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In pediatric cardiovascular surgery, the autologous pericardium is useful for pulmonary artery augmentation. However, in some cases, the autologous pericardium may not be available for various reasons. Since 2014, we have developed tissue-engineered vascular grafts (TEVGs) and obtained good results. We report a successful case of multiple pulmonary artery plasty with TEVGs for pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries. TEVGs are useful alternatives to autologous pericardium in pediatric cardiovascular operations that often require multistage procedures., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Patients with right isomerism have accompanying complex congenital heart disease, which is characterized by pulmonary atresia and total anomalous pulmonary venous return. Balanced regulation of the systemic and pulmonary circulation is essential for successful management, especially for cases complicated with necrotizing enterocolitis (NEC)., Case Presentation: A 6-day-old male neonate with a single ventricle, pulmonary atresia, patent ductus arteriosus (DA), and total anomalous pulmonary venous return associated with right isomerism was admitted because of dyspnea, cyanosis, and melena. The patient presented circulatory incompetence due to excessive pulmonary blood flow, resulting in NEC. The patient underwent DA banding and colectomy following continuous intravenous infusion of prostaglandin E1 at six days. Subsequently, his condition improved, reaching a systemic oxygen saturation of around 80%. He underwent a bidirectional Glenn procedure and closure of colectomy at the ages of 5 and 6 months, respectively., Conclusion: DA banding can be an alternative to placing an aortopulmonary shunt, which is conventional in patients with ductus-dependent pulmonary circulation, because DA banding is feasible without cardiopulmonary bypass., (© 2022. The Author(s).)

Objectives: Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery., Methods: This was a retrospective review of 69 patients with Alagille syndrome who underwent congenital heart surgery at our institution. The underlying diagnoses included PPAS (n = 29), tetralogy of Fallot with PPAS (n = 14), tetralogy with PA (n = 3), PA with ventricular septal defect and major aortopulmonary collateral arteries (n = 21) and one each with D-transposition and supravalvar aortic stenosis., Results: The median age at surgery was 16 months (range 0-228 months). Procedures performed included PPAS repair (n = 43), tetralogy with PA repair (n = 3), unifocalization procedures (n = 21) and other (n = 2). Forty-two (61%) patients had mild or no liver dysfunction, while 26 (38%) had moderate or severe liver dysfunction. The median cardiopulmonary bypass time was 345 min (341 with liver dysfunction, 345 without liver dysfunction). There were a total of 8 operative (12%) deaths and 3 late (4%) deaths. Six operative and 2 late deaths occurred in patients with liver dysfunction (combined 30.7%) versus 2 operative and 1 late death (combined 7.1%) for patients without liver dysfunction (P < 0.05)., Conclusions: These results suggest that liver dysfunction has a profound impact on survival in children with Alagille syndrome undergoing congenital heart surgery., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Pulmonary atresia with an intact ventricular septum typically occurs in patients with concordant atrioventricular and ventriculoarterial connections. When it does occur in patients with discordant connections, it is most frequently seen in association with congenitally corrected transposition. We present a rare case of transposition of the great arteries with a ventricular septal defect (VSD) detected in fetal life which evolved throughout pregnancy resulting in the development of pulmonary atresia and severe restriction of the VSD., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: The aim of this study was to evaluate the long-term outcome and freedom from pulmonary valve replacement (PVR) after initial repair of tetralogy of Fallot (TOF)., Patients and Methods: The cohort of 306 patients treated between 1980 and 2017 was divided into anatomical subgroups according to the diagnosis of TOF-pulmonary stenosis, TOF-pulmonary atresia and TOF-double outlet right ventricle. Patients were treated with transannular patch (TAP), valve sparing repair (VSR), or conduits from the right ventricle to the pulmonary arteries (RVPA conduits)., Results: There were 21 deaths (6.9%), 14 being hospital deaths (4.6%) after primary correction and four deaths (1.3%) occurred after PVR. One patient died after a non-cardiac operation (0.3%). There were two late deaths (0.7%). During the past 12 years no early mortality has been observed. Ninety-one patients (30.4%) received PVR after a median of 12.1 ± 7.0 years with an early mortality of 4.4% ( n  = 4) and no late mortality. A significant difference in freedom from reoperation after TAP, VSR, and RVPA-conduits could be identified. Multivariate analysis displayed transannular repair ( p  = 0.016), primary palliation ( p <0.001), the presence of major aortopulmonary collateral arteries (MAPCA; p  = 0.023), and pulmonary valve Z -scores < - 4.0 ( p  = 0.040) as significant risk factors for PVR., Conclusion: TOF repair has a beneficial long-term prognosis with low morbidity and mortality. Pulmonary valve Z -scores < - 4.0, transannular repair, and presence of MAPCAs are associated with earlier PVR. Non-VSRs and TOF-pulmonary atresia lead to earlier reoperation but have no negative impact on survival., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

A major aortopulmonary collateral artery is a rare and easily missed diagnosis that is usually associated with Tetralogy of Fallot or pulmonary atresia. We present two cases of major aortopulmonary collaterals associated with trisomy 21 and atrioventricular septal defect with balanced ventricles in which the diagnosis went undetected until after initial cardiac repair.

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although children with TOF share four characteristic features (subaortic ventricular septal defect, overriding aorta, right ventricular hypertrophy, pulmonary stenosis), the clinical spectrum and course are in fact greatly heterogeneous. Echocardiography remains the mainstay for diagnosis, presurgical planning and postoperative follow-up. However, with continued technological advances, CT now plays an increasing role in TOF evaluation and management, helping to minimize routine invasive catheter angiography. Preoperatively, CT is uniquely suited to assess associated pulmonary arterial, aortic and coronary anomalies as well as extra-cardiovascular structures and is particularly helpful for delineating complex anatomy in the TOF subtypes of absent pulmonary valve and pulmonary atresia with major aortopulmonary collaterals. Postoperatively, CT is useful for identifying surgical complications and for long-term monitoring including volumetry quantification, especially in children for whom MRI is contraindicated or limited by implanted devices such as pacemakers and stents. In this article, we review key clinical features and considerations in the pre- and postoperative TOF patient and the burgeoning role of CT for facilitating accurate diagnosis and personalized intervention., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pulmonary atresia with hypoplastic right ventricle and ventricular septal defect with right ventricle-dependent coronary circulation are very rare congenital heart anomalies. This report describes the case of a patient in whom aortic-right ventricle shunting was surgically established with a satisfactory postoperative course. Aortic-right ventricle shunting can enhance oxygenation of the sinusoidal communication and reduce the incidence of myocardial ischemia without influencing the left ventricular volume load through the ventricular septal defect despite preservation of the septal defect., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: We intend to describe an entirely transcatheter management pathway for patients with pulmonary atresia with intact ventricular septum (PA/IVS)., Background: PA/IVS is a rare cyanotic congenital heart lesion traditionally palliated with multiple procedures and surgeries. Entirely non-surgical transcatheter management from infancy to adulthood may be possible; however, the pathway from neonatal radiofrequency (RF) pulmonary valve perforation to later transcatheter pulmonary valve replacement (TPV) is not well described., Methods: This retrospective study was performed at a pediatric cardiac center between 2007-2018. All patients with PA/IVS who were managed exclusively with catheterization-based interventions as neonates (RF perforation, pulmonary valvuloplasty, or ductal stenting) were analyzed. Demographic, procedural and clinical data were collected., Results: Fifteen patients had exclusively catheterization-based RV decompression as neonates, 7 of whom did not require subsequent surgery. Six patients required a right ventricular outflow tract (RVOT) augmentation later in life; all were born before 2013. All 6 later had a TPV placed. Two of the patients underwent a surgical Glenn shunt alone. Of the 7 patients that never had surgery, 3 have since undergone a TPV, and 4 are awaiting candidacy for TPV placement. No patients with PA/IVS have had an RVOT augmentation at our institution since 2012., Conclusions: Patients with PA/IVS who undergo catheterization-based neonatal interventions can make it to TPV without requiring surgery. At our institution, there has been a shift in management over the last 8 years favoring entirely non-surgical transcatheter management from infancy to adulthood.

Objective: Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies., Methods: Retrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Descriptive statistics summarized patient characteristics and a multivariable Cox survival model was used to compare treatment strategies., Results: Of 119 patients, 62 (52.1%) were male and 13 (10.9%) had a chromosomal abnormality. BiV was pursued in 53.8% (64 out of 119) and SV in 46.2% (55 out of 119) with median tricuspid valve z scores of -1.59 (interquartile range, -3.03 to 0.21) and -5.12 (interquartile range, -5.60 to -4.06), respectively. The median follow-up was 6 years (interquartile range, 2-15 years). Overall survival at 1, 3, and 10 years was 82.4% (98 out of 119), 80.6% (96 out of 119) and 79.8% (95 out of 119), respectively. End states include 36 (30.3%) BiV, 33 (27.7%) SV, 22 (18.5%) alive without definitive end state, 21 (17.6%) death before end state, 4 (3.4%) 1-and-a-half ventricle, and 3 (2.5%) transplants. No SV were converted to BiV, whereas 4 out of 64 (6.3%) BiV were converted to SV. After adjusting for gender, chromosomal abnormalities, gestational age, and birth weight, SV patients had a significantly higher hazard of mortality (hazard ratio, 9.0; 95% CI, 2.65-30.69; P < .001). Mortality was higher in those with right ventricle-dependent coronary circulation (41.9% [13 out of 31]) compared with those without right ventricle-dependent coronary circulation (7.3% [6 out of 82]) (P < .001)., Conclusions: Pulmonary atresia with intact ventricular septum remains a challenging lesion for those patients on the SV pathway, particularly with right ventricle-dependent coronary circulation., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

We report the first successful Glenn procedure for pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries. This case was complicated by hypoplastic left ventricle and severe mitral regurgitation. Partial closure of the mitral valve at the time of Blalock-Taussig shunting resulted in improvement of heart failure.

Background: Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT., Methods: Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality., Results: Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p = .004). While waitlist mortality was similar between groups (p = .09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p = .03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p = .03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, p < .0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival., Conclusions: Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias., (© 2022 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)

A 12-year-old with a history of critical pulmonary stenosis and moderate right ventricular hypoplasia underwent neonatal pulmonary valve dilation, an aortopulmonary shunt, and an infant cavopulmonary anastomosis with aortopulmonary shunt takedown. During a diagnostic cardiac catheterization at 12 years of age, angiography showed interruption in the midportion of the coronary sinus, which required no intervention.

Recently, the outcome of severe Ebstein anomaly (EA) has improved with the prevalence of the Starnes procedure. However, time-dependent changes in the size of the central pulmonary artery (PA) have not been fully understood. A retrospective chart review of patients with EA who underwent the Starnes procedure during the neonatal period and those with pulmonary atresia with intact ventricular septum (PAIVS) was performed at Fukuoka Children's Hospital. There were 14 patients in the severe EA group and 36 in the PAIVS group, with mean observational periods of 3.8 and 4.2 years, respectively. No significant difference in survival was observed between the groups. However, the mean size of the central PA was smaller in the severe EA group at each surgical stage (after systemic-to-pulmonary shunt, after the bidirectional Glenn procedure, and after the Fontan procedure). A significantly larger ventricular volume was observed in the severe EA group after the Fontan procedure. The growth of the central PA was poor in patients with severe EA. Patients with severe EA should be carefully monitored in this regard both before and after undergoing the Fontan procedure. Further studies regarding long-term prognosis are expected., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect defined by membranous or muscular atresia of the right ventricular outflow tract where patients display varying degrees of hypoplasia of the right ventricle. This condition results in cyanosis due to an inability of blood to flow from the right ventricle to the pulmonary arteries, thus requiring immediate surgical intervention after birth. An iPSC line was generated from peripheral blood mononuclear cells of a 11-year-old male patient diagnosed with PA-IVS through Sendai virus-mediated reprogramming. This disease-specific iPSC line was characterized by immunocytochemistry, STR analysis, karyotype analysis, and mycoplasma testing., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Author(s). Published by Elsevier B.V. All rights reserved.)

We reported a case of a 14-year-old tetralogy of Fallot/pulmonary atresia patient, where a rare tubular aortopulmonary communication between the arterial trunks allowed for well-growth of the pulmonary vasculature. The patient underwent successful one-stage unifocalization, right ventricle-pulmonary artery conduit and ventricular septal defect closure. The tubular communication was considered an aortopulmonary window as opposed to the major aortopulmonary collateral, based on its exclusively intrapericardial course. We highlighted the importance of cross-section imaging in diagnosis and surgical decision-making in such situations., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries., Methods: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10)., Results: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group., Conclusion: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization., (© 2022 Wiley Periodicals LLC.)

To assess the relationships between pulmonary artery (PA) pressure and the PA: aortic systolic pressure ratio measured intraoperatively and at surveillance catheterization in patients achieving complete unifocalization and repair for tetralogy of Fallot with major aortopulmonary collateral arteries (TOF/MAPCAs). This was a single-center retrospective cohort analysis of all patients who underwent complete repair of TOF/MAPCAs from 2002 to 2019 and received a postoperative surveillance catheterization at our center 6-24 months after surgery. Associations between intraoperative and catheter hemodynamic data were analyzed. A total of 163 patients were included. Median systolic PA pressure was 30 (quartiles 26, 35) and 35 (28, 42) mm Hg intraoperatively and at catheterization respectively; systolic aortic pressure 90 (86, 100) and 84 (76, 92); and PA:aortic pressure ratio was 0.33 (0.28, 0.40) and 0.41 (0.34, 0.49). Moderate correlation was found between the intraoperative and catheter-based hemodynamics, with the majority of systolic PA pressures within 10 mm Hg and PA:Ao systolic ratios within 0.1. Changes in the ratio were influenced to a similar degree by differences in PA and aortic pressures. Surgical and/or catheter reinterventions were more common in patients with both higher intraoperative PA systolic pressure and PA:aortic systolic ratios and in those with a greater discrepancy between intraoperative and catheterization values. PA systolic pressure and the PA:aortic systolic pressure ratio measured immediately after repair remains useful metrics for assessing the initial operative PA reconstruction, and as indicators of longer-term hemodynamics. Initially elevated and subsequently discrepant PA systolic pressure and PA:aortic systolic pressure ratios were associated with higher rates of reintervention., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) represents an anatomically diverse and technically demanding spectrum of congenital disease. Here, we review a manuscript by Onalan et al. in the Journal of Cardiac Surgery detailing a retrospective, single-center cohort study of patients undergoing unifocalization for PA/VSD/MAPCA via either a pulmonary artery patch augmentation or pericardial roll technique. While they report statistically equivalent outcomes using both techniques, longer follow-up and increased sample size are necessary to determine efficacy and safety., (© 2022 Wiley Periodicals LLC.)

This report describes the cases of 2 neonates who had a diagnosis of pulmonary atresia with intact ventricular septum and muscular right ventricular outflow tract (RVOT) obstruction and who underwent modified hybrid therapy as initial palliation. This approach combined the traditional hybrid approach and off-pump RVOT reconstruction with a Foley balloon catheter. Both patients recovered well, with adequate pressure gradients over the RVOT and pulmonary valve. This modified hybrid therapy can serve as a feasible treatment approach for patients with pulmonary atresia with intact ventricular septum, especially patients with muscular RVOT stenosis., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable., Methods: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018., Results: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency., Conclusions: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature., (© 2022 Wiley Periodicals LLC.)

Background: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a relatively rare and complex form of congenital heart disease. Unifocalization of MAPCAs has been advocated by some groups for the treatment of this condition. The purpose of this study was to assess the midterm fate of unifocalized MAPCAs in a cohort of patients with retroesophageal MAPCAs., Methods: This was a retrospective review of 37 patients who underwent a unifocalization procedure. All patients in this study had 1 or more retroesophageal MAPCAs, and detailed mapping of the MAPCAs was made based on a combination of the cardiac catheterization and surgical findings., Results: The 37 patients had a total of 166 MAPCAs, or 4.5 MAPCAs per patient. One hundred twenty-nine (78%) MAPCAs were unifocalized, whereas 37 (22%) were ligated because they were dual supply. Median follow-up was 69 months. At follow-up cardiac catheterization, evaluation of the 129 unifocalized MAPCAs demonstrated that 123 (95%) had antegrade flow, whereas 6 were occluded. For the 123 MAPCAs with antegrade flow, 97 (80%) were widely patent, whereas 26 were stenotic. Thirteen of the 37 patients have subsequently undergone reintervention on MAPCAs that were determined to be stenotic following unifocalization. Seven of these patients had mild disease and had complete resolution with balloon (n = 5) or surgical revision (n = 2). Six patients with moderate or severe disease underwent surgical revision with confirmed resolution in 4 of 6., Conclusions: The data demonstrate that the majority of unifocalized MAPCAs remain widely patent following unifocalization. However, one-quarter of unifocalized MAPCAs develop stenoses or occlusion. These results suggest the fate for most unifocalized MAPCAs is favorable but highlight the need for close vigilance., (Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: We aimed to explore the relation between temporal right heart growth and long-term outcomes in patients with pulmonary atresia with an intact ventricular septum (PA/IVS) who underwent balloon pulmonary valvuloplasty (BPV)., Methods: We performed echocardiography to measure pulmonary valve diameter, right atrial end-systolic area (RAA), and right ventricular end-diastolic area (RVA) before BPV 1 and 5 years after BPV. Primary and secondary end points were to explore temporal changes in right heart structures and to determine echocardiographic parameters related to late adverse events (LAEs)., Results: In 31 patients, pulmonary valve diameter significantly increased after BPV, whereas tricuspid valve diameter remained unchanged throughout the 10.0-year follow-up (range, 5.8-14.0 years). After BPV, RAA temporally decreased, whereas RVA significantly increased. There were LAEs in 6 patients (19%), arrhythmias in 2, heart failure in 1, reintervention of the right ventricular outflow tract in 1, and reintervention for residual cyanosis in 2. The rate of freedom from LAEs at 5 and 10 years was 92% and 82%, respectively. Right atrial end-systolic area temporally decreased in patients without LAEs (P < .01); however, RAA remained unchanged throughout the period in patients with LAEs (P = .16). Moderate or severe pulmonary regurgitation (hazard ratio = 23.0; 95% confidence interval, 1.3-385; P = .03) and the ratio of RVA to RAA at 1 year after BPV (hazard ratio = 6.3 × 10 -11 ; 95% confidence interval, 2.1 × 10 -20 to 0.19; P = .03) were independent risk factors for LAEs., Conclusions: Disproportional right heart growth was observed in patients with pulmonary atresia with an intact ventricular septum after BPV. Pulmonary regurgitation and increased RAA are crucial for identifying the burden of LAEs among them., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We report a case of a 5-year-old child with tetralogy of Fallot-pulmonary atresia with anomalous origin of left pulmonary artery from ascending aorta and right lung supplied by collateral arteries. This case highlights the role of preoperative CT angiography in differentiating this entity from a common arterial trunk with discontinuous pulmonary arteries., (© 2022 Wiley Periodicals LLC.)

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery-pulmonary artery fistula. We present two cases with coronary artery-pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Objectives: This study aimed to identify clinical, hemodynamic, or echocardiographic predictive features of persistent duct-dependency of pulmonary circulation (PDDPC) after effective percutaneous relief of pulmonary atresia with the intact ventricular septum (PA-IVS) or critical pulmonary stenosis (CPS)., Methods: From 2010 to 2021, 55 neonates with PA-IVS or CPS underwent percutaneous right ventricle (RV) decompression at our Institution. After successfully relief of critical obstruction, 27 patients (group I) showed PDDPC, whereas RV was able to support the pulmonary circulation in the remaining 28 patients (group II). Clinical, hemodynamic, and echocardiographic features of these two groups were compared., Results: No significant difference in clinical and hemodynamic data was found between the groups, although the group I had a lower oxygen saturation at hospital admission. However, tricuspid valve (TV) diameter <8.8 mm, TV z-score ←2.12, tricuspid/mitral valve annular ratio <.78, pulmonary valve diameter <6.7 mm, pulmonary valve z-score ←1.17, end-diastolic RV area <1.35 cm 2 , end-systolic right atrium area >2.45 cm 2 , percentage amount of interatrial right-to-left shunt >69.5%, moderate/severe tricuspid regurgitation, RV systolic pressure >42.5 mmHg, tricuspid E/E' ratio >6.6 showed each significant predictive value of PDDPC. These parameters were used to build a composite echocardiographic score (PDDPC-score), assigning one point each above the respective cut-off value. A score ≥4.00 showed high sensitivity (100%) and specificity (86%) in predicting PDDPC., Conclusion: Clinical and hemodynamic features fail to predict the short-term fate of the pulmonary circulation after successful treatment of PA-IVS/CPS. However, a simple, composite echocardiographic score is useful to predict PDDPC and could be crucial in the management of this frail subset of patients., (© 2022 The Authors. Echocardiography published by Wiley Periodicals LLC.)

The Rastelli procedure is indicated for repair of tetralogy of Fallot (TOF) with pulmonary atresia. However, currently, there are no criteria available for concomitant surgical intervention for an enlarged ascending aorta in infancy. We report a rare case of TOF with pulmonary atresia complicated by severe enlargement of the ascending aorta in a girl aged 2 years and 5 months. We performed the Rastelli procedure with ascending aorta replacement. Pathological findings of the ascending aorta included fragmentation of elastic fibers and loss of smooth muscle cells. Postoperatively, the patient remained healthy and asymptomatic. This case indicates that ascending aortic enlargement in conotruncal anomalies may be associated with pathological lesion even in early childhood. Replacement of the diseased aorta could be considered as a concomitant procedure with definitive repair for conotruncal anomalies presenting with severe ascending aortic enlargement., (© 2021. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

Background: A staged thoracotomy unifocalization approach has not been the dominant option over the past 20 years primarily due to the introduction of midline one-stage complete unifocalization., Methods: In this issue of the Journal of Cardiac Surgery, van de Woestijne et al. publish their experience over the past 30 years in 39 consecutive patients with "consistent" staged unifocalization through a lateral thoracotomy in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCA)., Results: They report definitive repair completion in 76.3% of the patients and an overall survival after definitive repair of 96% at 20 years despite the study period ranging from 1989 to the present., Conclusion: Given the multiple variations one could have with PA/VSD/MPACA, a midline unifocalization approach may not always be possible. Surgeons should be familiar with the lateral thoracotomy unifocalization staged approach to PA/VSD/MAPCA., (© 2022 Wiley Periodicals LLC.)

Objectives: Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCAs) presents with variable anatomy with regard to the pulmonary vasculature, requiring personalized surgical treatment. A protocol consisting of staged unifocalization and correction was employed., Methods: Since 1989, 39 consecutive patients were included (median age at first operation 13 months). In selected cases, a central aorto-pulmonary shunt was performed as the first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure, and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Echocardiographic data were obtained postoperatively and at interval follow-up., Results: In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, of which four have since died. One patient is awaiting further correction. A correction was performed successfully in 28 patients. Operative mortality was 3% and late mortality was 11%. Median follow-up after the correction was 19 years. Eleven patients required homograft replacement. Freedom from conduit replacement was 88%, 73%, and 60% at 5, 10, and 15 years respectively. Right ventricular function was reasonable or good in 75% of patients. All but one patient were in NYHA Class I or II., Conclusions: After complete unifocalization 30/37 patients (81%) were considered correctable. The staged approach of PA, VSD, and SPCAs results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients., (© 2022 The Authors. Journal of Cardiac Surgery published by Wiley Periodicals LLC.)

Unifocalization of the major aortopulmonary collaterals (MAPCAs) followed by intracardiac repair with ventricular septal defect (VSD) closure and restoration of right ventricle-to-pulmonary artery continuity is the ultimate treatment goal in a case of VSD with pulmonary atresia and MAPCAs. It may be achieved in a single stage or may require multiple surgeries. We present a case of a 2-year-old boy with VSD with pulmonary atresia who underwent single-stage unifocalization of MAPCAs through the midline followed by intracardiac repair using 2 polytetrafluoroethylene tube grafts: one for unifocalization and other as a bicuspid valved right ventricle-to-pulmonary artery conduit., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Branch pulmonary artery (PA) stenosis due to ductus arteriosus (DA) tissue (DA-PS) contributes to the morbidity associated with pulmonary atresia (PAtr). We sought to identify preoperative echocardiographic features predictive of DA-PS. Patients consecutively encountered with PAtr and a DA-dependent pulmonary circulation at birth who underwent intervention in our program over a 5-year period were identified and records reviewed. Preoperative echocardiograms were reviewed to identify features that predicted postoperative DA-PS. Seventy patients with PAtr met inclusion criteria and 36 (51%) had DA-PS. At preoperative echocardiography, the proximal diameter of the PA ipsilateral to the DA was smaller in those with versus without DA-PS (Z-score - 4.8 ± 1.7 vs - 1.1 ± 1.7, respectively p < 0.001). PA origins could not be imaged on the same axial plane in 21/36 (58%) with versus 2/34 (6%) without DA-PS. Patients with DA-PS had an obtuse posterior angle of the PA bifurcation compared to those without (128 ± 17° and 87 ± 21°, p < 0.001), and a posterior angle of > 100° best predicted DA-PS with a sensitivity of 97% and specificity of 76%. An abnormal PA relationship and/or an obtuse posterior bifurcation angle had a sensitivity, specificity, positive and negative predictive value for DA-PS of 78%, 94%, 90% and 86%, respectively. Finally, DA insertion was into the ipsilateral PA in 26/36 (72%) of cases with DA-PS. A smaller proximal ipsilateral PA diameter, inability to image the PAs in the same plane, a posterior PA bifurcation angle of > 100°, and insertion of the DA in the ipsilateral PA demonstrated by echo are useful in identifying patients at risk for DA-PS., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: There is increasing use of catheter-based therapy as part of the neonatal treatment algorithm for pulmonary atresia with an intact ventricular septum (PAIVS). The management strategies utilised and outcomes of patients with PAIVS at our centre have not been examined., Methods: A retrospective case series was undertaken including all infants with PAIVS born January 2009 to July 2019 (follow-up to January 2020) managed at The Children's Hospital at Westmead, New South Wales. Demographic features, anatomical substrate, management pathway and subsequent clinical outcomes were examined., Results: Fifty-two (52) infants were included (male n=28, 53.8%). The right ventricular morphology was classified as normal, mildly, moderately and severely hypoplastic in 3 (5.8%), 13 (25.0%), 19 (36.5%) and 17 (32.7%) patients respectively. Thirty-seven (37) patients underwent an initial aortopulmonary (AP) shunt (surgical shunt or patent ductus arteriosus [PDA] stent). The remaining 15 patients underwent an initial intervention to decompress the right ventricle. Twenty (20) patients underwent a neonatal catheter-based intervention. An initial catheter-based intervention was more likely in the second half of the period. Sixteen (16) patients had an attempted pulmonary valve perforation, 12 as their initial procedure. Median follow-up was 62 months (range 3-119 months). Final circulation status was known in 37 patients; biventricular n=14 (37.8%), "1.5 ventricles" n=4 (10.8%), single n=19 (51.4%). There were five deaths during the period (9.6%), including two during the initial procedural admission attributed to tamponade requiring extracorporeal membrane oxygenation (ECMO) at the time of percutaneous pulmonary valve perforation., Conclusion: There has been an overall trend towards including catheter-based strategies in the neonatal period as part of management at our centre. Given the risk of bleeding and ECMO related to this, consideration should be given to the availability of multidisciplinary support when planning the timing of these procedures., (Crown Copyright © 2021. Published by Elsevier B.V. All rights reserved.)

Background: To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS)., Methods: 50 children with type I PA/IVS and 50 with type II PA/IVS who had undergone initial surgery were enrolled. Children with Type I were divided into groups A (n = 25) and B (n = 25). Group A had received BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, whereas group B had undergone BT shunt combined with PDA ligation and pulmonary valve incision. Children with type II were divided into groups C (n = 25) and D (n = 25). Group C had received BT shunt combined with PDA ligation, right ventricular outflow tract (RVOT) incision and transannular patch. Group D had undergone BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation. The differences in mechanical ventilation time, length of ICU stay, mortality rate, tricuspid Z value, tricuspid regurgitation, oxygen saturation, pulmonary regurgitation, McGoon ratio, pulmonary artery transvalvular pressure, survival rate were compared between groups A and B, between groups C and D respectively., Results: The ventilator assistance time and length of ICU stay were greater in group C than in group D (80.96 ± 8.42 h vs. 65.16 ± 4.85 h, P = 0.045; 222.00 ± 11.72 h vs. 162.48 ± 7.91 h, P = 0.048). The pulmonary artery transvalvular pressure was significantly higher in group A than in group B at 3, 6, 12, 24 and 36 months after surgery (64.86 ± 4.13 mmHg vs. 53.04 ± 5.64 mmHg, P = 0.045; 69.47 ± 1.93 mmHg vs. 55.95 ± 4.04 mmHg, P = 0.005; 80.16 ± 3.76 mmHg vs. 73.24 ± 2.34 mmHg, P = 0.035; 62.95 ± 5.64 mmHg vs. 48.47 ± 7.44 mmHg, P = 0.04; 53.69 ± 4.89 vs. 45.77 ± 3.26, P = 0.02). Furthermore, the tricuspid Z value was significantly greater in group B than in group A at 3 and 24 months after surgery (- (1.37 ± 0.04) vs. - (1.43 ± 0.06), P = 0.03; - (0.41 ± 0.06) vs. - (0.51 ± 0.11), P = 0.02)., Conclusions: The effect of BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, and the effect of BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation, RVOT incision and transannular patch., (© 2022. The Author(s).)

Objective: The study aims to evaluate the feasibility and effectiveness of an individualized procedure for right ventricular outflow tract (RVOT) reconstruction in pulmonary atresia with ventricular septal defect (PA-VSD)., Methods: RVOT was reconstructed using autologous pulmonary artery tissue preserved in situ as the posterior wall and a bovine jugular vein patch (BJVP) as the anterior wall in patients with PA-VSD (observation group). The size of the BJVP made from a bovine jugular vein conduit (BJVC) was individually calculated using a formula based on the child's weight and the size of the autologous pulmonary artery (the diameter of BJVC DB⁢J⁢V⁢C = Dt⁢h⁢e⁢o⁢r⁢e⁢t⁢i⁢c⁢a⁢l-W⁢z^-4π). Its effect was then compared with the conventional modified Rastelli procedure based on the BJVC (control group)., Results: A total of 22 patients that underwent the new procedure were simultaneously compared with the 25 patients in the control group. No deaths occurred in both groups. Notably, there were no significant differences in mechanical ventilation, ICU and postoperative residence, cardiopulmonary bypass, and aortic cross-clamp time. In the follow-up, which spanned for 8-12 years (mean 9.2 years), only four cases with moderate regurgitation were noted in the observation group without obstruction. In the control group, two patients had a conduit replacement. Three patients suffered from anastomotic stenosis, which was corrected by balloon dilatation., Conclusion: Individualized RVOT reconstruction with autologous pulmonary tissue preserved in situ as the posterior wall is adequate for treating PA-VSD., Competing Interests: The authors declare no conflict of interest., (© 2022 The Author(s). Published by IMR Press.)

Achieving an optimal surgical result in patients with major aortopulmonary collateral arteries (MAPCAs) requires a thorough preoperative evaluation of the anatomy and physiology of the pulmonary circulation. This review provides a detailed description of diagnostic catheterization in patients with MAPCAs, including a summary of catheterization techniques, an overview of commonly used terms, and a review of MAPCA and pulmonary artery angiographic anatomy., (© 2021 Wiley Periodicals LLC.)

Tetralogy of Fallot with pulmonary atresia is a group of congenital cardiac malformations, which is defined by the absence of luminal continuity between both ventricles and the pulmonary artery, and an interventricular communication. Pulmonary arterial supply in patients with tetralogy of Fallot with pulmonary atresia can be via the arterial duct or from collateral arteries arising directly or indirectly from the aorta (systemic-to-pulmonary artery collaterals), or rarely both. The rarest sources of pulmonary blood flow are aortopulmonary window and fistulous communication with the coronary artery.Herein, we describe an outflow tract malformation, tetralogy of Fallot with pulmonary atresia and aortopulmonary window, which was misdiagnosed as common arterial trunk. We emphasise the morphological differences.

Objective: Conotruncal anomalies can develop aortopathy and/or aortic valve (AV) disease and AV replacement (AVR) is occasionally needed. We report long-term results and examine factors affecting survival following AVR in this group., Methods: We queried the Pediatric Cardiac Care Consortium (PCCC, US database for interventions for congenital heart diseases) to identify patients with repaired conotruncal anomalies and AVR. Long-term outcomes were provided by the PCCC, the US National Death Index, and Organ Procurement and Transplantation Network. Competing risks analysis examined outcomes following AVR (death/transplantation, reoperation) and multivariable regression analysis assessed significant factors., Results: One hundred six children with repaired conotruncal anomalies underwent AVR (1982-2003). Underlying anomaly was truncus (n = 40), d-transposition (n = 22), type-B interrupted arch (n = 16), double-outlet right ventricle (n = 12), pulmonary atresia with ventricular septal defect (n = 9), tetralogy of Fallot (n = 6), corrected transposition (n = 1). 18 (17%) had prior aortic valvuloplasty (surgical = 12, percutaneous = 6). Median age at AVR was 6.9 years (interquartile range = 2.5-12.4). AV pathophysiology was regurgitation (n = 83, 78%), stenosis (n = 9, 9%), and mixed (n = 14, 15%). AVR type was mechanical (n = 72, 68%), homograft (n = 21, 20%), and Ross (n = 13, 12%). Operative mortality was 13(12%). Infant age at AVR was risk factor (odds ratio = 55, 95% confidence interval [CI] = 6-539, P  = .0006). On competing risks analysis, five years after AVR, 6% died or received transplantation, 20% had reoperation. Twenty-five years transplant-free survival was 53%. Factors associated with death after hospital discharge included mitral surgery (hazards ratio [HR] = 11, 95% CI = 3-39, P  = .0002), underlying defect (HR = 2, 95% CI = 1-5, P  = .446). Twenty years transplant-free survival in conotruncal anomalies group was inferior to matched children undergoing AVR for congenital non-conotruncal disease (61% vs 82%, P  = .0012)., Conclusions: Long-term survival following AVR in children with conotruncal anomalies is inferior to that of isolated congenital AV disease and is linked to an underlying cardiac defect. Although valve type was not associated with survival, infant age was a risk factor for operative mortality. Continuous attrition and high reoperation warrant vigilant monitoring.

We present a case of a 16-year-old cyanotic male patient with ventricular septal defect, pulmonary atresia and discordant atrioventricular connections where computed tomography angiography demonstrated a large tortuous coronary-to-pulmonary collateral artery arising from the superdominant morphologically right coronary artery and coursing cranially to supply the pulmonary parenchyma., (© 2022 Wiley Periodicals LLC.)

Background: The anatomy of major aortopulmonary collateral arteries (MAPCAs) can be highly variable with regard to number, anatomic origin, course, and relationship to the native pulmonary arteries. Some MAPCAs travel behind the esophagus (retroesophageal) and bronchus before entering the lung parenchyma. This study compared the physiologic and surgical characteristics of retroesophageal vs anterior located MAPCAs., Methods: This was a retrospective review of 42 patients who had 1 (n = 36) or 2 (n = 6) retroesophageal MAPCAs. These MAPCAs were then characterized as (1) single supply, meaning no connection to the pulmonary arteries; (2) dual supply, but inadequate connection to the distal pulmonary vascular bed; and (3) dual supply with adequate connection., Results: The 42 patients presented with 187 MAPCAs, or 4.5 MAPCAs per patient. Of these, 48 MAPCAs were retroesophageal, including 40 that were single supply, 6 were dual supply with inadequate connection, and 2 had dual supply with adequate connection. On the basis of this anatomy and physiology, 96% of retroesophageal MAPCAs were unifocalized. For the 139 anterior MAPCAs, 89 were single supply, 15 were dual supply with inadequate connection, and 35 were dual supply with adequate connection; thus, 75% of anterior MAPCAs were unifocalized (P < .01 compared with retroesophageal MAPCAs)., Conclusions: The data demonstrate that retroesophageal MAPCAs had very different anatomy and physiology compared with anterior MAPCAs. These results suggest that nearly every retroesophageal MAPCA should be unifocalized to incorporate the lung segments supplied., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Development of major aortopulmonary collateral arteries are strongly associated with cyanotic congenital heart disease. However, they have rarely been reported in noncyanotic congenital heart disease. We report a rare case of a newborn originally diagnosed with an atrial septal defect, a ventricular septal defect, and pulmonary arterial hypertension who underwent complete repair. Failure to progress postoperatively lead to the delayed diagnosis of aortopulmonary collateral arteries. Percutaneous embolization and surgical ligation of aortopulmonary collateral arteries resulted in rapid recovery., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)