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Neoaortic root dilation is a common phenomenon after the Norwood procedure, but the real incidence and its natural history are unclear. Regular surveillance in these patients after the operation is necessary. Herein, we present an 11-year-old boy born with tricuspid atresia, a discordant ventriculo-arterial connection and a hypoplastic aortic arch, who was palliated initially with a hybrid stage I procedure involving a reversed Blalock-Taussig shunt, followed by comprehensive stage II and then, an extra-cardiac fenestrated Fontan operation. The patient developed an aortic root aneurysm and severe aortic regurgitation. He was electively taken into the operating room, where cardiopulmonary bypass was established through a peripheral cannulation of the femoral vessels due to the high risk nature of the reoperation. A mechanical Bentall procedure was performed without residual lesions and the native ascending aorta was anastomosed as a single coronary button to the anterior wall of the graft., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Mortality associated with the modified Blalock-Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity., Design: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression., Results: Median age was 8 (0-126) days, weight 3.1(1.7-5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent had a sternotomy approach for shunt placement with 70% receiving a 3.5 mm graft. Mean graft index (shunt cross sectional area [mm 2 ]/BSA [m 2 ]) was 44.39 ± 8.04 and shunt size (mm) to body weight (kg) ratio 1.1 ± 0.2. Hospital mortality was 12%, with an interval mortality of 6%. Shunt thrombosis/stenosis occurred in 23% and pulmonary over circulation in 30%, while shunt reoperation was required in 12% and catheter intervention in 8% of the cohort. At 1-year, survival was 82.0% (95% CI [72.7%, 88.4%]), and survival free of major morbidity 61.4% (95% CI [50.7%, 70.5%]). Duct dependency predisposed to mortality (P = .01, HR 6.74 [1.54, 29.53]) and composite outcome (mortality and major morbidity) (P = .04, HR 2.15, CI [1.036, 4.466]) and higher graft index to mortality (P = .005, HR 1.07 [1.02, 1.12])., Conclusions: The commonest indication for a MBTS in the current era was single ventricle palliation. Morbidity and mortality was considerable, partly explained by the higher at risk population. Alternative methods to maintain pulmonary blood flow in place of a MBTS requires further investigation., (© 2017 Wiley Periodicals, Inc.)

Percutaneous, ultrasound-guided fetal cardiac intervention (FCI) is increasingly used to alter the prognosis of specific forms of congenital heart disease. Careful patient selection and postnatal management strategy are essential for optimal outcomes. This article discusses the rationale, patient selection criteria, procedural techniques, and contemporary results of FCI. Sources of information included published patient series, the International Fetal Cardiac Intervention Registry, and the Toronto experience as the Canadian referral center., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives: To assess the effectiveness of radiation-reduction measures implemented during pediatric catheterization, and provide data on the radiation doses for common interventional and diagnostic procedures, indexed to body weight., Background: Ionizing radiation exposure must be minimized to "as low as reasonably achievable," by instituting radiation-limiting techniques and knowledge of expected radiation exposure., Methods: Radiation-reduction measures included pulsed-fluoroscopy at 7.5 pulses/second (0.032-0.045 µGy/pulse), an air-gap magnification technique for children<20 kg, operator awareness, and additional exposure reduction techniques through projection optimization. Radiation doses for procedures performed between 2007 through 2014 were retrospectively reviewed, including dose area product (DAP) and DAP/kg of body weight for 25 procedural types. Median doses were compared with those previously published from other large centers and multi-institutional databases and assessed for changes over time., Results: Reviewed were 5,196 cases, which included 2,819 interventional, 710 endomyocardial biopsies and 1,667 diagnostic studies, documenting a significant difference in exposure between various procedures and body weights. The absolute exposure was significantly greater in larger children (e.g., for ductal closure median DAP/kg: 17 µGy*m2/kg 10-20 kg children vs. 37 µGy*m2 /kg for those>30 kg, P<0.001). Dose exposure using radiation-reduction techniques were the lowest reported in the literature for all procedure types compared (e.g. median DAP for pulmonary valvuloplasty 163 µGy*m2 vs. 405 to 1,230 µGy*m2 reported by 3 large centers). Reduction of fluoroscopy acquisition to 7.5 pulses/second nearly halved radiation exposure (P<0.001)., Conclusions: Implementing a radiation dose reduction and awareness program can lead to documented reduction in exposure, across a variety of procedures performed by multiple operators., (© 2015 Wiley Periodicals, Inc.)

Background: Percutaneous pulmonary valve implantation (PPVI) is a safe, less invasive alternative to surgical valve replacement for the congenital heart disease patient with right ventricular (RV) outflow tract dysfunction. The aim of this study was to determine whether reverse RV remodeling after PPVI was persistent in the longer term and whether timing of PPVI influenced outcomes., Methods and Results: Consecutive patients from the pediatric and adult congenital heart disease programs were enrolled. Cardiac MRI, echocardiography, metabolic exercise testing, chest radiography, and hemodynamics before intervention were compared with repeated follow-up measurements to assess changes over time. Fifty-one patients (including 23 patients <16 years old) were followed for a mean 4.5±1.9 (0.9-6.9) years after implantation, 59% of patients having available comparative cardiac MRI data. Freedom from any reintervention was 87% and 68% at 3 and 5 years, and freedom from surgery was 90% at 5 years. For every decade younger at implantation, there was an increase of 3.9%±1.0% in cardiac MRI left ventricular ejection fraction (P<0.001) and 2.4±0.9 mL/kg/min in maxVO2 (P=0.005) and a decrease of 0.7±0.2 cm in RV end-diastolic dimension (P<0.001) after intervention. Younger patients displayed an additional decline in the RV/left ventricular end-diastolic volume ratio (P=0.05) and trended toward improved RV ejection fraction in late follow-up (50%±7% versus 41%±12%, P=0.07)., Conclusions: This is the largest series to show that PPVI at a younger age yields incremental improvements in RV size and maximum oxygen consumption. Early valve implantation is associated with better RV function and should be considered in management planning for this population., (© 2015 American Heart Association, Inc.)

The Fontan operation allows for longer survival of those born with functionally single ventricle physiology. Although it effectively eliminates cyanosis, increased systemic venous pressure is an unavoidable consequence and low cardiac output is frequent. The abdomen is particularly vulnerable to these alterations in hemodynamics because the hepatic blood flow consists predominantly of portal venous flow, which relies on a low pressure gradient between the portal and hepatic veins. Therefore, any subtle increase in systemic venous pressure will adversely affect the hemodynamic balance of the liver as well as the intestine. As the clinical manifestations and routine laboratory findings of abdominal complications can lag behind the hemodynamic and pathologic changes in the abdominal organs, regular imaging surveillance is critical. Magnetic resonance (MR) provides excellent visualization of both cardiovascular and abdominal systems. It provides robust anatomic and hemodynamic data which can be used for timely implementation of treatment options. In this review paper, we discuss the pathogenesis and MR findings of abdominal complications following the Fontan operation. Specifically we explore the utility of phase-contrast MR for assessment of the abdominal circulation in children following the Fontan palliation.

Background: The use of a fenestration in the Fontan pathway remains controversial, partly because its hemodynamic effects and clinical consequences are insufficiently understood. The objective of this study was to quantify the magnitude of fenestration flow and to characterize its hemodynamic consequences after an intermediate interval after surgery., Methods: Twenty three patients with a fenestrated extracardiac conduit prospectively underwent investigation by cardiac magnetic resonance (CMR), echocardiography, and invasive manometry under the same general anesthetic 12 ± 4 months after Fontan surgery. Fenestration flow was determined using phase contrast CMR by subtracting flow in the Fontan pathway above the fenestration from Fontan flow below the fenestration., Results: Fenestration flow constituted a mean of 31 ± 12% (range 8-50%) of ventricular preload. It was associated with a lower Qp/Qs (r = -0.64, p=0.001) and oxygen saturation (r = -0.74, p<0.0001). Fenestration flow volume was correlated with pulmonary vascular resistance (r = 0.45, p = 0.04) and markers of ventricular diastolic function (early diastolic strain rate r = 0.57, p = 0.008 and ventricular untwist rate r = 0.54, p = 0.02). In 14 patients (61%) all of the net inferior vena cava flow and part of the superior vena cava flow were diverted into the systemic atrium and did not reach the lungs., Conclusions: Fenestration flow can be measured accurately with CMR. In two-thirds of the patients not only all of the inferior vena cava flow, but also some of the superior vena cava flow is diverted through the fenestration. Fenestration flow is driven by a balance between pulmonary vascular resistance and early diastolic ventricular function., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Objective: Retrograde aortic arch malperfusion after ductal stenting can be life-threatening after univentricular hybrid palliation. Arch perfusion can be maintained with a main pulmonary artery to innominate artery shunt placed during the stage I procedure: a "reverse Blalock-Taussig shunt.", Methods: A retrospective review of 37 infants who underwent hybrid palliation from January 2004 to March 2010 was performed. The infants were divided into 2 groups, those with (group I, n = 16) and those without (group II, n = 21) a reverse Blalock-Taussig shunt., Results: At the initial palliation, no differences were found in the demographics, systolic or diastolic pressures, or ventricular or atrioventricular valve function between the 2 groups. Group I had more infants with aortic atresia (P < .01) and smaller ascending aortas (P < .01). Before stage II, the retrograde aortic Doppler flow velocity increased in group I (P < .01) and was unchanged in group II. The reintervention rates before stage II were similar between the 2 groups. Before stage II, the ventricular end-diastolic pressure, left and right pulmonary artery pressures and diameters, and mixed venous and arterial saturations were similar between the 2 groups. The complication rates between the 2 groups were not significantly different, although a nonsignificant trend toward more neurologic complications was noted in group I. The Kaplan-Meier survival estimate at 1 year was similar between the 2 groups (63% for group I vs 71% for group II)., Conclusions: The presence of a reverse Blalock-Taussig shunt was not associated with more adverse events than those without. Gradual retrograde arch obstruction occurs commonly in palliated infants with aortic atresia. A reverse Blalock-Taussig shunt might play an important role to address the potential of retrograde obstruction, augmenting arch blood flow., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Aortopulmonary collaterals are a frequent phenomenon in patients after bidirectional cavopulmonary connection. The aortopulmonary collateral flow volume can be quantified using cardiac magnetic resonance imaging. However, the significance of aortopulmonary collateral flow for the postoperative outcome after total cavopulmonary connection is unclear and was sought to be determined., Methods: The data from 33 patients were prospectively studied with cardiac magnetic resonance, echocardiography, and cardiac catheterization before the total cavopulmonary connection operation. The early postoperative outcomes after total cavopulmonary connection completion were recorded., Results: Aortopulmonary collateral flow was 1.59 L/min/m(2) ± 0.65 L/min/m(2) (range, 0.54 L/min/m(2)-3.34 L/min/m(2)), constituting 43% ± 13% (range, 12-87%) of pulmonary blood flow and 35% ± 12% (range, 11-62%) of the cardiac index, resulting in a pulmonary blood flow/systemic blood flow ratio of 1.06 ± 0.17 (range, 0.79-1.55). The aortopulmonary collateral flow correlated with pulmonary blood flow/systemic blood flow ratio (r = 0.69, P < .0001), oxygen saturation (r = 0.42, P = .018), and cardiac index (r = 0.53, P = .002). Of the 36 patients, 24 underwent fenestrated total cavopulmonary connection during the study period. The aortopulmonary collateral flow, relative to the cardiac index, correlated with the duration of hospital stay (r = 0.48, P = .02) and pleural drainage (r = 0.45, P = .03). Patients whose pleural drainage lasted 1 week or less had less aortopulmonary collateral flow before the Fontan operation than those with a longer period until chest tube removal (1.23 L/min/m(2) ± 0.38 L/min/m(2) vs 1.73 L/min/m(2) ± 0.76 L/min/m(2); P = .03). Compared with a contemporary group of total cavopulmonary connection patients with fenestration in their extracardiac conduit who were studied prospectively, with a similar protocol, the bidirectional cavopulmonary connection had a greater amount of aortopulmonary collateral flow (1.59 L/min/m(2) ± 0.65 L/min/m(2) vs 1.30 L/min/m(2) ± 0.57 L/min/m(2), P = .04)., Conclusions: Patients after bidirectional cavopulmonary connection routinely acquire a large amount of aortopulmonary collateral flow. The hemodynamic consequences of aortopulmonary collateral flow translate into adverse outcomes early after total cavopulmonary connection completion., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: This study was to review an institutional experience with transcatheter closure of Fontan fenestrations and its impact on clinical care., Background: An interatrial fenestration improves postoperative outcomes in high-risk children undergoing a Fontan repair. While technical feasibility has been well defined, the clinical impact of subsequent closure is not well defined., Methods: Transcatheter closure of a surgically created or additional interatrial communication was attempted in 152 children at a median interval of 13.8 months after surgery. The clinical records were reviewed for demographic and anatomical characteristics, previous surgeries; catheterization data, and status at latest follow-up., Results: Mean oxygen saturation and right atrial pressure increased acutely from 87% +/- 5% to 96% +/- 3% (P < 0.001) and 12 +/- 2 mm Hg to 13 +/- 3 mm Hg (P < 0.001), respectively. Higher systemic venous atrial pressures after occlusion correlated with higher pulmonary artery pressures (P = 0.05) before the Fontan procedure and with higher right (P < 0.001) and left atrial (P = 0.001) and ventricular end-diastolic pressures (P < 0.001) immediately before occlusion. Complications included device malposition in 2 children, 1 child each had an air embolism and post-procedural bleeding, and each self-limiting and 1 child had acute ST elevation in inferior ECG leads because of occlusion of the acute marginal branch which was treated with angioplasty and placement of a stent. At follow-up (median 4.5 years), the mean oxygen saturation was 95% +/- 3%. Residual interatrial leaks were noted echocardiographically in 9%. Two children developed protein-losing enteropathy after fenestration closure. No deaths or strokes were observed in follow-up., Conclusions: Transcatheter occlusion of Fontan fenestrations is safe with acute and persistent improvements in oxygen saturations.