Abstract
Adults and children have similarities as well as differences in their ventricular arrhythmias. The most similar are the definition of the arrhythmias; although the rate of ventricular tachycardia in an infant may be as high as 500/minute with a QRS duration of 40 msec. Also similar are the techniques of intracardiac electrophysiology study. However, there are major differences in the etiology, prognosis and treatment of ventricular arrhythmias in children. There are four major areas of difference. 1) Idiopathic incessant ventricular tachycardia occurs in nfants less than the age of three years. It does not respond to conventional investigational antiarrhythmic agents. The majority of patients have Purkinje cell tumors causing the tachycardia. These tumors cannot be detected by echocardiography or angiography. Electrophysiologically directed surgery is curative. 2) In children with ventricular tachycardia who are thought to have an otherwise normal heart, the heart is usually not ‘normal’. When anatomic and hemodynamic cardiac catheterization is performed in these patients, in 70% either arrhythmogenic right ventricle or congestive cardiomyopathy is found. If the heart is truly normal, the prognosis is excellent. On the other hand, children with arrhythmogenic right ventricle or congestive cardiomyopathy, especially those who have exercise-related ventricular tachycardia, may die suddenly. 3) The idiopathic long QT syndrome occurs more commonly in children than adults. Sudden death may occur without preceding symptoms and treatment is recommended for even the asymptomatic child who has the diagnosis of prolonged QT syndome (in addition to the long QT interval, the diagnosis of the syndrome requires either a positive family history, bradycardia, deafness, or typical T wave morphology). 4) Patients who have had repair of congenital heart disease die suddenly due to ventricular arrhythmias. Treatment of these arrhythmias based on results of the Holter have resulted in a significant decrease in morality. The drugs that are successful in the treatment of arrhythmias in these patients (phenytoin, mexiletine) are different from the traditionally effective drugs in adults with ischemic heart disease. This observation has led to the hypothesis that the cellular mechanism of the initiating ventricular arrhythmias in patients after repair of congenital heart disease (triggered activity) may be different from the mechanism in those with ischemic heart disease (reentry). This hypothesis is extended to other patients with the common findings of ventriculr hypertrophy, cellular disarray and fibrosis such as aortic stenosis/aortic insufficiency, hypertrophic cardiomyopathy and systemic arterial hypertension. This hypothesis applies to all patients regardless of their age, perphaps pointing out a major similarity in arrhythmia mechanism. Therefore, the differences between children and adults may be less than is immediately apparent.
Dr. Garson is an Established Investigator of the American Heart Association, Dallas, Texas
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Reference
Garson A, Gillette PC, Titus JL, Hawkins EP, Kearney D, Ott D, Cooley DA, McNamara DG. 1984. Surgical treatment of ventricular tachycardia in infants. N Engl J Med 310:1443–45.
Gillette PC, Smith RT, Garson A, Mullins CE, Gutgesell HP, Goh TH, Cooley DA, McNamara DG. 1985. Chronic supraventricular tachycardia: A curable cause of congestive cardiomyopathy. J Am Med Assoc 253: 391–92.
Garson A, Gillette PC, McNamara DG. 1981. Supraventricular tachycardia in children: Clinical features, response to treatment and long-term follow-up in 217 patients. J Pediatr 98: 875–82.
James TN, Beeson CW, Sherman EB. 1975. De Subitaneis Mortibus: XIII Multifocal Purkinje cell tumors of the heart. Circulation 52: 333–44.
Ferrans VJ, McAllister HA, Haese WH. 1976. Infantile cardiomyopathy with histiocytoid change in cardiac muscle cells: Report of six patients. Circulation 53: 708–19.
Jacobsen J, Garson A, Gillette PC, McNamara DG. 1978. Premature ventricular contractions in normal children. J Pediatr 92: 36–8.
Deal BJ, Miller SM, Prechel D, Scagliotti D, Gallastegui L. 1985. Ventricular tachycardia in the young without overt heart disease. Circulation 72 (Suppl III): 340.
Dunnigan A, Pierpont ME, Smith SA, Breningstall G, Benditt DG, Benson DW, Jr. 1984. Cardiac and skeletal myopathy associated with cardiac dysrhythmias. Am J Cardiol 53(6): 731–7.
Garson A, Gillette PC, Porter CJ, McNamara DG. 1982. Ventricular tachycardia in children with a normal heart. Circulation 66 (Suppl II): 170.
Schwartz PJ. 1985. Idiopathic long QT syndrome: progress and questions. Am Heart J 109 (2): 399–411.
Bazett HC. 1918. Analysis of the time relations of electrocardiograms. Heart 7: 353–8.
Ross BA, Garson A, McNamara DG. 1984. Factors affecting outcome in children with long QT syndrome. Circulation 70 (Suppl II): 320.
Bricker T, Garson A, Gillette PC. 1984. A history of seizures in the family associated with sudden cardiac deaths. Am J Dis Chid 138: 866–8.
Takamizawa K, Takao A. 1985. Mexiletine for successful treatment of the long QT syndrome. Proc Second World Congress of Pediatric Cardiology 101.
Kay GN, Plumb VJ, Arcinigas JG, Henthorn RW, Waldo AL. 1983. Torsade-de-pointes: the long — short initiating sequence and other clinical features. J Am Coll Cardiol 2: 806–17.
Locati E, Schwartz P J, Moss A J, Crapton RS. 1985. Long-term survival after left cervico-thoracic sympathectomy in high risk long QT syndrome patients with refractory ventricular arrhythmias. J Am Coll Cardiol 7: 235.
Moller JH, Patton C, Varco RL, Lillehei CW. 1985. Postoperative ventricular septal defect: 24–30 years follow-up of 232 patients. Proc Second World Congress of Pediatric Cardiology: 20.
Garson A, Nihill MR, McNamara DG, Cooley DA. 1979. Status of the adult and adolescent following repair of tetralogy of Fallot. Circulation 59: 1232–40.
Fyfe DA, Driscoll DJ, Danielson GK, Mair DD. 1985. Truncus arteriosus with single pulmonary artery: influence of pulmonary vascular obstructive disease on early and late operative results. J Am Coll Cardiol 5: 1168–72.
Garson A, Smith RT, Moak JP, Ross BA, McNamara DG. 1985. Sudden death in children related to ventricular arrhythmias. J Am Coll Cardiol 5: 130B-4B.
Garson A, Gillette PC, Gutgesell HP, McNamara DG. 1980. Stress-induced ventricular arrhythmias after tetralogy of Fallot repair. Am J Cardiol 46: 1006–12.
Kavey RE, Blackman MS, Sondheimer HM. 1982. Incidence and severity of chronic ventricular dysrhythmias after repair of tetralogy of Fallot. Am Heart J 103 (3): 342–50.
Garson A, Porter CJ, Gillette PC, McNamara DG. 1983. Induction of ventricular tachycardia during electrophysiologic study after repair of tetralogy of Fallot. J Am Coll Cardiol 1:1493–1502.
Garson A, Randall DC, McVey P, Smith RT, Moak JP, Gillette PC, McNamara DG. 1985. Prevention of sudden death after repair of tetralogy of Fallot: treatment of ventricular arrhythmias. J Am Coll Cardiol 6: 221–7.
Wolff GS, Rowland TW, Ellison RC. 1972. Surgically induced right bundle branch block with left anterior hemiblock. Circulation 45: 587–93.
Fuster V, McGoon DC, Kennedy MA. 1980. Long-term evaluation (12–22 years) of open heart surgery for tetralogy of Fallot. Am J Cardiol 46: 635–40.
Garson A, Kugler JD, Gillette PC, Simonelli A, McNamara DG. 1980. Control of late postoperative ventricular arrhythmias with phenytoin in young patients. Am J Cardiol 46: 290–4.
Moak JP, Gillette PC, Garson A. 1984. Mexiletine: an alternative to dilantin for pediatric ventricular arrhythmias. Circulation 70 (Suppl II): 207.
Kugler JD, Cheatham JP, Gumbiner CH, Hofschire PJ, Latson LA. 1985. Results of phenytoin and propranolol drug electrophysiology studies for ventricular tachycardia in patients having repaired lesions with tetralogy of Fallot physiology. Circulation 72 (II): 341.
Deal BJ, Miller SM, Scagliotti D, Gallastegiu JM. 1985. Electrophysiologic drug testing in ventricular arrhythmias following tetralogy of Fallot repair. Circulation 72 (II): 197.
Byrum CJ, Sondheimer HM, Kavey REW, Blackman MS. 1985. Ventricular arrhythmia non-therapy guided by electrophysiologic tesing. Am Heart J 110: 707.
Deanfield JE, Franklin RCG, Dickie S, McKenna W, Gersony W, Hallidie-Smith K. 1985. Prognostic significance of ventricular arrhythmias after repair of tetralogy of Fallot: a prospective study. Proc Second World Congress of Pediatric Cardiology: 14.
Deanfield JE, McKenna WJ, Hallidie-Smith KA. 1980. Detection of late arrhythmia and conduction disturbance after correction of tetralogy of Fallot. Br Heart J 44 (3): 248–53.
Spinelli W, Rosen M. 1986. Developmental and use-dependent effects of phenytoin on neonatal and adult Purkinje fibers. J Am Coll Cardiol 7:123.
Rosen MR, Gelband H, Merker C, Hoffman BF. 1973. Mechanisms of digitalis toxicity: effects of ouabain on phase four of canine Purkinje fibers transmembrane potential. Circulation 47: 681–6.
Amerini S, Carbonin P, Cerbai E, Giotti A, Mugelli A, Pahor M. 1985. Electrophysiological mechanisms for the antiarrhythmic action of mexiletine on digitalis, reperfusion and reoxygenation-induced arrhythmias. Br J Pharmacol 86 (4): 805–15.
Tseng G, Wit AL. 1985. Arrhythmogenic effects of quinidine on triggered activity in atrial muscle. Circulation 72 (Suppl III): 380.
Moak JP, Rosen MR. 1982. The effects of pacing on ouabain-induced sustained rhythmic activity. Circulation 66 (Suppl II): 79.
Johnson N, Danilo P, Wit A, Rosen MR. 1985. Response to pacing of triggered activity occurring in catecholamine-treated canine coronary sinus. Circulation 72 (Suppl III): 381.
Benito F, Aguado AG, Fernandez A, Moreno F. 1985. Delayed afterdepolarizations in patients post-repair of tetralogy of Fallot: could be considered a mechanism in the genesis of ventricular arrhythmias? Proc Second World Congress of Pediatric Cardiology: 111.
Spicer RL, Rocchini AP, Crowley DC, Rosenthal A. 1984. Chronic verapamil treatment in young adult patients with hypertrophic cardiomyopathy. Am J Cardio 53:1614–19.
Gillette PC, Garson A. 1977. Electrophysiologic and pharmacologic characteristics of automatic ectopic supraventricular tachycardia. Circulation 56: 571–75.
Zipes DP, Foster PR, Troup PJ, Pedersen DH. 1979. Atrial induction of ventricular tachycardia: reentry versus triggered automaticity. Am J Cardiol 44 (1): 1–8.
Aronson RS. 1981. Afterpotentials and triggered activity in hypertrophied myocardium from rats with renal hypertension. Circ Res 48: 720–8.
Kugler JD, Pinsky WW, Cheatman JP, Hofschire PJ, Mooring PK, Fleming WH. 1983. Sustained ventricular tachycardia after repair of tetralogy of Fallot: new electrophysiologic findings. Am J Cardiol 51 (7): 1137–43.
Harken AH, Horowitz LN, Josephson ME. 1980. Surgical correction of recurrent sustained ventricular tachycardia following complete repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 80 (5): 779–81.
Kawashima Y, Matsuda H, Hirose H, Nakano S, Shirakura R, Kobayashi J. 1985. Ninety consecutive corrective operations for tetralogy of Fallot with or without minimal right ventriculotomy. J Thorac Cardiovasc Surg 90 (6): 856–63.
Moak JP, Garson A. 1986. Late electrophysiologic effects of experimental right ventriculotomy. J Am Coll Cardiol 7: 49.
Garson A. 1984. Ventricular arrhythmias after congenital heart surgery: a canine model. Pediatr Res 18:1112–20.
Deanfield J, McKenna W, Rowland E. 1985. Local abnormalities of right ventricular depolarization after repair of tetralogy of Fallot: a basis for ventricular arrhythmia. Am J Cardiol 55 (5): 522–5.
Waxman MB, Wald RW, Finley JP, Bonet JF, Downar E, Sharma AD. 1980. Valsalva termination of ventricular tachycardia. Circulation 62 (4): 843–51.
Cassidy DM, Vassallo JA, Miller JM, Josephson ME. 1985. Differences in endocardial electrophysiologic characteristics in coronary artery disease patients with ventricular tachycardias versus cardiac arrest. Circulation 72 (Suppl III): 160.
Benchimol A, Matsuo S, Wang TP. 1972. Phasic coronary arterial flow velocity during arrhythmias in man. Am J Cardiol 29: 604–15.
Tepper D, Sarda MA, Capasso JM, Somberg JC. 1986. Does hypertensive hypertrophy cause myocardial electrical instability? J Am Coll Cardiol 7:111A.
Palik I, Graham TP, Burger J. 1986. Ventricular function in patients with obstructed right ventricular pulmonary artery conduits. J Am Coll Cardiol 7: 201A.
McRae JR, Wagner GC, Rogers MC. 1974. Paroxysmal familial ventricular fibrillation. J Pediatr 84: 515–20.
Fyke FE, Vlietstra RE, Danielson GK, Beynen FM. 1983. Verapamil for refractory ventricular fibrillation during cardiac operations in patients with cardiac hypertrophy. J Thorac Cardiovasc Surg 86 (1): 108–11.
Reduto LA, Berger HJ, Johnstone DE. 1978. Radionuclide assessment of exercise right and left ventricular performance following total correction of tetralogy of Fallot. Circulation 58:145–50.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1987 Martinus Nijhoff Publishers, Dordrecht
About this chapter
Cite this chapter
Garson, A. (1987). Ventricular arrhythmias in the young: clinical and experimental findings. In: Aliot, E., Lazzara, R. (eds) Ventricular Tachycardias. Developments in Cardiovascular Medicine, vol 71. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-3323-1_10
Download citation
DOI: https://doi.org/10.1007/978-94-009-3323-1_10
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-010-7992-1
Online ISBN: 978-94-009-3323-1
eBook Packages: Springer Book Archive