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Br Heart J. 1989 February; 61(2): 198–203.
PMCID: PMC1216641

Impaired left ventricular diastolic filling in patients with familial amyloid polyneuropathy: a pulsed Doppler echocardiographic study.


To assess left ventricular diastolic filling in patients with amyloid heart disease 12 patients with familial amyloid polyneuropathy and 15 normal subjects were studied by pulsed Doppler echocardiography. None of the patients had clinical evidence of overt heart disease or restrictive cardiomyopathy and only two of them showed ventricular wall thickening. The peak flow velocity of rapid diastolic filling and the acceleration rate of early diastolic inflow were significantly lower in patients with familial amyloid polyneuropathy than in controls. The pressure half time was significantly longer in patients than in controls. In addition, the peak flow velocity during atrial contraction and the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity were significantly greater in patients than in controls. Although there were no significant correlations between measurements of diastolic filling and clinical findings in patients with familial amyloid polyneuropathy, the ratio of atrial peak flow velocity to rapid diastolic peak flow velocity was significantly related to left ventricular posterior wall thickness. These findings suggest that in patients with cardiac amyloidosis without restrictive cardiomyopathy, abnormal left ventricular diastolic filling, manifested by a reduction in the rate and volume of rapid diastolic filling with enhanced atrial contraction, can be seen even in the early stage of the disease.

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Selected References

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  • Hongo M, Ikeda S. Echocardiographic assessment of the evolution of amyloid heart disease: a study with familial amyloid polyneuropathy. Circulation. 1986 Feb;73(2):249–256. [PubMed]
  • Hongo M, Hirayama J, Fujii T, Yamada H, Okubo S, Kusama S, Ikeda S. Early identification of amyloid heart disease by technetium-99m-pyrophosphate scintigraphy: a study with familial amyloid polyneuropathy. Am Heart J. 1987 Mar;113(3):654–662. [PubMed]
  • Kim SG. The management of patients with life-threatening ventricular tachyarrhythmias: programmed stimulation or Holter monitoring (either or both)? Circulation. 1987 Jul;76(1):1–5. [PubMed]
  • Ikeda S, Hanyu N, Hongo M, Yoshioka J, Oguchi H, Yanagisawa N, Kobayashi T, Tsukagoshi H, Ito N, Yokota T. Hereditary generalized amyloidosis with polyneuropathy. Clinicopathological study of 65 Japanese patients. Brain. 1987 Apr;110(Pt 2):315–337. [PubMed]
  • Sahn DJ, 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. [PubMed]
  • Thomas JD, Weyman AE. Doppler mitral pressure half-time: a clinical tool in search of theoretical justification. J Am Coll Cardiol. 1987 Oct;10(4):923–929. [PubMed]
  • Meaney E, Shabetai R, Bhargava V, Shearer M, Weidner C, Mangiardi LM, Smalling R, Peterson K. Cardiac amyloidosis, contrictive pericarditis and restrictive cardiomyopathy. Am J Cardiol. 1976 Nov 4;38(5):547–556. [PubMed]
  • Swanton RH, Brooksby IA, Davies MJ, Coltart DJ, Jenkins BS, Webb-Peploe MM. Systolic and diastolic ventricular function in cardiac amyloidosis. Studies in six cases diagnosed with endomyocardial biopsy. Am J Cardiol. 1977 May 4;39(5):658–664. [PubMed]
  • Chew C, Ziady GM, Raphael MJ, Oakley CM. The functional defect in amyloid heart disease. The "stiff heart" syndrome. Am J Cardiol. 1975 Oct 6;36(4):438–444. [PubMed]
  • St John Sutton MG, Reichek N, Kastor JA, Giuliani ER. Computerized M-mode echocardiographic analysis of left ventricular dysfunction in cardiac amyloid. Circulation. 1982 Oct;66(4):790–799. [PubMed]
  • Borer JS, Henry WL, Epstein SE. Echocardiographic observations in patients with systemic infiltrative disease involving the heart. Am J Cardiol. 1977 Feb;39(2):184–188. [PubMed]
  • Buja LM, Khoi NB, Roberts WC. Clinically significant cardiac amyloidosis. Clinicopathologic findings in 15 patients. Am J Cardiol. 1970 Oct;26(4):394–405. [PubMed]
  • Bhandari AK, Nanda NC. Myocardial texture characterization by two-dimensional echocardiography. Am J Cardiol. 1983 Mar 1;51(5):817–825. [PubMed]
  • Smith RR, Hutchins GM. Ischemic heart disease secondary to amyloidosis of intramyocardial arteries. Am J Cardiol. 1979 Sep;44(3):413–417. [PubMed]
  • Saffitz JE, Sazama K, Roberts WC. Amyloidosis limited to small arteries causing angina pectoris and sudden death. Am J Cardiol. 1983 Apr;51(7):1234–1235. [PubMed]
  • Cueto-Garcia L, Tajik AJ, Kyle RA, Edwards WD, Wood DL, Seward JB. Echocardiographic features of amyloid ischemic heart disease. Am J Cardiol. 1985 Feb 15;55(5):606–607. [PubMed]

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