Inger Olson, MD

Abstract

In this manuscript, we describe the design and rationale of a randomized controlled trial in pediatric Fontan patients to test the hypothesis that a live-video-supervised exercise (aerobic+resistance) intervention will improve cardiac and physical capacity; muscle mass, strength, and function; and endothelial function. Survival of children with single ventricles beyond the neonatal period has increased dramatically with the staged Fontan palliation. Yet, long-term morbidity remains high. By age 40, 50% of Fontan patients will have died or undergone heart transplantation. Factors that contribute to onset and progression of heart failure in Fontan patients remain incompletely understood. However, it is established that Fontan patients have poor exercise capacity which is associated with a greater risk of morbidity and mortality. Furthermore, decreased muscle mass, abnormal muscle function, and endothelial dysfunction in this patient population is known to contribute to disease progression. In adult patients with two ventricles and heart failure, reduced exercise capacity, muscle mass, and muscle strength are powerful predictors of poor outcomes, and exercise interventions can not only improve exercise capacity and muscle mass, but also reverse endothelial dysfunction. Despite these known benefits of exercise, pediatric Fontan patients do not exercise routinely due to their chronic condition, perceived restrictions to exercise, and parental overprotection. Limited exercise interventions in children with congenital heart disease have demonstrated that exercise is safe and effective; however, these studies have been conducted in small, heterogeneous groups, and most had few Fontan patients. Critically, adherence is a major limitation in pediatric exercise interventions delivered on-site, with adherence rates as low as 10%, due to distance from site, transportation difficulties, and missed school or workdays. To overcome these challenges, we utilize live-video conferencing to deliver the supervised exercise sessions. Our multidisciplinary team of experts will assess the effectiveness of a live-video-supervised exercise intervention, rigorously designed to maximize adherence, and improve key and novel measures of health in pediatric Fontan patients associated with poor long-term outcomes. Our ultimate goal is the translation of this model to clinical application as an "exercise prescription" to intervene early in pediatric Fontan patients and decrease long-term morbidity and mortality.

View details for DOI 10.1016/j.ahj.2023.02.006

View details for PubMedID 36796574

Abstract

BACKGROUND: The transition from residency to paediatric cardiology fellowship is challenging due to the new knowledge and technical skills required. Online learning can be an effective didactic modality that can be widely accessed by trainees. We sought to evaluate the effectiveness of a paediatric cardiology Fellowship Online Preparatory Course prior to the start of fellowship.METHODS: The Online Preparatory Course contained 18 online learning modules covering basic concepts in anatomy, auscultation, echocardiography, catheterisation, cardiovascular intensive care, electrophysiology, pulmonary hypertension, heart failure, and cardiac surgery. Each online learning module included an instructional video with pre-and post-video tests. Participants completed pre- and post-Online Preparatory Course knowledge-based exams and surveys. Pre- and post-Online Preparatory Course survey and knowledge-based examination results were compared via Wilcoxon sign and paired t-tests.RESULTS: 151 incoming paediatric cardiology fellows from programmes across the USA participated in the 3 months prior to starting fellowship training between 2017 and 2019. There was significant improvement between pre- and post-video test scores for all 18 online learning modules. There was also significant improvement between pre- and post-Online Preparatory Course exam scores (PRE 43.6 11% versus POST 60.3 10%, p < 0.001). Comparing pre- and post-Online Preparatory Course surveys, there was a statistically significant improvement in the participants' comfort level in 35 of 36 (97%) assessment areas. Nearly all participants (98%) agreed or strongly agreed that the Online Preparatory Course was a valuable learning experience and helped alleviate some anxieties (77% agreed or strongly agreed) related to starting fellowship.CONCLUSION: An Online Preparatory Course prior to starting fellowship can provide a foundation of knowledge, decrease anxiety, and serve as an effective educational springboard for paediatric cardiology fellows.

View details for DOI 10.1017/S1047951122003626

View details for PubMedID 36440543

Abstract

Cor triatriatum may be associated with abnormalities of pulmonary venous anatomy. This case report describes a unique form of partial anomalous pulmonary venous connection. The patient presented at 5 weeks of age with symptoms of tachypnea and poor feeding. Echocardiography demonstrated cor triatriatum and partial anomalous pulmonary venous drainage of the right upper lung. The patient underwent urgent repair of cor triatriatum. It was elected to not address the partial anomalous pulmonary venous connection at that time. The patient returned at age 19 months for elective repair of the anomalous pulmonary venous connection. There was also a large vein connecting the right lower pulmonary veins to the superior vena cava. This was repaired by dividing the superior vena cava along a vertical axis to redirect the flow of the anomalous pulmonary veins through the connecting vein to the left atrium. This report describes the anatomy and surgical approach to a unique form of anomalous pulmonary venous connection.

View details for DOI 10.1177/21501351211046907

View details for PubMedID 34647493

Abstract

Background Pediatric heart transplant recipients have high-risk cardiovascular profiles that can affect their long-term outcomes; however, promoting exercise and healthy diet has not been a major focus in the field. The objective of this study was to test the feasibility and impact of a supervised exercise and diet intervention delivered via live videoconferencing in this population. Methods and Results Patients 8 to 19years of age at least 1year post heart transplantation were enrolled. The 12- to 16-week intervention phase included live video-supervised exercise (3/week) and nutrition (1/week) sessions. The 12- to 16-week maintenance phase included 1/week live video-supervised exercise and nutrition sessions and 2/week self-directed exercise sessions. Cardiac, vascular, nutritional, and functional health indices were obtained at baseline, after intervention, and after maintenance. Fourteen patients (median age, 15.2; interquartile range, 14.3-16.7years) at a median of 3.3 (interquartile range, 1.5-9.7) years after heart transplant completed the intervention. Patients attended 89.611% of exercise and 88.410% of nutrition sessions during the intervention and 93.411% of exercise and 92.311% of nutrition sessions during maintenance. After intervention, body mass index percentile (median, -27%; P=0.02), endothelial function (median, +0.29; P=0.04), maximum oxygen consumption (median, +2mL/kg per minute; P=0.002). Functional Movement Screening total score (median, +2.5; P=0.002) and daily consumption of saturated fat (median, -6g; P=0.02) improved significantly. After maintenance, improvements in maximum oxygen consumption (median, +3.2mL/kg per minute; P=0.02) and Functional Movement Screening total score (median, +5; P=0.002) were sustained. Conclusions In pediatric heart transplant recipients, a live video-supervised exercise and diet intervention is feasible. Our results demonstrate excellent adherence with significant improvements in cardiovascular and functional health. Clinical Trial Registration URL: http://www.clinicaltrials.gov. Unique identifier: NCT02519946.

View details for DOI 10.1161/JAHA.119.013816

View details for PubMedID 31973598

View details for DOI 10.1161/JAHA.119.013816

View details for DOI 10.1111/petr.13316

View details for Web of Science ID 000457579500010

View details for Web of Science ID 000528619406319

Abstract

BACKGROUND: Pediatric heart transplant (PedHtx) patients have increased cardiovascular risk profiles that affect their long-term outcomes and quality of life. We designed a 12- to 16-week diet and exercise intervention delivered via live video conferencing to improve cardiovascular health. Our methodology and baseline assessment of the first 13 enrolled patients are reported.METHODS: Inclusion criteria are as follows: (a) 8-19years old; (b) heart transplant >12months; (c) ability to fast overnight; (d) cardiac clearance by cardiologist; and (e) presence of an adult at home during exercise sessions for patients <14years old. Exclusion criteria are as follows: (a) acute illness; (b) latex allergy; (c) transplant rejection <3months ago; and (d) multi-organ transplantation. The intervention consists of one diet and three exercise sessions weekly via live video conferencing. Study visits are conducted at baseline, intervention completion, and end of maintenance period.RESULTS: A total of 13 participants (15.2 [2.3]years) have been enrolled. Median percent-predicted VO2 max was 56.8 [20.7]% (10 patients <70%). Ten patients had abnormal endothelial function (reactive hyperemia index <1.9; 1.4 [0.325]) and 11 patients had stiff arteries (pulse wave velocity 5.5m/s for 15-19years, 4.5m/s for 8-14years; 5.6 [0.7]m/s). Patients had suboptimal diets (saturated fat: 22.7 [23.8]g/d, sodium: 2771 [1557]mg/d) and were sedentary at a median of 67.5 [13.8]% of their time.CONCLUSIONS: Baseline assessment confirms that PedHtx patients have abnormal cardiac, vascular, and functional health indices, poor dietary habits, and are sedentary. These results support the rationale to test the feasibility and impact of a non-pharmacologic lifestyle intervention in this patient population.

View details for PubMedID 30393915

View details for DOI 10.1017/S1047951118000707

View details for Web of Science ID 000439937200005

Abstract

OBJECTIVES: The goal of this study was to assess the utility of serial electrocardiograms in routine follow-up of paediatric Marfan patients.METHODS: Children 18 years who met the revised Ghent criteria for Marfan syndrome and received a 12-lead electrocardiogram and echocardiogram within a 3-month period were included. Controls were matched by age, body surface area, gender, race, and ethnicity, and consisted of patients assessed in clinic with a normal cardiac evaluation. Demographic, clinical, echocardiographic, and electrocardiographic data were collected.RESULTS: A total of 45 Marfan patients (10.8 [2.4-17.1] years) and 37 controls (12.8 [1.3-17.1] years) were included. Left atrial enlargement and left ventricular hypertrophy were more frequently present on 12-lead electrocardiogram of Marfan patients compared with controls (12 (27%) versus 0 (0%), p<0.001; and 8 (18%) versus 0 (0%), p=0.008, respectively); however, only two patients with left atrial enlargement on 12-lead electrocardiogram were confirmed to have left atrial enlargement by echocardiogram, and one patient had mild left ventricular hypertrophy by echocardiogram, not appreciated on 12-lead electrocardiogram. QTc interval was longer in Marfan patients compared with controls (42716 versus 41722 ms, p=0.03), with four Marfan patients demonstrating borderline prolonged QTc intervals for gender.CONCLUSIONS: While Marfan patients exhibited a higher frequency of left atrial enlargement and left ventricular hypertrophy on 12-lead electrocardiograms compared with controls, these findings were not supported by echocardiography. Serial 12-lead electrocardiograms in routine follow-up of asymptomatic paediatric Marfan patients may be more appropriate for a subgroup of Marfan patients only, specifically those with prolonged QTc interval at their baseline visit.

View details for PubMedID 29972109

View details for DOI 10.1016/S0735-1097(18)33163-2

View details for Web of Science ID 000429659705072

Abstract

To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had 3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.51.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P=.12). Patients with 3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P=.001), longer CVICU lengths of stay (22 vs 3 days; P<.0001), longer overall hospitalizations (28 vs 7 days; P<.0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P<.0001) than those with <3 TTEs. Eight patients with 3 TTEs did not survive, compared with 3 with <3 TTEs (P=.0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

View details for DOI 10.1016/j.jpeds.2016.09.061

View details for Web of Science ID 000390028100018

Abstract

To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had 3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.51.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P=.12). Patients with 3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P=.001), longer CVICU lengths of stay (22 vs 3 days; P<.0001), longer overall hospitalizations (28 vs 7 days; P<.0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P<.0001) than those with <3 TTEs. Eight patients with 3 TTEs did not survive, compared with 3 with <3 TTEs (P=.0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

View details for DOI 10.1016/j.jpeds.2016.09.061

View details for PubMedID 28029346

Abstract

To evaluate adherence of overweight and obese adolescents to a live video lifestyle intervention. The impact on vascular and functional health was also assessed.Twenty adolescents 14.52.1years of age with body mass index z-score 1.940.43 were enrolled. The 12-week intervention included 3-times-weekly videoconference sessions with a trainer and weekly diet consultations. Adherence was evaluated by completion rate and percentage of sessions attended. Vascular health indices and traditional cardiovascular risk factors were obtained at baseline and study end.Seventeen participants (85%) completed the intervention. The participants attended 9311% of scheduled sessions. Reasons for absences included illness/injury (23%), school activities (21%), holidays (18%), forgetting the appointment (8%), Internet connectivity issues (7%), and family emergency (7%). Significant changes were observed in waist-hip ratio (0.870.08 vs 0.840.08, P=.03), total (15927 vs 14723mg/dL, P=.004) and low-density lipoprotein cholesterol levels (9120 vs 8118mg/dL, P=.004), volume of inspired oxygen per heartbeat at peak exercise (6916 vs 7215%, P=.01), and functional movement score (132 vs 171, P<.001). Participants with abnormal vascular function at baseline showed improvement in endothelial function and arterial stiffness indices (P=.01 and P=.04, respectively).A 12-week live video intervention promotes adherence among overweight and obese adolescents and shows promise for improving vascular and functional health. Integrating telehealth into preventive care has the potential to improve cardiovascular health in the youth at risk.

View details for DOI 10.1016/j.jpeds.2015.06.015

View details for PubMedID 26154402

Abstract

The coronary sinus can become obstructed with any instrumentation at or near the ostium such as in atrioventricular canal defect repairs. This complication may lead to a wide range of consequences including dyspnea, angina, myocardial infarction, and sudden death. The following report illustrates the importance of careful perioperative echocardiographic evaluation of the coronary sinus in procedures that may affect the sinus and its ostium.

View details for DOI 10.1111/chd.12096

View details for PubMedID 23682752

View details for Web of Science ID 000337579800030

Abstract

Supine cycling may be an important alternative modality for exercise testing. Subtle differences in supine and upright exercise physiology have been suggested but not fully explored in the pediatric population. The aim of this study was to compare peak and submaximal metabolic data in the upright and supine exercise positions. Healthy children (N=100) performed cardiopulmonary exercise tests using supine and upright cycle ergometry. Recruitment was governed by grant funding and not based on sample size calculations. Subjects exercised to exhaustion. Paired Student's t-tests were used to compare upright and supine data; simple linear regression analyses examined correlations between the two modalities. Peak heart rate was similar in both testing positions. Although peak oxygen uptake (peak VO2), oxygen uptake at anaerobic threshold (VO2@AT), VO2 when the respiratory exchange ratio is consistently at or above 1.0 (VO2@RER1.0), and the oxygen uptake efficiency slope were significantly higher in the upright position, no difference was considered of practical significance when applying Cohen's effect size criteria. Therefore, it may be reasonable for pediatric exercise laboratories to use established normative data and predictions for upright cycling position and apply them to metabolic measures obtained in supine exercise testing. Supine cycling with submaximal parameters may allow objective assessment of exercise capacity in children who undergo exercise echocardiography or cannot perform upright exercise testing.

View details for DOI 10.1007/s00246-013-0841-y

View details for PubMedID 24253612

Abstract

Echocardiography has been used to determine ventricular function, segmental wall motion abnormality, and pulmonary artery pressure before and after peak exercise. No prior study has investigated systolic and diastolic function using echocardiography at various phases of exercise in children. The aim of this study was to determine the fractional shortening (FS), systolic-to-diastolic (S/D) ratio, heart rate-corrected velocity of circumferential fiber shortening (VCFc), circumferential wall stress (WS), ratio of mitral passive inflow to active inflow (E/A), ratio of passive inflow by pulsed-wave to tissue Doppler (E/E'), and right ventricular-to-right atrial pressure gradient from tricuspid valve regurgitation jet velocity (RVP) and time duration at various phases of exercise in children.In an 8-month period (December 2007 to July 2008), 100 healthy children were evaluated, and 97 participants aged 8 to 17 years who performed complete cardiopulmonary exercise stress tests using supine cycle ergometry were prospectively enrolled. The participants consisted of 48 female and 49 male subjects with various body sizes, levels of exercise experience, and physical capacities. The cardiopulmonary exercise stress test consisted of baseline pulmonary function testing, continuous gas analysis and monitoring of blood pressure and heart rate responses, electrocardiographic recordings, and oxygen saturation measurement among participants who pedaled against a ramp protocol based on body weight. All participants exercised to exhaustion. Echocardiography was performed during exercise at baseline, at a heart rate of 130 beats/min, at a heart rate of 160 beats/min, at 5 min after exercise, and at 10 min after exercise. FS, S/D ratio, VCFc, WS, E/A, E', E/E', and RVP at these five phases were compared in all subjects.All echocardiographic parameters differed at baseline from 160 beats/min (P < .0001) except E/E', which remained at 5.4 to 5.8. Specifically, FS (from 37% to 46%), S/D ratio, VCFc (from 1.1 to 1.6), WS (from 200 to 258 g/cm(2)), E' (from 0.2 to 0.3), and RVP (from 18 to 35 mm Hg) increased from baseline to 160 beats/min and then subsequently decreased to at or near baseline, while tricuspid valve regurgitation duration decreased (from 370 to 178 msec).Normal values for systolic and diastolic echocardiographic measurements of function are now available. FS, VCFc, WS, and RVP increase with exercise and then return to near baseline levels. The E/E' ratio is unaltered with exercise in normal subjects.

View details for DOI 10.1016/j.echo.2012.03.007

View details for Web of Science ID 000305701600013

View details for PubMedID 22521368

Abstract

Although exercise testing is an important objective method used to assess cardiopulmonary function, subjective assessment often is used as a proxy in the clinical setting. This study aimed to determine whether responses to a subjective functional capacity questionnaire administered to parents and patients in a pediatric exercise laboratory correlate with objective assessment of functional capacity, measured by peak oxygen consumption during maximal voluntary exercise testing.Subjective questionnaire responses and exercise test results collected over 10 years were retrospectively analyzed. Symptoms and physical capabilities were assessed using a 6-point Likert scale regarding the ability to attend school/work, walk, climb stairs, and run, as well as the frequency of fatigue, palpitations, and chest pain. Values of 0 to 3 were considered abnormal, whereas values of 4-5 were regarded as normal. Exercise testing was performed on a stationary cycle ergometer with a continuous ramping protocol. Heart rate and oxygen saturation were continuously monitored. Blood pressures and electrocardiograms (ECGs) were obtained at 2-min intervals. Metabolic gas analysis was performed using a breath-by-breath method. The results of the exercise testing were normalized for body size and expressed as a percentage of predicted peak oxygen consumption (%pVO(2)).Very weak but statistically significant correlations ( < 0.25; P < 0.05) between the scores of the school/work, walking, stair climbing, running, and fatigue items and %pVO(2) were found using Kendall's rank correlations.The subjective Likert scales used to assess basic physical capacity and cardiac-associated symptoms have limited ability to predict actual functional capacity as measured by %pVO(2) achieved. The very weak rank-order correlation between %pVO(2) achieved and the subjective reporting of the ability to attend school/work, walk, climb stairs, and run has low clinical significance and will not be useful in predicting functional capacity within the clinic setting.

View details for DOI 10.1007/s00246-010-9761-2

View details for Web of Science ID 000282424800015

View details for PubMedID 20811883

Abstract

Prominent azygos veins (PAVs) have been described with interrupted inferior venae cavae (IVCs) with heterotaxy. At the authors' institution, cases of PAVs with uninterrupted IVCs have been noted. The aim of this study was to determine the occurrence rate of PAVs and associated lesions by echocardiography.All patients with PAVs were collected; those with interrupted and uninterrupted IVCs were assigned to groups 1 and 2. Normal controls were assigned to group 3.Among 15,849 patients from January 1, 2001, to March 31, 2008, 55 (0.3%) had PAVs, 42 (76%) in group 1 and 13 (24%) in group 2. Heterotaxy was prominent in group 1, whereas patients in group 2 had no heterotaxy. Patients in group 2 had more structurally normal hearts than those in group 1 (46% vs 14%, P<.01), partial anomalous pulmonary venous return, and one single ventricle. IVC measurements were the same in groups 2 and 3 (P=.65).This study demonstrates that a PAV without IVC interruption is not associated with heterotaxy. Patients with PAVs should be carefully examined for partial anomalous pulmonary venous return.

View details for DOI 10.1016/j.echo.2009.11.025

View details for Web of Science ID 000275221900007

View details for PubMedID 20138465

Abstract

Maximal oxygen consumption (VO2max) is the "gold standard" by which to assess functional capacity; however, it is effort dependent. VO2@RER1.0 is defined when VO2 = VCO2. Between December 22, 1997 and November 9, 2004, 305 pediatric subjects underwent cycle ergometer cardiopulmonary exercise testing, exercised to exhaustion, and reached a peak respiratory exchange ratio > or = 1.10. Group 1 subjects achieved a peak VO2 > or = 80% of predicted VO2max; group 2 subjects achieved a peak VO2 < or = 60% of predicted VO2max; and group 3 subjects achieved a peak VO2 between 61 and 79% of predicted VO2max. Linear regression analysis was performed for VO2@RER1.0 as a function of predicted VO2 for group 1 subjects. A -2 SD regression line and equation was created. VO2@RER1.0 data from groups 2 and 3 were plotted onto the normative graph. Contingency table and relative-risk analysis showed that an abnormal VO2@RER1.0 predicted an abnormal peak VO2(positive-predictive value 83%, negative-predictive value 85%, sensitivity 84%, and specificity 84%). VO2@RER1.0 is a highly sensitive, specific, and predictive submaximal index of functional capacity. This submaximal index is easy to identify without subjectivity. This index may aid in the evaluation of subjects who cannot exercise to maximal parameters.

View details for DOI 10.1007/s00246-009-9544-9

View details for Web of Science ID 000273675400010

View details for PubMedID 19812880