Clinical Investigation
Congenital Heart Disease
Three-Dimensional Mechanical Dyssynchrony and Myocardial Deformation of the Left Ventricle in Patients with Tricuspid Atresia after Fontan Procedure

https://doi.org/10.1016/j.echo.2012.01.003Get rights and content

Background

The aim of this study was to test the hypothesis that dyssynchronous contraction of functional single ventricles occurs in Fontan patients and is related to indices of myocardial deformation and global ventricular function.

Methods

Twenty patients with tricuspid atresia (mean age, 23.5 ± 7.1 years) were studied 17.8 ± 3.8 years after undergoing the Fontan procedure. Three-dimensional echocardiographic data were acquired for determination of left ventricular (LV) volumes and systolic dyssynchrony indices. LV myocardial deformation was determined using speckle-tracking echocardiography. Calibrated integrated backscatter intensity was measured as an index of myocardial fibrosis. The results were compared with those in 20 controls.

Results

Compared with controls, patients had significantly greater systolic dyssynchrony indices (6.13 ± 1.32% vs 4.06 ± 0.84%, P < .001). The prevalence of LV mechanical dyssynchrony (systolic dyssynchrony index > 5.74%) in patients was 55% (95% confidence interval, 32%–77%). LV global systolic longitudinal, radial, and circumferential strain (P < .001 for all), longitudinal systolic (P < .001) and early diastolic (P < .001) strain rate, and circumferential systolic (P < .001) and early diastolic (P = .009) strain rate were significantly lower in patients than in controls, while the average calibrated integrated backscatter was higher (P < .001). Patients with LV dyssynchrony (n = 11) had lower global LV longitudinal strain (P = .02), reduced LV ejection fractions (P = .002), and higher average calibrated integrated backscatter (P = .03) compared with those without LV dyssynchrony (n = 9).

Conclusions

A high proportion of patients with tricuspid atresia after the Fontan operation exhibit LV mechanical dyssynchrony, which may in part be related to myocardial fibrosis and has implications for myocardial deformation and global ventricular function.

Section snippets

Subjects

Twenty-five of the 32 Fontan patients with tricuspid atresia identified from the congenital heart disease database agreed to participate in this study. Five patients who required pacing or were not in sinus rhythm at the time of study were excluded. The following data were retrieved from the case records: cardiac diagnosis, need for systemic-to-pulmonary shunt insertion or pulmonary arterial banding, type of Fontan procedure, age at operation, and duration of follow-up since Fontan procedure.

Subjects

A total of 20 patients (seven men, 13 women; mean age, 23.5 ± 7.1 years) were studied. All but one patient had situs solitus with absent right atrioventricular connection, while the remaining patient had situs inversus totalis. The ventriculoarterial connection was concordant in 13 patients, double-outlet from the left ventricle in five, and discordant in two. Surgical interventions before the Fontan procedure included systemic-to-pulmonary arterial shunt insertion in nine patients, pulmonary

Discussion

The present study demonstrates a high prevalence of systemic LV mechanical dyssynchrony and impaired myocardial deformation in three dimensions in Fontan patients with tricuspid atresia. Additionally, LV systolic mechanical dyssynchrony was found to correlate negatively with magnitude and rate of myocardial deformation and cIB intensity. To our knowledge, this is the first study to quantify three-dimensional dyssynchronous contraction of functional single ventricles and to explore its

Conclusions

A high proportion of patients with tricuspid atresia after Fontan operation exhibit LV mechanical dyssynchrony, which may in part be related to myocardial fibrosis and has implications on myocardial deformation and global ventricular function.

References (49)

  • M.A. Binotto et al.

    Left ventricular remodeling in hearts with tricuspid atresia: morphologic observations and possible basis for ventricular dysfunction after surgery

    J Thorac Cardiovasc Surg

    (2003)
  • R.H. Rathod et al.

    Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation

    J Am Coll Cardiol

    (2010)
  • A.J. Chin et al.

    Changes in ventricular geometry early after Fontan operation

    Ann Thorac Surg

    (1993)
  • D.J. Penny et al.

    Diastolic ventricular function after the Fontan operation

    Am J Cardiol

    (1992)
  • N. Moiduddin et al.

    Two-dimensional speckle strain and dyssynchrony in single right ventricles versus normal right ventricles

    J Am Soc Echocardiogr

    (2010)
  • F. Labombarda et al.

    Health-e-Child project: mechanical dyssynchrony in children with dilated cardiomyopathy

    J Am Soc Echocardiogr

    (2009)
  • J. Wang et al.

    Systolic and diastolic dyssynchrony in patients with diastolic heart failure and the effect of medical therapy

    J Am Coll Cardiol

    (2007)
  • E.A. Bacha et al.

    Ventricular resynchronization by multisite pacing improves myocardial performance in the postoperative single-ventricle patient

    Ann Thorac Surg

    (2004)
  • H. Senzaki et al.

    Cardiac resynchronization therapy in a patient with single ventricle and intracardiac conduction delay

    J Thorac Cardiovasc Surg

    (2004)
  • N.S. Khoo et al.

    Novel insights into RV adaptation and function in hypoplastic left heart syndrome between the first 2 stages of surgical palliation

    JACC Cardiovasc Imaging

    (2011)
  • E. Gayat et al.

    Reproducibility and inter-vendor variability of left ventricular deformation measurements by three-dimensional speckle-tracking echocardiography

    J Am Soc Echocardiogr

    (2011)
  • C. Sonne et al.

    Real-time 3-dimensional echocardiographic assessment of left ventricular dyssynchrony: pitfalls in patients with dilated cardiomyopathy

    JACC Cardiovasc Imaging

    (2009)
  • M. Gewillig

    The Fontan circulation

    Heart

    (2005)
  • Y.F. Cheung et al.

    Serial assessment of left ventricular diastolic function after Fontan procedure

    Heart

    (2000)
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