Abstract
Forty children with presumed ventricular tachyarrhythmic syncopes in the absence of structural heart disease were studied.
Twenty-nine patients, one of whom was deaf, had a prolonged QT-interval in the resting electrocardiogram (Group 1); eleven patients had a normal QT-interval (Group 2). The median QTc-interval was 0.51s in Group 1 and 0.40s in Group 2. Familial occurence suggesting autosomal dominant inheritance was found in 21 of 28 normally hearing patients in Group 1 and in 2 of 11 patients in Group 2. Syncopes were definitely stress-induced in 22 patients in Group 1 and in all 11 patients in Group 2. Of 23 patients in Group 1 in whom an electrocardiogram was obtained during physical exercise, only one showed severe ventricular dysrhythmia. In contrast, all eleven patients in Group 2 developed severe ventricular dysrhythmia with exercice. Treatment with beta-blocking medication prevented further syncopes in 15 of 19 patients with several previous attacks in Group 1 and in 3 of 5 patients of Group 2. Four of the 29 patients in Group 1 died suddenly and one more remained apallic after an attack. Of the 11 patients in Group 2, four died suddenly and one retains severe cerebral damage after resuscitation from ventricular fibrillation.
We conclude that, besides the group of patients with the long QT-syndrome, there may be a distinct group of patients with a consistently normal QT-interval and severe ventricular dysrhythmia with exercise. Patients of both groups are threatened by sudden death and are improved by treatment with beta-blocking medication.
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References
Alimurung MM, Joseph LG, Craige E, Masseli BF (1950) The QT interval in normal infants and children. Circulation 1:1329–1337
Athanasiou DJ (1976) Das Jervell- und Lange-Nielsen-Syndrom. Eine klinisch-elektrokardiographische Einheit. Forum des praktischen Arztes, 15
Benzing F, Stopfkuchen H, Jüngst B-K (1978) Das Syndrom der langen QT-Dauer als kardiologischer Notfall. Monatsschr Kinderheilk 126:320–321
von Bernuth G, Belz GG, Evertz W, Stauch M (1973) QTU-abnormalities, sinus bradycardia and Adams-Stokes attacks due to ventricular tachyarrhythmia. Acta paediat Scand 62:675–679
von Bernuth G, Lang D, Hofstetter R (1977) Tachyarrhythmische Synkopen unter Belastung bei Sinusbradykardie und normalem QT-Intervall in Ruhe. Z Kardiol 66:55–60
Burgess MJ, Millar K, Abildskov JA (1969) Cancellation of electrocardiographic effects during ventricular recovery. J Electrocardiol 2:101–108
Coumel P, Fidelle J, Lucet V, Attuel P, Bouvrain Y (1978) Catecholamine-induced severe ventricular arrhythmias with Adams-Stokes syndrome in children: report of four cases. Br Heart J 40: 28–37
Crampton R (1979) Preeminence of the left stellate ganglion in the long QT syndrome. Circulation 59:769–778
Davignon A, Routaharju R, Boiselle E, Sounus F, Méjélas M, Choquette A (1979/1980) Normal ECG standards for infants and children. Pediat Cardiol 1:123–152
Jervell A, Lange-Nielsen F (1957) Congenital deafmutism, functional heart disease with prolongation of the QT interval, and sudden death. Am Heart J 54:59–68
Jüngst B-K, Stopfkuchen H, Benzing F, Schranz D, Meireis H (1982) Ventrikuläre Tachyarrhythmien als Ursache kardialer Synkopen unter körperlicher Belastung im Kindesalter. Dtsch Z Sportment (in press)
Kallfelz HC (1968) Über ein neues EKG-Syndrom bei Kindern mit synkopalen Anfällen und plötzlichem Tod. Dtsch Med Wochenschr 93:1046–1052
Kallfelz HC (1974) Tachykardie und Extrasystolie im Kindesalter. In: Antoni H, Effert S (eds) Herzrhythmusstörungen. Neue experimentelle Ergebnisse und klinisch-therapeutische Gesichtspunkte. Stuttgart New York, pp 247–255
Romano C, Gemme G, Pongiglione R (1963) Artimie cardiache rare dell'età pediatrica. Clin Pediatr 45:656–683
Schwartz PJ, Periti M, Malliani A (1974) The long QT-syndrome. Am Heart J 89:378–390
Ward OC (1964) New familial cardiac syndrome in children. J Ir Med Assoc 54:103–106
Weber H, Grimm T, Rupprath G, Beuren AJ (1981) Neue Erkenntnisse in der Pathogenese des QT-Syndroms mit synkopalen Anfällen. Intrakardiale EKG-Ableitungen während programmierter Vorhofstimulation bei 4 Patienten. Z Kardiol 70:131–137
Weiner C (1975) Synkopale Anfälle und plötzlicher Tod durch Kammerflimmern. Eine Variante des Jervell-und Lange-Nielsen-Syndroms? Klin Pädiatr 187:448–460
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Presented in part on the World Congress of Paediatric Cardiology, London 1980
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von Bernuth, G., Bernsau, U., Gutheil, H. et al. Tachyarrhythmic syncopes in children with structurally normal hearts with and without QT-prolongation in the electrocardiogram. Eur J Pediatr 138, 206–210 (1982). https://doi.org/10.1007/BF00441203
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DOI: https://doi.org/10.1007/BF00441203