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West J Med. 1986 January; 144(1): 33–41.
PMCID: PMC1306503

Familial Automaticity-Conduction Disorder With Associated Cardiomyopathy

Abstract

An unusually large family of European descent was afflicted over four generations by an automaticity and conduction disorder with an associated dilated cardiomyopathy of variable expression. Ten living members affected with the disorder and three presumed affected but dead members were identified. Typically, the disorder presented as a sinoatrial bradyarrhythmia/tachyarrhythmia syndrome, followed by atrial enlargement and, variably, ventricular enlargement and dysfunction. Three family members required pacemaker implantation. Longevity did not seem to be greatly affected, but the demonstrated potential for embolic cerebrovascular events stresses an associated morbidity. The familial incidence was best explained by autosomal dominant inheritance with incomplete penetrance (greater in males and usually occurring first in adolescence) and variable expressivity. The large size of the family, frequency and profile of disease manifestations and disease tracking through at least four generations are unusual features of the familial disease described.

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Selected References

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  • GRIFFITH GC, ZINN WJ, VURAL IL. FAMILIAL CARDIOMYOPATHY: HEART BLOCK AND STOKES-ADAMS ATTACKS TREATED BY PACEMAKER IMPLANTATION. Am J Cardiol. 1965 Aug;16:267–272. [PubMed]
  • James TN. Congenital deafness and cardiac arrhythmias. Am J Cardiol. 1967 May;19(5):627–643. [PubMed]
  • Tsagaris TJ, Bustamante RA, Friesendorff RA. Familial heart disease. Dis Chest. 1967 Aug;52(2):153–158. [PubMed]
  • Allensworth DC, Rice GJ, Lowe GW. Persistent atrial standstill in a family with myocardial dease. Am J Med. 1969 Nov;47(5):775–784. [PubMed]
  • Kariv I, Kreisler B, Sherf L, Feldman S, Rosenthal T. Familial cardiomyopathy. A review of 11 families. Am J Cardiol. 1971 Dec;28(6):693–706. [PubMed]
  • Williams DO, Jones EL, Nagle RE, Smith BS. Familial atrial cardiomyopathy with heart block. Q J Med. 1972 Oct;41(164):491–508. [PubMed]
  • DEFOREST RE. Four cases of benign left bundle branch block in the same family. Am Heart J. 1956 Mar;51(3):398–404. [PubMed]
  • GOULD WL. Auricular fibrillation; report on a study of a familial tendency, 1920-1956. AMA Arch Intern Med. 1957 Dec;100(6):916–926. [PubMed]
  • WRIGHT FS, ADAMS P, Jr, ANDERSON RC. Congenital artrioventricular dissociation due to complete or advanced atrioventricular heart block; clinical and cardiac catheterization findings in twelve children without cardiac malformations, including three siblings. AMA J Dis Child. 1959 Jul;98(1):72–79. [PubMed]
  • BACOS JM, EAGAN JT, ORGAIN ES. Congenital familial nodal rhythm. Circulation. 1960 Nov;22:887–895. [PubMed]
  • SEGALL HN. Congenital arrhythmias and conduction abnormalities in a father and four children. Can Med Assoc J. 1961 Jun 10;84:1283–1296. [PMC free article] [PubMed]
  • COMBRINK JM, DAVIS WH, SNYMAN HW. Familial bundle branch block. Am Heart J. 1962 Sep;64:397–400. [PubMed]
  • PHAIR WB. FAMILIAL ATRIAL FIBRILLATION. Can Med Assoc J. 1963 Dec 21;89:1274–1276. [PMC free article] [PubMed]
  • KHORSANDIAN RS, MOGHADAM AN, MUELLER OF. FAMILIAL CONGENITAL A-V DISSOCIATION. Am J Cardiol. 1964 Jul;14:118–124. [PubMed]
  • GAZES PC, CULLER RM, TABER E, KELLY TE. CONGENITAL FAMILIAL CARDIAC CONDUCTION DEFECTS. Circulation. 1965 Jul;32:32–34. [PubMed]
  • Wagner CW, Jr, Hall RJ. Congenital familial atrioventricular dissociation. Report of three siblings. Am J Cardiol. 1967 Apr;19(4):593–596. [PubMed]
  • Spellberg RD. Familial sinus node disease. Chest. 1971 Sep;60(3):246–251. [PubMed]
  • Sarachek NS, Leonard JL. Familial heart block and sinus bradycardia. Classification and natural history. Am J Cardiol. 1972 Apr;29(4):451–458. [PubMed]
  • Livesley B, Catley PF, Oram S. Familial sinuatrial disorder. Br Heart J. 1972 Jul;34(7):668–670. [PMC free article] [PubMed]
  • Schaal SF, Seidensticker J, Goodman R, Wooley CF. Familial right bundle-branch block, left axis deviation, complete heart block, and early death. A heritable disorder of cardiac conduction. Ann Intern Med. 1973 Jul;79(1):63–66. [PubMed]
  • Vallianos G, Sideris DA. Familial conduction defects. Cardiology. 1974;59(3):190–197. [PubMed]
  • Waxman MB, Catching JD, Felderhof CH, Downar E, Silver MD, Abbott MM. Familial atrioventricular heart block; an autosomal dominant trait. Circulation. 1975 Feb;51(2):226–233. [PubMed]
  • Greenspahn BR, Denes P, Daniel W, Rosen KM. Chronic bifascicular block: evaluation of familial factors. Ann Intern Med. 1976 May;84(5):521–525. [PubMed]
  • Nordenberg A, Varghese PJ, Nugent EW. Spectrum of sinus node dysfunction in two siblings. Am Heart J. 1976 Apr;91(4):507–512. [PubMed]
  • Jacovella G, Santini M, Floris B, Masini V. Malattia del nodo del seno familiare. Osservazioni su tre casi personali. G Ital Cardiol. 1976;6(1):112–117. [PubMed]
  • Caralis DG, Varghese PJ. Familial sinoatrial node dysfunction. Increased vagal tone a possible aetiology. Br Heart J. 1976 Sep;38(9):951–956. [PMC free article] [PubMed]
  • Brodsky M, Wu D, Denes P, Rosen KM. Familial atrial tachyarrhythmia with short PR interval. Arch Intern Med. 1977 Feb;137(2):165–169. [PubMed]
  • Schneider MD, Roller DH, Morganroth J, Josephson ME. The syndromes of familial atrioventricular block with sinus bradycardia: prognostic indices, electrophysiologic and histopathologic correlates. Eur J Cardiol. 1978 Jul;7(5-6):337–351. [PubMed]
  • Tan AT, EE BK, Mah PK, Choo MH, Chia BL. Diffuse conduction abnormalities in an adolescent with familial sinus node disease. Pacing Clin Electrophysiol. 1981 Nov;4(6):645–649. [PubMed]
  • Fuhrman BP, Bass JL, Lawrence C, Lucas RV., Jr Progressive cardiac conduction defect and cardiomyopathy in siblings with syncope. Pediatr Cardiol. 1982;2(1):57–62. [PubMed]
  • Kariv I, Szeinberg A, Fabian I, Sherf L, Kreisler B, Zeltzer M. A family with cardiomyopathy. Am J Med. 1966 Jan;40(1):140–148. [PubMed]
  • Ferrer MI. The sick sinus syndrome. Circulation. 1973 Mar;47(3):635–641. [PubMed]
  • Anderson JL, Carlquist JF, Lutz JR, DeWitt CW, Hammond EH. HLA A, B and DR typing in idiopathic dilated cardiomyopathy: a search for immune response factors. Am J Cardiol. 1984 May 1;53(9):1326–1330. [PubMed]
  • Savage DD, Levy D, Garrison RJ, Castelli WP, Kligfield P, Devereux RB, Anderson SJ, Kannel WB, Feinleib M. Mitral valve prolapse in the general population. 3. Dysrhythmias: the Framingham Study. Am Heart J. 1983 Sep;106(3):582–586. [PubMed]
  • Ryder LP, Svejgaard A, Dausset J. Genetics of HLA disease association. Annu Rev Genet. 1981;15:169–187. [PubMed]

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