insufficiency ranging from dizziness to syncope and convulsions in 20 patients, and signs and symptoms of congestive heart failure in four patients. Thirteen patients had complete A-V block as a complication of surgical repair of a congenital heart defect (table 1). Nine patients had congenital complete A-V block (table 2) and in four of them this was an associated congenital cardiac defect. Complete A-V block was "acquired" spontaneously in two patients. One of these had onset of A-V block at age nine years associated with Refsum's disease'7 (chronic polyneuropathy associated with ichthyosis, deafness, and retinitis pigmentosa secondary to lipoid storage). Atrioventricular block had occurred in another patient at 20 years of age during her second trimester of pregnancy. The resting ventricular rate in the 24 patients ranged from 31 to 85, average 46 beats/min. The QRS duration and morphology was normal in seven patients; 11 had left bundle branch block and six had right bundle branch block. Four patients exhibited an unstable cardiac pacemaker, shifting from a junctional rhythm to an idioventricular rhythm.
Materials and Methods
The 11 pulse generators implanted from 1962 through 1967 were each of the fixed-rate type. Beginning in 1968 the demand type became available and was used in the other 13 patients. The pacemaker was implanted epicardially in all patients in this series.
Pacemakers
Patients During
'the I11-year period,
from
July
1962
through
September 1973, 24 patients ranging in age from eight days to 21 years (average 9.7 years), underwent implantation of a cardiac pacemaker at the Texas Children's Hospital, St.
Results Patients
From the Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas, and the Divisions of Cardiology and Surgery, Texas Heart Institute and St. Luke's Episcopal Hospital, Houston, Texas. Supported in part by Grant HL 5756 from the National Institutes of Health, USPHS, and by USPH Grant RR-00188 from the General Clinical Research Branch, National Institutes of Health. Address for reprints: Paul C. Gillette, M.D., Texas Children's Hospital, 6621 Fannin, Houston, Texas 77025. Received June 26, 1975; revision accepted for publication September 5, 1975.
The 24 patients were followed from one year to 12 years (average five years). Six patients died (25%), five of whom had associated complex congenital heart defects and one of whom had Refsum's disease (table 3). Death was sudden and unexpected in three patients. Two patients died from heart failure despite pacing and in one patient death resulted from intractable ventricular fibrillation during the replacement of a defective pulse generator. 245
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VOL. 53, No. 2, FEBRUARY 1976
CIRCULATION
246
TAB3LE 1. Feat-tres of 1 Patients who Experienced Postsorgical Heart Block as a Complication of Surgical Repair of a Congenital Heart Defect Pt no.
1 2 3 4 5 6 7 8 9 10 11 12 13
Sex
M M 1M M M F F M M M F M F
Age (yrs)
21 12
3 13 2 20 4 5 4 17 14 20
Ventricular rate
Congenital heart lesion
Ebstein's disease VSD, subaortic st. VSI) (type 2) VSI) (type 2) VSI) (type 2) VSD (type 2) Tetralogy A-V canal Tetralogy Ostium primum Common ventricle Aortic stenosis Ostium primtlm
Surgery
Symptoms
(beats/min)
TVIt VSD clos. res. memb. VSI) closure VS D closure VSD closure VSD closure Total repair
Syncope Syncope Syncope
42 50 48 85 32 46 33 56 46 36 60 60 45
MVIR Total repair Patch closure VSD closure AVR ASI) closure
Syllcope
SyIIcope CHF Syncope CHF Syncope
Syncope Syncope Syncope Syncope
Al)breviations: ASD = atrial septal defect; AVR = aortic valve replacement; CHF = congestive heart failure; MVR mitral valve replacement; res. meml). = resection of subaortic membrane; st. = stenosis; TVR = tricuspid valve replacement; VSD = ventricular septal defect.
The nine patients with congenital complete A-V block are alive and improved by the pacemaker, as are eight of 13 patients with surgical A-V block and one patient with idiopathic A-V block which developed during pregnancy. An example of the improvement possible is that of a 9year-old girl with isolated congenital A-V block who was considered by her parents to be asymptomatic. During a treadmill exercise test she became exhausted after only one minute. After the pacemaker was inserted the parents noted increased physical activity and the child was able to exercise for nine minutes on the treadmill. Pacemakers
The average duration of pacemaker function at the completion of the follow-up study was 703 days (range 43 to 1,725 days) (table 4). In 12 patients only one unit was implanted; five of them died but in seven the initial pacemaker was functioning at the end of the study. These seven are the most recent patients in the series. Pacemaker malfunction was observed 28 times in 12 patients. The type of malfunction included: 1) failure to capture the ventricles, 2) sudden spontaneous loss of synchronization, 3) progressive decay of pacemaker rate, and 4)
non-sensing by the pacemaker of the R waves falling during the alert period. Failure of the impulse generator occurred 23 times, 87.5% of the failures. The fault in the generator was usually depletion of the battery. Each of the deteriorating pacemakers demonstrated a change in rate as failure occurred. Electrodyne units showed an increase in interval from 10 to 150 msec at the time of failure. Three of the malfunctioning Medtronic units demonstrated repetitive firing; fortunately in these cases the impulse was below threshold for tachyarrhythmias. Other arrhythmias included premature ventricular contractions in five patients (escape bigeminy), paroxysmal ventricular tachycardia in three, and ventricular fibrillation in two. Wire fractures, loss of contact between wire and wire in splices and between wires and metal adaptors, and dislodgement of the electrode occurred five times, 12.5% of the late failure of pacemakers in this group of patients (table 4). Discussion Reports of permanent cardiac pacing in children have been infrequent. Several reasons have been offered to explain the paucity of such cases in the literature: 1) the in-
TABLE 2. Features of Nine Patients with Congenital Heart Block Pt no.
Sex
Age (yrs)
Congenital heart lesion
14
M
13
TGA
15
M
10
VSD,PS
16
M
1
CTGA
17
M
3
VSD, PS, ASD
18 19 20 21
F F F F F
22
10 4 8 days 9 10
None None None None None
Ventri cular rate
Surgery
Symptoms
(beats/min)
W/C shunt PM impl. VSD, PV PM impl.
Syncope
50
CHF
52
PA banding PM impl. Total correction PM impl. PM impl. PM impl. PM impl. PM impl. PM impl.
Syncope
52
Syncope
43
Syncope Syncope CHF Syncope Syncope
50 52 32 41 30
Abbreviations: ASD = atrial septal defect; CHF = congestive heart failure; CTGA = corrected transposition of the great arteries; = pulmonary artery; PM impl. = pacemaker implantation; PS = pulmonic stenosis; PV = pulmonary valvotomy; TGA = transposition of the great arteries; VSD = ventricular septal defect; WV/C = WVaterston-Cooley shunt.
PA
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PACEMAKERS IN INFANTS AND CHILDREN/Benrey et al.
247
TABLE 3. Features and Cause of Death in Six Patents Pt
Aae
no.
Sex
(yrs)
1 14 15 2
M M M M
21 13 10 12
16 23
M M
1 13
Congenital heart lesion
Ebsteiii's d. TGA Tetralogy VSI), subaortic stenosis CTGA Refsum's d.
Type of heart
Sturgery
block Surgical Congenital Surgical Surgical
TVIt W/C shunt Total repair VSI) closure, res. memb. PA banding None
Survival
Congenital Acquired
(days)
Cause of death
43 70 1342 466
CHF Sudden death CHF Sudden death
137
1533
Sudden death V. Fib.
Ahl)reviations: CHF = congestive lheart failure; CTGA = corrected transposition of the great arteries; d. = disease; PA = pulmonarv artery; res. meml). = resection of subaortic membrane; TGA = transposition of the great arteries: TVR = tricuspid valve replacement; V. Fil). = ventricular fibrillation; VSD = ventricular septa! defect; \V/C = Waterston-Cooley shunt.
cidence of complete heart block in children is lower than in adults,'4 2) most patients with congenital complete heart block tend to do well and do not experience symptoms or major difficulty,'18' and 3) long-term pacing in infants and children is difficult because of the complications that arise from the small size of the patient compared to the size of the pacemaker." The anticipated need for multiple operations to replace batteries or wires also discourages the use of pacemakers in children. We did not find that the pacemaker upset the family life. Our patients averaged one hospitalization for one week per two years. The prognosis in children with complete A-V block is related to the presence or absence of associated cardiovascular defects,'8 stability of the cardiac pacemaker,'8 and the etiology of the disease process. Although Stokes-Adams attacks are uncommon in children with congenital complete A-V block, in cases when they have occurred many have been fatal.20"28 According to Scarpelli and Rudolph,27 Stokes-Adams attacks are the most common mechanism of death in children with complete A-V block. In a collective series reported by Michaelsson and Engle,28
nine of 418 (2%) patients with congenital complete A-V block died suddenly. Five deaths occurred in patients who had experienced previous episodes of dizziness or syncope but did not have a pacemaker implanted. Since all of our symptomatic patients with congenital complete A-V block who required a pacemaker are alive and well, we believe pacemakers have improved their prognosis. Complete A-V block following surgical repair of intracardiac malformations is a serious complication. The prognosis of surgical complete A-V block is almost always poor. In a series reported by Squarcia et al.29 seven patients of 911 had persistent complete heart block after leaving the operating room, five of whom died within 24 hours after surgery and the remaining two died soon after discharge. Ten of our 13 patients with surgical complete A-V block are alive. Thus the prognosis of surgical complete A-V block can be significantly improved by the use of pacemakers. Acquired nonsurgical complete A-V block was observed in a patient with Refsum's disease in our series who exhibited syncope. Patients with Refsum's disease are prone to sudden death;'0 to our knowledge no case of complete A-V block in a patient with Refsum's disease has been documented previously. The newborn period is a vulnerable time for death, especially in children with congenital third degree A-V block. Reports of mortality occurring in the newborn period sug-
gest that such infants with ventricular rates of less than 50 per minute are the most likely to succumb.21-23 An example is illustrated by our youngest case, an eight-day-old newborn who was admitted to Texas Children's Hospital with a ventricular rate of 32 per minute and signs of severe congestive heart failure. After successful pacemaker implantation, the child's progress has been good. The congestive heart failure cleared promptly and subsequent development of the infant has been normal. Initial management of the infant or child with complete heart block, in whom pacing is indicated, usually involves insertion of a transvenous intracardiac electrode into the right ventricle and stimulation of the heart from an external battery-operated pulse generator. This technique protects the patient from serious arrhythmias occurring during the administration of anesthesia. In the management of a pacemaker which has failed, a temporary transvenous pacemaker should be placed before surgery. In a pacemaker which is threatening to fail, i.e., the TABLE 4. Battery Replacement in 12 Patients Pt
Battery change
no.
3
1 2 3 4
5 6 7 8 1 1 2 3 1
6 4
7 8
1
9
1
17
1 2 3 4 1 2 3 1 1
24
12 5
2 3
13 23
PM
1 1 =
Cause
PM failure PM failure Runaway PM Broken wire Infection PM failure PM failure PM failure PM failure PM failure PM failure PM failure PM failure PM failure Infection PM failure PM failure PM failure PM failure PM failure PM failure PM failure Runaway PM Broken wire PM failure Runaway PM Broken wire PM failure
pacemaker.
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Duration
(days)
320 452 538 712 243 464 148 790 760 435 769 1021 1155 1030 373 790 573 540 816 828 930 1020 970 122 1285 635 1138 1535
Type of pacemaker
Electrodyne Atricor
Electrodyne Atricor Atricor Atricor Medtronic Medtronic Gen. Electric Electrodyne Electrodyne Cordis Gen. Electric Medtronic Medtronic
Electrodyne Electrodyne Medtronic Medtronic Cordis Medtronic
Nuclear PM Medtronic Electrodyne Gen. Electric Medtronic Medtronic Medtronic
248
CIRCULATION
rate is decreasing, it is usually not necessary to use a temporary pacemaker. We have found that by using regular check-ups in a pacemaker clinic, and telephone checks, emergency pacemaker changes can be largely avoided. The results of our 12-year-experience with long-term pacing in 24 children with complete A-V block are good, not only in extending life but also in relieving symptoms. Because of the increasing reliability of the generator units, we believe a permanent cardiac pacemaker should be used in the young patient who has symptoms. Based on the above data and those from the literature, we have developed the following criteria for implantation of a permanent pacemaker in children, of which any one of the following is an indication for implantation: 1) Surgical A-V block after two weeks 2) Stokes-Adams attack in any patient with complete A-V block 3) Congestive heart failure in a patient with complete A-V block 4) Moderately severe exercise intolerance 5) A ventricular rate less than 50 beats/min in a newborn 6) An atrial rate greater than 150 beats/min in a newborn.
References 1. Callahan JC, Bigelow WG: An electrical artificial pacemaker for standstill of the heart. Am Surg 134:8, 1951 2. Zoll PM: Resuscitation of the heart in ventricular standstill by external electric stimulation. N Engl J Med 247:768, 1952 3. Weirich WL, Gott VL, Lillehei CW: The treatment of complete heart block by the continued use of a myocardial electrode and an artificial pacemaker. Surg Forum 8:360, 1957 4. Glenn WWL, Mauro A, Longo E, Lavretes PH, Mackay FJ: Remote stimulation of the heart by radio frequency transmission. N Engl J Med 216:948, 1959 5. Chardack WM, Gage AA, Greatbach W: Correction of complete heart block by a self-contained and subcutaneously implanted pacemaker: Clinical experience with 15 patients. J Thorac Cardiovasc Surg 42:814, 1961 6. Zoll PM, Frank HA, Zarsky LRN, Linenthal AJ, Belgard AH: Longterm electric stimulation of the heart for Stokes-Adams disease. Am J Surg 154:330, 1961 7. Kantrowitz A, Cohen R, Raillard H, Schmidt J, Feldman DS: The treatment of complete heart block with an implanted, controllable pacemaker. Surg Gynecol Obstet 115:415, 1962 8. Chardack WM, Gage A, Frederico AJ, Schmert GI, Greatbach W: The long-term treatment of heart block. Prog Cardiovasc Dis 9:105, 1966
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9. Gadboys HG, Lubkan S, Litwak RS: Long-term follow-up of patients with cardiac pacemaker. Am J Cardiol 21:55, 1968 10. Dack S, Donoso E: Heart block with Stokes-Adams syndrome: Predications and results of cardiac pacing. Ann N Y Acad Sci 167:519, 1 969 11. Liu L, Griffiths P, Gest PH: Implanted pacemaker in children: A report on their application in five patients. Am J Cardiol 20:639, 1967 12. Trusler GA, Mustard WT, Keith JD: The role of pacemaker therapy in congenital complete heart block: Report of three cases. J Thorac Cardiovasc Surg 55:105, 1968 13. Martin MV: Implantation of a Chardack-Greatbach adjustable rate and current pacemaker in a 4 month old infant. Pediatrics 37:323, 1966 14. Glenn WL, de Leuchlenberg N, Van Heeckeven DW, Sato G, Holcomb WA, Paisson K: Heart block in children. J Thorac Cardiovasc Surg 58: 361, 1969 15. Mouguin M, Maysse J, Duraund M, Lauvent D, Sprovior K: Implantation d'un stimulatent interne pour correction d'un bloc auriculoventriculare chiriogucal chez une enfant de 7 ans. Arch Neal Cocur 55: 241, 1962 16. Kangor JJ, Griffiths SP, Blumenthal S: Congenital complete heart block. Am J Cardiol 20:632, 1967 17. Steinberg D, Vroom FQ, Engel WK, Cammermeyer J, Mize CE, Avigan J: Refsum's disease: A recently characterized lipidosis involving the nervous system. Ann Intern Med 66:365, 1967 18. Ayers CR, Boineau JP, Spach MS: Congenital complete heart block in children. Am Heart J 72:381, 1966 19. Campbell M, Emanuel R: Six cases of congenital complete heart block followed for 34-40 years. Br Heart J 29:577, 1967 20. Nakamura FF, Nadas AS: Complete heart block in infants and children. N Engl J Med 270:1261, 1964 21. Moltham ME, Miller RM, Hastreiter AR, Paul MH: Congenital heart block with fatal Adams-Stokes attacks in childhood. Pediatrics 30:32, 1962 22. Wallgren A, Winlslad A: Congenital heart block. Acta Paediat 20:175, 1937 23. Crittenden IH, Latta H, Ticinovich DA: Familial congenital heart block. Am J Dis Child 108:104, 1964 24. Landtman B, Londer E, Hjelt L, Tuteri L: Congenital complete heart block. Ann Paediat Fenn 10:99, 1964 25. Brested JH: Edwin Smith surgical papyrus. In Facsimile and Hieroglyphic Transliteration with Translation and Commentary, 2 vols. Chicago, The University of Chicago Oriental Institute Publications, 1930, p 372 26. Katz LN, Shaffer AB: The effect of cardiac arrhythmias on the performance of the heart: In Mechanisms and Therapy of Cardiac Arrhythmias, edited by Dreyfus LS and Likoff W. New York, Grune and Stratton, l966, p 28 27. Scarpelli EM, Rudolph AM: The hemodynayiics of congenital heart block. Prog Cardiovasc Dis 6:327, 1964 28. Michaelsson M, Engle MA: Congenital Complete Heart Block, An International Study of the Natural History. In Pediatric Cardiology, vol. 4, no. 3, edited by Brest AN, Engle MA (guest ed). Philadelphia, F. A. Davis Company, 1972, p 86 29. Squarcia U, Merideth J, McGoon DC, Weidman WH: Prognosis of transient atrioventricular conduction disturbances complicating open heart surgery for congenital heart defects. Am J Cardiol 28:648, 1971
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Permanent pacemaker implantation in infants, children, and adolescents. Long-term follow-up. J Benrey, P C Gillette, A T Nasrallah and G L Hallman Circulation. 1976;53:245-248 doi: 10.1161/01.CIR.53.2.245 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1976 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539
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