Mark A Fogel

The Children's Hospital of Philadelphia, Filadelfia, Pennsylvania, United States

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Publications (203)1100.51 Total impact

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    ABSTRACT: Patients with single ventricle can develop aortic-to-pulmonary collaterals (APCs). Along with systemic-to-pulmonary artery shunts, these structures represent a direct pathway from systemic to pulmonary circulations, and may limit cerebral blood flow (CBF). This study investigated the relationship between CBF and APC flow on room air and in hypercarbia, which increases CBF in patients with single ventricle. 106 consecutive patients with single ventricle underwent 118 cardiac magnetic resonance (CMR) scans in this cross-sectional study; 34 prior to bidirectional Glenn (BDG) (0.50±0.30 years old), 50 prior to Fontan (3.19±1.03 years old) and 34 3-9 months after Fontan (3.98±1.39 years old). Velocity mapping measured flows in the aorta, cavae and jugular veins. Analysis of variance (ANOVA) and multiple linear regression were used. Significance was p<0.05. A strong inverse correlation was noted between CBF and APC/shunt both on room air and with hypercarbia whether CBF was indexed to aortic flow or body surface area, independent of age, cardiopulmonary bypass time, Po2 and Pco2 (R=-0.67--0.70 for all patients on room air, p<0.01 and R=-0.49--0.90 in hypercarbia, p<0.01). Correlations were not different between surgical stages. CBF was lower, and APCs/shunt flow was higher prior to BDG than in other stages. There is a strong inverse relationship between CBF and APC/shunt flow in patients with single ventricle throughout surgical reconstruction on room air and in hypercarbia independent of other factors. We speculate that APC/shunt flow may have a negative impact on cerebral development and neurodevelopmental outcome. Interventions on APC may modify CBF, holding out the prospect for improving neurodevelopmental trajectory. NCT02135081. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
    Heart (British Cardiac Society) 06/2015; DOI:10.1136/heartjnl-2014-307311 · 6.02 Impact Factor
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    ABSTRACT: To determine the cardiovascular effects of obesity on patients with tetralogy of Fallot (TOF) repair. Ventricular performance measures were compared between obese (body mass index [BMI] ≥95%), overweight (85% ≤BMI <95%), and normal weight subjects (BMI <85%) in a retrospective review of patients with TOF who underwent cardiac magnetic resonance from 2005-2010. Significance was P < .05. Of 260 consecutive patients with TOF, 32 were obese (12.3%), 48 were overweight (18.5%), and 180 were normal weight (69.2%). Biventricular mass was increased in obese compared with normal weight patients with right ventricular mass more affected than left ventricular mass. Obese patients demonstrated decreased biventricular end-diastolic volume (EDV) and stroke volume (SV) when indexed to body surface area (BSA) with an increased heart rate when compared with normal weight patients; cardiac index, ejection fraction, and pulmonary regurgitation fraction were similar. When indexed to ideal BSA, biventricular EDV and SV were similar. EDV and SV for overweight patients were nearly identical to normal weight patients with ventricular mass in between the other 2 groups. Approximately 12% of patients after TOF repair referred for cardiac magnetic resonance in a tertiary referral center are obese with increased biventricular mass. Obese patients and normal weight patients have similar cardiac indices, however, when indexed to actual BSA, obese patients demonstrate decreased EDV and SV with increased heart rate and similar cardiac indices. When indexed to ideal BSA, no differences in biventricular volumes were noted. Copyright © 2015. Published by Elsevier Inc.
    The Journal of pediatrics 05/2015; DOI:10.1016/j.jpeds.2015.04.018 · 3.74 Impact Factor
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    ABSTRACT: Anomalous origin of a coronary artery from the contralateral sinus of Valsalva is associated with exercise-induced ischemia and sudden death. That is thought to be due to aortic enlargement in patients with an elliptical ostium. We hypothesize that virtual angioscopy can identify abnormal coronary ostial morphology in these patients. We retrospectively analyzed 55 consecutive pediatric coronary artery magnetic resonance imaging studies from January 2006 to January 2010 with the diagnosis of anomalous right (n = 20), or left (n = 7) coronary artery, or normal coronary origins (n = 28). One postmortem heart specimen with anomalous left coronary artery was imaged and analyzed to validate our technique. Virtual angioscopy analysis was used for visualization and measurement of the coronary ostia. Distinct aortic origins of the right and left coronaries were seen in all 55 studies. An elliptical orifice with a longer superior-inferior dimension was seen in all anomalous ostia, in contrast to a circular ostium in all normal origins. That was quantified in anomalous ostia with a long-axis to short-axis ratio of 2.5 ± 0.5 (right) and 2.4 ± 0.5 (left) compared with 1.1 ± 0.2 (right) and 1.0 ± 0.3 (left) in controls (p < 0.001 for right and left ostia comparisons). Ostial morphology was confirmed in all 9 patients who underwent operative repair and in 1 patient at autopsy. Virtual angioscopy identifies abnormal ostial morphology in anomalous coronary artery patients, which is important for characterizing the diagnosis of patients who may be at risk for sudden death. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
    The Annals of thoracic surgery 05/2015; DOI:10.1016/j.athoracsur.2015.02.031 · 3.65 Impact Factor
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    ABSTRACT: There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m(2) vs. 118 ± 30 mL/m(2)), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation.
    Pediatric Radiology 05/2015; DOI:10.1007/s00247-015-3358-0 · 1.65 Impact Factor
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    ABSTRACT: In Bi-directional Glenn (BDG) physiology, the superior systemic circulation and pulmonary circulation are in series. Consequently, only blood from the superior vena cava is oxygenated in the lungs. Oxygenated blood then travels to the ventricle where it is mixed with blood returning from the lower body. Therefore, incremental changes in oxygen extraction ratio (OER) could compromise exercise tolerance. In this study, the effect of exercise on the hemodynamic and ventricular performance of BDG physiology was investigated using clinical patient data as inputs for a lumped parameter model coupled with oxygenation equations. Changes in cardiac index, Qp/Qs, systemic pressure, oxygen extraction ratio and ventricular/vascular coupling ratio were calculated for three different exercise levels. The patient cohort (n=29) was sub-grouped by age and pulmonary vascular resistance (PVR) at rest. It was observed that the changes in exercise tolerance are significant in both comparisons, but most significant when sub-grouped by PVR at rest. Results showed that patients over 2 years old with high PVR are above or close to the upper tolerable limit of OER (0.32) at baseline. Patients with high PVR at rest had very poor exercise tolerance while patients with low PVR at rest could tolerate low exercise conditions. In general, ventricular function of SV patients is too poor to increase CI and fulfill exercise requirements. The presented mathematical model provides a framework to estimate the hemodynamic performance of BDG patients at different exercise levels according to patient specific data. Published by Elsevier Ltd.
    Journal of Biomechanics 04/2015; 48(10). DOI:10.1016/j.jbiomech.2015.03.034 · 2.75 Impact Factor
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    ABSTRACT: Children with single ventricle heart disease are at risk for developing systemic to pulmonary arterial collateral vessels that adversely impact short-term outcomes, although the effect on long-term outcomes remains unclear. Collateral flow (CollF) can be quantified using cardiac magnetic resonance (CMR) flow quantification. The velocity-time integral (VTI), obtained from spectral Doppler tracings, has been used in "runoff" lesions like aortic regurgitation to quantify insufficiency. We hypothesized that the VTI ratio of the proximal descending aorta (DAo) after cavopulmonary anastomosis (CPA) would estimate CollF. A retrospective cross-sectional study was conducted. Patients who had a superior CPA or total CPA and underwent CMR between April 2008 and December 2012 were included. Those with greater than trivial semilunar valve insufficiency or aortic arch obstruction were excluded. In a subset (n = 88), spectral Doppler tracings of the DAo were analyzed to determine the VTI ratio. In another subset (n = 112), CMR was used to determine the ratio of retrograde to antegrade flow in the DAo. There was no linear correlation between VTI ratio and CollF (r (2) = .006, P = .46). There was a weakly positive correlation with CollF (r (2) = .07, P = .007) and the CMR measured ratio of retrograde to antegrade flow. Holodiastolic flow reversal by echo did not predict higher CollF (P = .40), but those with holodiastolic flow reversal by CMR had significantly higher CollF (P = .04). The ratio of reverse to forward flow in the DAo as determined by Doppler echo does not accurately reflect CollF in children with single ventricle after CPA. © The Author(s) 2014.
    04/2015; 6(2-2):209-214. DOI:10.1177/2150135114563937
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    ABSTRACT: The investigators recently validated a method of quantifying systemic-to-pulmonary arterial collateral flow using phase-contrast magnetic resonance imaging velocity mapping. Cross-sectional data suggest decreased collateral flow in patients with total cavopulmonary connections (TCPCs) compared with those with superior cavopulmonary connections (SCPCs). However, no studies have examined serial changes in collateral flow from SCPCs to TCPCs in the same patients. The aim of this study was to examine differences in collateral flow between patients with SCPCs and those with TCPCs. Collateral flow was quantified by 2 independent measures from 250 single-ventricle studies in 219 different patients (115 SCPC and 135 TCPC studies, 31 patients with both) and 18 controls, during routine studies using through-plane phase-contrast magnetic resonance imaging. Collateral flow was indexed to body surface area, aortic flow, and pulmonary venous flow. Regardless of indexing method, SCPC patients had significantly higher collateral flow than TCPC patients (1.64 ± 0.8 vs 1.03 ± 0.8 L/min/m(2), p <0.001). In 31 patients who underwent serial examinations, collateral flow as a fraction of aortic flow increased early after TCPC completion. In TCPC patients, indexed collateral flow demonstrated a significant negative correlation with time from TCPC. In conclusion, SCPC and TCPC patients demonstrate substantial collateral flow, with SCPC patients having higher collateral flow than TCPC patients overall. On the basis of the paired subset analysis, collateral flow does not decrease in the short term after TCPC completion and trends toward an increase. In the long term, however, collateral flow decreases over time after TCPC completion. Copyright © 2015 Elsevier Inc. All rights reserved.
    The American journal of cardiology 03/2015; 115(12). DOI:10.1016/j.amjcard.2015.03.022 · 3.43 Impact Factor
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    ABSTRACT: The authors’ reply We read with interest the response1 to our study.2 The authors speculate: “What if indexed power loss (iPL) is dependent on body surface area (BSA)?” We wish to make the following points in response: The data extracted from our paper2 that includes 30 patients shows no statistically significant correlation (p=0.167) between iPL and BSA (see online supplementary figure). This clarifies the speculation and supports our conclusion: ‘iPL correlates with exercise capacity’. We believe this is not surprising given …
    Heart (British Cardiac Society) 02/2015; 101(7). DOI:10.1136/heartjnl-2015-307484 · 6.02 Impact Factor
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    ABSTRACT: Single ventricle lesions are associated with gradual attrition after surgical palliation with the total cavopulmonary connection (TCPC). Ventricular dysfunction is frequently noted, particularly impaired diastolic performance. This study seeks to relate TCPC hemodynamic energy losses to single ventricle volumes and filling characteristics. Cardiac magnetic resonance (CMR) data were retrospectively analyzed for 30 single ventricle patients at an average age of 12.7 ± 4.8 years. Cine ventricular short-axis scans were semiautomatically segmented for all cardiac phases. Ventricular volumes, ejection fraction, peak filling rate, peak ejection rate, and time to peak filling were calculated. Corresponding patient-specific TCPC geometry was acquired from a stack of transverse CMR images; relevant flow rates were segmented from through-plane phase contrast CMR data at TCPC inlets and outlets. The TCPC indexed power loss was calculated from computational fluid dynamics simulations using a validated custom solver. Time-averaged flow conditions and rigid vessel walls were assumed in all cases. Pearson correlations were used to detect relationships between variables, with p less than 0.05 considered significant. Ventricular end-diastolic (R = -0.48) and stroke volumes (R = -0.37) had significant negative correlations with the natural logarithm of a flow-independent measure of power loss. This power loss measure also had a significant positive relationship to time to peak filling rate (normalized to cycle time; R = 0.67). Flow-independent TCPC power loss is inversely related with ventricular end-diastolic and stroke volumes. Elevated power losses may contribute to impaired diastolic filling and limited preload reserve in single ventricle patients. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
    The Annals of Thoracic Surgery 01/2015; 99(3). DOI:10.1016/j.athoracsur.2014.10.043 · 3.63 Impact Factor
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    ABSTRACT: -Patients with repaired tetralogy of Fallot (TOF) experience variable outcomes for reasons that are incompletely understood. We hypothesize that genetic variants contribute to this variability. We sought to investigate the association of 22q11.2 deletion status with clinical outcome in patients with repaired TOF. -We performed a cross sectional study of TOF subjects who were tested for 22q11.2 deletion, and underwent cardiac magnetic resonance (CMR), exercise stress test (EST) and review of medical history. We studied 165 subjects (12.3 ± 3.1 years), of which 30 (18%) had 22q11.2 deletion syndrome (22q11.2DS). Overall, by CMR the right ventricular (RV) ejection fraction was 60±8%, pulmonary regurgitant fraction 34±17%, and RV end-diastolic volume 114±39 cc/m(2). On EST, maximum oxygen consumption (mVO2) was 76±16% predicted. Despite comparable RV function and pulmonary regurgitant fraction, on EST the 22q11.2DS had significantly lower percent predicted: forced vital capacity (61.5 ± 16 vs. 80.5 ± 14, p< 0.0001); mVO2 (61±17 vs. 80±12, p<0.0001); and work (64±18 vs. 86±22, p=0.0002). Similarly, the 22q11.2DS experienced more hospitalizations (6.5 [5; 10] vs. 3 [2; 5], p<0.0001), saw more specialists (3.5 [2; 9] vs. 0 [0; 12], p<0.0001) and used one or more medications (67 vs. 34%, p <0.001). -22q11.2DS is associated with restrictive lung disease, worse aerobic capacity, and increased morbidity, and may explain some of the clinical variability seen in TOF. These findings may provide avenues for intervention to improve outcomes, and should be re-evaluated longitudinally as these associations may become more pronounced with time.
    Circulation Cardiovascular Genetics 01/2015; 8(1). DOI:10.1161/CIRCGENETICS.114.000819 · 5.34 Impact Factor
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    ABSTRACT: OBJECTIVE. Anomalous left coronary artery from the inappropriate aortic sinus with intraseptal course is generally benign but can be confused on imaging studies with the potentially lethal interarterial, intramural anomalous left coronary artery. The purpose of this study was to assess normal ostial morphologic features and intraseptal course using cardiac MRI and CT in pediatric patients with intraseptal anomalous left coronary artery. MATERIALS AND METHODS. A retrospective review was conducted of the medical records of 14 children with the diagnosis of intraseptal anomalous left coronary artery between November 2009 and March 2013. Coronary artery origin and course were evaluated with cardiac MRI or CT, and 3D assessment of coronary ostial morphologic features was performed with virtual angioscopy. RESULTS. The patient ages ranged from 5 to 18 years at diagnosis; 10 (71.4%) were boys. The right and left coronary origins were the right sinus of Valsalva as a common origin (n = 9) or a single coronary artery (n = 5). Anomalous intraseptal left main coronary was found in 13 patients, and one patient had anomalous left anterior descending with retroaortic circumflex coronary artery. Anomalous coronary ostia were round and without stenosis in all studies. The anomalous vessel was identified with echocardiography, but the anomalous left coronary artery was not delineated, and a normal ostium was not adequately portrayed in any instance. CONCLUSION. By use of cardiac MRI and CT, the anomalous course of round coronary ostia was confirmed and visualized in a pediatric cohort with intraseptal anomalous left coronary artery. The data provide the basis for understanding the benign clinical course and showing that surgery is unnecessary for this coronary anomaly.
    American Journal of Roentgenology 01/2015; 204(1):W104-9. DOI:10.2214/AJR.14.12953 · 2.74 Impact Factor
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    ABSTRACT: Utilization of cardiovascular magnetic resonance (CMR) is limited in young children because of the need for sedation or general anesthesia (GA). It has been previously shown that CMR can be performed without sedation or GA in young infants who are prone to fall asleep after being fed and swaddled. The purpose of this study was to prospectively prove the feasibility of the feed-and-sleep CMR technique in larger cohorts in the two institutions where the technique was initially developed. This was a prospective dual-center cohort study over a two-year period. All infants younger than 6 months old with complex congenital cardiovascular anomaly who required CMR were recruited for this study. The exclusion criteria included mechanical ventilation, oxygen dependence, feeding difficulties, and any contraindication to CMR. The feed-and-sleep study was performed by fasting the infant for a period of 4 h prior to the scan, placing the infant in a vacuum immobilizer, and feeding the infant just prior to the CMR. The CMR sequences were prioritized to target the area of most importance first. A study was considered complete and diagnostic if the clinical question was answered. A total of 60 infants (39 from center A and 21 from center B) were recruited for this study, 32 male and 28 female, ages ranging from 1 to 177 days (50 ± 54). The CMR studies were diagnostic and answered the clinical questions in all patients. All infants tolerated the procedure well, and no complications were noted in any of the patients. The CMR duration ranged between 4-132 minutes (45 ± 21). The feed-and-sleep approach in selected patients obviates the need of sedation or GA for CMR in infants younger than 6 months old. Therefore, CMR can be utilized whenever echocardiography fails to provide the complete information required for the patients' management.
    Pediatric Cardiology 12/2014; 36(4). DOI:10.1007/s00246-014-1084-2 · 1.55 Impact Factor
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    ABSTRACT: Pulmonary insufficiency (PI) is associated with right ventricular (RV) dilation, dysfunction, and exercise intolerance in patients with tetralogy of fallot (TOF). We sought to compare RV function and exercise performance in patients with valvar pulmonary stenosis (VPS) following pulmonary balloon valvuloplasty to those with repaired TOF with similar degrees of PI. We performed a cross-sectional study of patients with VPS and TOF. Cardiac magnetic resonance (CMR) and exercise stress test were performed. Subjects were matched by time from initial procedure and severity of PI using propensity scores. After matching, there were 16 patients with VPS and 16 with TOF for comparison, with similar demographics. Time from initial procedure was 14 years (12-16), p = 0.92, and pulmonary regurgitant fraction was 19 % (6-31), p = 0.94, Patients with TOF had lower ejection fraction [58 % (53-66) vs. 65 % (60-69), p = 0.04] and more RV hypertrophy [69 g/m(2) (52-86) vs. 44 g/m(2) (32-66), p = 0.04] compared to those with VPS. Aerobic capacity was worse in patients with TOF [68 ± 19 % mVO2 (56-84) vs. 82 ± 9.2 % (74-89) in VPS, p = 0.01], with a trend for less habitual physical activity [0.9 (0-12) vs. 8 h/week (4-12), p = 0.056], respectively. With similar degrees of PI, patients with TOF demonstrate worse RV function and aerobic capacity as compared to patients with just VPS. Habitual exercise may in part explain differences in exercise performance and should be further explored.
    Pediatric Cardiology 12/2014; 36(4). DOI:10.1007/s00246-014-1087-z · 1.55 Impact Factor
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    ABSTRACT: Pulse-wave velocity (PWV), a measure of arterial stiffness, is a known independent risk factor for cardiovascular events. Patients with single ventricle who undergo aortic to pulmonary anastomosis (recon) have noncompliant patch material inserted into the neoaorta, possibly increasing vessel stiffness and afterload. The purpose of this study is to determine if PWV in patients with single ventricle differed between those who did and those who did not undergo aortic reconstruction (nonrecon). We retrospectively reviewed cardiac magnetic resonance anatomic, cine, and phase contrast evaluations in the ascending aorta and descending aorta (DAo) at the level of the diaphragm data from 126 patients with single ventricle (8.6 ± 8.0 years) from January 2012 to May 2013. Significance = p <0.05. Seventy-five patients underwent recon and 51 did not. PWV in recon was significantly higher than in nonrecon (3.9 ± 0.9 m/s vs 3.2 ± 1.0 m/s, p = 0.008); in recon, patients >13 years old had a higher PWV than those <7 years (4.5 ± 0.6 vs 3.5 ± 0.7 m/s, p = 0.004). Whether <7 or >13 years old, PWV of those with recon was higher than nonrecon DAo distensibility was similar between both groups. There was no difference in age, body surface area, or cardiac index between recon and nonrecon. No correlations between various hemodynamic and ventricular function parameters with PWV were noted. In conclusion, PWV in recon is higher than in nonrecon with similar DAo distensibility implicating the aortic reconstruction as a possible cause of increased afterload; older recon patients have stiffer aortas than younger ones, possibly imposing an additional cardiovascular risk in the future. Other biomaterials may potentially moderate PWV if clinical outcome is adversely affected. Copyright © 2014 Elsevier Inc. All rights reserved.
    The American Journal of Cardiology 12/2014; 114(12):1902-7. DOI:10.1016/j.amjcard.2014.09.032 · 3.43 Impact Factor
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    ABSTRACT: As patients with a single-ventricle physiology age, long-term complications inherent to this population become more evident. Previous studies have focused on correlating anatomic and hemodynamic performance, but there is little information of how these variables change with time. Vessel growth and flow rate changes were quantified using cardiac magnetic resonance and their effects on hemodynamics were assessed, which could affect the long-term outcome. Forty-eight patients with a lateral tunnel or extracardiac conduit Fontan who underwent two cardiac magnetic resonance scans (average interval, 5.1 ± 2.3 years) were studied. Total cavopulmonary connection anatomic and flow variables were reconstructed and normalized to body surface area(1/2). Total cavopulmonary connection hemodynamic efficiency (indexed power loss) was obtained through computational fluid dynamic modeling. Absolute vessel diameters increased with time, normalized diameters decreased, and vessel mean flow rates remained unchanged. Indexed power loss changed significantly in the cohort, as well as in patients in whom the minimum normalized left pulmonary artery decreased. Age at first scan and connection type (lateral tunnel or extracardiac conduit) were not associated with changes in indexed power loss. We present the largest serial cardiac magnetic resonance Fontan cohort to date. Although flow rates increased proportionally to body surface area, vessel diameters did not match somatic growth. As a result, energy losses increased significantly with time in the cohort analyzed. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
    The Annals of Thoracic Surgery 11/2014; 99(1). DOI:10.1016/j.athoracsur.2014.08.046 · 3.63 Impact Factor
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    ABSTRACT: The American College of Cardiology (ACC) participated in a joint project with the American Society of Echocardiography, the Society of Pediatric Echocardiography, and several other subspecialty societies and organizations to establish and evaluate Appropriate Use Criteria (AUC) for the initial use of outpatient pediatric echocardiography. Assumptions for the AUC were identified, including the fact that all indications assumed a first-time transthoracic echocardiographic study in an outpatient setting for patients without previously known heart disease. The definitions for frequently used terminology in outpatient pediatric cardiology were established using published guidelines and standards and expert opinion. These AUC serve as a guide to help clinicians in the care of children with possible heart disease, specifically in terms of when a transthoracic echocardiogram is warranted as an initial diagnostic modality in the outpatient setting. They are also a useful tool for education and provide the infrastructure for future quality improvement initiatives as well as research in healthcare delivery, outcomes, and resource utilization. To complete the AUC process, the writing group identified 113 indications based on common clinical scenarios and/or published clinical practice guidelines, and each indication was classified into 1 of 9 categories of common clinical presentations, including palpitations, syncope, chest pain, and murmur. A separate, independent rating panel evaluated each indication using a scoring scale of 1 to 9, thereby designating each indication as "Appropriate" (median score 7 to 9), "May Be Appropriate" (median score 4 to 6), or "Rarely Appropriate" (median score 1 to 3). Fifty-three indications were identified as Appropriate, 28 as May Be Appropriate, and 32 as Rarely Appropriate.
    Journal of the American College of Cardiology 11/2014; 64(19):2039-2060. DOI:10.1016/j.jacc.2014.08.003 · 15.34 Impact Factor
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    ABSTRACT: Background Radiation exposure in the pediatric population may increase the risk of future malignancy. Children with congenital heart disease who often undergo repeated catheterizations are at risk. One possible strategy to reduce radiation is to use X-ray Magnetic Resonance Fusion (XMRF) to facilitate cardiac catheterization. Methods Catheterization data of patients who underwent diagnostic XMRF procedures between January 1, 2009 and February 1, 2012 were reviewed. Cases were matched 1:1 to contemporary controls who did not undergo XMRF based on weight and diagnosis and were compared in radiation exposure, contrast dose, and procedural and anesthesia times. ResultsForty-four matched pairs were included. Baseline demographics were similar in both groups. Patients in the XMRF group had lower indices of radiation exposure measured by fluoroscopy time (14 vs. 16.4 vs. P=0.047), dose-area product from fluoroscopy (513.2 vs. 589.1 mu Gym(2), P=0.042), total dose-area product (625.8 vs. 995.2 mu Gym(2), P=0.027), and total air kerma dose (94.5 vs. 153.8 mGy, P=0.017). There was also a significant reduction in contrast dose (2 vs. 3.3 cc/kg, P <0.001). Procedural time tended to be shorter in the XMRF group but anesthesia time was significantly longer. Conclusion Select diagnostic cardiac catheterization cases that utilized XMRF used less radiation and contrast than similar cases where XMRF was not used. Future work is needed to determine whether similar benefits can be extended to other types of diagnostic and complex interventional procedures. (c) 2014 Wiley Periodicals, Inc.
    Catheterization and Cardiovascular Interventions 11/2014; 84(5). DOI:10.1002/ccd.25473 · 2.40 Impact Factor
  • Mark Fogel · Matthew Harris · Kevin Whitehead
    2014 American Academy of Pediatrics National Conference and Exhibition; 10/2014
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    ABSTRACT: Objective 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. Methods 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. Results 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=0.9671x3.0263, p<0.0001). Conclusions 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.
    Heart (British Cardiac Society) 09/2014; 101(2). DOI:10.1136/heartjnl-2014-306337 · 6.02 Impact Factor
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    ABSTRACT: Background. Single-ventricle patients undergoing surgical reconstruction experience a high rate of brain injury. Incidental findings on preoperative brain scans may result in safety considerations involving hemorrhage extension during cardiopulmonary bypass that result in surgical postponement. Methods. Single-ventricle patients were studied with brain scans immediately preoperatively, as part of a National Institutes of Health study, and were reviewed by neuroradiology immediately before cardiopulmonary bypass. Results. Of 144 consecutive patients recruited into the project, 33 were studied before stage I (3.7 +/- 1.8 days), 34 before bidirectional Glenn (5.8 +/- 0.5 months), and 67 before Fontan (3.3 +/- 1.1 years) operations. Six operations (4.5%), 2 before stage I, 3 before bidirectional Glenn, and 1 before Fontan, were postponed because of concerning findings on brain magnetic resonance imaging. Five were due to unexpected incidental findings of acute intracranial hemorrhage, and 1 was due to diffuse cerebellar cytotoxic edema; none who proceeded to operation had these lesions. Prematurity and genetic syndromes were not present in any patients with a postponed operation. Four of 4 before bidirectional Glenn/Fontan with surgical delays had hypoplastic left heart syndrome compared with 44 of 97 who did not (p = 0.048). After observation and follow-up, all eventually had successful operations with bypass. Conclusions. Preoperative brain magnetic resonance imaging performed in children with single ventricles disclosed injuries in 4.5% leading to surgical delay; hemorrhagic lesions were most common and raised concerns for extension during the operation. The true risk of progression and need for delay of the operation due to heparinization associated with these lesions remains uncertain. (C) 2014 by The Society of Thoracic Surgeons
    The Annals of Thoracic Surgery 08/2014; 98(5). DOI:10.1016/j.athoracsur.2014.05.079 · 3.63 Impact Factor

Publication Stats

3k Citations
1,100.51 Total Impact Points

Institutions

  • 1995–2015
    • The Children's Hospital of Philadelphia
      • • Department of Pediatrics
      • • Division of Cardiology
      Filadelfia, Pennsylvania, United States
  • 1993–2014
    • William Penn University
      Filadelfia, Pennsylvania, United States
  • 2012
    • University of Pennsylvania
      • Department of Physics and Astronomy
      Philadelphia, PA, United States
  • 2002–2011
    • Georgia Institute of Technology
      • Department of Biomedical Engineering
      Atlanta, Georgia, United States
  • 1999–2011
    • Hospital of the University of Pennsylvania
      • Department of Pediatrics
      Philadelphia, Pennsylvania, United States