Usefulness of Cardiac Magnetic Resonance Imaging Combined With Low-Dose Dobutamine Stress to Detect an Abnormal Ventricular Stress Response in Children and Young Adults After Fontan Operation at Young Age

doi:10.1016/j.amjcard.2008.01.050

The American Journal of Cardiology

Volume 101, Issue 11, 1 June 2008, Pages 1657-1662

https://doi.org/10.1016/j.amjcard.2008.01.050 Get rights and content

After Fontan operation, patients are limited in increasing cardiac output and in exercise capacity. This has been related to impaired preload or other factors leading to decreased global ventricular performance with stress. To study these factors, the stress responses of functionally univentricular hearts were assessed at rest and during low-dose dobutamine stress using cardiovascular magnetic resonance imaging. Thirty-two patients after Fontan completion at young age were included (27 with total cavopulmonary connection, 5 with atriopulmonary connection; mean age 13.3 years, range 7.5 to 22.2; 23 male patients; median follow-up after Fontan operation 8.1 years, range 5.2 to 17.8). A multiphase short-axis stack of 10 to 12 contiguous slices of the systemic ventricle was obtained at rest and during low-dose dobutamine stress cardiovascular magnetic resonance imaging (maximum 7.5 μg/kg/min). With stress-testing, heart rate, ejection fraction, and cardiac index increased adequately (p <0.001). There was an abnormal decrease in end-diastolic volume and an adequate decrease in end-systolic volume (p <0.001). Stroke volume did not change with stress testing (p = 0.15). At rest, dominant left ventricles had higher ejection fractions than dominant right ventricles (p = 0.01), but this difference disappeared with stress testing. In conclusion, a functionally univentricular heart after Fontan completion at young age has an adequate increase in ejection fraction with β-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate.

Section snippets

Methods

A cross-sectional study of patients after Fontan completion was performed. The following inclusion criteria were used: (1) patients after initial Fontan completion who were seen for regular follow-up at 3 tertiary referral centers, (2) age at Fontan completion ≤7 years, and (3) duration of follow-up since Fontan completion ≥5 years. Exclusion criteria were (1) contraindications for CMR imaging and (2) mental retardation. Medical records were reviewed for patient characteristics and anatomic and

Results

Sixty-eight patients were eligible for participation in this study. Thirty-two patients gave informed consent. Statistical analysis did not show any differences between participants and nonresponders when compared for patient characteristics and clinical status at most recent follow-up. The characteristics of the 32 patients are listed in Table 1. At the latest follow-up before this study, none of the patients had a residual outflow tract obstruction on echocardiography.

CMR scanning was well

Discussion

The results of this study of patients who underwent Fontan operations for univentricular hearts at young age show that with low-dose dobutamine stress-testing, EF increases adequately, demonstrating a good contractile capacity, and SVI does not change. This is caused by an abnormal decrease in EDVI with stress, whereas ESVI decreases adequately. Consequently, the systemic ventricle increases its output only by increasing heart rate. The normal response to low-dose dobutamine stress in children

Acknowledgment

We would like to thank Saskia Luijnenburg, MD, for her assistance in assessing the interobserver variability.

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Cited by (47)

Stress imaging in patients with a Fontan circulation: A systematic review
2023, International Journal of Cardiology
The aims of this study were to provide an overview of the cardiac stress response in Fontan patients and of the use, safety and clinical value of stress imaging in Fontan patients.
Studies evaluating cardiac function using stress imaging in Fontan patients published up until 12 December 2021 were included in this review.
From 1603 potential studies, 32 studies met the inclusion criteria. In total, stress imaging tests of 728 Fontan patients were included. Cardiac function was most often measured using physical stress (61%), all other studies used dobutamine-induced stress.
Stroke volume (SV) increased in most studies (71%), mean SV at rest ranged from 27 mL/m² to 60 mL/m² versus 27 mL/m² to 101 mL/m² during stress, and increased with an average of 4%. Ejection fraction increased in almost all studies, whereas both end-systolic volume and end-diastolic volume decreased during stress. Higher heart rates were obtained with physical stress (82–180) compared to dobutamine induced stress (73–128). Compared to controls, increases in heartrate and SV were lower and end-diastolic volume decreased abnormally in 75% of reporting studies. No major adverse events were reported. Poorer cardiac stress response was related to decreased exercise capacity and higher risk for long-term (adverse) outcomes in Fontan patients.
Cardiac stress response in Fontan patients differs from healthy subjects, reflected by lower increases in heart rate, diminished preload and decreased cardiac output, especially during higher levels of exercise. Stress imaging is safe, however the added clinical value needs to be investigated in more detail.
Single ventricle systolic function and cardiopulmonary exercise performance in adult patients with Fontan circulation
2020, International Journal of Cardiology
We employed echocardiographic tissue characterization for the assessment of single ventricle systolic function and examined its relationship with cardiopulmonary exercise testing (CPET) performance in the Fontan circulation.
40 consecutive adult Fontan patients (16 female, 24 male; mean age 26.3 ± 5.9) were enrolled in our prospective cross-sectional study. All patients underwent echocardiographic assessment including tissue characterization and CPET within 3 days from each other.
LAPSE (lateral annular plane systolic excursion) was higher in patients with a dominant left ventricle compared to a dominant right ventricle (12.3 + −2.6 vs 9.8 + −1.1 mm, p = .008) and in patients with AP (right atrium-pulmonary artery) connection than with lateral tunnel or extracardiac conduits (13.9 ± 1.7 vs 11.3 ± 2.6 and 10.9 ± 2.2 mm, p = .014 and p = .014, respectively). Maximal oxygen pulse (O₂ pulse max) was significantly higher in patients with a dominant left or common ventricle compared to patients with a dominant right ventricle (10.3 and 12.0 vs 6.6 ml/beat, p = .002 and p = .032, respectively). There was a positive correlation between O₂ pulse max and LAPSE (r = 0.442, p = .009), atrioventricular valve annulus systolic velocity (S′) (r = 0.606, p < .0001), lateral wall basal segment systolic velocity (bas vel s) (r = 0.469, p = .009) and lateral wall mid segment systolic velocity (mid vel s) (r = 0.452, p = .012). In the multivariable regression model, S′ (r = 0.606, p < .0001) remained predictive of O₂ pulse max.
LAPSE and S′ are suitable parameters for the assessment of systolic function in Fontan patients. Subclinical systolic dysfunction of longitudinal fibers is associated with worse CPET performance. O₂ pulse max seems to be the best parameter reflecting Fontan patients' physical performance.
Relation of Fontan Baffle Stroke Volume to Fontan Failure and Lower Exercise Capacity in Patients With an Atriopulmonary Fontan
2019, American Journal of Cardiology
Fontan failure remains a significant problem, especially in patients with an atriopulmonary Fontan. Fontan baffle volume change during the cardiac cycle (Fontan baffle stroke volume) may affect outcomes in Fontan circulation. Assuming that increased Fontan baffle stroke volume is associated with increased energy loss in the baffle, we hypothesized that higher baffle stroke volume is associated with worse exercise capacity and increased incidence of Fontan failure. Patients from 6 centers with an atriopulmonary or lateral tunnel Fontan operation were included if they had a cardiac magnetic resonance (CMR) study and an adequate cardiopulmonary exercise test. Fontan baffle stroke volume was defined as the difference between maximum and minimum Fontan baffle volumes. Fontan failure was defined as death, listing for transplantation, heart failure symptoms requiring medications, or peak VO₂ below 16 ml/kg/min. The study group consisted of 107 patients (median age 19 years, interquartile range, 14 to 29 years). Most patients (84%) had lateral tunnel procedure. During a median follow-up period of 6.8 [interquartile range: 3.2 to 8.8] years after the CMR, 25 (23%) patients had Fontan failure (7 deaths, 3 listed for transplantation, and 15 with heart failure symptoms). Predictors of Fontan failure on multivariable analysis were ventricular tachycardia, protein losing enteropathy, and additionally in atriopulmonary Fontan only, larger Fontan baffle stroke volume. Predictors of lower peak VO₂ on multivariable analysis were older age at CMR and additionally in atriopulmonary Fontan only, larger Fontan baffle stroke volume. In conclusion, larger Fontan baffle stroke volume was independently associated with lower peak VO₂ and Fontan failure in atriopulmonary Fontan.
Cardiac Physiology and Pharmacology
2019, A Practice of Anesthesia for Infants and Children
This chapter describes important features of the fetal circulation, essential changes at birth and other age-specific developments of the cardiovascular system. The incidence, prevalence and basic pathophysiological principles of congenital heart defects are presented. Several special situations are discussed in more detail: the exercise physiology of patients with repaired congenital heart disease, the Fontan physiology and the transplanted heart. Indications and practical considerations for the use of various vasoactive and antiarrhythmic drugs are reviewed.
Cardiac Physiology and Pharmacology
2018, A Practice of Anesthesia for Infants and Children
This chapter describes important features of the fetal circulation, essential changes at birth and other age-specific developments of the cardiovascular system. The incidence, prevalence and basic pathophysiological principles of congenital heart defects are presented. Several special situations are discussed in more detail: the exercise physiology of patients with repaired congenital heart disease, the Fontan physiology and the transplanted heart. Indications and practical considerations for the use of various vasoactive and antiarrhythmic drugs are reviewed.
Pressure–volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitations in cardiac output in children with hypoplastic left heart syndrome
2017, International Journal of Cardiology
Citation Excerpt :
However, measuring cardiac function solely under baseline conditions in patients with HLHS may mask dysfunction seen only during exertion. Pharmacological stress therefore offers an alternative method to simulate some of the physiological effects of higher heart rates (HR) in this group of patients [5,18]. In this study, we assessed a group of children with HLHS exhibiting symptoms of diminished exercise capacity.
Children with a single systemic right ventricle, such as in hypoplastic left heart syndrome (HLHS), frequently experience reduced exercise capacity. Elucidating the causes could help with optimising treatment strategies.
Prospective data from 10 consecutive symptomatic patients with HLHS undergoing clinical cardiac magnetic resonance with catheterisation (XMR) were analysed. Mean age 8.6 years (range 3.5–11.6 years), mean time since Fontan completion 5.5 years. MR-compatible catheters were placed in the systemic right ventricle and branch pulmonary arteries to record pressures at rest, with dobutamine infusion at 10 mcg/kg/min and at 20 mcg/kg/min. Cine short-axis stacks of the ventricle were performed at each condition and used to construct pressure–volume loops.
Compared to rest, cardiac index increased with low-dose dobutamine (p < 0.01) with no further rise at peak stress despite a further, albeit, blunted rise in heart rate (p = 0.002). A fall in stroke volume occurred (p = 0.014) despite good contractility (74% increase, p = 0.045) and a well-coupled ventriculo-arterial ratio. End-diastolic pressure and early active relaxation, markers of diastolic function, were normal at rest. However, preload fell at peak stress (p < 0.008) while pulmonary vascular resistance (PVR) was low throughout. This group of HLHS patients demonstrated a fall in SV at peak stress, coinciding with a fall in preload.
Markers of systolic and diastolic function remained normal. Failure to adequately fill the ventricle implies a ceiling of maximal flow through the Fontan circuit despite low PVR.

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Congenital heart diseaseUsefulness of Cardiac Magnetic Resonance Imaging Combined With Low-Dose Dobutamine Stress to Detect an Abnormal Ventricular Stress Response in Children and Young Adults After Fontan Operation at Young Age

Section snippets

Methods

Results

Discussion

Acknowledgment

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Ann Thorac Surg

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Thorac Cardiovasc Surg

Left ventricular characteristics during exercise in patients after Fontan's operation for tricuspid atresia

Heart Vessels

Comparison of cardiopulmonary adaptation during exercise in children after the atriopulmonary and total cavopulmonary connection Fontan procedures

Circulation

Congenital heart disease
Usefulness of Cardiac Magnetic Resonance Imaging Combined With Low-Dose Dobutamine Stress to Detect an Abnormal Ventricular Stress Response in Children and Young Adults After Fontan Operation at Young Age