Skip to main content
British Heart Journal logoLink to British Heart Journal
. 1981 Feb;45(2):225–229. doi: 10.1136/hrt.45.2.225

Invasive electrophysiological study in the Jervell and Lange-Nielsen syndrome.

G O Hartzler, M J Osborn
PMCID: PMC482516  PMID: 7459181

Abstract

Repeated invasive electrophysiological studies in a 7-year-old boy with the classic Jervell and Lange-Nielsen syndrome disclosed increased ventricular refractoriness, unusual late diastolic endocardial waveforms, and the inability to induce ventricular fibrillation. Despite apparently beneficial electrophysiological responses to left stellate block, surgical left cervical sympathectomy was followed by spontaneous ventricular fibrillation, an unchanged QT interval and ventricular refractoriness, and persistence of late diastolic endocardial waveforms. These electrophysiological observations, which are unique, may reflect temporal and spatial inhomogeneity of repolarisation resulting from cardiac autonomic imbalance. The absence of electrophysiological change despite left stellate block is disconcerting and re-emphasises our incomplete understanding of the pathogenesis of syndromes involving long QT intervals. Invasive electrophysiological study should be considered in the assessment of future patients with this disorder.

Full text

PDF
225

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Guss S. B., Kastor J. A., Josephson M. E., Schare D. L. Human ventricular refractoriness. Effects of cycle length, pacing site and atropine. Circulation. 1976 Mar;53(3):450–455. doi: 10.1161/01.cir.53.3.450. [DOI] [PubMed] [Google Scholar]
  2. Hartzler G. O., Maloney J. D. Programmed ventricular stimulation in management of recurrent ventricular tachycardia. Mayo Clin Proc. 1977 Nov;52(11):731–741. [PubMed] [Google Scholar]
  3. JERVELL A., LANGE-NIELSEN F. Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J. 1957 Jul;54(1):59–68. doi: 10.1016/0002-8703(57)90079-0. [DOI] [PubMed] [Google Scholar]
  4. Moss A. J., McDonald J. Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long QT interval syndrome. N Engl J Med. 1971 Oct 14;285(16):903–904. doi: 10.1056/NEJM197110142851607. [DOI] [PubMed] [Google Scholar]
  5. Moss A. J., Schwartz P. J. Sudden death and the idiopathic long Q-T syndrome. Am J Med. 1979 Jan;66(1):6–7. doi: 10.1016/0002-9343(79)90473-x. [DOI] [PubMed] [Google Scholar]
  6. ROMANO C., GEMME G., PONGIGLIONE R. ARITMIE CARDIACHE RARE DELL'ETA' PEDIATRICA. II. ACCESSI SINCOPALI PER FIBRILLAZIONE VENTRICOLARE PAROSSISTICA. (PRESENTAZIONE DEL PRIMO CASO DELLA LETTERATURA PEDIATRICA ITALIANA) Clin Pediatr (Bologna) 1963 Sep;45:656–683. [PubMed] [Google Scholar]
  7. UEDA H., YANAI Y., MURAO S., HARUMI K., MASHIMA S., KUROIWA A., SUGIMOTO T., SHIMOMURA D. ELECTROCARDIOGRAPHIC AND VECTORCARDIOGRAPHIC CHANGES PRODUCED BY ELECTRICAL STIMULATION OF THE CARDIAC NERVES. Jpn Heart J. 1964 Jul;5:359–372. doi: 10.1536/ihj.5.359. [DOI] [PubMed] [Google Scholar]
  8. WARD O. C. A NEW FAMILIAL CARDIAC SYNDROME IN CHILDREN. J Ir Med Assoc. 1964 Apr;54:103–106. [PubMed] [Google Scholar]
  9. Yanowitz F., Preston J. B., Abildskov J. A. Functional distribution of right and left stellate innervation to the ventricles. Production of neurogenic electrocardiographic changes by unilateral alteration of sympathetic tone. Circ Res. 1966 Apr;18(4):416–428. doi: 10.1161/01.res.18.4.416. [DOI] [PubMed] [Google Scholar]

Articles from British Heart Journal are provided here courtesy of BMJ Publishing Group

RESOURCES