A Cross-Sectional Study of Exercise Performance During the First 2 Decades of Life After the Fontan Operation Stephen M. Paridon, Paul D. Mitchell, Steven D. Colan, Richard V. Williams, Andrew ...Blaufox, Jennifer S. Li, Renee Margossian, Seema Mital, Jennifer Russell, Jonathan Rhodes, for the Pediatric Heart Network Investigators Exercise testing in a large group of Fontan survivors was performed, and clinical factors influencing exercise performance were assessed. Ramp cycle ergometry with metabolic cart measurements was performed on 411 subjects (mean age 12.4 ± 3.2 years). Peak oxygen consumption (VO2 ) was decreased at 65% of predicted for age and gender. Peak VO2 at ventilatory anaerobic threshold was better preserved at 78% of predicted than peak VO2 . Higher O2 pulse was associated with better exercise performance.
Background In multisystem inflammatory syndrome in children, there is paucity of longitudinal data on cardiac outcomes. We analyzed cardiac outcomes 3 to 4 months after initial presentation using ...echocardiography and cardiac magnetic resonance imaging. Methods and Results We included 60 controls and 60 cases of multisystem inflammatory syndrome in children. Conventional echocardiograms and deformation parameters were analyzed at 4 time points: (1) acute phase (n=60), (2) subacute phase (n=50; median, 3 days after initial echocardiography), (3) 1-month follow-up (n=39; median, 22 days), and (4) 3- to 4-month follow-up (n=25; median, 91 days). Fourteen consecutive cardiac magnetic resonance imaging studies were reviewed for myocardial edema or fibrosis during subacute (n=5) and follow-up (n=9) stages. In acute phase, myocardial injury was defined as troponin-I level ≥0.09 ng/mL (>3 times normal) or brain-type natriuretic peptide >800 pg/mL. All deformation parameters, including left ventricular global longitudinal strain, peak left atrial strain, longitudinal early diastolic strain rate, and right ventricular free wall strain, recovered quickly within the first week, followed by continued improvement and complete normalization by 3 months. Median time to normalization of both global longitudinal strain and left atrial strain was 6 days (95% CI, 3-9 days). Myocardial injury at presentation (70% of multisystem inflammatory syndrome in children cases) did not affect short-term outcomes. Four patients (7%) had small coronary aneurysms at presentation, all of which resolved. Only 1 of 9 patients had residual edema but no fibrosis by cardiac magnetic resonance imaging. Conclusions Our short-term study suggests that functional recovery and coronary outcomes are good in multisystem inflammatory syndrome in children. Use of sensitive deformation parameters provides further reassurance that there is no persistent subclinical dysfunction after 3 months.
This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation ...(ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients. In simple D-TGA: 1) familial recurrence risk is low; 2) children diagnosed pre-natally have improved cognitive skills compared with those diagnosed post-natally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and prostaglandin E1 may not be indicated in all cases; 5) early ASO improves outcomes and reduces costs with a low mortality; 6) single or intramural coronary arteries remain risk factors; 7) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 8) coronary insufficiency and arrhythmias are rare but are associated with sudden death; 9) early- and late-onset ND abnormalities are common; 10) aortic regurgitation and aortic root dilation are well tolerated; and 11) the aging ASO patient may benefit from "exercise-prescription" rather than restriction. Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO.
Children and young adults with single-ventricle physiology have abnormal exercise capacity after the Fontan operation. A medication capable of decreasing pulmonary vascular resistance should allow ...improved cardiac filling and improved exercise capacity.
This study was a double-blind, placebo-controlled, crossover trial conducted in children and young adults after Fontan. Subjects were randomized to receive placebo or sildenafil (20 mg three times daily) for 6 weeks. After a 6-week washout, subjects crossed over for an additional 6 weeks. Each subject underwent an exercise stress test at the start and finish of each phase. After taking sildenafil, subjects had a significantly decreased respiratory rate and decreased minute ventilation at peak exercise. At the anaerobic threshold, subjects had significantly decreased ventilatory equivalents of carbon dioxide. There was no change in oxygen consumption during peak exercise, although there was a suggestion of improved oxygen consumption at the anaerobic threshold. Improvement at the anaerobic threshold was limited to the subgroup with single left or mixed ventricular morphology and to the subgroup with baseline serum brain natriuretic peptide levels ≥100 pg/mL.
In this cohort, sildenafil significantly improved ventilatory efficiency during peak and submaximal exercise. There was also a suggestion of improved oxygen consumption at the anaerobic threshold in 2 subgroups. These findings suggest that sildenafil may be an important agent for improving exercise performance in children and young adults with single-ventricle physiology after the Fontan operation. Clinical Trial Registration- URL: http://clinicaltrials.gov. Unique identifier: NCT00507819.
Elevated energy loss in the total cavopulmonary connection (TCPC) is hypothesised to have a detrimental effect on clinical outcomes in single-ventricle physiology, which may be magnified with ...exercise. This study investigates the relationship between TCPC haemodynamic energy dissipation and exercise performance in single-ventricle patients.
Thirty consecutive Fontan patients with TCPC and standard metabolic exercise testing were included. Specific anatomies and flow rates at rest and exercise were obtained from cardiac MR (CMR) and phase-encoded velocity mapping. Exercise CMR images were acquired immediately following supine lower limb exercise using a CMR-compatible cycle ergometer. Computational fluid dynamics simulations were performed to determine power loss of the TCPC anatomies using in vivo anatomies and measured flows.
A significant negative linear correlation was observed between indexed power loss at exercise and (a) minute oxygen consumption (r=-0.60, p<0.0005) and (b) work (r=-0.62, p<0.0005) at anaerobic threshold. As cardiac output increased during exercise, indexed power loss increased in an exponential fashion (y=0.9671x(3.0263), p<0.0001).
This is the first study to demonstrate the relationship between power loss and exercise performance with the TCPC being one of the few modifiable factors to allow for improved quality of life. These results suggest that aerobic exercise tolerance in Fontan patients may, in part, be a consequence of TCPC power loss.
It is known that respiration modulates cavopulmonary flows, but little data compare mean flows under breath-holding and free-breathing conditions to isolate the respiratory effects and effects of ...exercise on the respiratory modulation.
Real-time phase-contrast magnetic resonance combined with a novel method to track respiration on the same image acquisition was used to investigate respiratory effects on Fontan caval and aortic flows under breath-holding, free-breathing, and exercise conditions. Respiratory phasicity indices that were based on beat-averaged flow were used to quantify the respiratory effect.
Flow during inspiration was substantially higher than expiration under the free-breathing and exercise conditions for both inferior vena cava (inspiration/expiration: 1.6 ± 0.5 and 1.8 ± 0.5, respectively) and superior vena cava (inspiration/expiration: 1.9 ± 0.6 and 2.6 ± 2.0, respectively). Changes from rest to exercise in the respiratory phasicity index for these vessels further showed the impact of respiration. Total systemic venous flow showed no significant statistical difference between the breath-holding and free-breathing conditions. In addition, no substantial difference was found between the descending aorta and inferior vena cava mean flows under either resting or exercise conditions.
This study demonstrated that inferior vena cava and superior vena cava flow time variance is dominated by respiratory effects, which can be detected by the respiratory phasicity index. However, the minimal respiration influence on net flow validates the routine use of breath-holding techniques to measure mean flows in Fontan patients. Moreover, the mean flows in the inferior vena cava and descending aorta are interchangeable.
To identify mediators of health status and quality of life (QOL) in children and adolescents aged 8-18 years old following surgical repair for tetralogy of Fallot (TOF), including resource use, ...exercise performance, and 22q11.2 deletion status.
We performed a corollary study to a cross-sectional analysis of subjects following repair for TOF that completed cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and instruments assessing health status and QOL. General linear models were used to test for mediation.
A total of 29 of 151 (19%) patients carried a 22q11.2 deletion. Parents of children with a deletion compared with those without a deletion reported worse physical and psychosocial functioning on the Child Health Questionnaire. The patients with a 22q11.2 deletion and their parents reported lower total and Disease Impact scores compared with the group without a deletion on the Pediatric Cardiac Quality of Life Inventory. Medical care use negatively correlated with measures of health status/QOL. Greater maximum work correlated with better patient health status and QOL, regardless of deletion status. Exercise performance mediated the association between deletion status and parent-reported outcomes (unstandardized effects ranging from 2.4 to 4.2) and patient-reported Disease Impact (0.99; 95% CI 0.02-2.70).
Children and adolescents following repair for TOF seem to suffer significant challenges to their health status and QOL, which is amplified markedly in the context of the 22q11.2 deletion syndrome, and related to exercise performance.
Evaluation of Myocardial Ischemia After Surgical Repair of Anomalous Aortic Origin of a Coronary Artery in a Series of Pediatric Patients Julie A. Brothers, Michael G. McBride, Mohamed A. Seliem, ...Bradley S. Marino, Ryan S. Tomlinson, Miguel H. Pampaloni, J. William Gaynor, Thomas L. Spray, Stephen M. Paridon Anomalous aortic origin of a coronary artery that courses between the great vessels is a rare anomaly associated with increased myocardial ischemia and sudden death risk in children. We prospectively assessed children with exercise stress test, stress echocardiogram, and stress myocardial perfusion scan. One anomalous origin of left main coronary artery patient and 8 anomalous right coronary (ARCA) patients had positive ischemia evaluations. Subclinical changes suggestive of ischemia might occur despite patent neo-coronary ostia, notably after ARCA repair.
Background Anomalous aortic origin of a coronary artery with an interarterial and intramural course (AAOCA) is a rare anomaly with increased risk of sudden cardiac death during or just after exercise ...among otherwise healthy youth. Risk stratification and management remain controversial, especially for the asymptomatic child with an anomalous right coronary artery from the left coronary sinus (ARCA). Medium-term surgical and quality-of-life (QOL) outcome data are lacking in this population. Methods We performed medical record review on 24 subjects who underwent AAOCA repair between 2001 and 2007 at The Children's Hospital of Philadelphia. QOL was prospectively assessed with age-appropriate questionnaires. Results Median age at follow-up was 18 (range, 11-25) years, median follow-up from surgery was 63 (range, 12-110) months, and 16 (67%) had ARCA. All were alive without exercise restriction. Thirteen (54%) complained of cardiac-type symptoms postoperatively, most commonly chest pain, none correlating with evidence of ischemia on testing. Of the 13 patients, 7 (54%) reported the same symptoms preoperatively; and of these, 5 had ARCA. Postoperative morbidity occurred in 16 (67%), including pericardial effusion (n = 11), wound infection (n = 2), and development of mild aortic insufficiency (n = 4). QOL questionnaires were sent to 21 subjects; 12 (57%) were returned. Average QOL was normal for all subjects. Conclusions In the medium-term after AAOCA repair, cardiac-type symptoms frequently persist and morbidity is common, but these do not impair QOL. The significance of these findings in the long-term is unknown and warrants continued follow-up.
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