Low Ventricular Stiffness Is Associated With Suboptimal Outcomes in Patients With a Single Right Ventricle After the Fontan Operation: A Novel Phenotype.

BACKGROUND: Despite a rigorous screening process, including cardiac catheterization, a subset of patients with a single right ventricle (SRV) demonstrates suboptimal short-term outcomes after the Fontan operation. The goal of this study was to perform a comprehensive assessment of diastolic function in pre-Fontan patients with an SRV using invasive reference-standard measures and determine their associations with post-Fontan outcomes. METHODS AND RESULTS: Children aged 2 to 6 years with SRV physiology undergoing pre-Fontan heart catheterization were recruited prospectively. Patients were divided into those who had an optimal or suboptimal outcome. A suboptimal outcome was defined as length of stay ≥14 days or heart transplant/cardiac death in first year after Fontan. Patients underwent pressure-volume loop analysis using reference-standard methods. The measure of ventricular stiffness, ß, was obtained via preload reduction. Cardiac magnetic resonance imaging for extracellular volume and serum draws for matrix metalloproteinase activity were performed. Of 19 patients with an SRV, 9 (47%) had a suboptimal outcome. Mean age was 4.2±0.7 years. Patients with suboptimal outcomes had lower ventricular stiffness (0.021 [0.009-0.049] versus 0.090 [0.031-0.118] mL-1; P=0.02), lower extracellular volume (25% [28%-32%] versus 31% [28%-33%]; P=0.02), and lower matrix metalloproteinase-2 (90 [79-104] versus 108 [79-128] ng/mL; P=0.01) compared with patients with optimal outcomes. The only invasive measure that had an association with suboptimal outcome was ß (P=0.038). CONCLUSIONS: Patients with an SRV with suboptimal outcome after the Fontan operation had lower ventricular stiffness and evidence of maladaptive extracellular matrix metabolism compared with patients with optimal outcome. This appears to be a novel phenotype that may have important clinical implications and requires further study.

Diastolic Dysfunction With Preserved Ejection Fraction After the Fontan Procedure.

Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross-sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e'/end-diastolic volume) was >90th percentile (≥0.26 mL-1). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO2. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P<0.01. DD patients had lower predicted peak VO2 compared with controls (52% [20] versus 67% [23], P<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7), P<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. OBJECTIVES: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. METHODS: CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. RESULTS: Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CONCLUSIONS: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Challenges With Left Ventricular Functional Parameters: The Pediatric Heart Network Normal Echocardiogram Database.

BACKGROUND: The reliability of left ventricular (LV) systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of LV size and systolic function in children with normal cardiac anatomy and qualitatively normal function. METHODS: The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race. A core lab used two-dimensional echocardiograms to measure LV dimensions from which a separate data coordinating center calculated LV volumes and systolic functional indices. To evaluate interobserver variability, two independent expert pediatric echocardiographic observers remeasured LV dimensions on a subset of studies, while blinded to calculated volumes and functional indices. RESULTS: Of 3,215 subjects with measurable images, 552 (17%) had a calculated LV shortening fraction (SF) < 25% and/or LV ejection fraction (EF) < 50%; the subjects were significantly younger and smaller than those with normal values. When the core lab and independent observer measurements were compared, individual LV size parameter intraclass correlation coefficients were high (0.81-0.99), indicating high reproducibility. The intraclass correlation coefficients were lower for SF (0.24) and EF (0.56). Comparing reviewers, 40/56 (71%) of those with an abnormal SF and 36/104 (35%) of those with a normal SF based on core lab measurements were calculated as abnormal from at least one independent observer. In contrast, an abnormal EF was less commonly calculated from the independent observers' repeat measures; only 9/47 (19%) of those with an abnormal EF and 8/113 (7%) of those with a normal EF based on core lab measurements were calculated as abnormal by at least one independent observer. CONCLUSIONS: Although blinded measurements of LV size show good reproducibility in healthy children, subsequently calculated LV functional indices reveal significant variability despite qualitatively normal systolic function. This suggests that, in clinical practice, abnormal SF/EF values may result in repeat measures of LV size to match the subjective assessment of function. Abnormal LV functional indices were more prevalent in younger, smaller children.

Diagnostic Accuracy of Noncontrast Self-navigated Free-breathing MR Angiography versus CT Angiography: A Prospective Study in Pediatric Patients with Suspected Anomalous Coronary Arteries.

RATIONALE AND OBJECTIVES: To evaluate the diagnostic accuracy of a prototype noncontrast, free-breathing, self-navigated 3D (SN3D) MR angiography (MRA) technique for the assessment of coronary artery anatomy in children with known or suspected coronary anomalies, using CT angiography (CTA) as the reference standard. MATERIALS AND METHODS: Twenty-one children (15 male, 12.3 ± 2.6 years) were prospectively enrolled between July 2014 and August 2016 in this IRB-approved, HIPAA-compliant study. Patients underwent same-day unenhanced SN3D-MRA and contrast-enhanced CTA. Two observers rated the visualization of coronary artery segments and diagnostic confidence on a 3-point scale and assessed coronary arteries for anomalous origin, as well as interarterial and intramural course. Sensitivity, specificity, positive (PPV) and negative predictive values (NPV) of SN3D-MRA for the detection of coronary artery abnormalities were calculated. Interobserver agreement was assessed using Intraclass Correlation Coefficients (ICC). RESULTS: Fourteen children showed coronary artery abnormalities on CTA. The visualization of coronary segments was rated significantly higher for CTA compared to MRA (p <0.015), except for the left main coronary artery (p = 0.301), with good to excellent interobserver agreement (ICC = 0.62-0.94). Diagnostic confidence was higher for CTA (p = 0.046). Sensitivity, specificity, PPV, and NPV of MRA were 92%, 92%, 96%, and 87% for the detection of coronary artery anomalies, 85%, 85%, 74%, and 92% for high origin, 71%, 92%, 82%, and 87% for interarterial, and 41%, 96%, 87%, and 80% for intramural course. CONCLUSIONS: Noncontrast SN3D-MRA is highly accurate for the detection of coronary artery anomalies in pediatric patients while diagnostic confidence and coronary artery visualization remain superior with CTA.

Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome.

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed. Stiffness index (SI) and elastic modulus (EM) were calculated for aortic root and ascending aorta. Data were analyzed using multivariable mixed effects modeling and Cox regression. Heart rate-corrected aortic-root SI over 3 years decreased with atenolol but did not change with losartan (-0.298 ± 0.139 vs 0.141 ± 0.139/year, p = 0.01). In the entire cohort, above-median aortic-root SI (>9.1) and EM (>618 mm Hg) predicted a smaller annual decrease in ARz (p ≤0.001). Upper-quartile aortic-root EM (>914 mm Hg) predicted the composite outcome of aortic-root surgery, dissection, or death (hazard ratio 2.17, 95% confidence interval 1.02 to 4.63, p = 0.04). Crude 3-year event rates were 10.4% versus 3.2% for higher versus lower EM groups. In conclusion, atenolol was associated with a decrease in aortic-root SI, whereas losartan was not. Higher baseline aortic-root SI and EM were associated with a smaller decrease in ARz and increased risk for clinical outcomes. These data suggest that noninvasive aortic stiffness measures may identify patients at higher risk of progressive aortic enlargement and adverse clinical outcomes, potentially allowing for closer monitoring and more aggressive therapy.

Echocardiographic Detection of Increased Ventricular Diastolic Stiffness in Pediatric Heart Transplant Recipients: A Pilot Study.

BACKGROUND: Pediatric heart transplant recipients are at risk for increased left ventricular (LV) diastolic stiffness. However, the noninvasive evaluation of LV stiffness has remained elusive in this population. The objective of this study was to compare novel echocardiographic measures of LV diastolic stiffness versus gold-standard measures derived from pressure-volume loop (PVL) analysis in pediatric heart transplant recipients. METHODS: Patients undergoing left heart catheterization were prospectively enrolled. PVLs were obtained via conductance. The end-diastolic pressure-volume relationship was obtained via balloon occlusion. The stiffness constant, ß, was calculated. Echocardiographic measures of diastolic function were derived from spectral and tissue Doppler and two-dimensional speckle-tracking. Ventricular volumes were measured using three-dimensional echocardiography. The novel echocardiographic estimates of ventricular stiffness included E:e'/end-diastolic volume (EDV) and E:early diastolic strain rate/EDV. RESULTS: Of 24 children, 18 were heart transplant recipients. Six control patients had hemodynamically insignificant patent ductus arteriosus or coronary fistula. The mean age was 9.1 ± 5.6 years. Median end-diastolic pressure was 9 mm Hg (interquartile range, 8-13 mm Hg). Lateral E:e'/EDV (r = 0.59, P < .01), septal E:e'/EDV (r = 0.57, P < .01), and (E:circumferential early diastolic strain rate)/EDV (r = 0.54, P < .01) correlated with ß. Lateral E:e'/EDV displayed a C statistic of 0.93 in detecting patients with abnormal LV stiffness (ß > 0.015 mL-1). A lateral E:e'/EDV of >0.15 mL-1 had 89% sensitivity and 93% specificity in detecting an abnormal ß. CONCLUSIONS: Echocardiographic estimates of ventricular stiffness may be accurate compared with the gold standard in pediatric heart transplant recipients. The clinical usefulness of these noninvasive measures in assessing LV stiffness merits further study in children.

Longitudinal measures of deformation are associated with a composite measure of contractility derived from pressure-volume loop analysis in children.

Aims: The relationship between echocardiographic measures of left ventricular (LV) systolic function and reference-standard measures have not been assessed in children. The objective of this study was to assess the validity of echocardiographic indices of LV systolic function via direct comparison to a novel composite measure of contractility derived from pressure-volume loop (PVL) analysis. Methods and results: Children with normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. A composite invasive composite contractility index (ICCI) was developed using data reduction strategies to combine four measures of contractility derived from PVL analysis. Echocardiograms were performed immediately after PVL analysis under the same anesthetic conditions. Conventional and speckle-tracking echocardiographic measures of systolic function were measured. Of 24 patients, 18 patients were heart transplant recipients, 6 patients had a small patent ductus arteriosus or small coronary fistula. Mean age was 9.1 ± 5.6 years. Upon multivariable regression, longitudinal strain was associated with ICCI (ß = -0.54, P = 0.02) while controlling for indices of preload, afterload, heart rate, and LV mass under baseline conditions. Ejection fraction and shortening fraction were associated with LV mass and load indices, but not contractility. Conclusion: Speckle-tracking derived longitudinal strain is associated ICCI in children with normal loading conditions. Longitudinal measures of deformation appear to accurately assess LV contractility in children.

Coronary artery assessment using self-navigated free-breathing radial whole-heart magnetic resonance angiography in patients with congenital heart disease.

OBJECTIVES: To evaluate a self-navigated free-breathing three-dimensional (SNFB3D) radial whole-heart MRA technique for assessment of main coronary arteries (CAs) and side branches in patients with congenital heart disease (CHD). METHODS: SNFB3D-MRA datasets of 109 patients (20.1±11.8 years) were included. Three readers assessed the depiction of CA segments, diagnostic confidence in determining CA dominance, overall image quality and the ability to freeze cardiac and respiratory motion. Vessel sharpness was quantitatively measured. RESULTS: The percentages of cases with excellent CA depiction were as follows (mean score): left main, 92.6 % (1.92); left anterior descending (LAD), 88.3 % (1.88); right (RCA), 87.8 % (1.85); left circumflex, 82.8 % (1.82); posterior descending, 50.2 % (1.50) and first diagonal, 39.8 % (1.39). High diagnostic confidence for the assessment of CA dominance was achieved in 56.2 % of MRA examinations (mean score, 1.56). Cardiac motion freezing (mean score, 2.18; Pearson's r=0.73, P<0.029) affected image quality more than respiratory motion freezing (mean score, 2.20; r=0.58, P<0.029). Mean quantitative vessel sharpness of the internal thoracic artery, RCA and LAD were 53.1, 52.5 and 48.7 %, respectively. CONCLUSIONS: Most SNFB3D-MRA examinations allow for excellent depiction of the main CAs in young CHD patients; visualisation of side branches remains limited. KEY POINTS: • Self-navigated free-breathing three-dimensional magnetic resonance angiography (SNFB3D-MRA) sufficiently visualises coronary arteries (CAs). • Depiction of main CAs in patients with congenital heart disease is excellent. • Visualisation of CA side branches using SNFB3D-MRA is limited. • SNFB3D-MRA image quality is especially correlated to cardiac motion freezing ability.

Utility of Cardiac Magnetic Resonance Imaging in the Management of Adult Congenital Heart Disease.

The increasing number of patients with adult congenital heart disease (ACHD) calls for the development of noninvasive imaging techniques that allow a long-term evaluation of native and postsurgical anatomy and function. Echocardiography remains the imaging modality of choice for congenital heart disease, but it is affected by limited acoustic windows and poor tissue characterization. Cardiac computed tomography and cardiac catheter angiography are 2 valid alternatives for the anatomic and functional assessment of ACHD; however, both use ionizing radiation, and cardiac catheter angiography requires an invasive approach. Cardiac magnetic resonance (CMR), noninvasively and in the absence of ionizing radiation, has the ability to evaluate the biventricular function, quantify flows, characterize tissue, and provide information on cardiac anatomy. Despite the long acquisition time and lower spatial resolution compared with cardiac computed tomography, CMR represents the ideal technique for long-term follow-up of ACHD. CMR is now widely utilized and is well described in the literature with regard to diagnosis, identification of complications, timing of surgery, and postoperative prognosis in ACHD. CMR represents a fundamental technique for the evaluation of patients with ACHD.

Systolic-diastolic functional coupling in healthy children and in those with dilated cardiomyopathy.

Systolic and diastolic function affect dilated cardiomyopathy (DCM) outcomes. However, systolic-diastolic coupling, as a distinct characteristic, may itself affect function but is poorly characterized. We hypothesized that echocardiographic left ventricular (LV) longitudinal systolic tissue velocities (S') correlate with diastolic longitudinal velocities (E') and that their relationship is associated with ventricular function and that this relationship is impaired in pediatric DCM. We analyzed data from the Pediatric Heart Network Ventricular Volume Variability study, using linear regression and generalized additive modeling to assess relationships between S' and E' at the lateral and septal mitral annulus. We explored relationships between the systolic:diastolic (S:D) coupling ratio (S':E' relative to age) and ventricular function. Up to 4 echocardiograms from 130 DCM patients (mean age: 9.3 ± 6.1 yr) and 1 echocardiogram from each of 591 healthy controls were analyzed. S' and E' were linearly related in controls (r = 0.64, P < 0.001) and DCM (r = 0.83, P < 0.001). In DCM, the magnitude of association between S' and E' was reduced with progressive ventricular remodeling. The S:D ratio was more strongly associated with LV function in controls vs. DCM. The septal S:D ratio was higher (presumed worse) in DCM vs. controls (0.69 ± 0.13 vs. 0.62 ± 0.12, P = 0.001). A higher septal S:D ratio was associated with worse LV dimensions (parameter estimate: 0.0061, P = 0.004), mass (parameter estimate: 0.0074, P = 0.002), ejection fraction (parameter estimate: -0.0303, P = 0.024), and inflow propagation (parameter estimate: -0.3538, P < .001). S:D coupling becomes weaker in DCM with LV remodeling and dysfunction. The S:D coupling ratio may be useful to assess coupling, warranting study in relation to patient outcomes.

Safety of Magnetic Resonance Imaging After Implantation of Stainless Steel Embolization Coils.

Stainless steel embolization coils (SSEC) have been used for over four decades for vascular occlusion. Recently, the safety of these coils in a magnetic resonance environment has been called into question, with important ramifications for thousands of patients with existing coils in place. We performed a retrospective chart review at five tertiary care pediatric centers evaluating all children and young adults with implanted SSEC who underwent magnetic resonance imaging (MRI). Data reviewed included demographics, coil implantation, MRI studies, and follow-up evaluations. Complications such as heating, discomfort, or device migration were specifically sought. Two hundred and ninety-seven patients with implanted SSEC underwent 539 MRI examinations. The median age at SSEC implantation was 2.3 years (1 week-23.2 years). The MRI studies were performed a median of 7.4 years (4 days-23.1 years) after implantation. No patients experienced any reported complications associated with their MRI examinations during the study or at median follow-up post-MRI of 4.8 years (1 day-23 years). In this large, retrospective review of patients with implanted SSEC undergoing MRI, there were no reported adverse events. These findings support the recent change by Cook Medical Inc. of their standard embolization coils from a designation of magnetic resonance unsafe to conditional.

Impact of obesity on ventricular size and function in children, adolescents and adults with Tetralogy of Fallot after initial repair.

Obesity is epidemic in congenital heart disease, with reported rates of 16% to 26% in children and 54% in adults. The aim of this study was to evaluate the impact of obesity on ventricular function and size in patients after initial repair for tetralogy of Fallot (TOF). Cardiac magnetic resonance studies in normal-weight (body mass index percentile <85th) and obese (body mass index percentile ≥95th) children and adults with repaired tetralogy of Fallot were reviewed. The left ventricular ejection fraction, the right ventricular ejection fraction, left and right ventricular end-diastolic volumes indexed to actual body surface area, to height, and to body surface area using ideal body weight were evaluated in 36 obese patients and 72 age-matched normal-weight patients. Compared with normal-weight patients, obese patients had lower right ventricular ejection fractions (mean 46 ± 9% vs 51 ± 7%, p = 0.003) and left ventricular ejection fractions (mean 57 ± 9% vs 61 ± 6%, p = 0.017), higher right ventricular end-diastolic volumes indexed to height (mean 160 ± 59 vs 135 ± 41 ml/m, p = 0.015) and left ventricular end-diastolic volumes indexed to height (mean 86 ± 25 vs 70 ± 20 ml/m, p = 0.001), and higher right ventricular end-diastolic volumes indexed to ideal body weight (mean 166 ± 55 vs 144 ± 38 ml/m², p = 0.020) and left ventricular end-diastolic volumes indexed to ideal body weight (mean 90 ± 22 vs 75 ± 15 ml/m², p <0.001). In conclusion, obesity is a modifiable risk factor associated with worsened biventricular systolic function and biventricular dilation in patients with repaired tetralogy of Fallot. The standard method of indexing ventricular volumes using actual body surface area may underestimate volume load in obese patients.

Doppler flow patterns in the right ventricle-to-pulmonary artery shunt and neo-aorta in infants with single right ventricle anomalies: impact on outcome after initial staged palliations.

BACKGROUND: A Pediatric Heart Network trial compared outcomes in infants with single right ventricle anomalies undergoing Norwood procedures randomized to modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS). Doppler patterns in the neo-aorta and RVPAS may characterize physiologic changes after staged palliations that affect outcomes and right ventricular (RV) function. METHODS: Neo-aortic cardiac index (CI), retrograde fraction (RF) in the descending aorta and RVPAS conduit, RVPAS/neo-aortic systolic ejection time ratio, and systolic/diastolic (S/D) ratio were measured early after Norwood, before stage II palliation, and at 14 months. These parameters were compared with transplantation-free survival, length of hospital stay, and RV functional indices. RESULTS: In 529 subjects (mean follow-up period, 3.0 ± 2.1 years), neo-aortic CI and descending aortic RF were significantly higher in the MBTS cohort after Norwood. The RVPAS RF averaged <25% at both interstage intervals. Higher pre-stage II descending aortic RF was correlated with lower RV ejection fraction (R = -0.24; P = .032) at 14 months for the MBTS cohort. Higher post-Norwood CI (5.6 vs 4.4 L/min/m(2), P = .04) and lower S/D ratio (1.40 vs 1.68, P = .01) were correlated with better interstage transplantation-free survival for the RVPAS cohort. No other Doppler flow patterns were correlated with outcomes. CONCLUSIONS: After the Norwood procedure, infants tolerated significant descending aortic RF (MBTS) and conduit RF (RVPAS), with little correlation with clinical outcomes or RV function. Neo-aortic CI, ejection time, and S/D ratios also had limited correlations with outcomes or RV function, but higher post-Norwood neo-aortic CI and lower S/D ratio were correlated with better interstage survival in those with RVPAS.

Effect of branch pulmonary artery stenosis on right ventricular volume overload in patients with tetralogy of fallot after initial surgical repair.

Right ventricular (RV) volume overload secondary to pulmonary regurgitation is common in patients after initial repair of tetralogy of Fallot (TOF) and is associated with adverse long-term outcomes. The objective of the present study was to determine the effect of branch pulmonary artery stenosis on the RV volume in patients with repaired TOF. We reviewed 178 cardiac magnetic resonance imaging studies in patients with repaired TOF. We defined bilateral stenosis as a Nakata index of ≤200 mm(2)/m(2) and concordant branch pulmonary artery cross-sectional area, unilateral stenosis as 1 branch pulmonary artery cross-sectional area ≤100 mm(2)/m(2) and 1 branch pulmonary artery cross-sectional area >100 mm(2)/m(2), and restrictive physiology as prograde main pulmonary artery diastolic flow. Of the 178 patients, 20 (11%) had bilateral stenosis, 47 (26%) unilateral stenosis, and 111 (63%) had no stenosis. The RV end-diastolic volume was lower in patients with bilateral (125 ± 27 ml/m(2)) or unilateral (131 ± 43 ml/m(2)) stenosis than in those without stenosis (149 ± 35 ml/m(2), p = 0.021 and p = 0.019, respectively). The main pulmonary artery regurgitant fraction was greater in patients without stenosis (47%, range 2% to 69%) than in those with bilateral (33%, range 9% to 59%; p = 0.009) or unilateral stenosis (40%, range 0% to 71%; p = 0.033). Restrictive physiology was more common in patients with bilateral (13 of 15, 87%) or unilateral (21 of 38, 55%) stenosis than in those without stenosis (28 of 85, 33%; p <0.001 and p = 0.017, respectively). In conclusion, in patients with repaired TOF, bilateral and unilateral branch pulmonary artery stenosis was associated with a greater main pulmonary artery regurgitant fraction and smaller RV end-diastolic volume than those in patients without stenosis, likely owing to the development of restrictive physiology. Branch pulmonary artery stenosis might effectively delay the referral for pulmonary valve replacement.

ALCAPA: the role of myocardial viability studies in determining prognosis.

BACKGROUND: ALCAPA is optimally treated by coronary artery reimplantation early in neonatal life. Delayed diagnosis, however, is not infrequent, because symptoms often do not manifest until about 3 months of age, coinciding with the physiological nadir in pulmonary vascular resistance. With delayed diagnosis, there is potential for coronary steal and irreversible myocardial injury, which worsens outcome. OBJECTIVE: To assess the utility of MRI in determining prognosis in children with surgically corrected ALCAPA. MATERIALS AND METHODS: A retrospective chart review was performed in two children with ALCAPA who underwent coronary reimplantation and postoperative cardiac MRI. Both children subsequently underwent cardiac transplantation. The imaging findings and pathological findings at explant are presented. RESULTS: In both children, there was severe, globally depressed left ventricular systolic function and abnormal delayed enhancement in a predominantly subendocardial distribution. Pathological examination of the cardiac explants showed extensive fibrotic tissue, which correlated with areas of abnormal delayed enhancement on MRI. CONCLUSION: Severe reduction in systolic function and presence of delayed enhancement indicate extensive myocardial injury and pathologically correlate with irreversible fibrotic changes, which may help identify a subgroup of children who will not recover ventricular function and ultimately require heart transplantation.

Elucidating the relationship between cardiac preload and renal perfusion under pneumoperitoneum.

INTRODUCTION: Pneumoperitoneum is associated with a well-described decrease in renal blood flow, but it remains unclear whether a decrease in cardiac preload is responsible. Our aim was to characterize the relationship between cardiac preload and renal perfusion during pneumoperitoneum. METHODS: Eleven pigs were submitted to three 30 minute study periods: 1) Baseline (n=11): no interventions, 2) Pneumoperitoneum (n=11): 12 mmHg CO2 pneumoperitoneum, 3) Preload Reduction: pneumoperitoneum and nitroglycerin infusion (n=8); or pneumoperitoneum and hemorrhage to a mean arterial pressure (MAP) of 40 mmHg (n=3). Echocardiographic measurements of left ventricular end-diastolic diameter (LVEDD) were used as an index of preload. Renal cortical perfusion (RCP) was measured using laser doppler flowmetry. RESULTS: LVEDD decreased from 4.2 +/- 0.5 to 4.1 +/- 0.6 cm (p=0.02) with pneumoperitoneum and then to 4.0 +/- 0.5 cm (p=0.03) with the addition of nitroglycerin. There was no statistically significant change in RCP with pneumoperitoneum (33.5 +/- 8.4 to 28.5 +/- 8.4 ml/min/100g tissue, p=0.2), but it decreased to 18.5 +/- 11.3 ml/min/100g tissue (p=0.001) with the addition of nitroglycerin. The correlation between RCP and LVEDD was weak (0.35, p=0.003), whereas correlation between RCP and MAP was superior (R=0.59, p<0.0001). CONCLUSIONS: While decreasing preload under extreme lab conditions also decreases RCP, simply creating a pneumoperitoneum of 12 mmHg does not. The decrease in renal blood flow associated with pneumoperitoneum is likely not solely a function of preload.

Electrocardiogram interpretation and management in a pediatric emergency department.

OBJECTIVES: To determine the accuracy of electrocardiogram (ECG) interpretation by pediatric emergency physicians through comparison with a pediatric cardiologist and to determine the intrarater and interrater reliability for pediatric emergency physicians and cardiologists. METHODS: This was a prospective cohort study in which pediatric emergency physicians ordering an ECG completed a standardized questionnaire. The same emergency physician, a second emergency physician, and a pediatric cardiologist also completed the questionnaire for all ECGs at a later time. A randomly selected subset of ECGs was also interpreted by the same cardiologist and a second pediatric cardiologist. Major outcome variables were (1) whether the ECG was normal or abnormal, and if abnormal, (2) whether the abnormality represented a minor or major concern, and (3) whether the ECG warranted referral to a pediatric cardiologist. RESULTS: For pediatric emergency physicians, the intrarater and interrater kappa values were 0.56 and 0.24 for the presence of an abnormality, 0.49 and 0.36 for level of concern, and 0.63 and 0.25 for need of cardiology follow-up. For pediatric cardiologists, the intrarater and interrater kappa values were 0.82 and 0.92 for the presence of an abnormality, 0.71 and 1.00 for level of concern, and 0.82 and 0.91 for need of cardiology follow-up. A comparison of the initial emergency physician and cardiologist interpretations yielded kappa values of 0.42 for the presence of an abnormality, 0.16 for level of concern, and 0.31 for need of cardiology follow-up. CONCLUSIONS: When compared with interpretation by a pediatric cardiologist, ECG interpretation by pediatric emergency physicians was relatively inaccurate; intrarater and interrater agreement among emergency physicians was good and poor, respectively, and the intrarater and interrater agreement among pediatric cardiologists was excellent.

Kawasaki disease and scald injuries: a possible association.

The cause of Kawasaki disease (KD) is still unknown. Evidence is available to support both a conventional infection, as well as a superantigen-mediated illness. The cases of two children who were hospitalized with scald injuries are presented. Both patients became febrile without an infectious source and subsequently developed typical KD, that responded to treatment with intravenous immunoglobulin, without cardiac sequelae. The temporal association between the thermal injury and the onset of KD could be explained by the entry of an infectious agent(s), toxin or superantigen through the compromised skin barrier, leading to an inflammatory vasculitis and the clinical manifestations of KD.