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Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited, progressive form of cardiomyopathy, which is characterized by fibrofatty replacement of the myocardium. While the gold standard for diagnosis remains pathologic evaluation of biopsy, advances in noninvasive imaging, including cardiac magnetic resonance imaging (CMRI), have led to improved clinical diagnosis. Case report: We report three additional cases of pediatric patients that have pathologically confirmed ARVC/D with CMRI images, demonstrating extensive macroscopic fatty infiltration of the right and left ventricular myocardium. The identification using CMRI allowed timely transplantation and patient survival. Conclusion: Our study is designed to highlight how fibrofatty changes are minimal using CMRI in the pediatric population and how this can be a valuable tool to provide an additional method of diagnosis.

For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic. In this statement, we provide background on several NMDs in which there is cardiac involvement, highlighting unique features of NMD-associated myocardial disease that require clinicians to tailor their approach to prevention and treatment of heart failure. Undoubtedly, further investigations are required to best inform future guidelines on NMD-specific cardiovascular health risks, treatments, and outcomes., Competing Interests: The American Heart Association makes every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest., (© 2017 American Heart Association, Inc.)

Vascular ring in the form of a double aortic arch is a rare anomaly that can cause airway compression. It occasionally occurs with unusual head and neck vessel branching. A 5-year-old boy with chronic respiratory symptoms was referred because of a tracheal indentation on his chest x-ray. Magnetic resonance imaging showed a double aortic arch with arch origins of a common carotid, vertebral, and subclavian on the right and internal and external carotids, vertebral, and subclavian arteries on the left. Our case represents, to our knowledge, the first report of a double aortic arch with 7 separate vessels arising from the transverse arches., (Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Improved delineation of vascular structures is a common indication for cardiovascular magnetic resonance (CMR) in children and requires high spatial resolution. Currently, pre-contrast 3D, respiratory navigated, T2-prepared, fat saturated imaging with a bSSFP readout (3D bSSFP) is commonly used; however, these images can be limited by blood pool inhomogeneity and exaggeration of metal artifact. We compared image quality of pediatric vasculature obtained using standard 3D bSSFP to 3D, respiratory navigated, inversion recovery prepared imaging with a gradient echo readout (3D IR GRE) performed after administration of gadofosveset trisodium (GT), a blood pool contrast agent., Methods: For both sequences, VCG triggering was used with acquisition during a quiescent period of the cardiac cycle. 3D bSSFP imaging was performed pre-contrast, and 3D IR GRE imaging was performed 5 min after GT administration. We devised a vascular imaging quality score (VIQS) with subscores for coronary arteries, pulmonary arteries and veins, blood pool homogeneity, and metal artifact. Scoring was performed on axial reconstructions of isotropic datasets by two independent readers and differences were adjudicated. Signal- and contrast-to-noise (SNR and CNR) calculations were performed on each dataset., Results: Thirty-five patients had both 3D bSSFP and 3D IR GRE imaging performed. 3D IR GRE imaging showed improved overall vascular imaging compared to 3D bSSFP when comparing all-patient VIQS scores (n = 35, median 14 (IQR 11-15), vs 6 (4-10), p < 0.0001), and when analyzing the subset of patients with intrathoracic metal (n = 17, 16 (14-17) vs. 5 (2-9), p < 0.0001). 3D IR GRE showed significantly improved VIQS subscores for imaging the RCA, pulmonary arteries, pulmonary veins, and blood pool homogeneity. In addition, 3D IR GRE imaging showed reduced variability in both all-patient and metal VIQS scores compared to 3D bSSFP (p < 0.05). SNR and CNR were higher with 3D IR GRE in the left ventricle and left atrium, but not the pulmonary arteries., Conclusions: Respiratory navigated 3D IR GRE imaging after GT administration provides improved vascular CMR in pediatric patients compared to pre-contrast 3D bSSFP imaging, as well as improved imaging in patients with intrathoracic metal. It is an excellent alternative in this challenging patient population when high spatial resolution vascular imaging is needed.

Background: Using a bifurcated Y-graft as the Fontan baffle is hypothesized to streamline and improve flow dynamics through the total cavopulmonary connection (TCPC). This study conducted numerical simulations to evaluate this hypothesis using postoperative data from 5 patients., Methods: Patients were imaged with cardiac magnetic resonance or computed tomography after receiving a bifurcated aorto-iliac Y-graft as their Fontan conduit. Numerical simulations were performed using in vivo flow rates, as well as 2 levels of simulated exercise. Two TCPC models were virtually created for each patient to serve as the basis for hemodynamic comparison. Comparative metrics included connection flow resistance and inferior vena caval flow distribution., Results: Results demonstrate good hemodynamic outcomes for the Y-graft options. The consistency of inferior vena caval flow distribution was improved over TCPC controls, whereas the connection resistances were generally no different from the TCPC values, except for 1 case in which there was a marked improvement under both resting and exercise conditions. Examination of the connection hemodynamics as they relate to surgical Y-graft implementation identified critical strategies and modifications that are needed to potentially realize the theoretical efficiency of such bifurcated connection designs., Conclusions: Five consecutive patients received a Y-graft connection to complete their Fontan procedure with positive hemodynamic results. Refining the surgical technique for implementation should result in further energetic improvements that may help improve long-term outcomes., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Cardiac MRI has become widespread to characterize cardiac lesions in children. No study has examined the role of deep sedation performed by non-anesthesiologists for this investigation., Objective: We hypothesized that deep sedation provided by non-anesthesiologists can be provided with a similar safety and efficacy profile to general anesthesia provided by anesthesiologists., Materials and Methods: This is a retrospective chart review of children who underwent cardiac MRI over a 5-year period. The following data were collected from the medical records: demographic data, cardiac lesion, American Society of Anesthesiologists (ASA) physical status, sedation type, provider, medications, sedation duration and adverse events or interventions. Image and sedation adequacy were recorded., Results: Of 1,465 studies identified, 1,197 met inclusion criteria; 43 studies (3.6%) used general anesthesia, 506 (42.3%) had deep sedation and eight (0.7%) required anxiolysis only. The remaining 640 studies (53.5%) were performed without sedation. There were two complications in the general anesthesia group (4.7%) versus 17 in the deep sedation group (3.4%). Sedation was considered inadequate in 22 of the 506 deep sedation patients (4.3%). Adequate images were obtained in 95.3% of general anesthesia patients versus 86.6% of deep sedation patients., Conclusion: There was no difference in the incidence of adverse events or cardiac MRI image adequacy for children receiving general anesthesia by anesthesiologists versus deep sedation by non-anesthesiologists. In summary, this study demonstrates that an appropriately trained sedation provider can provide deep sedation for cardiac MRI without the need for general anesthesia in selected cases.

Objective: Optimizing flow and diminishing power loss in the Fontan circuit can improve hemodynamic efficiency, potentially improving the long-term outcomes. Computerized modeling has predicted improved energetics with a Y-graft Fontan., Methods: From August to December 2010, 6 consecutive children underwent completion Fontan (n=3) or Fontan revision (n=3) using a bifurcated polytetrafluoroethylene Y-graft (18×9×9 mm in 2, 20×10×10 mm in 4) connecting the inferior vena cava to the right and left pulmonary arteries with separate graft limbs. The patents underwent magnetic resonance imaging (n=5) or computed tomography (n=1). Computational fluid dynamics assessed Fontan hemodynamics, power loss, and inferior vena cava flow splits to the branch pulmonary arteries. The clinical parameters were compared with those from 12 patients immediately preceding the present series who had undergone a lateral Fontan procedure., Results: Despite longer crossclamp and bypass times (not statistically significant), the Y-graft Fontan patients had postoperative courses similar to those of the conventional Fontan patients. Other than 2 early readmissions for pleural effusions managed with diuretics, at 6 to 12 months of follow-up (mean, 8 months), all 6 patients had done well. Postoperative flow modeling demonstrated a balanced distribution of inferior vena cava flow to both pulmonary arteries with minimal flow disturbance. Improvements in hemodynamics and efficiency were noted when the Y-graft branches were anastomosed distally and aligned tangentially with the branch pulmonary arteries., Conclusions: The present preliminary surgical experience has demonstrated the clinical feasibility of the bifurcated Y-graft Fontan. Computational fluid dynamics showed acceptable hemodynamics with low calculated power losses and a balanced distribution of inferior vena cava flow to the pulmonary arteries as long as the branch grafts were anastomosed distally., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

This article introduces a novel method named "Parallel Imaging and Noquist in Tandem" (PINOT) for accelerated image acquisition of cine cardiac magnetic resonance imaging. This method combines two prior information formalisms, the SPACE-RIP implementation of parallel imaging and the Noquist method for reduced-data image reconstruction with prior knowledge of static and dynamic regions in the field of view. The general theory is presented, and supported by results from experiments using time-resolved two-dimensional simulation data and retrospectively sub-sampled magnetic resonance imaging data with acceleration factors around 4. A signal-to-noise ratio analysis and a comparison study with TSENSE and k-t SENSE show that PINOT performs favorably in preserving edge detail, at a cost in signal-to-noise ratio and computational complexity., (Copyright © 2010 Wiley-Liss, Inc.)

Background: Congenital bicuspid aortic valve (BAV) is a significant risk factor for serious complications including valve dysfunction, aortic dilatation, dissection, and sudden death. Clinical tools for identification and monitoring of BAV patients at high risk for development of aortic dilatation, an early complication, are not available., Methods: This paper reports an investigation in 18 pediatric BAV patients and 10 normal controls of links between abnormal blood flow patterns in the ascending aorta and aortic dilatation using velocity-encoded cardiovascular magnetic resonance. Blood flow patterns were quantitatively expressed in the angle between systolic left ventricular outflow and the aortic root channel axis, and also correlated with known biochemical markers of vessel wall disease., Results: The data confirm larger ascending aortas in BAV patients than in controls, and show more angled LV outflow in BAV (17.54 +/- 0.87 degrees) than controls (10.01 +/- 1.29) (p = 0.01). Significant correlation of systolic LV outflow jet angles with dilatation was found at different levels of the aorta in BAV patients STJ: r = 0.386 (N = 18, p = 0.048), AAO: r = 0.536 (N = 18, p = 0.022), and stronger correlation was found with patients and controls combined into one population: SOV: r = 0.405 (N = 28, p = 0.033), STJ: r = 0.562 (N = 28, p = 0.002), and AAO r = 0.645 (N = 28, p < 0.001). Dilatation and the flow jet angle were also found to correlate with plasma levels of matrix metallo-proteinase 2., Conclusions: The results of this study provide new insights into the pathophysiological processes underlying aortic dilatation in BAV patients. These results show a possible path towards the development of clinical risk stratification protocols in order to reduce morbidity and mortality for this common congenital heart defect.

Background: After tetralogy of Fallot (TOF) repair, severe pulmonary insufficiency is known to impair biventricular function. Pulmonary valve replacement (PVR) alleviates symptoms, normalizes right ventricular volumes, and improves ventricular function. Most studies addressing the role of PVR have examined older adolescents or adults. Less is known about the potential benefits of PVR in preadolescents with TOF and significant right ventricular dilatation., Methods: We reviewed the clinical data for all preadolescents (< or = 13 years) with TOF who underwent cardiac magnetic resonance imaging (cMRI) or PVR, or both. Serial cMRI data were analyzed to determine the change in indexed right ventricular end-diastolic volume (RVEDV) and biventricular ventricular ejection fractions. Available cMRI data after PVR were compared with data before PVR., Results: During the study period, 101 preadolescents with TOF had cMRI. The median age of complete repair was 6 months (range, 6 days to 3.4 years). The mean RVEDV at the first study was 135 +/- 39 mL/m(2). For 32 with serial cMRI studies, the RVEDV increased at a mean yearly rate of 9 mL/m(2). Ventricular systolic function was impaired in 46 (46%). Forty-two patients underwent PVR at a mean age of 8 +/- 3 years. No hospital deaths occurred, and no pulmonary valve reinterventions have been required., Conclusions: Significant right ventricular dilatation is common in preadolescents after transannular patch repair of TOF. Routine follow-up of this population should incorporate cMRI. Further studies will be needed to determine whether a strategy of early PVR might improve intermediate-term outcome., (2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Our goal was to determine flow distribution in the cavopulmonary connections of patients with and without bilateral superior venae cavae who had the Fontan procedure. No large series exists that establishes the flow distributions in Fontan patients, which would be an important resource for everyday clinical use and may affect future surgical reconstruction., Methods: We studied 105 Fontan patients (aged 2-24 years) with through-plane phase contrast velocity mapping to determine flow rates in the inferior and superior venae cavae and left and right pulmonary arteries. Superior caval anastomosis type included 40 bidirectional Glenn shunts (of which 15 were bilateral) and 53 hemi-Fontan anastomoses; Fontan type included 69 intra-atrial baffles, 28 extracardiac conduits, and 4 atriopulmonary connections., Results: Total caval flow was 2.9 +/- 1.0 L x min(-1) x m(-2), with an inferior vena cava contribution of 59% +/- 15%. Total pulmonary flow was 2.5 +/- 0.8 L x min(-1) x m(-2), statistically less than caval flow and not explained by fenestration presence. The right pulmonary artery contribution (55% +/- 13%) was statistically greater than the left. In patients with bilateral superior cavae, the right cava accounted for 52% +/- 14% of the flow, with no difference in pulmonary flow splits (50% +/- 16% to the right). Age and body surface area correlated with percent inferior caval contribution (r = 0.60 and 0.74, respectively). Superior vena cava anastomosis and Fontan type did not significantly affect pulmonary flow splits., Conclusions: Total Fontan cardiac index was 2.9 L x min(-1) x m(-2), with normal pulmonary flow splits (55% to the right lung). Inferior vena caval contribution to total flow increases with body surface area and age, consistent with data from healthy children.

Particle image velocimetry (PIV) and phase contrast magnetic resonance imaging (PC-MRI) have not been compared in complex biofluid environments. Such analysis is particularly useful to investigate flow structures in the correction of single ventricle congenital heart defects, where fluid dynamic efficiency is essential. A stereolithographic replica of an extracardiac total cavopulmonary connection (TCPC) is studied using PIV and PC-MRI in a steady flow loop. Volumetric two-component PIV is compared to volumetric three-component PC-MRI at various flow conditions. Similar flow structures are observed in both PIV and PC-MRI, where smooth flow dominates the extracardiac TCPC, and superior vena cava flow is preferential to the right pulmonary artery, while inferior vena cava flow is preferential to the left pulmonary artery. Where three-component velocity is available in PC-MRI studies, some helical flow in the extracardiac TCPC is observed. Vessel cross sections provide an effective means of validation for both experiments, and velocity magnitudes are of the same order. The results highlight similarities to validate flow in a complex patient-specific extracardiac TCPC. Additional information obtained by velocity in three components further describes the complexity of the flow in anatomic structures.

Background: Echocardiographic assessment of left ventricular (LV) contractility and dimensions is important in the management of patients with congenital heart disease. Conventional two-dimensional measures are limited because of volume or pressure-overloaded right ventricles that may distort the septal planes. Real-time three-dimensional echocardiography (RT3DE) has overcome these limitations; however, postprocessing image reconstruction and analysis are required. We compared LV indices calculated by new online RT3DE software with those obtained by magnetic resonance imaging (MRI) in patients with congenital heart disease., Methods: Twelve patients (ages 1-33 years, median age = 15.9 years) with congenital heart disease underwent RT3DE and cardiac MRI. End-diastolic and end-systolic LV volumes, stroke volume, ejection fraction, and mass were calculated online using biplane method-of-discs and semiautomated border detection echocardiographic techniques., Results: All RT3DE volumes correlated strongly with MRI (r = 0.93-0.99, P < .001). Ejection fraction had a lower correlation (r = 0.69, P = .013). There was no significant underestimation or overestimation of MRI values by RT3DE. Both biplane method-of-discs and semiautomated border detection echocardiographic techniques had excellent volume correlation (r = 0.94-0.99, P < .001). Interobserver variability was 7%., Conclusions: Combined RT3DE acquisition and analysis machines can accurately assess the LV in patients with congenital heart disease, thus impacting clinical management and perhaps obviating the need for MRI in some cases.

Background: Unfavorable cardiac mechanics in children with hypoplastic left heart syndrome (HLHS) when compared with other single-ventricle defects may affect long-term morbidity and outcome. Using noninvasive phase contrast magnetic resonance imaging (PC MRI), we examined cardiac mechanics in children with HLHS and compared the results to other single-ventricle defects., Methods: Eighteen children with HLHS and 18 children with other single-ventricle defects were studied after the Fontan operation. Phase contrast MRI scans were obtained perpendicular to the ascending aorta, and flow was quantified using an in-house segmentation and reconstruction scheme. The total power output was determined using the modified Bernoulli equation along with cardiac output and systemic vascular resistance index., Results: Compared with non-HLHS congenital heart defects, children with HLHS had significantly lower power output (1.40 +/- 0.39 versus 1.78 +/- 0.38 W/m2, p < 0.004) and cardiac index (3.15 +/- 0.97 versus 4.09 +/- 1.23 L x Min(-1) x m(-2), p < 0.009) with a concomitant higher systemic vascular resistance index (28.94 +/- 11.5 versus 22.7 +/- 8.53 WU, p < 0.03) despite generating similar systolic blood pressures (112.9 +/- 22.4 versus 115.2 +/- 23 mm Hg, p > 0.05)., Conclusions: Minimally invasive measurements with PC MRI in children with HLHS showed significantly lower power output and cardiac index when compared with other single-ventricle physiologies. Abnormal aortic flow patterns may contribute to power loss and may have long-term survival and morbidity implications associated with the Fontan procedure. Elevated systemic vascular resistance index despite similar blood pressure opens avenues for therapeutic intervention for afterload reduction.

In adults, pulmonary value replacement (PVR) shows improvement in right ventricular (RV) volume and function and reduces QRS duration. In addition, RV volume correlates with QRS duration and QRS change. This has not been shown in pediatric patients. The purpose of this study was to evaluate serial magnetic resonance imaging (MRI) and electrocardiogram measurements before and after early PVR in a pediatric population with repaired Tetralogy of Fallot and whether QRS duration and QRS change correlated with RV volume. A retrospective review of MRIs and electrocardiograms was conducted on 10 patients. Median age at repair was 2.1 +/- 0.7 years, and median age at PVR was 11.5 +/- 2.0 years. There were significant decreases in RV end diastolic volume (EDV)/body surface area (BSA) (p < 0.0004), end systolic volume (ESV)/BSA (p = 0.02), RVEDV/left ventricular (LV) EDV (p < 0.001), RV ejection fraction (p < 0.04), RV stroke volume (SV)/BSA (p < 0.0002), and (RVSV - LVSV)/BSA (p = 0.0007). No significant change in QRS duration occurred (p = 0.08). QRS duration (pre-r = 0.44, p = 0.20; post-r = 0.34, p = 0.33) and QRS change (r = -0.08, p = 0.83) did not correlate with RVEDV. We propose early consideration of PVR in pediatric patients. PVR improves RV volumes and function and may provide beneficial electromechanical effects by slowing the progression of QRS duration.

Background: During primary coarctation repair, collateral blood vessels contribute significantly to distal perfusion. We sought to determine if velocity-encoded cine magnetic resonance imaging (VENC-MRI) could provide insight into anatomy and hemodynamics of collateral flow in patients with unrepaired coarctation., Methods: Sixteen patients (median age, 6.2 years; range, 1 to 18) with discrete coarctation (65% severe, 29% mild-moderate) and 10 controls (median age, 12.0 years; range, 9 to 15) without left-sided heart lesions were referred for cardiac MRI. Flow volumes were calculated from VENC-MRI images at the coarctation (proximal), diaphragm (distal), and midway between the two points (midpoint). A means model, repeated-measure analysis, was performed for volumes., Results: In coarctation patients, flow volumes increased by 59% (p = 0.0002) from coarctation to diaphragm, primarily between the proximal and midpoint sites (by 77%, p < 0.0001). In controls, flow volumes decreased by 11% along the entire aortic study length. Coarctation volumes were lower than controls by 54% (p = 0.0003) at the proximal site but showed no statistical difference at the midpoint or diaphragm., Conclusions: Coarctation flow volumes maximally increase in the upper thoracic aorta, but approach normal flow volumes in the lower thoracic region. Arteries arising from mid and lower thoracic level, such as those supplying the anterior spinal cord, may have nearly normal flow if collaterals are present. Velocity-encoded MRI can evaluate flow in patients who have poor collateral circulation to improve surgical planning and decrease neurologic complications of coarctation repair.

In pediatric ventricular assist device (VAD) design, the process of matching device characteristics and dimensions to the relevant disease conditions poses a formidable challenge because the disease spectrum is more highly varied than for adult applications. One example arises with single-ventricle congenital defects, which demand palliative surgeries that create elevated systemic venous pressure and altered pulmonary hemodynamics. Substituting a mechanical pump as a right ventricle has long been proposed to eliminate the associated early and postoperative anomalies. A pulsatile lumped-parameter model of the single-ventricle circulation was developed to guide the preliminary design studies. Two special modules, the pump characteristics and the total cavopulmonary connection (TCPC) module, are introduced. The TCPC module incorporates the results of three-dimensional patient-specific computational fluid dynamics calculations, where the pressure drop in the TCPC anastomosis is calculated at the equal vascular lung resistance operating point for different cardiac outputs at a steady 60/40 inferior vena cava/superior vena cava flow split. Preliminary results obtained with the adult parameters are presented with no ventricle remodeling or combined larger-size single ventricle. A detailed literature review of single-ventricle function is provided. Coupling a continuous pump to the single-ventricle circulation brought both the pulmonary and systemic venous pressures back to manageable levels. Selected VADs provided an acceptable cardiac output trace of the single left ventricle, after initial transients. Remodeling of the systemic venous compliance plays a critical role in performance and is included in this study. Pulsatile operation mode with rotational speed regulation highlighted the importance of TCPC and pulmonary artery compliances. Four different pumps and three patient-specific anatomical TCPC pathologies were studied. Magnitudes of the equivalent TCPC resistances were found to be comparable to the vascular resistances of the normal baseline circulation, significantly affecting both the VAD design and hemodynamics.

In a cohort of 56 school-aged children with repaired tetralogy of Fallot, significant (moderate to severe) tricuspid regurgitation was common (32% of patients) and was related to both tricuspid annulus dilatation and structural valve abnormalities that were potentially related to previous surgery. Even after adjusting for pulmonary regurgitation, tricuspid regurgitation was significantly correlated with right ventricular volume (r= 0.39, p = 0.009), suggesting that tricuspid regurgitation as well as pulmonary regurgitation may contribute significantly to progressive right ventricular dilatation in this population.

The ability to noninvasively diagnose even the most complex congenital heart disease is one of the greatest advances in the care of children with cardiac defects. Initially, two-dimensional echocardiography displayed anatomy; later, Doppler allowed the quantitation of pressure and flow. Using these modalities, cardiac catheterization is unnecessary for most children with cardiac defects. Echocardiography also is routinely used for intraoperative and fetal cardiac imaging. Three-dimensional magnetic resonance imaging can now quantitate volumes of irregular shaped heart chambers (eg, the right ventricle) and blood flow (eg, as from a leaking valve). As technology moves further into the digital age, developments will continue to facilitate noninvasive diagnosis and treatment of children with congenital heart disease.

Background: Surgical repair of obstructive lesions of the right ventricular outflow tract (RVOT) in children commonly creates pulmonary valve incompetence that may eventually require pulmonary valve replacement (PVR). We reviewed our experience with PVR late after RVOT reconstruction., Methods: We performed 100 PVRs in 93 children 1.1 months to 22.4 years (median 8) after RVOT reconstruction. Children with right ventricular to pulmonary artery conduits and primary PVRs were excluded. Age at PVR was 4.5 months to 27.9 years (median 9.5 years). Initial diagnosis was tetralogy of Fallot and variants, 62; critical pulmonary stenosis, 15; pulmonary atresia with intact ventricular septum, 7; and others, 9. Eleven patients had a redo PVR. A total of 62 PVRs were homografts; 38 were porcine valves., Results: There was one early death. On follow-up of 5 months to 12.4 years (mean 4.9 years) there were no late deaths although 1 child underwent cardiac transplantation. Actuarial freedom from redo PVR at 8 years was 100% for porcine valves but 70% for homograft valves (p = 0.17). For children younger than 3 years at PVR, freedom from reoperation was 76% at 1 year and 39% at 8 years compared with freedom from redo PVR at 8 years of 100% for children older than 3 years. On latest echocardiogram 97% of porcine valves had mild or no pulmonary regurgitation compared with 72% of homograft valves., Conclusions: PVR after RVOT reconstruction can be performed with low risk. Porcine valves may be superior to homograft valves although this advantage may be due to older age at time of PVR.

Objectives: This study evaluated the accuracy, advantages and clinical efficacy of magnetic resonance (MR) phase-shift velocity mapping, in delineating the site and the hemodynamic severity of pulmonary venous (PV) obstruction in patients with congenital heart disease (CHD)., Background: Magnetic resonance phase-shift velocity mapping of normal pulmonary veins and of obstructed PV pathways have been previously reported in a mainly adult population., Methods: The study population (33 pts) underwent MR phase-shift velocity mapping of their PV pathways. These results were compared with cardiac catheterization and Doppler echocardiography data., Results: The study population (0.4 to 19.5 years) consisted of a study group (PV pathway obstruction, n = 7) and a control group (no PV obstruction, n = 26). No patients had any left-to-right shunt lesions. The MR imaging displayed precise anatomical detail of the pulmonary veins. Phase velocities in the control group ranged from 20 to 71 cm/s, whereas velocities in the study group ranged from 100 to 250 cm/s (p = 0.002). The MR phase velocities (154 +/- 0.53 cm/s) compared favorably with Doppler echocardiography (147 +/- 0.54 cm/s), (r = 0.76; p = 0.05). The MR velocity mapping was 100% specific and 100% sensitive in detecting PV obstruction, although the absolute gradient measurements among MR phase mapping, echocardiographic Doppler and catheterization did not show statistically significant correlation., Conclusions: In the absence of any associated left-to-right shunt lesions, PV velocities of 100 cm/s and greater indicated significant obstruction. The MR phase-shift velocity mapping, together with MR spin echocardiography and MR angiography, provides comprehensive anatomic and physiologic data that may obviate the need for further invasive studies.

Background: Excessive pulmonary blood flow increases ventricular volume work in the face of inadequate systemic cardiac output, low diastolic blood pressure, and inadequate coronary perfusion. Using the smallest available 3-mm polytetrafluoroethylene shunts have been successful, although catastrophic shunt thrombosis has occasionally been observed. To avoid thrombosis with a smaller conduit, saphenous vein homografts (SVG) were used to construct the modified Blalock-Taussig (BT) shunts., Methods: From January 1998 to April 1999, 25 patients weighing 3.1 kg (3.0 kg or less, n = 9), at a mean age of 8.9 days, underwent stage I Norwood using an SVG BT shunt. Common heart defects were aortic atresia (n = 8), mitral atresia and double-outlet right ventricle (n = 5), and unbalanced AVC (n = 5). Mean BT shunt size was 3.2 mm, with 12 patients having shunts that were 3 mm or smaller., Results: Thirty-day hospital mortality was 8% (2 of 25). No shunt thrombosis was seen, despite banding the BT shunt in 3 patients. One patient had BT revision because of an anatomic issue not directly related to the shunt material., Conclusions: Excellent results may be achieved using SVG BT shunts in the Norwood operation. This conduit seems less likely to thrombose, both acutely and chronically, allowing the use of appropriately smaller-sized shunts in small neonates.

Government health policy for malaria control in Solomon Islands has three main objectives: (1) early diagnosis and treatment of malaria at a health service; (2) reduction of human-vector contact through widespread use of insecticide-impregnated bed nets; and (3) provision of malaria chemoprophylaxis for pregnant women. Social research was carried out in thirteen villages in central Malaita to determine local attitudes toward malaria and to estimate the level of participation in malaria control activities. Interviews with 124 care-givers who had children 0-10 years of age, 20 focus group discussions and four evening structured observations were research methods used. Antimalarial drugs were the most favoured treatment, and use of traditional medicines and healers were reportedly minimal. Twenty-five percent of respondents reported keeping chloroquine at home and 42% said they would use chloroquine before seeking diagnosis and treatment from a health service. Structured observations suggest that protection against mosquitoes is poor during the evening. Fifty-two percent of respondents reported using fire and 32% said they used bed nets to protect themselves from mosquitoes. Participants had contradictory beliefs on the threat of malaria during pregnancy and the safety of taking chloroquine prophylaxis. Implications of malaria treatment and prevention practices are discussed, and recommendations for improving malaria control are presented.

The present study investigated the use of insecticide-impregnated bed nets by communities in central Malaita, Solomon Islands. Qualitative and quantitative data were collected by: (1) questionnaire administration to 124 care-givers of children aged 0-10 years of age; (2) 20 focus group discussions; (3) two structured observations of bed net re-impregnation, and (4) interviews with key informants. Ninety-four percent of all care-givers had bed nets, but only 62% had sufficient bed nets for all household members. Fifty-two percent used bed nets throughout the year and 70% of care-givers reported that all their children slept under bed nets. Although coastal householders considered malaria and mosquitoes more of a problem than inland householders, overall bed net compliance did not differ. Factors affecting bed net ownership were cost and community expectation of free bed nets. Bed net use was affected by four factors: (1) seasonality (99% used bed nets during the rainy season, 52% used them all year); (2) mosquito nuisance (59% of respondents reported that protection against mosquitoes was the main reason for using a bed net); (3) weather (68% of care-givers would not use a bed net if the weather was hot), and (4) low density of mosquitoes (respondents who used bed nets as protection against mosquito nuisance were more likely not to use bed nets when mosquitoes were few than those who used bed nets for malaria protection (odds ratio (OR), 3.9; 95% confidence interval (CI), 1.4-12.0). Protection against malaria was the main reason children slept under bed nets. Children from households where bed nets were used for malaria protection were more likely to sleep under bed nets than children from households where nets were used as protection from mosquitoes only (OR, 2.7; 95% CI, 1.3-5.9). Other factors that affected children's bed net use were, age (users were significantly younger than non-users; chi(2)=7.9, degrees of freedom=1, P=0.005) and sufficiency of bed nets (OR, 2.0; 95% CI, 1. 3-7.0).

Background: Right ventricular dysfunction occurs in many patients with significant pulmonary valve regurgitation late after initial total repair of tetralogy of Fallot. Methods to predict which of these patients are at increased risk of late morbidity and mortality are not yet known., Hypothesis: This study evaluated electrocardiographic (ECG) predictors of severe right ventricular dilatation determined by magnetic resonance imaging (MRI) volumes in patients with tetralogy of Fallot late after initial corrective repair., Methods: We retrospectively reviewed the ECGs and MRI right ventricular volume measurements of 20 patients (age 4.4 to 19.3 years, mean 10.0 years) with significant pulmonary valve regurgitation late after repair of tetralogy of Fallot. All patients had enlarged, hypokinetic right ventricles by echocardiography. The patients were grouped based on an indexed right ventricular end-diastolic volume (RVEDV/BSA) of < 102 ml/m2 (Group 1) or > or = 102 ml/m2 (Group 2). We determined the sensitivity, specificity, positive and negative predictive values of QRS duration, and mean frontal plane QRS axis for predicting right ventricular volumes., Results: A maximal QRS duration of > or = 150 ms or a northwest quadrant frontal plane QRS axis had 85% sensitivity, 86% specificity, 92% positive predictive value, and 75% negative predictive value for predicting an RVEDV/BSA of > or = 102 ml/m2. The mean QRS duration was significantly longer in Group 2 than in Group 1 patients (156 ms vs. 125 ms, p = 0.005)., Conclusions: In patients late after repair of tetralogy of Fallot with significant pulmonary valve regurgitation a maximal manually measured QRS duration of > or = 150 ms and/or a frontal plane QRS northwest quadrant axis can predict patients with marked right ventricular enlargement. The presence of either of these findings on the ECG signifies patients who require further evaluation and consideration for pulmonary valve replacement.

Measurement of right ventricular volume and function by two-dimensional echocardiography is unreliable because of the asymmetric shape of the right ventricle. The purpose of this study was to validate the accuracy of transthoracic three-dimensional echocardiography in assessing right ventricular volumes in children with congenital heart disease after surgical repair of the defects, by comparison with those measured by magnetic resonance imaging. We examined 13 children after repair of tetralogy of Fallot (10), hypoplastic left heart syndrome (2), or atrial septal defect (1). Each underwent magnetic resonance imaging followed by three-dimensional echocardiography done with a transthoracic 5 MHz, prototype internally rotating omniplane transducer. In both methods, endocardial borders were manually traced and volumetric slices were summated. Close correlation was observed between the two methods (R2 0.91 for end-systolic volumes, 0.90 for end-diastolic volumes, 0.64 for ejection fraction, and 0.92 for interobserver variability). A limits-of-agreement analysis showed no adverse trend between the two methods under values of 100 ml and low variation around the mean values. We conclude that three-dimensional echocardiography measurement of right ventricular volumes correlates closely with magnetic resonance imaging in children with operated congenital heart disease and may allow accurate serial evaluation in these patients.

Purpose: To determine whether breath holding can be eliminated in two-dimensional magnetic resonance (MR) imaging of the coronary arteries by using real-time respiratory gating., Materials and Methods: Thirty-one subjects (20 healthy volunteers, 11 patients) underwent MR imaging. In 13 subjects, a respiratory monitoring belt was used, and in 18 subjects, a navigator echo was used. MR imaging was performed with breath holding, respiratory gating, and respiratory gating with two signals acquired. Three reviewers conducted a blinded review of the images, and overall image quality was rated on a scale from 1 (poor) to 5 (excellent)., Results: Respiratory gating with two signals acquired provided image quality superior to that with breath-hold imaging (3.7 vs 3.0, respectively; P < .05). Measurements of signal-to-noise ratio (14.5 for respiratory gating with two signals acquired and 11.9 for breath holding) supported the results of the image review. Navigator-echo gating provided better image quality than the monitoring belt (3.7 vs 3.1, respectively; P < .05)., Conclusion: Breath holding may be eliminated by gating image acquisition to a real-time monitor of respiratory position. Respiratory gating enables improved resolution by means of acquisition of multiple signals, provides aligned sections of coronary arteries, and improves patient tolerance.

Objectives: This study evaluated whether magnetic resonance imaging (MRI) and magnetic resonance (MR) phase velocity mapping could provide accurate estimates of stenosis severity and pressure gradients in aortic coarctation., Background: Clinical management of aortic coarctation requires determination of lesion location and severity and quantification of the pressure gradient across the constricted area., Methods: Using a series of anatomically accurate models of aortic coarctation, the laboratory portion of this study found that the loss coefficient (K), commonly taken to be 4.0 in the simplified Bernoulli equation delta P = KV2, was a function of stenosis severity. The values of the loss coefficient ranged from 2.8 for a 50% stenosis to 4.9 for a 90% stenosis. Magnetic resonance imaging and MR phase velocity mapping were then used to determine coarctation severity and pressure gradient in 32 patients., Results: Application of the new severity-dependent loss coefficients found that pressure gradients deviated from 1 to 17 mm Hg compared with calculations made with the commonly used value of 4.0. Comparison of MR estimates of pressure gradient with Doppler ultrasound estimates (in 22 of 32 patients) and with catheter pressure measurements (in 6 of 32 patients) supports the conclusion that the severity-based loss coefficient provides improved estimates of pressure gradients., Conclusions: This study suggests that MRI could be used as a complete diagnostic tool for accurate evaluation of aortic coarctation, by determining stenosis location and severity and by accurately estimating pressure gradients.

Purpose: To evaluate the use of spiral computed tomographic (CT) angiography for the diagnosis of pediatric great vessel anomalies., Materials and Methods: Techniques used in adult CT angiography were modified to allow imaging of 15 children aged 1 month to 12 years. Two-dimensional axial images and three-dimensional shaded surface display images of the airway and great vessels were generated from volumetric CT data and evaluated prospectively. Findings were correlated with results obtained at surgery (five patients), autopsy (one patient), bronchoscopy (one patient), or other imaging studies (eight patients)., Results: In 14 patients, CT angiography accurately demonstrated vessel anomalies, including double or right aortic arch (seven cases), aberrant subclavian artery (six cases), innominate artery compression syndrome (one case), unilateral pulmonary artery agenesis (one case), and pulmonary artery enlargement (three cases). One patient had normal vasculature., Conclusion: CT angiography is feasible in children and can be used to delineate abnormal great vessel anatomy.

Objectives: Magnetic resonance angiography with three-dimensional surface rendering was performed to determine its value in assessing anatomic detail in patients with suspected aortic aneurysms., Background: Dacron patch aortoplasty repair of coarctation of the aorta carries an inherent risk of aneurysm development. Sudden death from aortic rupture prompted discontinuing this operation and evaluating 39 patients (16 girls; mean age 6.3 years, range 10 days to 14.5 years) undergoing repair between January 1976 and October 1987. The aorta ruptured in 10 patients; 6 died at a mean interval of 8.1 years (range 0.75 to 12.4) after repair. All 33 survivors were interviewed and examined., Methods: Conventional magnetic resonance imaging was performed in 26 patients, magnetic resonance angiography in 18. Angiographic slices were used to reconstruct three-dimensional images. No catheterization or contrast angiography was performed. Surgical intervention was based on clinical findings and magnetic resonance images., Results: Twenty patients (11 girls) developed aneurysms, of which nine were detected in patients studied by magnetic resonance. Ruptures occurred in eight female patients, three of whom were pregnant. Surface renderings accurately defined aortic anatomy or aneurysms in all patients. On follow-up, no aneurysms have been detected in patients with negative magnetic resonance study results. Precise anatomic correlation with operative findings was reported., Conclusions: Magnetic resonance angiography with three-dimensional surface rendering provides noninvasive, radiation-free and contrast agent-free high resolution images of the thoracic aorta. These images can be reviewed and have three-dimensional form and perspective. These techniques were preferred over invasive angiography by surgeons and clinicians as definitive, risk-free procedures before surgical intervention.

The mainstays of initial evaluation of the airway in infants and children are chest radiography and esophagography. Magnetic resonance (MR) imaging is frequently used next to diagnose specific abnormalities and obviates angiocardiography. MR imaging usually allows distinction between a double aortic arch and a right aortic arch with an aberrant left subclavian artery. In cases of pulmonary artery sling, MR imaging enables full evaluation of the vascular anatomy and may also demonstrate the tracheobronchial anomalies. MR imaging may aid in diagnosis of innominate artery compression syndrome by demonstrating the extent of the tracheal luminal narrowing, the tracheal configuration, the structure causing the compression, and the size of the thymus. Finally, MR imaging usually allows distinction of long-segment tracheal stenosis from tracheomalacia and is especially helpful in cases of isolated stenosis. Three-dimensional reconstructions are also useful in assessing relationships between vascular structures and the adjacent trachea.

Magnetic resonance imaging (MRI) of the airway has recently emerged as a diagnostic tool for evaluating tracheobronchial obstruction in pediatric patients. Computer-assisted reconstructions of transaxial images have enabled three-dimensional reconstructions of the airway to be manipulated in any plane and visualized in relation to adjacent anatomical structures. Eight patients in whom magnetic resonance imaging was used to diagnose a variety of airway obstructive lesions are presented. A protocol for the evaluation of pediatric tracheobronchial obstruction is presented, with the role of three dimensionally reconstructed MR imaging emphasized.

A 7-year-old male presenting with Wolff-Parkinson-White syndrome and tachycardia was suspected by echocardiographic and magnetic resonance imaging evaluation to have an associated pericardial cyst anterior to the right atrium and ventricle. Electrophysiological evaluation demonstrated short antegrade and retrograde accessory connection refractory periods, with inducible orthodromic atrioventricular reentrant tachycardia. Surgical observation revealed a rare congenital right atrial diverticulum bridging the anterior right atrioventricular groove, with the functional accessory connection lateralized to the medial aspect of this structure. Endocardial and epicardial incisions and cryolesions placed along the anterior right atrioventricular groove initially appeared successful, but preexcitation recurred within 4 weeks postoperatively.

The use of prostaglandin-E1 (PGE1) to maintain patency of the ductus arteriosus in infants with ductal-dependent congenital heart disease is now well established. A 2.5-month-old child with cyanotic heart disease who required long-term PGE1 infusions; developed widespread periosteal reactions during the course of therapy. Prostaglandin-induced subperiosteal hyperostosis should now be considered in the differential diagnosis of neonatal cortical proliferation.