Hostname: page-component-78c5997874-ndw9j Total loading time: 0 Render date: 2024-11-10T09:31:42.372Z Has data issue: false hasContentIssue false
  • English
  • Français

The early response of the systemic ventricle during transition to the Fontan circulation—an acute hypertrophic cardiomyopathy?

Published online by Cambridge University Press:  19 August 2008

Daniel J. Penny ,
Christopher Lincoln ,
Darryl F. Shore ,
Han B. Xiao ,
Michael L. Rigby  and
Andrew N. Redington
Daniel J. Penny
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospital, London
Christopher Lincoln
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospital, London
Darryl F. Shore
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospital, London
Han B. Xiao
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospital, London
Michael L. Rigby
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospital, London
Andrew N. Redington*
Affiliation:
Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospital, London
*
Dr. Andrew N. Redington, Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospital, Sydney Street, London SW3 6NP, United Kingdom. Tel. 71-352-8121
Get access Rights & Permissions [Opens in a new window]

Summary

Using combined epicardial echocardiography and high-fidelity intraventricular pressure recordings, the acute effects of transition to a Fontan circulation were studied in 10 patients. Measurements were made before and after cardiopulmonary bypass. The Fontan operation had no significant effect on load-dependent indices, or on a load independent index (Vmax) of ventricular systolic function. Large changes were observed in ventricular geometry. Maximum and minimum cavity dimensions were reduced, while maximum and minimum thicknesses of the posterior wall were increased.While simultaneous Doppler/intraventricular pressure measurements suggested that the compliance of the ventricle was unchanged, the pattern of atrioventricular flow changed from predominantly early diastolic (E wave) to predominantly atrial systolic (A wave) as a result of surgery in four patients. The time constant of ventricular relaxation was prolonged in all patients and five developed Doppler echocardiographic evidence of incoordinate relaxation of the ventricle, with intraventricular flow occurring during isovolumic relaxation. Thus, the Fontan operation causes acute hypertrophy of the systemic ventricle due to a sudden reduction in ventricular preload in the presence of a maintained shortening fraction. While global ventricular diastolic compliance may be unchanged by the operative procedure, filling of the ventricle during early diastole is altered as a result of incoordinate relaxation and a prolonged time constant of ventricular relaxation.

Keywords

Fontan operationsystemic ventricleventricular function
Type
Original Articles
Information
Cardiology in the Young , Volume 2 , Issue 1 , January 1992 , pp. 78 - 84
Copyright
Copyright © Cambridge University Press 1992

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Hurwitt, ES, Young, D, Escher, DJW. The rationale of anastomosis of the right auricular appendage to the pulmonary artery in the treatment of tricuspid atresia. J Thorac Cardiovasc Surg 1955; 30: 503–512.Google Scholar
2.Fontan, F, Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240–248.CrossRefGoogle ScholarPubMed
3.Choussat, A, Fontan, F, Besse, P, Vallot, F, Chauve, A, Bricaud, H. Selection criteria for Fontan's procedure. In: Anderson, RH, Shinebourne, EA (eds). Paediatric Cardiology 1977. Churchill Livingstone, Edinburgh, 1977, pp 559–566.Google Scholar
4.Gewillig, MH, Lundstrom, UR, Deanfield, JE, Bull, C, Franklin, RC, Graham, TP, Wyse, RK. Impact of Fontan operation on left ventricular size and contractility in tricuspid atresia. Circulation 1990; 81: 118–127.CrossRefGoogle ScholarPubMed
5.Kirklin, JK, Blackstone, EH, Kirklin, JW, Pacífico, AD, Bargeron, LM. The Fontan operation. Ventricular hypertrophy, age and date of operation as risk factors. J Thorac Cardiovasc Surg 1986; 92: 1049–1064.CrossRefGoogle ScholarPubMed
6.Seliem, M, Muster, AJ, Paul, MH, Benson, W. Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia. J Am Coll Cardiol 1989; 14: 750–755.CrossRefGoogle ScholarPubMed
7.de Leval, MR, Kilner, P, Gewillig, M, Bull, C. Total cavopulmonary connection: A logical alternative to atriopulmonary connection for complex Fontan operations. J Thorac Cardiovasc Surg 1988; 96: 682–695.CrossRefGoogle ScholarPubMed
8.Gibson, DG, Brown, DJ. Measuremenr of peak rates of left ventricular wall movement in man. Br Heart J 1975; 37: 677–683.CrossRefGoogle ScholarPubMed
9.Mason, DT, Spann, JF, Zelis, R. Quantification of the contractile state of the intact human heart. Am J Cardiol 1970; 26: 248–257.CrossRefGoogle ScholarPubMed
10.Raff, GL, Glantz, SA. Volume loading slows left ventricular isovolumic relaxation rate in the intact dog heart. Circ Res 1981; 48: 813–824.CrossRefGoogle ScholarPubMed
11.Penny, DJ, Rigby, ML, Redington, AN. Abnormal patterns of intraventricular flow and diastolic filling after the Fontan operation: Evidence for incoordinate ventricular wall motion. Br Heart J 1991. [in press]Google ScholarPubMed
12.Heath, D, Edwards, JE. The pathology of hypertensive pulmonary vascular disease. A description of six grades of structural changes in the pulmonary artery with special reference to congenital cardiac septal defect. Circulation 1958; 18: 533–547.CrossRefGoogle Scholar
13.Hagler, DJ, Seward, JB, Tajik, JRitter, DG. Functional assessment of the Fontan operation: Combined M-mode, Twodimensional and Doppler Echocardiographic Studies. J Am Coll Cardiol 1984; 4: 756–764.CrossRefGoogle ScholarPubMed
14.Penny, DJ, Redington, AN. Angiographic demonstration of incoordinate motion of the ventricular wall after the Fontan operation. Br Heart J 1991. [in press]Google ScholarPubMed
15.Braunwald, E, Ross, J. Control of cardiac performance. In: Berne, RM (ed), Handbook of Physiology, Volume 1, The Heart. American Physiological Society, Maryland, 1979, pp 533–580.Google Scholar
16.Sullivan, ID, Taylor, JFN. Hearts with one ventricle: current concepts and management. Arch Dis Child 1989; 64: 166–171.CrossRefGoogle Scholar
17.Mirsky, I, Pasipoularides, A. Clinical assessment of diastolic function. Prog Cardiovasc Dis 1990; 32: 291–318.CrossRefGoogle ScholarPubMed
18.Aoyagi, T, lizuka, M, Takahashi, T, Ohya, T, Serizawa, T, Momomura, S, Sato, H, Mochizuki, T, Matsui, H, Ikenouchi, H, Shin, I, Ma, Y, Sugimoto, T. Wall motion asynchrony prolongs time constant of left ventricular relaxation. Am J Physiol 1989; 257: H883–H890.Google ScholarPubMed
19.Perrone-Filardi, P, Betocchi, S, Giustino, G, Piscione, F, Indolfi, C, Salvatore, M, Chiariello, M. Influence of left ventricular asynchrony on filling in coronary artery disease. Am J Cardiol 1988; 62: 523–527.CrossRefGoogle ScholarPubMed
20.Hanrath, P, Mathey, DG, Siegert, R, Bleifeld, W. Left ventricular relaxation and filling pattern in different forms of ventricular hypertrophy: An echocardiographic study. Am J Cardiol 1980; 45: 15–23.CrossRefGoogle ScholarPubMed
21.Murgo, JP. The hemodynamic evaluation in hypertrophic cardiomyopathy: Systolic and diastolic dysfunction. In: Shaver, JA (ed): Cardiomyopathies: Clinical presentation, differential diagnosis and management. Current Science Ltd., Philadelphia, 1989, pp 193–220.Google Scholar
22.Sasson, Z, Hatle, L, Appleton, CP, Jewett, M, Alderman, EL, Popp, RL. Intraventricular flow during isovolumic relaxation: Description and characterization by Doppler echocardiography. J Am Coll Cardiol 1987; 10: 539–546.CrossRefGoogle ScholarPubMed
23.Bridges, ND, Lock, JE, Castaneda, AR. Baffle fenestration with subsequent transcatheter closure. Circulation 1990; 82: 1681–1689.CrossRefGoogle ScholarPubMed
24.Penny, DJ, Hayek, Z, Shinebourne, EA, Redington, AN. Pulmonary blood flow after righr heart bypass. The role of the lungs. Br Heart J 1991; 66: 53 [Abstract].CrossRefGoogle Scholar