Exercise capacity and biventricular function in adult patients with repaired tetralogy of Fallot

doi:10.1016/j.ahj.2008.02.005

American Heart Journal

Volume 156, Issue 1, July 2008, Pages 100-105

https://doi.org/10.1016/j.ahj.2008.02.005 Get rights and content

Background

Adult patients with repaired tetralogy of Fallot (rTOF) often have diminished exercise capacity. The primary objective of this study was to examine whether abnormalities of biventricular function play a role in exercise limitation in patients with rTOF.

Methods

This was a retrospective review of 99 adult patients with rTOF. Right ventricular (RV) and left ventricular (LV) function were assessed echocardiographically using the myocardial performance index (MPI). Maximal oxygen consumption (VO₂ Max) was measured during a level 1 cardiopulmonary exercise test.

Results

The mean age of the cohort was 34 ± 11 years (50% females). Although most of the patients reported good functional capacity, the peak Vo₂max was decreased at 22 ± 6 mL/kg per minute (66% ± 13% predicted Vo₂max for age and sex). The mean RV and LV MPI were 0.30 ± 0.07 and 0.42 ± 0.09, respectively. In the multivariate model, higher RV MPI (P = .04) and LV MPI (P = .005) values, representing impaired ventricular function, were associated with diminished Vo₂max. There was a significant correlation between the RV and LV MPI (r = 0.54, P = .001).

Conclusions

Impairment of RV and LV function, as measured by MPI, is associated with diminished exercise capacity in patients with repaired tetralogy of Fallot. Furthermore, there is a linear relationship between the RV and LV function suggesting that ventricular interactions are contributing to the limited exercise capacity in this group of patients. Strategies aimed at preserving biventricular function or improving adverse ventricular interactions could help to improve functional capacity in these patients.

Section snippets

Methods

This was a retrospective analysis of 99 patients with repaired tetralogy of Fallot seen at the Toronto Congenital Cardiac Centre for Adults, Toronto, Canada, between July 1, 2003, and July 1, 2005. All patients undergoing echocardiographic assessment and a level 1 cardiopulmonary testing on the same day of their routine annual clinic visit were included in the study. Patients with prosthetic mitral or aortic valves or with additional congenital heart lesions were excluded. Assessment of

Results

The mean age of the cohort was 34 ± 11 years (range 19-72 years). Thirty-one percent (31/99) of patients had reoperation with a homograft implantation, and the median duration of homograft implantation before the study was 12 years. Severe residual PR was present in 49% (48/99) of patients. Ninety-three percent (93/99) of patients were classified as New York Heart Association (NYHA) functional class I. Twenty-six percent of these patients had a Vo₂max value <20 mL/kg per minute. Additional

Discussion

In this study we found that patients with repaired tetralogy of Fallot have diminished exercise capacity. The factors that have been associated with limited exercise capacity in earlier studies of repaired tetralogy of Fallot, including older age, age at tetralogy of Fallot repair, chonotropic incompetence, and abnormal lung function, were confirmed in our study. However, we were unable to show an association between the RV size or the degree of pulmonary regurgitation and diminished exercise

Conclusions

Many patients with repaired tetralogy of Fallot had diminished exercise capacity. Impairment in RV and LV function is associated with diminished exercise capacity. Furthermore, there is a linear relationship between the RV and LV function, suggesting that ventricular interactions are contributing to the limited exercise capacity in this group of patients. Clinicians therefore need to be attentive to the function of both the RV and the LV in this group of patients. Strategies aimed at preserving

References (29)

P.M. Fredriksen et al.
Aerobic capacity in adults with tetralogy of Fallot
Cardiol Young
(2002)
G.P. Diller et al.
Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication
Circulation
(2005)
A. Frigiola et al.
Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot
Circulation
(2004)
A. Giardini et al.
Impact of pulmonary regurgitation and right ventricular dysfunction on oxygen uptake recovery kinetics in repaired tetralogy of Fallot
Eur J Heart Fail
(2006)
C. Kondo et al.
Left ventricular dysfunction on exercise long-term after total repair of tetralogy of Fallot
Circulation
(1995)
A.A. Roest et al.
Exercise MR imaging in the assessment of pulmonary regurgitation and biventricular function in patients after tetralogy of Fallot repair
Radiology
(2002)
G.K. Singh et al.
Right ventricular function and exercise performance late after primary repair of tetralogy of Fallot with the transannular patch in infancy
Am J Cardiol
(1998)
T. Geva et al.
Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging
J Am Coll Cardiol
(2004)
American Thoracic Society
Lung function testing: selection of reference values and interpretive strategies
Am Rev Respir Dis
(1991)
N.L. Jones

C. Tei et al.

New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function—a study in normals and dilated cardiomyopathy

J Cardiol

(1995)

C. Tei et al.

Noninvasive Doppler-derived myocardial performance index: correlation with simultaneous measurements of cardiac catheterization measurements

J Am Soc Echocardiogr

(1997)

B.W. Eidem et al.

Usefulness of the myocardial performance index for assessing right ventricular function in congenital heart disease

Am J Cardiol

(2000)

W.A. Zoghbi et al.

Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography

J Am Soc Echocardiogr

(2003)

Cited by (24)

Echocardiographic surveillance in children after tetralogy of Fallot repair: Adherence to guidelines?
2020, International Journal of Cardiology
Citation Excerpt :
Long-term outcomes of patients after repair of tetralogy of Fallot (TOF) are impacted by residual anatomic and hemodynamic abnormalities such as pulmonary valve regurgitation or stenosis, right ventricular (RV) dilation and biventricular dysfunction [1,2]. There is an increased incidence of arrhythmias (∼43%), exercise intolerance (28% had severely decreased exercise capacity with <50% predicted peak oxygen consumption), heart failure (12% NYHA functional class ≥ III), and sudden cardiac death beginning in early adulthood, which are often attributed to these abnormalities [3–5]. Therefore, longitudinal clinical surveillance is crucial in this patient population.
Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations.
This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines.
We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53–70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported.
Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.
Subaortic right ventricular characteristics and relationship to exercise capacity in congenitally corrected transposition of the great arteries
2012, Journal of the American Society of Echocardiography
Citation Excerpt :
The stage 1 cardiopulmonary tests were performed on an ergometer cycle (Elema, Solna, Sweden). The protocol used at our center has been previously described.12 Briefly, heart rate, oxygen saturation (finger probe), and ventilation are measured continuously, and measurements are averaged over 30-sec intervals.
In congenitally corrected transposition of the great arteries (cc-TGA), the morphologic right ventricle acts as the subaortic (systemic) ventricle, and deterioration of the ventricle over time is a well-known complication. The objective of this study was to characterize the right ventricle and explore factors that may be contributing to ventricular dilation and dysfunction and the relationship with exercise capacity.
This was a prospective study of adults with cc-TGA. All patients underwent cardiopulmonary stress testing, ventricular volume and fibrosis assessment by cardiac magnetic resonance imaging, and Velocity Vector Imaging strain echocardiography.
Twenty-six patients were included (mean age, 38 ± 16 years; 54% women). Exercise capacity was significantly reduced in patients with cc-TGA compared with normal subjects (20.9 ± 6.0 vs 30.8 ± 9.2 mL/kg/min, P = .001). The majority of patients (61%) had right ventricular (RV) ejection fractions ≤ 40%. There was no evidence of fibrosis on cardiac magnetic resonance imaging. There was a significant difference in diastolic volumes among those with RV ejection fractions > 40% versus ≤ 40% (173 ± 29mL vs 233 ± 65 mL, P = .02) and moderate or severe versus no or mild tricuspid regurgitation (240 ± 80mL vs 190 ± 38mL, P = .04). RV apical longitudinal and mid free wall circumferential strain was decreased compared with these values in controls.
In this relatively “well” cc-TGA population, subaortic RV dilation, dysfunction, and exercise intolerance are a common problem. Significant systemic tricuspid atrioventricular valvular regurgitation is an important contributor to the problem. In this study, subaortic RV myocardial deformation parameters were found to be abnormal, suggesting that there is a failure of the ventricle to adapt to systemic pressures, and therapies to reduce afterload should be explored. Recurrent ischemia resulting in fibrosis likely does not contribute to RV dilation or dysfunction as demonstrated by the magnetic resonance imaging findings in this study.
Right ventricular-left ventricular interaction in adults with Tetralogy of Fallot: A combined cardiac magnetic resonance and echocardiographic speckle tracking study
2012, International Journal of Cardiology
Citation Excerpt :
We speculate that this discrepancy is due to the contribution of circumferential right ventricular fibers – which could not be assessed as part of the current study – or due to the contribution of enlarged, hypokinetic outflow tract portions with preserved inflow RV function. Previous studies have demonstrated reduced LV ejection fraction and myocardial performance index in ToF patients [8,33]. Although Ghai and colleagues have shown a worse prognosis in ToF patients with moderately or severely reduced LV ejection fraction [16], the majority of contemporary ToF patients have preserved LV ejection fraction [8].
To assess ventricular dysfunction and ventricular interaction after repair of Tetralogy of Fallot (ToF) employing echocardiography speckle-tracking and cardiac magnetic resonance imaging (CMR).
Severe pulmonary regurgitation and right ventricular (RV) dysfunction are common after repair of ToF and may also affect the shape and function of the left ventricle (LV). Recent studies suggest that LV dysfunction may be of particular prognostic value.
Twenty-one consecutive adults with repaired ToF (15 male, mean age 38 ± 11 years, 7 with severe PR) underwent a comprehensive echocardiographic exam including speckle-tracking analysis, CMR and cardiopulmonary exercise testing. Twenty-one subjects without relevant heart disease served as controls. Echocardiographically measured RV diameters correlated with RV volumes obtained from CMR (r = 0.63; p = 0.006). In addition, a close correlation was found between RV and LV function on CMR (r = 0.74, p = 0.002), speckle-tracking LV and RV peak longitudinal 2D strain (r = 0.66, p = 0.003) and mitral and tricuspid annular plain systolic excursion (r = 0.71, p = 0.0003). While LV ejection fraction was normal in the majority of patients and not different from controls, LV longitudinal strain was significantly reduced in ToF patients (− 16.5 ± 3.3 vs. -20.5 ± 2.7%, p = 0.0001).
Left and right ventricular function both by CMR and speckle-tracking is interrelated in adults with repaired ToF. Despite normal LV ejection fraction, 2D longitudinal strain is significantly reduced in ToF patients, suggesting subclinical LV myocardial damage. Considering the potential prognostic value of LV dysfunction in ToF, this measurement may gain importance and should be included in future outcome studies.
Longitudinal exercise capacity of patients with repaired tetralogy of Fallot
2011, American Journal of Cardiology
Citation Excerpt :
Several cross-sectional studies of patients with rTOF have reported that older patients tend to have more compromised exercise function than their younger peers. In a study of 99 adults with rTOF, Samman et al6 documented the %VO2 peak to be depressed at 66 ± 13% of predicted. They found that older age, age at rTOF, chronotropic incompetence, and abnormal lung function were associated with limited exercise capacity.
Patients with repaired tetralogy of Fallot have a reduced percentage of predicted peak oxygen consumption (VO₂) and percentage of oxygen pulse (O₂P%) compared to healthy controls. Because data regarding the progression of exercise intolerance in these patients is limited, we sought to analyze the serial exercise data from patients with Tetralogy of Fallot to quantify the changes in their exercise capacity over time and to identify associations with clinical and cardiac magnetic resonance imaging variables. The data from cardiopulmonary exercise tests (CPXs) from 2002 to 2010 for patients with repaired tetralogy of Fallot with ≥2 CPXs separated by ≥12 months were analyzed. Tests occurring after interventional catheterization or surgery were excluded. A total of 70 patients had 179 CPXs. They had a median age at the initial study of 23.6 years and an interval between the first and last CPX of 2.8 years. At the initial CPX, the peak VO₂ was 27.6 ± 8.8 ml/kg/min (78 ± 19% of predicted), and the peak O₂P% was 89 ± 22% of predicted. At the most recent study, the peak VO₂ averaged 25.0 ± 7.4 ml/kg/min (73 ± 16% of predicted), and the peak O₂P% averaged 83 ± 20% (p <0.01) for each versus the initial CPX. The decrease in the peak VO₂ was strongly associated with a decrease in O₂P% and an increase (worsening) in the slope of the minute ventilation-versus-carbon dioxide production relation. Changes in the peak VO₂ did not correlate with concomitant changes in any other CPX variable. The rate of decrease was not related to a history of shunt palliation, age at CPX, or any other baseline clinical parameter, including cardiac magnetic resonance measurements. In conclusion, the exercise capacity of patients with repaired tetralogy of Fallot tends to decrease over time. This deterioration is variable and unpredictable and is primarily related to a decrease in the forward stroke volume at peak exercise.
Pulmonary Homograft Monocusp Reconstruction of the Right Ventricular Outflow Tract: Outcomes to the Intermediate Term
2010, Annals of Thoracic Surgery
Citation Excerpt :
This natural history was documented in early animal studies [17], and has been confirmed as patients have had closer follow-up with advanced imaging techniques such as cardiac magnetic resonance imaging. The clinical course of tetralogy patients with RV dilation secondary to nonvalved transannular patching has been well documented [18–24]. Complications ranging from exercise intolerance, growth retardation, arrhythmia, and sudden death have been noted.
There is limited information on longer-term outcomes of pulmonary homograft monocusp (PHM) reconstruction of the right ventricular outflow tract (RVOT).
A retrospective review of 131 consecutive patients undergoing RVOT reconstruction with PHM was completed.
Median age was 7.6 months (range, 1 day to 14 years) and weight was 7.3 kg (range, 2 to 65 kg). Most patients (108 of 131; 82%) underwent repair for Tetralogy. After PHM, median duration of mechanical ventilation was 1 day (range, 0 to 89) and hospital stay was 6.5 days (range, 2 to 137). Hospital mortality was 2% (3 of 131) with 1 patient undergoing early replacement of PHM. Echocardiogram at hospital discharge demonstrated peak RVOT gradient of 16 mm Hg (range, 4 to 64 mm Hg); and pulmonary insufficiency was absent/trivial in 40%, mild in 42%, moderate in 16%, and severe in 2%. Follow-up is completed in 91% of hospital survivors at a median of 5 years (range, 1 to 12). There were 5 late deaths, with an actuarial survival of 96% ± 3.7%, 94% ± 4.6%, and 89% ± 9.2% at 1 year, 5 years, and 10 years, respectively. There were 24 reinterventions, including 10 pulmonary valve replacements. Median time to valve replacement was 1.9 years (range, 0.4 to 4.6). Actuarial freedom from pulmonary valve replacement was 97% ± 3.0%, 90% ± 6.1%, and 85% ± 10.3% at 1 year, 5 years, and 10 years, respectively. Echocardiogram at last follow-up demonstrated no increase in RVOT gradient compared with hospital discharge (16 mm Hg), but there was significant increase in pulmonary insufficiency (mild 27%, moderate 39%, severe 34%).
Pulmonary homograft monocusp reconstruction is an alternative strategy for RVOT reconstruction and provides early but gradually diminishing protection against pulmonary insufficiency without a risk of stenosis. As expected, PHM function decreases over time as the RVOT grows and the homograft tissue undergoes structural deterioration.
Usefulness of maximal oxygen pulse in timing of pulmonary valve replacement in patients with isolated pulmonary regurgitation
2016, Cardiology in the Young

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Clinical InvestigationValvular and Congenital Heart DiseaseExercise capacity and biventricular function in adult patients with repaired tetralogy of Fallot

Background

Methods

Results

Conclusions

Section snippets

Methods

Results

Discussion

Conclusions

Aerobic capacity in adults with tetralogy of Fallot

Cardiol Young

Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication

Circulation

Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of Fallot

Circulation

Impact of pulmonary regurgitation and right ventricular dysfunction on oxygen uptake recovery kinetics in repaired tetralogy of Fallot

Eur J Heart Fail

Left ventricular dysfunction on exercise long-term after total repair of tetralogy of Fallot

Circulation

Exercise MR imaging in the assessment of pulmonary regurgitation and biventricular function in patients after tetralogy of Fallot repair

Radiology

Right ventricular function and exercise performance late after primary repair of tetralogy of Fallot with the transannular patch in infancy

Am J Cardiol

Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging

J Am Coll Cardiol

Lung function testing: selection of reference values and interpretive strategies

Am Rev Respir Dis

New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function—a study in normals and dilated cardiomyopathy

J Cardiol

Noninvasive Doppler-derived myocardial performance index: correlation with simultaneous measurements of cardiac catheterization measurements

J Am Soc Echocardiogr

Usefulness of the myocardial performance index for assessing right ventricular function in congenital heart disease

Am J Cardiol

Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography

J Am Soc Echocardiogr

Clinical Investigation
Valvular and Congenital Heart Disease
Exercise capacity and biventricular function in adult patients with repaired tetralogy of Fallot