BACKGROUND:Selective antegrade cerebral perfusion (SACP) is increasingly used during neonatal aortic arch surgery, yet practices remain inconsistent across surgeons and institutions due to limited evidence and lack of consensus. This study aimed to characterize deep hypothermic circulatory arrest (DHCA) and SACP practices among congenital heart surgeons internationally, including temperature, flow rate, and monitoring. METHODS:A 124-question electronic survey was distributed to congenital heart surgeons worldwide through the World Society for Pediatric and Congenital Heart Surgery from November 2024 to February 2025. Responses were limited to currently practicing or recently retired surgeons. RESULTS:The survey was completed by 104 surgeons (95.2% active, 4.8% retired). Respondents were predominantly men (93.3%) with a median age of 53 years and 19 years in practice. SACP was used exclusively by 69.2% and DHCA by 9.6%, whereas 21.2% selected between techniques based on anatomy or anticipated arrest duration. A change in individual practice was reported by 41% of the respondents, most transitioning from DHCA to SACP-particularly among older surgeons (median age 60 vs 48 years, P < .001). Among those using SACP, 78.3% used it alone and 21.7% combined it with lower body perfusion. Target SACP temperature ranged widely from <20 °C to 35 °C, with lower targets for longer procedures. Flow determination also varied: 59.6% used weight-based calculations, whereas others adjusted by pressure or near-infrared spectroscopy. After initiation, flow was titrated to right radial artery pressure (38.0%) or near-infrared spectroscopy (55.4%). CONCLUSIONS:Substantial variability persists in neuroprotective strategies during neonatal aortic arch repair, emphasizing the need for evidence-based standardization and high-fidelity neuromonitoring strategies.

Background: Rheumatic heart disease (RHD) continues to disproportionately affect young Māori and Pacific Peoples in Aotearoa New Zealand (NZ) [...]

Background: To improve understanding of indications and outcomes for cardiac surgery for rheumatic aortic valvular disease in the young. Methods: Single institution retrospective cohort aged < 18 years with rheumatic heart disease who underwent surgery for isolated rheumatic aortic valve disease between 2000 and 2019. Baseline, intermediate follow-up, and late follow-up data were collected. Left ventricular (LV) dysfunction defined as LV ejection fraction < 55% or LV shortening fraction < 27%. Results: Thirty-nine patients who were 8 to 18 years of age were included (median age 14 years), weighing 27 to 157 kg (median 78 kg) with follow up of 2 to 15 years (median 7 years). Index operations were valve repair 6 of 39 (15%), valve replacement 33 of 39 (84%) with homograft (n = 20), and mechanical valve (13). The 30-day mortality was zero. Overall mortality was 8 of 39 (20%); 17 of 39 (43%) underwent reoperation. At intermediate and late follow up, 27% (7/26) and 53% (18/34) had persistent dysfunction, respectively. Baseline LV dysfunction increased the risk of death (hazard ratio 13.3 [1.52-115.5], P = .003), which increased adjusting for higher body surface area (BSA). All those with baseline weight > 105 kg either died or had LV dysfunction at late follow up (P = .001). Freedom from late valve-related complications at 5, 10, and 15 years was 72%, 31%, and 23%, respectively. Conclusions: Preoperative LV dysfunction is associated with an increased risk of death for pediatric patients undergoing isolated rheumatic aortic valve surgery. This risk increases further, adjusting for BSA. There was a high rate of reoperations and late complications.

Objectives: To describe longer-term survival and morbidity outcomes after hospital discharge in a binational cohort of children who required extracorporeal membrane oxygenation after cardiac surgery. Methods: This was a retrospective cohort study from the Australia and New Zealand Congenital Outcomes Registry for Surgery database. All patients younger than 18 years of age (n = 12,290) undergoing pediatric cardiac surgical procedures between January 1, 2013, and December 31, 2021, who required post-cardiotomy extracorporeal membrane oxygenation in the same admission were included. Results: Among 376 (3%) patients who required post-cardiotomy extracorporeal membrane oxygenation, 242 (64.4%) survived to hospital discharge. Median follow-up in survivors posthospital discharge was 4.2 years (interquartile range, 2.6-8 years). Kaplan-Meier survival at 1, 3, 5, and 8 years was 62%, 58.5%, 55.8%, and 52.6%. Postdischarge survival was 95.5%, 90.7%%, 87.1%, and 81.9% at 3, 5, and 8 years, respectively. Survival was significantly poorer in patients with single-ventricle physiology. In total, 43.3% (n = 105) of hospital survivors had at least 1 major morbidity at the last follow-up. The most common major morbidities were developmental delay, permanent neurologic deficits, and growth restriction. Conclusions: In total, 38% of children who required ECMO after cardiac surgery did not survive beyond 1 year. However, of patients who survived to hospital discharge, 95% were alive at 1 year. Approximately 80% of patients were alive at 5 years after hospital discharge. Outcomes were poorer in patients with single ventricle physiology. In total, 40% of patients had significant morbidity after hospital discharge. (J Thorac Cardiovasc Surg 2025;169:1548-58)

In this issue of The Annals of Thoracic Surgery, the report by Cheung and colleagues 1 Cheung E.W. Mastropietro C.W. Flores S. et al. Procedural outcomes of pulmonary atresia with intact ventricular septum in neonates: a multicenter study. Ann Thorac Surg. 2023; 115: 1470-1478 Abstract Full Text Full Text PDF Scopus (3) Google Scholar of early outcomes in pulmonary atresia with intact ventricular septum shows improvement in mortality compared with earlier multicenter reports. 1 Cheung E.W. Mastropietro C.W. Flores S. et al. Procedural outcomes of pulmonary atresia with intact ventricular septum in neonates: a multicenter study. Ann Thorac Surg. 2023; 115: 1470-1478 Abstract Full Text Full Text PDF Scopus (3) Google Scholar It is good to know outcomes are better but the use of major adverse cardiac events (MACE), a combined morbidity-mortality score, demonstrates that we still face major challenges in these neonates, with 20% having major adverse events. Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter StudyThe Annals of Thoracic SurgeryVol. 115Issue 6PreviewMulticenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE). Full-Text PDF

Objectives To better understand the relative influence of fetal and maternal factors in determining the choice-of-care pathway (CCP) and outcome in the fetus with hypoplastic left heart syndrome (HLHS).Design A retrospective, population-based study of fetuses with HLHS from a national dataset with near-complete case ascertainment from 20 weeks’ gestation. Fetal cardiac and non-cardiac factors were recorded from the patient record and maternal factors from the national maternity dataset. The primary endpoint was a prenatal decision for active treatment after birth (intention-to-treat). Factors associated with a delayed diagnosis (≥24 weeks’ gestation) were also reviewed. Secondary endpoints included proceeding to surgical treatment, and 30-day postoperative mortality in liveborns with an intention-to-treat.Setting New Zealand population-wide.Participants Fetuses with a prenatal diagnosis of HLHS between 2006 and 2015.Results Of 105 fetuses, the CCP was intention-to-treat in 43 (41%), and pregnancy termination or comfort care in 62 (59%). Factors associated with intention-to-treat by multivariable analysis included a delay in diagnosis (OR: 7.8, 95% CI: 3.0 to 20.6, p<0.001) and domicile in the maternal fetal medicine (MFM) region with the most widely dispersed population (OR: 5.3, 95% CI: 1.4 to 20.3, p=0.02). Delay in diagnosis was associated with Māori maternal ethnicity compared with European (OR: 12.9, 95% CI: 3.1 to 54, p<0.001) and greater distance from the MFM centre (OR: 3.1, 95% CI: 1.2 to 8.2, p=0.02). In those with a prenatal intention-to-treat, a decision not to proceed to surgery was associated with maternal ethnicity other than European (p=0.005) and the presence of major non-cardiac anomalies (p=0.01). Thirty-day postoperative mortality occurred in 5/32 (16%) and was more frequent when there were major non-cardiac anomalies (p=0.02).Conclusions Factors associated with the prenatal CCP relate to healthcare access. Anatomic characteristics impact treatment decisions after birth and early postoperative mortality. The association of ethnicity with delayed prenatal diagnosis and postnatal decision-making suggests systemic inequity and requires further investigation.

In this issue of World Journal for Pediatric and Congenital Heart Surgery, Maeda et al. report excellent results in mitral valve repair for regurgitation in 18 consecutive infants operated on at their institution in Hyogo, Japan from 2005 to 2019. There were no early deaths and only 1 late death with a median follow-up of 7 years and 9 months. At both 5 and 10 years, they had survival of 94.4%, freedom from reoperation of 83.0%, freedom from valve replacement of 88.9%, and freedom from recurrent significant mitral regurgitation of 83.3%. These outcomes are better than those recently reported by Geoffrion et al. particularly the reintervention-free survival which in Dallas, Texas was 73.3% and 48.9% at 5 and 10 years. Similarly in the paper by Isaacson et al. comparing infant and child outcomes in Wisconsin, the 5-year survival (death/transplant free) was 78%, freedom from reoperation 72%, and mitral valve disease recurrence 47%, respectively. Of note, in these 2 papers from the United States centers, there were no infants with acquired mitral regurgitation, and congenital mitral stenosis (either mixed or isolated) featured in at least a third of their cohorts. Geoffrion’s paper compared outcomes in those with primary mitral stenosis versus mitral regurgitation and found no difference, although numbers were small. Maeda and associates stipulate in the discussion that their institutional preference is to perform the Norwood procedure in those with significant mitral stenosis presenting in infancy. Hence, those patients do not feature in this Japanese series, so perhaps this selectivity contributes to the better late outcomes. Looking closely at this group of 18 infants, not only are the congenital patients all pure mitral regurgitation, but 10 of the patients have acquired, acute pathology. For many of us working outside of Asia, the condition of acute mitral chordal rupture has never been encountered, but in Japan where it was first described by Mishima et al. in 1996, it is now a well-known entity. Matsumoto et al. in 1999, wrote up a series of 16 children describing how to repair the regurgitant mitral valve by reconstructing artificial chordae tendineae including 1 infant. He included a useful diagram of the technique, with reported outcomes at a median follow-up of 14.8 months showing no valve failures and 100% survival, which are outstanding for both congenital and acquired cases. A nationwide survey in Japan reported by Shiraishi et al. in 2014 documents 95 children having acute rupture of mitral chordae with 85% of them being between 4 and 6 months of age. Although 10 were caused by Kawasaki disease and 2 by maternally derived SSA antibodies, the majority had no formal etiologic diagnosis. In the 28 who had histologic evaluation, there were acute inflammatory reactions in some, and fibrosis or myxoid changes in others. They also report an 8.4% mortality in patients prior to getting to the operating theatre and persistent neurologic complications in 11% of survivors. This explains why Maeda describes these infants being operated on within 24 h of admission, with 9 of the 10 being on mechanical ventilation prior to surgery. The damage to coronary arteries in some patients with Kawasaki disease is well known but many patients also have mild mitral regurgitation (63% in a Japanese survey and 71% in a Chinese survey). It affects other valves too, and tends to resolve over the following year. Macrophages and cytotoxic T cells were reported to have invaded mitral tissue in 1 infant operated on a year after the initial diagnosis. These histopathologic findings, and the link with ethnicity, remind us of rheumatic valve disease, a far more common global cause of acquired mitral regurgitation in children, which is triggered by an immune reaction that activates T cells and is now demonstrated to correlate with certain genes that code for the proteins which mediate the immune responses. Histologic examination of any tissue removed at these acute infant operations may improve our understanding of the causative factors. Apart from the Japanese publications, there appears to be just 1 abstract describing mitral valve repairs in infants in China in 2017 in which 22 patients or 55% were under a year of age and their reported early outcomes were good. Yoshizawa et al. from Hyogo published in the WJPCHS the first paper that selectively reports on a group of infants with acute mitral chordal rupture. There is a detailed description of the typical presentation and the use of polytetrofluoroethylene chordal implantation, edge-to-edge suture, and Kay annuloplasty. They stress the need for prompt transfer to theatre and for 48 h of sedation afterward to avoid hypertension in the early phase postoperatively. These 6 cases, now included in the current paper, have no doubt enabled the surgeons to gain confidence in constructing chords at the correct length, even in these small infant hearts; however, this technique was not applied to their congenital infant cases. The concern that the use of polytetrofluoroethylene chord in an infant may impact the durability of the repair that has been addressed by Sai et al. in their careful echo follow-up of 8 infants aged 1 to 7 months over a mean of 5.8 years. Freedom from reoperation is 100% and any postoperative mitral regurgitation tended to improve with time, with an left ventricular end diastolic dimension that remained normal over 5 years. They measured the length of the associated papillary muscle and showed that it grows in length as expected and correlates with body surface area. Isaacson et al. in his description of the index procedures for congenital mitral repair, does not use chordal insertion in infants and Geoffrion reports only 1 in their 25 infants. This Japanese report demonstrating the safety of polytetrofluoroethylene chordal insertion in infants should encourage us to use this technique in the congenital infants and children we encounter with more confidence in its durability. We can also be prepared, just in case, in this global community we now live in, we one day encounter an infant case of acute acquired mitral regurgitation.

Background Leaflet escape is a rare complication of mechanical heart valves. The following case is the first report of leaflet escape from an On-X mechanical mitral valve in a child. Case summary A 12-year-old boy presented with acute pulmonary oedema one year after mitral valve replacement with an On-X valve for rheumatic heart disease. Transoesophageal echocardiogram diagnosed an absent mechanical mitral valve leaflet and severe mitral regurgitation for which the patient successfully underwent urgent mitral valve replacement. Computed tomography located fragments of the embolised mitral valve leaflet at the bifurcation of the abdominal aorta and within the right common iliac artery. In the absence of impaired lower limb perfusion, the leaflet fragments were left in situ. He was discharged without complication and remains asymptomatic three months post-discharge. Discussion Leaflet fracture and escape is a known but rare complication of mechanical valve prostheses. The first-line investigation for suspected mechanical valve dysfunction is transoesophageal echocardiogram which elicited the diagnosis on this occasion.

Objective: A small percentage of infants with d-loop transposition of the great ar-teries with intact intraventricular septum have life-threatening refractory hypox-emia often due to coexistent persistent pulmonary hypertension of the newborn. In this case series we describe the outcomes of a "rescue" emergency arterial switch operation (ASO).Methods: We undertook a retrospective medical record analysis of infants with d-loop transposition of the great arteries with intact intraventricular septum who underwent an ASO in New Zealand from January 1, 1996, to April 30, 2017. Data were compared for those who received an emergency ASO and those with a nonemergency ASO for descriptive purposes. An emergency ASO was defined as one that was undertaken for life-threatening refractory hypoxemia when the only alternative stabilization strategy was preoperative extracorporeal life support. Pri-mary outcome measures were 30-day postoperative mortality and abnormal neuro-developmental outcome in the survivors. Secondary outcomes were low cardiac output, arrhythmia, renal dysfunction, postoperative seizures, and length of stay. Other known risk factors for morbidity and mortality were also assessed.Results: Two hundred seventy-two infants underwent an ASO with 25 (9%) who received an emergency ASO. No infants received preoperative extracorporeal life support. The emergency group had greater 30-day postoperative mortality (8.0% vs 0.4%; P = .01) with no difference in abnormal neurodevelopmental outcome among the survivors (17.4% vs 13.8%; P = .35). The emergency group had more therapies for low cardiac output syndrome, more postoperative seizures, and a longer length of stay.Conclusions: An emergency ASO is a definitive rescue therapy that can be under-taken with acceptable mortality and neurodevelopmental outcome with consider-ation of the preoperative clinical state. (J Thorac Cardiovasc Surg 2023;166:183-90)

BACKGROUND:There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary.METHODS:All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle.RESULTS:A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days (P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival.CONCLUSION:Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

The vast majority of New Zealanders who were diagnosed with significant congenital heart disease (CHD) from the 1950s found themselves sent to Green Lane Hospital for evaluation and potentially surgery. The Cardiothoracic centre at Green Lane in Auckland grew up adjacent to a large park around the base of Maungakiekie/One Tree Hill, one of Auckland's many volcanic maunga or cones. Since 1960, largely through immigration, New Zealand's population has doubled to a heady 5 million. The majority of our patients however are still those who were originally evaluated and operated here. We are fortunate to have access to their original documents, describing in detail symptoms and signs at presentation and thereafter how a diagnosis was determined, often with earlier ingenious techniques. The surgical reports detail the operative findings and techniques of repair and (hopefully) thereafter discharge letter and clinic reports describe gradual recovery and an unfolding life.Overall survival from earlier years has been excellent and as current clinicians we are aware each day of the legacy we have inherited from pioneering colleagues, patients and families. This paper reflects on the past of Green Lane Hospital and the experiences of staff and patients in those earlier days and how these influence our Service in the present. We recognise that understanding this history will better enable (and inspire) us to improve the care we deliver to new arrivals and to the cohorts surviving from earlier eras, many now well into middle age and beyond.

A significant percentage of rheumatic heart disease (RHD) patients eventually require surgery, mainly in young patients, sometimes affecting children less than 10 years old. Those with severe functional valvular changes and dilated ventricles have progressive cardiac deterioration. Timely and appropriately performed surgery and perioperative care will be required. Indications and techniques for surgery of aortic, mitral, and tricuspid disease are discussed. Techniques of mitral valve repair have evolved significantly, and results are now much better; hence, comparison with replacement is favorable. With the exception of percutaneous treatment mitral stenosis, new technologies are, yet, not generally applicable in rheumatic disease. Further developments are expected. Cardiac surgery needs complex infrastructures and skills. In a significant number of low-middle income countries, especially in Africa, these facilities are not available and, where available, they are usually incapable to cope with the very large demand.

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

Objective To establish the impact that timing of diagnosis and place of birth have on neonatal outcomes in those with readily treatable critical congenital heart disease. Study design This was a population-based study with a complete national cohort of live-born infants with transposition of the great arteries and aortic arch obstruction in New Zealand between 2006 and 2014. Timing of diagnosis, place of birth, survival to surgery, in-hospital events, and neonatal mortality were reviewed. Live births with a gestation of >= 35 weeks and without associated major extracardiac anomalies were included for analysis. Results A total of 166 live-born infants with transposition of the great arteries and 87 with aortic arch obstruction were included. Antenatal detection increased from 32% in the first 3 years to 47% in the last 3 years (P = .05). During the same period, neonatal mortality decreased from 9% to 1% (P = .02). No deaths occurred after surgical intervention. An antenatal diagnosis was associated with decreased mortality (1/97 [1%] vs 11/156 [7%]; P = .03) and birth outside the surgical center was associated with increased risk of mortality (11/147 [7%] vs 1/106 [1%]: P = .02). Those with an antenatal diagnosis required fewer hours of mechanical ventilation (P = .02) and had shorter durations of hospital stay (P = .05) compared with those diagnosed >48 hours after birth. Conclusions The mortality risk for transposition of the great arteries and critical aortic arch obstruction is greatest before cardiac surgery. Improved antenatal detection allowing delivery at a surgical center is associated with reduced mortality.

The natural history of a systemic right ventricle after an atrial switch procedure has yet to be fully characterised. We describe the case of the longest surviving patient at our institution who underwent a Mustard Baffle correction for dextro-transposition of great arteries in childhood. Over following decades he was reviewed regularly with deteriorating systemic right ventricle function. At around 50 years of age he developed worsening heart failure on maximal medical therapy. He was subsequently assessed for cardiac transplantation which he underwent successfully at the age of 55 years.