Skip to main content
NCBI home page
British Heart Journal logoLink to British Heart Journal
. 1982 Jun;47(6):588–595. doi: 10.1136/hrt.47.6.588

Familial hypertrophic cardiomyopathy: vectorcardiographic findings in echocardiographically unaffected relative.

F Loperfido, R Fiorilli, A Digaetano, M Di Gennaro, P Santarelli, F Bellocci, E Coppola, P Zecchi
PMCID: PMC481186  PMID: 7200794

Abstract

The electrocardiographic and vectorcardiographic (Frank system) features of the first degree relatives of subjects with documented familial hypertrophic cardiomyopathy were analysed. A total of nine affected members and 29 relatives were examined in four families. THe subjects were considered to be affected when the septal to free posterior wall thickness ratio exceeded 1.3 at M-mode echocardiography. Four relatives had asymmetric septal hypertrophy. Among 25 relatives without evidence of asymmetric septal hypertrophy, two over 20 years and 10 under 20 years of age showed increased voltage of QRS anterior forces (Qz amplitude greater than 0.80 mV) on the orthogonal electrocardiogram. The vectorcardiographic data of the relatives under 20 years of age without evidence of asymmetric septal hypertrophy (18 subjects) were compared with those of 38 normal control subjects of comparable age range. The young relatives without disproportionate septal hypertrophy had significantly greater Qz amplitude and Q/Rz ratio than the normal control subjects. In contrast, the echocardiographic data were not significantly different. We suggest that the electrocardiographic finding of abnormal anterior forces in one or more first degree relatives of subjects with documented hypertrophic cardiomyopathy may constitute a valuable aid in ascertaining the genetic transmission of the disease and in recognising affected members without echocardiographic evidence of hypertrophic cardiomyopathy.

Full text

PDF
588

Images in this article

589Image
on p.589
593Image
on p.593

Selected References

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

  1. Allen J. W., Kim S. J., Edmiston W. A., Venkataraman K. Problems in ultrasonic estimates of septal thickness. Am J Cardiol. 1978 Jul;42(1):89–96. doi: 10.1016/0002-9149(78)90990-6. [DOI] [PubMed] [Google Scholar]
  2. Bahl O. P., Walsh T. J., Massie E. Electrocardiography and vectorcardiography in idiopathic hypertrophic subaortic stenosis. Am J Med Sci. 1970 Apr;259(4):262–271. doi: 10.1097/00000441-197004000-00004. [DOI] [PubMed] [Google Scholar]
  3. Brackbill T. A., Shah P. M. Vectorcardiographic comparison of left ventricular hypertrophy in idiopathic hypertrophic subaortic stenosis, aortic stenosis, and aortic regurgitation. Am Heart J. 1974 Sep;88(3):269–276. doi: 10.1016/0002-8703(74)90458-x. [DOI] [PubMed] [Google Scholar]
  4. Chen C. H., Kawai C. Anterior QRS loop in hypertrophic cardiomyopathy. J Electrocardiol. 1978 Apr;11(2):129–136. doi: 10.1016/s0022-0736(78)80102-2. [DOI] [PubMed] [Google Scholar]
  5. Chen C. H., Nobuyoshi M., Kawai C. ECG pattern of left ventricular hypertrophy in nonobstructive hypertrophic cardiomyopathy: the significance of the mid-precordial changes. Am Heart J. 1979 Jun;97(6):687–695. doi: 10.1016/0002-8703(79)90002-4. [DOI] [PubMed] [Google Scholar]
  6. Clark C. E., Henry W. L., Epstein S. E. Familial prevalence and genetic transmission of idiopathic hypertrophic subaortic stenosis. N Engl J Med. 1973 Oct 4;289(14):709–714. doi: 10.1056/NEJM197310042891402. [DOI] [PubMed] [Google Scholar]
  7. DRAPER H. W., PEFFER C. J., STALLMANN F. W., LITTMANN D., PIPBERGER H. V. THE CORRECTED ORTHOGONAL ELECTROCARDIOGRAM AND VECTORCARDIOGRAM IN 510 NORMAL MEN (FRANK LEAD SYSTEM). Circulation. 1964 Dec;30:853–864. doi: 10.1161/01.cir.30.6.853. [DOI] [PubMed] [Google Scholar]
  8. DeMaria A. N., Bommer W., Lee G., Mason D. T. Value and limitations of two dimensional echocardiography in assessment of cardiomyopathy. Am J Cardiol. 1980 Dec 18;46(7):1224–1231. doi: 10.1016/0002-9149(80)90292-1. [DOI] [PubMed] [Google Scholar]
  9. Doi Y. L., Deanfield J. E., McKenna W. J., Dargie H. J., Oakley C. M., Goodwin J. F. Echocardiographic differentiation of hypertensive heart disease and hypertrophic cardiomyopathy. Br Heart J. 1980 Oct;44(4):395–400. doi: 10.1136/hrt.44.4.395. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Doi Y. L., McKenna W. J., Gehrke J., Oakley C. M., Goodwin J. F. M mode echocardiography in hypertrophic cardiomyopathy: diagnostic criteria and prediction of obstruction. Am J Cardiol. 1980 Jan;45(1):6–14. doi: 10.1016/0002-9149(80)90213-1. [DOI] [PubMed] [Google Scholar]
  11. Engler R. L., Smith P., LeWinter M., Gosink B., Johnson A. The electrocardiogram in asymmetric septal hypertropy. Chest. 1979 Feb;75(2):167–173. doi: 10.1378/chest.75.2.167. [DOI] [PubMed] [Google Scholar]
  12. Fowles R. E., Martin R. P., Popp R. L. Apparent asymmetric septal hypertrophy due to angled interventricular septum. Am J Cardiol. 1980 Sep;46(3):386–392. doi: 10.1016/0002-9149(80)90005-3. [DOI] [PubMed] [Google Scholar]
  13. Gibson D. G., Traill T. A., Hall R. J., Brown D. J. Echocardiographic features of secondary left ventricular hypertrophy. Br Heart J. 1979 Jan;41(1):54–59. doi: 10.1136/hrt.41.1.54. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Goodwin J. F. Hypertrophic cardiomyopathy: a disease in search of its own identity. Am J Cardiol. 1980 Jan;45(1):177–180. doi: 10.1016/0002-9149(80)90236-2. [DOI] [PubMed] [Google Scholar]
  15. Henry W. L., Clark C. E., Epstein S. E. Asymmetric septal hypertrophy. Echocardiographic identification of the pathognomonic anatomic abnormality of IHSS. Circulation. 1973 Feb;47(2):225–233. doi: 10.1161/01.cir.47.2.225. [DOI] [PubMed] [Google Scholar]
  16. Ishikawa K., Tamura T., Inoue M. Orthogonal electrocardiographic study on progressive muscular dystrophy of the Duchenne type. J Electrocardiol. 1979;12(2):163–167. doi: 10.1016/s0022-0736(79)80025-4. [DOI] [PubMed] [Google Scholar]
  17. Kansal S., Roitman D., Sheffield L. T. Interventricular septal thickness and left ventricular hypertrophy. An echocardiographic study. Circulation. 1979 Nov;60(5):1058–1065. doi: 10.1161/01.cir.60.5.1058. [DOI] [PubMed] [Google Scholar]
  18. Larter W. E., Allen H. D., Sahn D. J., Goldberg S. J. The asymmetrically hypertrophied septum. Further differentiation of its causes. Circulation. 1976 Jan;53(1):19–27. doi: 10.1161/01.cir.53.1.19. [DOI] [PubMed] [Google Scholar]
  19. Maron B. J., Epstein S. E. Hypertrophic cardiomyopathy. Recent observations regarding the specificity of three hallmarks of the disease: asymmetric septal hypertrophy, septal disorganization and systolic anterior motion of the anterior mitral leaflet. Am J Cardiol. 1980 Jan;45(1):141–154. doi: 10.1016/0002-9149(80)90232-5. [DOI] [PubMed] [Google Scholar]
  20. Maron B. J., Gottdiener J. S., Bonow R. O., Epstein S. E. Hypertrophic cardiomyopathy with unusual locations of left ventricular hypertrophy undetectable by M-mode echocardiography. Identification by wide-angle two-dimensional echocardiography. Circulation. 1981 Feb;63(2):409–418. doi: 10.1161/01.cir.63.2.409. [DOI] [PubMed] [Google Scholar]
  21. Maron B. J., Gottdiener J. S., Epstein S. E. Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients. Am J Cardiol. 1981 Sep;48(3):418–428. doi: 10.1016/0002-9149(81)90068-0. [DOI] [PubMed] [Google Scholar]
  22. Maron B. J., Henry W. L., Clark C. E., Redwood D. R., Roberts W. C., Epstein S. E. Asymetric septal hypertrophy in childhood. Circulation. 1976 Jan;53(1):9–19. doi: 10.1161/01.cir.53.1.9. [DOI] [PubMed] [Google Scholar]
  23. PRESCOTT R., QUINN J. S., LITTMANN D. ELECTROCARDIOGRAPHIC CHANGES IN HYPERTROPHIC SUBAORTIC STENOSIS WHICH SIMULATE MYOCARDIAL INFARCTION. Am Heart J. 1963 Jul;66:42–48. doi: 10.1016/0002-8703(63)90067-x. [DOI] [PubMed] [Google Scholar]
  24. Ronan J. A., Jr, Perloff J. K., Bowen P. J., Mann O. [The vectorcardiogram in Duchenne's progressive muscular dystrophy]. Am Heart J. 1972 Nov;84(5):588–596. doi: 10.1016/0002-8703(72)90172-x. [DOI] [PubMed] [Google Scholar]
  25. Sahn D. J., DeMaria A., Kisslo J., Weyman A. Recommendations regarding quantitation in M-mode echocardiography: results of a survey of echocardiographic measurements. Circulation. 1978 Dec;58(6):1072–1083. doi: 10.1161/01.cir.58.6.1072. [DOI] [PubMed] [Google Scholar]
  26. Savage D. D., Seides S. F., Clark C. E., Henry W. L., Maron B. J., Robinson F. C., Epstein S. E. Electrocardiographic findings in patients with obstructive and nonobstructive hypertrophic cardiomyopathy. Circulation. 1978 Sep;58(3 Pt 1):402–408. doi: 10.1161/01.cir.58.3.402. [DOI] [PubMed] [Google Scholar]
  27. Wei J. Y., Weiss J. L., Bulkley B. H. The heterogeneity of hypertrophic cardiomyopathy: an autopsy and one dimensional echocardiographic study. Am J Cardiol. 1980 Jan;45(1):24–32. doi: 10.1016/0002-9149(80)90215-5. [DOI] [PubMed] [Google Scholar]
  28. Wigle E. D., Baron R. H. The electrocardiogram in muscular subaortic stenosis. Effect of a left septal incision and right bundle-branch block. Circulation. 1966 Oct;34(4):585–594. doi: 10.1161/01.cir.34.4.585. [DOI] [PubMed] [Google Scholar]
  29. Yamaguchi H., Ishimura T., Nishiyama S., Nagasaki F., Nakanishi S., Takatsu F., Nishijo T., Umeda T., Machii K. Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients. Am J Cardiol. 1979 Sep;44(3):401–412. doi: 10.1016/0002-9149(79)90388-6. [DOI] [PubMed] [Google Scholar]
  30. ten Cate F. J., Hugenholtz P. G., van Dorp W. G., Roelandt J. Prevalence of dignostic abnormalities in patients with genetically transmitted asymmetric septal hypertrophy. Am J Cardiol. 1979 Apr;43(4):731–737. doi: 10.1016/0002-9149(79)90071-7. [DOI] [PubMed] [Google Scholar]
  31. van Dam R. T., Roos J. P., Durrer D. Electrical activation of ventricles and interventricular septum in hypertrophic obstructive cardiomyopathy. Br Heart J. 1972 Jan;34(1):100–112. doi: 10.1136/hrt.34.1.100. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. van Dorp W. G., ten Cate F. J., Vletter W. B., Dohmen H., Roelandt J. Familial prevalence of asymmetric septal hypertrophy. Eur J Cardiol. 1976 Sep;4(3):349–357. [PubMed] [Google Scholar]

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

RESOURCES