To evaluate the feasibility of dobutamine stress magnetic resonance (DSMR) in pediatric patients.... more To evaluate the feasibility of dobutamine stress magnetic resonance (DSMR) in pediatric patients. The medical records of all DSMR studies performed on patients < or =22 years old at a single institution were retrospectively reviewed. The DSMR protocol included dobutamine doses up to 40 microg/kg/minute and atropine to attain the target heart rate [0.85 . (220 - age)]. Thirty-two DSMR studies were performed in 28 patients (median age = 7.3 years; range = 0.8-22 years). Twenty of the studies were performed under general anesthesia. The protocol was completed in 26 studies, technical problems and interruptions were few, and image quality scores (1-5) for all ventricular wall segments were high (mean = 4.2). A heart rate > or =160 bpm was attained in 84% of the studies, a rate pressure product > or =20,000 beats . mm Hg in 87%, and a heart rate greater than or equal to the target heart rate in 19%. No serious adverse events occurred. One patient had an inducible wall motion abnormality. Interobserver agreement was 100% (kappa = 1.0) for test positivity and 92% (kappa = 0.72) for wall motion scores. DSMR in pediatric patients is feasible and provides high-quality imaging of all ventricular wall segments with low interobserver variability. Further exploration of DSMR in pediatric patients is warranted, particularly for those children who are unable to cooperate sufficiently for exercise stress or have poor acoustic windows.
To investigate the pathophysiology of chronic venous insufficiency in adults with a Fontan circul... more To investigate the pathophysiology of chronic venous insufficiency in adults with a Fontan circulation. Chronic venous insufficiency (CVI) is prevalent in adults with a Fontan circulation, however the underlying pathophysiology has not been identified. We defined the prevalence of venous reflux and obstruction in a Fontan population, identified associated factors, and assessed the hypothesis that venous reflux correlates with clinical CVI in adults with a Fontan circulation. Fifty-one adult Fontan subjects from the Congenital Heart Disease in Adults: Lower Extremity Systemic Venous Health in Fontan Patients (CALF) study, ten control subjects without cardiac disease, and ten comparator subjects with repaired tetralogy of Fallot underwent lower extremity Doppler venous ultrasound. We investigated 10 venous segments in each subject for venous obstruction and reflux. The prevalence of venous reflux was significantly greater in the Fontan population compared to healthy controls (51% vs. 10%, p=0.03). Venous flow patterns demonstrated a range of pulsatility and biphasic flow in many subjects. There was no evidence of venous obstruction in any subject. Venous reflux did not correlate with clinical venous insufficiency; 49% of Fontan subjects with observed venous reflux demonstrated no clinical signs of venous disease. Predictors of venous reflux in multivariate analysis were single right ventricle (p=0.03), use of anti-arrhythmic medications (p=0.04), and family history of venous disease (p=0.003). Venous reflux is highly prevalent and venous obstruction is absent in adults with a Fontan circulation. Clinical CVI underestimates pathophysiologic venous insufficiency in adults with a Fontan circulation.
Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform cl... more Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform clinical decision-making. The objective of this study was to identify priorities for clinical research in ACHD. A list of 45 research questions was developed by the Alliance for Adult Research in Congenital Cardiology (AARCC), compiled into a survey, and administered to ACHD providers. Patient input was sought via the Adult Congenital Heart Association at community meetings and online forums. The 25 top questions were sent to ACHD providers worldwide via an online survey. Each question was ranked based on perceived priority and weighted based on time spent in ACHD care. The top 10 topics identified are presented and discussed. The final online survey yielded 139 responses. Top priority questions related to tetralogy of Fallot (timing of pulmonary valve replacement and criteria for primary prevention ICDs), patients with systemic right ventricles (determining the optimal echocardiographic techniques for measuring right ventricular function, and indications for tricuspid valve replacement and primary prevention ICDs), and single ventricle/Fontan patients (role of pulmonary vasodilators, optimal anticoagulation, medical therapy for preservation of ventricular function, treatment for protein losing enteropathy). In addition, establishing criteria to refer ACHD patients for cardiac transplantation was deemed a priority. The ACHD field is in need of prospective research to address fundamental clinical questions. It is hoped that this methodical consultation process will inform researchers and funding organizations about clinical research topics deemed to be of high priority.
An increasing number of adults with congenital heart disease (CHD) require implantable cardiovert... more An increasing number of adults with congenital heart disease (CHD) require implantable cardioverter-defibrillators (ICDs), yet little is known about their impact on psychological well-being and sexual function. To assess shock-related anxiety in adults with CHD and its association with depression and sexual function. A prospective, multicenter, cross-sectional study was conducted on adult patients with CHD with (ICD(+)) and without (ICD(-)) ICDs. The Florida Shock Anxiety Scale was administered to patients with ICD(+) and the Beck Depression Inventory-II to all patients. Men completed the Sexual Health Inventory for Men, and women completed the Female Sexual Function Index. A total of 180 adults with CHD (ICD(+): n = 70; ICD(-): n = 110; median age 32 years [interquartile range 27-40 years]; 44% women) were enrolled. The complexity of CHD was classified as mild in 32 (18%), moderate in 93 (52%), and severe in 54 (30%) subjects. In ICD recipients, a high level of shock-related anxiety was identified (Florida Shock Anxiety Scale score 16; interquartile range 12-23.5), which was slightly higher than the median score for ICD recipients in the general population (P = .057). A higher level of shock-related anxiety was associated with poorer sexual function scores in both men (Spearman's ρ =-.480; P<.001) and women (Spearman's ρ =-.512; P<.01). It was also associated with self-reported depressive symptomatology (Spearman's ρ = .536; P< .001). Adults with CHD and ICDs demonstrate a high level of shock-related anxiety, which is associated with lower sexual functioning scores in men and women. These results underscore the need for increased clinical attention related to ICD-related shock anxiety and impaired sexual function in this population.
The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile ... more The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages. The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age. The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.
Typically, a Fontan connection is constructed as either a lateral tunnel (LT) pathway or an extra... more Typically, a Fontan connection is constructed as either a lateral tunnel (LT) pathway or an extracardiac (EC) conduit. The LT is formed partially by atrial wall and is assumed to have growth potential, but the extent and nature of LT pathway growth have not been well characterized. A quantitative analysis was performed to evaluate this issue. Retrospective serial cardiac magnetic resonance data were obtained for 16 LT and 9 EC patients at 2 time points (mean time between studies, 4.2 ± 1.6 years). Patient-specific anatomies and flows were reconstructed. Geometric parameters of Fontan pathway vessels and the descending aorta were quantified, normalized to body surface area (BSA), and compared between time points and Fontan pathway types. Absolute LT pathway mean diameters increased over time for all but 2 patients; EC pathway size did not change (2.4 ± 2.2 mm vs 0.02 ± 2.1 mm, p < 0.05). Normalized LT and EC diameters decreased, while the size of the descending aorta increased proportionally to BSA. Growth of other cavopulmonary vessels varied. The patterns and extent of LT pathway growth were heterogeneous. Absolute flows for all vessels analyzed, except for the superior vena cava, proportionally to BSA. Fontan pathway vessel diameter changes over time were not proportional to somatic growth but increases in pathway flows were; LT pathway diameter changes were highly variable. These factors may impact Fontan pathway resistance and hemodynamic efficiency. These findings provide further understanding of the different characteristics of LT and EC Fontan connections and set the stage for further investigation.
To evaluate the feasibility of dobutamine stress magnetic resonance (DSMR) in pediatric patients.... more To evaluate the feasibility of dobutamine stress magnetic resonance (DSMR) in pediatric patients. The medical records of all DSMR studies performed on patients < or =22 years old at a single institution were retrospectively reviewed. The DSMR protocol included dobutamine doses up to 40 microg/kg/minute and atropine to attain the target heart rate [0.85 . (220 - age)]. Thirty-two DSMR studies were performed in 28 patients (median age = 7.3 years; range = 0.8-22 years). Twenty of the studies were performed under general anesthesia. The protocol was completed in 26 studies, technical problems and interruptions were few, and image quality scores (1-5) for all ventricular wall segments were high (mean = 4.2). A heart rate > or =160 bpm was attained in 84% of the studies, a rate pressure product > or =20,000 beats . mm Hg in 87%, and a heart rate greater than or equal to the target heart rate in 19%. No serious adverse events occurred. One patient had an inducible wall motion abnormality. Interobserver agreement was 100% (kappa = 1.0) for test positivity and 92% (kappa = 0.72) for wall motion scores. DSMR in pediatric patients is feasible and provides high-quality imaging of all ventricular wall segments with low interobserver variability. Further exploration of DSMR in pediatric patients is warranted, particularly for those children who are unable to cooperate sufficiently for exercise stress or have poor acoustic windows.
To investigate the pathophysiology of chronic venous insufficiency in adults with a Fontan circul... more To investigate the pathophysiology of chronic venous insufficiency in adults with a Fontan circulation. Chronic venous insufficiency (CVI) is prevalent in adults with a Fontan circulation, however the underlying pathophysiology has not been identified. We defined the prevalence of venous reflux and obstruction in a Fontan population, identified associated factors, and assessed the hypothesis that venous reflux correlates with clinical CVI in adults with a Fontan circulation. Fifty-one adult Fontan subjects from the Congenital Heart Disease in Adults: Lower Extremity Systemic Venous Health in Fontan Patients (CALF) study, ten control subjects without cardiac disease, and ten comparator subjects with repaired tetralogy of Fallot underwent lower extremity Doppler venous ultrasound. We investigated 10 venous segments in each subject for venous obstruction and reflux. The prevalence of venous reflux was significantly greater in the Fontan population compared to healthy controls (51% vs. 10%, p=0.03). Venous flow patterns demonstrated a range of pulsatility and biphasic flow in many subjects. There was no evidence of venous obstruction in any subject. Venous reflux did not correlate with clinical venous insufficiency; 49% of Fontan subjects with observed venous reflux demonstrated no clinical signs of venous disease. Predictors of venous reflux in multivariate analysis were single right ventricle (p=0.03), use of anti-arrhythmic medications (p=0.04), and family history of venous disease (p=0.003). Venous reflux is highly prevalent and venous obstruction is absent in adults with a Fontan circulation. Clinical CVI underestimates pathophysiologic venous insufficiency in adults with a Fontan circulation.
Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform cl... more Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform clinical decision-making. The objective of this study was to identify priorities for clinical research in ACHD. A list of 45 research questions was developed by the Alliance for Adult Research in Congenital Cardiology (AARCC), compiled into a survey, and administered to ACHD providers. Patient input was sought via the Adult Congenital Heart Association at community meetings and online forums. The 25 top questions were sent to ACHD providers worldwide via an online survey. Each question was ranked based on perceived priority and weighted based on time spent in ACHD care. The top 10 topics identified are presented and discussed. The final online survey yielded 139 responses. Top priority questions related to tetralogy of Fallot (timing of pulmonary valve replacement and criteria for primary prevention ICDs), patients with systemic right ventricles (determining the optimal echocardiographic techniques for measuring right ventricular function, and indications for tricuspid valve replacement and primary prevention ICDs), and single ventricle/Fontan patients (role of pulmonary vasodilators, optimal anticoagulation, medical therapy for preservation of ventricular function, treatment for protein losing enteropathy). In addition, establishing criteria to refer ACHD patients for cardiac transplantation was deemed a priority. The ACHD field is in need of prospective research to address fundamental clinical questions. It is hoped that this methodical consultation process will inform researchers and funding organizations about clinical research topics deemed to be of high priority.
An increasing number of adults with congenital heart disease (CHD) require implantable cardiovert... more An increasing number of adults with congenital heart disease (CHD) require implantable cardioverter-defibrillators (ICDs), yet little is known about their impact on psychological well-being and sexual function. To assess shock-related anxiety in adults with CHD and its association with depression and sexual function. A prospective, multicenter, cross-sectional study was conducted on adult patients with CHD with (ICD(+)) and without (ICD(-)) ICDs. The Florida Shock Anxiety Scale was administered to patients with ICD(+) and the Beck Depression Inventory-II to all patients. Men completed the Sexual Health Inventory for Men, and women completed the Female Sexual Function Index. A total of 180 adults with CHD (ICD(+): n = 70; ICD(-): n = 110; median age 32 years [interquartile range 27-40 years]; 44% women) were enrolled. The complexity of CHD was classified as mild in 32 (18%), moderate in 93 (52%), and severe in 54 (30%) subjects. In ICD recipients, a high level of shock-related anxiety was identified (Florida Shock Anxiety Scale score 16; interquartile range 12-23.5), which was slightly higher than the median score for ICD recipients in the general population (P = .057). A higher level of shock-related anxiety was associated with poorer sexual function scores in both men (Spearman's ρ =-.480; P<.001) and women (Spearman's ρ =-.512; P<.01). It was also associated with self-reported depressive symptomatology (Spearman's ρ = .536; P< .001). Adults with CHD and ICDs demonstrate a high level of shock-related anxiety, which is associated with lower sexual functioning scores in men and women. These results underscore the need for increased clinical attention related to ICD-related shock anxiety and impaired sexual function in this population.
The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile ... more The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages. The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age. The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.
Typically, a Fontan connection is constructed as either a lateral tunnel (LT) pathway or an extra... more Typically, a Fontan connection is constructed as either a lateral tunnel (LT) pathway or an extracardiac (EC) conduit. The LT is formed partially by atrial wall and is assumed to have growth potential, but the extent and nature of LT pathway growth have not been well characterized. A quantitative analysis was performed to evaluate this issue. Retrospective serial cardiac magnetic resonance data were obtained for 16 LT and 9 EC patients at 2 time points (mean time between studies, 4.2 ± 1.6 years). Patient-specific anatomies and flows were reconstructed. Geometric parameters of Fontan pathway vessels and the descending aorta were quantified, normalized to body surface area (BSA), and compared between time points and Fontan pathway types. Absolute LT pathway mean diameters increased over time for all but 2 patients; EC pathway size did not change (2.4 ± 2.2 mm vs 0.02 ± 2.1 mm, p < 0.05). Normalized LT and EC diameters decreased, while the size of the descending aorta increased proportionally to BSA. Growth of other cavopulmonary vessels varied. The patterns and extent of LT pathway growth were heterogeneous. Absolute flows for all vessels analyzed, except for the superior vena cava, proportionally to BSA. Fontan pathway vessel diameter changes over time were not proportional to somatic growth but increases in pathway flows were; LT pathway diameter changes were highly variable. These factors may impact Fontan pathway resistance and hemodynamic efficiency. These findings provide further understanding of the different characteristics of LT and EC Fontan connections and set the stage for further investigation.
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