World Journal for Pediatric and Congenital Heart Surgery

Objectives: Volume overload due to ventricular septal defect (VSD) can cause mitral valve dilation, resulting in mitral regurgitation (MR). We aimed to support the clinical decision-making on mitral valvuloplasty during VSD closure by reviewing patients with VSD and MR. Methods: Of the 1524 patients who underwent VSD closure from 2004-2024, 30 had moderate or worse MR. Among them, 14 underwent VSD closure-alone, while 16 underwent concomitant mitral valve repair. Primary outcome was long-term mitral valve-related events. Secondary outcome was serial valve function changes. The median follow-up period was 6.7 years. Results: The median age and weight at the time of surgery were 5.4 months and 5.6 kg. In the VSD closure-alone group; 13 patients had moderate MR and 1 patient had severe MR preoperatively. At follow-up, 12 patients had mild or less MR, one patient had moderate MR, and the outcome was unknown in one. No reoperations were required. In the VSD closure with mitral valvuloplasty group, 12 patients had moderate MR and 4 patients had severe MR preoperatively. At follow-up, ten patients had mild or less MR; three patients had moderate MR, two patients had severe MR, and the outcome was unknown in one. Three patients required mitral valve replacement. Freedom from reoperation at one, five, and ten years was 96.3%, 87.3%, and 87.3%, respectively. Preoperative MR severity and Carpentier type IIIa were risk factors for the progression of MR and reoperation. Conclusions: Ventricular septal defect closure-alone can reduce functional MR in most cases, but patients with structural MR are at higher risk for progressive MR and may eventually require mitral valve replacement. Decision-making should consider preoperative MR severity and structural valve abnormalities.

Background: The World Database for Pediatric and Congenital Heart Surgery (WDPCHS) was utilized to define the incidence of one-year mortality and identify associated risk factors following surgery for congenital heart disease. Methods: The WDPCHS was queried from January 2017 to September 2022 for all tier 1 surgeries submitted to the database. Out-of-hospital death was defined as a death between 30 days postoperatively and up to one year. Preoperative risk factors and other demographics were evaluated. Chi-square test was used to evaluate associations, a multivariable logistic regression model was performed and the P value for statistical significance was set at <.05. Results: Among 9764 patients alive at hospital discharge (or 30 days), overall, one-year mortality was 1.47% (n = 144). Mortality was highest in STAT 5, 22.88% (27/118) (P < .0001). Risk factors with a higher incidence of one-year mortality included: non-Trisomy 21 genetic syndromes 6.38% (18/282), more than three preoperative risk factors 12.12% (16/132), more than three postoperative complications 9.81% (37/377) all P < .0001. Highest mortality by procedure was seen with the Norwood procedure 22.88% (27/118) and truncus arteriosus repair 8.97% (7/78), both P < .0001. By multivariable analysis, the risk of one-year mortality remained significantly higher with more than three preoperative risk factors (OR 5.85, 95% CI 3.12-10.96), non-Trisomy 21 chromosomal abnormalities (OR 3.04, 95% CI 1.75-5.29), and STAT 5 cases (OR 28.4, 95% CI 15.02-53.71). Conclusions: Based on these findings, there are certain risk factors (multiple preoperative risk factors, chromosomal abnormalities, STAT 5 category) that warrant targeted increased surveillance to improve one-year outcomes.

Background: This study aimed to compare the long-term outcomes of a modified réparation à l’étage ventriculaire (REV) and the Rastelli repair for ventricular septal defect (VSD) and pulmonary outflow tract obstruction without ventriculoarterial concordance. Methods: The study included 100 consecutive patients who underwent a modified REV (n = 50) or Rastelli repair (n = 50) for transposition of the great arteries, double outlet right ventricle, or double outlet left ventricle with VSD and pulmonary outflow tract obstruction. The mean ages of the patients who underwent the modified REV and Rastelli repair were 2.50 years (range: 0.30-12.48) and 5.91 years (range: 0.36-46.15), respectively (P < .05). Results: Actuarial survival and arrhythmia-free survival rates were 83.4% ± 4.0% (P > .05) and 72.4% ± 5.0%, respectively at 37.25 years (P > .05). The reoperation-free survival was 18.7% ± 4.6% at 31.82 years (P < .05). The freedom from the first, second, third, and fourth reoperations after the modified REV versus the Rastelli repair was 35.9% ± 8.3% at 29.8 years versus 6.7% ± 4.3% at 31.8 years (P < .05); 83.2%±6.3% at 29.8 years versus 21.2% ± 15.6% at 35.8 years (P < .05); 94.1% ± 4.1% at 29.8 years versus 56.1% ± 10.9% at 37.3 years (P < .05); and 97.0% ± 3.0% at 29.8 years versus 74.3% ± 10.2% at 37.3 years (P < .05), respectively. The most common causes of reoperation after the Rastelli repair were pulmonary stenosis, left ventricular outflow tract obstruction, and arrhythmia. However, the most common cause of reoperation after the modified REV was pulmonary regurgitation. Conclusion: Modified REV and Rastelli repair have shown satisfactory results in long-term follow-up. However, the Rastelli repair could not avoid repeated reoperations, especially for biventricular outflow tract obstruction and arrhythmia.

Background: Delayed sternal closure (DSC) is frequently utilized to facilitate the recovery of myocardial function and edema following the Norwood procedure. At our institution, most patients undergo primary sternal closure (PSC), unless specified high-risk characteristics are present. We sought to analyze the outcomes of our approach. Methods: A retrospective review was performed of patients who underwent the Norwood procedure from 2017 to 2022. Patients were divided into two groups—PSC and DSC. Baseline demographics, clinical characteristics, and perioperative details were compared. The primary outcomes of interest were operative survival and infectious complications. Results: The Norwood procedure was performed in 116 neonates, of whom 80 (68.9%) underwent PSC. Baseline clinical characteristics and echocardiographic findings were not different between groups, except for lower preoperative inotrope use in the PSC group (13/80 [16.3%] vs 15/36 [41.7%], P = .003). Perioperatively, PSC patients were less likely to have multiple bypass runs (3/80 [3.8%] vs 9/36 [25.0%], P < .001) and less postoperative mechanical circulatory support (6/80 [7.5%] vs 12/36 [33.3%], P < .001). Hospital survival was higher (73/80 [[91.3%] vs 24/36 [66.7%], P = .04), and length of stay was shorter (29.0 vs 78.0 days, P < .001) with PSC. Of the 80 patients in the PSC group 5 (6.2%) required reopening of the sternum. By multivariable analysis, DSC was not an independent risk factor for operative mortality (OR 1.42 [95% CI 0.33-6.02], P = .63) or infection (OR 2.22 [95% CI 0.74-6.60], P = .15). Conclusions: Primary sternal closure can be performed in most patients following the Norwood procedure with favorable outcomes. Selected patients with high-risk preoperative or intraoperative features may benefit from DSC. Delayed sternal closure does not independently increase the risk of mortality or infectious complications.

Background: This analysis evaluates the longitudinal impact of extracorporeal membrane oxygenation (ECMO) and ventricular assist device (VAD) on the progression of motor delay and cognitive delay in pediatric heart transplant recipients. Methods: The United Network for Organ Sharing Registry was queried for pediatric patients (<18 years) who received a heart transplant between 2008 and 2022 and were bridged-to-transplantation with either ECMO or VAD. Patients were further stratified based on the progression of delay status pretransplant to post-transplant. Results: A total of 827 patients were included in the final VAD cohort and 68 in the final ECMO cohort with 187/827 (22.6%) and 20/68 (29.4%) having progression of delay, respectively. Patients with progression of delay were more likely than those without progression of delay to have had a stroke prior to discharge (VAD: 14/187 (7.5%) vs. 16/640 (2.5%), P = .003; ECMO: 5/20 (25%) vs. 3/48 (6.25%), P = .043). VAD patients more often developed isolated delays (motor or cognitive only), whereas patients bridged-to-transplantation on ECMO more often had combined delays, with a cumulative incidence of combined delay at 3-years of 20.0% (95% CI = 8.94–34.20). Among VAD supported patients, those with progression of delay had worse 3-year survival compared with those without (91.20% [95% CI = 87.04–95.57%] vs. 94.97% [95% CI = 93.19–96.78], log-rank P = .04). Postoperative stroke was associated with an increased risk of progression of delay (OR = 2.39 [95% CI = 1.13–5.02] P = .021), and progression of delay was found to be an independent predictor of mortality (HR = 2.10 [95% CI = 1.35–3.25], P < .001). Conclusions: While overall rates of neurodevelopmental delay are similar between those bridged-to-transplantation with ECMO or VAD, the type of delay and associated survival varies.

Objective: To analyze in-hospital mortality in children undergoing congenital heart interventions in the only public referral center in Amazonas, North Brazil, between 2014 and 2022. Methods: This retrospective cohort study included 1041 patients undergoing cardiac interventions for congenital heart disease, of whom 135 died during hospitalization. Records were reviewed to obtain demographic, clinical, and surgical data. Descriptive statistics were applied for categorical and continuous variables. Results: In-hospital mortality was 12.96% (135/1041). Most deaths occurred in infants (97/135, 71.8%), and the median survival postsurgery was 1 day. The majority of patients were male (70/135, 51.8%), mixed-race (119/135, 88.2%), and born in Manaus (88/135, 65%). Only 5/135 (3.7%) underwent fetal echocardiography. Extracardiac malformations were present in 76/135 (56.3%), and 123/135 (91%) required mechanical ventilation postoperatively. Blalock-Taussig-Thomas shunt was the most frequent surgery among those who died. The RACHS-1 Category 3 concentrated 51.8% (70/135) of deaths. Temporal trends showed increased mortality after 2016, coinciding with the rise in neonatal and infant surgeries. Surgical complications (81/135, 60%) and hospital-acquired infections (34/135, 25%) were the leading causes of death. Cardiopulmonary bypass was used in 92/135 (68%) of cases, with a mean duration of 84 min. Conclusion: In-hospital mortality following congenital heart surgery in the Brazilian Amazon remains elevated, particularly among infants and in intermediate-risk RACHS-1 categories. These findings suggest that factors beyond surgical complexity—such as late diagnosis, referral delays, and resource limitations—contribute to adverse outcomes. Strengthening early diagnosis, improving perioperative care, and expanding regional surgical capacity are essential to reducing disparities in pediatric cardiac care.

Background: The purpose of this study is to identify 35-year trends in adult congenital heart disease (ACHD) heart transplant volume, transplant centers, patient characteristics, and longitudinal survival up to ten years. Methods: We performed a retrospective review of ACHD patients (≥18 years) who underwent heart transplantation (N = 2,297 transplants) between January 1, 1988, and December 31, 2022, using the United Network for Organ Sharing Database. Trends in transplant volume, transplant centers, patient characteristics, and longitudinal survival were analyzed. To allow for distinct comparisons between contemporary and historical outcomes, two eras were created (Era 1[N = 1,202 transplants]: January 1, 1988-December 31, 2011; Era 2[N = 1,095 transplants]: January 1, 2012-December 31, 2022). Results: Over a 35-year period, 2,297 heart transplants were performed in ACHD patients. During this time, ACHD heart transplant volume steadily increased. In 1988, 26 heart transplants were performed in ACHD patients nationally, while in 2022, 165 heart transplants were performed in ACHD patients nationally. Simultaneously, the number of transplant centers also increased, but at a slower pace. Era 2 transplants were performed in patients with increased rates of pretransplant cerebrovascular disease, inotropic support, and mechanical circulatory support; nevertheless, patients transplanted in Era 2 had improved longitudinal survival through ten years (log-rank P < .001). Conclusions: Our analysis of ACHD patients undergoing cardiac transplantation over 35 years (January 1, 1988-December 31, 2022) demonstrates that in the more recent era, ACHD transplant patients have increased pretransplant rates of cerebrovascular disease, inotropic support, and mechanical circulatory support; nevertheless, patients transplanted in the most recent ten years of our analysis had improved longitudinal posttransplant survival through ten years in comparison with those transplanted earlier.

Objectives: Durability of aortic valve neocuspidization (AVNeo) in children, adolescents, and young adults is a matter of debate. We sought to assess its viability in these age groups and identify the best fields of application. Methods: A retrospective cohort study (07/2016-08/2024) analyzed survival, reoperation rates, and AVNeo function across different age groups and also initial valve disease, surgical complexity, and material used. Results: The study included 49 patients (median age, 13.8, IQR 10.9-16.3 years), 14 (28.6%) children (1-10 years), 31 (63.2%) adolescents (11-18 years), and 4 (8.2%) young adults (19-25 years). Thirteen patients had previous surgeries. Valve leaflets were made from autologous (36/49, 73.5%) or heterologous pericardium (13/49, 26.5%). Forty-three of 49 (87.8%) patients underwent neocuspidization ± simple procedures, while 6/49 (12.2%) had complex procedures. One complex patient died, and 11 patients needed early or late AVNeo replacement. Median follow-up was 37.8 months. Redo neocuspidization was associated with higher reoperation rates (7/12, 58%) compared with first-time procedures (4/36, 11%) (P = .002). Reoperation was more frequent with heterologous pericardium (7/12, 58.3%) than autologous pericardium (4/36, 11.1%) (P < .001). AVNeo function worsened over time, with increases in both peak gradient (P = .005) and indexed vena contracta (P = .014). Preoperative aortic annular diameter inversely correlated with both peak gradient (P < .001) and indexed vena contracta (P = .022) at follow-up. Conclusions: Medium-term AVNeo dysfunction progression had no clear link with patient age, although a small aortic annulus, which is common in children, significantly impacted AVNeo function. Additionally, children had a higher reintervention rate. Therefore, neocuspidization in children should be considered only when no alternatives are available. AVNeo as a reoperation is associated with worse outcomes. Nonautologous pericardium should be avoided.

Background: We aimed to evaluate the clinical performance of the Inspiris Resilia valve in the pulmonic position. Methods: This study includes adult patients with congenital heart disease (CHD) who underwent pulmonary valve replacement (PVR) using the Inspiris Resilia or Carpentier-Edwards (CE) valve at Kyushu University Hospital from 2004 to 2023, as well as adult patients who underwent aortic valve replacement (AVR) using the Inspiris Resilia valve from 2018 to 2023. We compared the outcomes of the Inspiris Resilia and CE valves at the pulmonic position, and the outcomes of the Inspiris Resilia valves in the pulmonic and aortic position. The primary endpoint was freedom from mild valve regurgitation. Results: There was no significant difference in the freedom from at least moderate pulmonary regurgitation (PR) or stenosis between the Inspiris Resilia and CE valves (P = .65). However, the progression to mild PR in the Inspiris Resilia valve occurred significantly earlier (P = .03), particularly when implanted in the native right ventricular outflow tract (RVOT) (P = .01). The freedom from at least mild aortic regurgitation following AVR with or without using a composite valve graft showed no significant difference (P = .92). Conclusions: This study showed good results for PVR with the Inspiris Resilia valve in adult patients with CHD, but also early onset of mild PR after PVR raising concerns about the long-term durability of the Inspiris Resilia valve, especially when it is implanted in the native RVOT. We recommend that patients who have undergone PVR with the Inspiris Resilia valve in the native RVOT be closely monitored.

Background:Tetralogy of Fallot (TOF) with absent pulmonary valve (PV) accounts for 3% to 6% of TOF cases. It is physiologically distinct due to tracheobronchial compression caused by dilatation of the pulmonary arteries. We reviewed the surgical outcomes of TOF with absent PV at our center using various strategies. Methods: A retrospective analysis was conducted on all patients diagnosed with TOF with absent PV who underwent surgery from May 2018 to December 2024. Data on demographics, surgical techniques, in-hospital mortality, ventilation duration, intensive care unit (ICU) and hospital stay, and early follow-up outcomes were collected. The surgical technique was individualized to the patient based on the severity of the clinical presentation, age, and radiologic evidence of tracheobronchial compression. Results:Twenty-eight patients were included (median age: 9 months; median weight: 5 kg); six (21.4%) were neonates. Branch pulmonary artery reduction was performed in 75% (21/28) of cases. Surgical approaches included valveless transannular patch (5/28, 17.8%), 0.1 mm polytetrafluoroethylene (PTFE) bicuspid valve with transannular patch (12/28, 42.8%), PTFE bicuspid valve with LeCompte's maneuver (6/28, 21.4%), and valved conduit repair (5/28, 17.8%). One patient (3.6%) experienced in-hospital mortality. Median mechanical ventilation time was 11 h (range: 4-82 h) with neonates requiring prolonged ventilation (>72 h, median 74.5 h). Median ICU stay was 24 h, and median hospital stay was six days. One patient (3.5%) required reintervention during follow-up. Conclusion:Tailoring surgical therapy to each patient's clinical presentation and anatomy yields reasonable outcomes in TOF with absent PV. Early presentation and severe airway compression needs an aggressive surgical strategy. Neonates demonstrated a need for prolonged ventilatory support.

Background: Neonatal congenital heart surgery (CHS) patients may have coexisting gastrointestinal disorders and/or complications that may require abdominal surgery. We sought to identify the outcomes of these patients. Methods: The Pediatric Health Information System database was queried to identify neonatal CHS patients. Patients undergoing bowel, hepatobiliary, or gastric operations during their neonatal CHS hospitalization were categorized as the abdominal surgery group and compared with nonabdominal surgery neonatal CHS. Results: A total of 12 437 neonatal CHS patients were included, with 389 (3%) undergoing abdominal surgery during the same hospitalization. Within the abdominal surgery group, 78% (303/389) underwent abdominal surgery after, 19% (74/389) before, and 3% (12/389) both before and after neonatal CHS. The abdominal surgery group was more likely to be preterm (21% [82/389] vs 12% [1446/12 048]), have complex lesions (hypoplastic left heart syndrome: 27.3% [106/389] vs 22.9% [2769/12 048]), and were more likely to develop necrotizing enterocolitis (20.5% [80/389] vs 4% [479/12 048]), all P < .05. In-hospital mortality was significantly higher in the abdominal surgery group (22% [87/389] vs 8% [967/12 048], P < .001). After adjusting for confounders, abdominal surgery was associated with more than 3-fold increase in mortality (OR: 3.34, 95% CI: 2.33-4.78). Odds of mortality remained elevated regardless of whether abdominal surgery occurred before or after neonatal CHS (P < .05). Hospitalization costs were more than doubled in the abdominal surgery group ($401 201 vs $182 036, P < .001). Among survivors of neonatal CHS hospitalization, the abdominal surgery group continued to exhibit lower survival rates at one (94% vs 97%) and five years (85% vs 94%), log-rank P < .001. Conclusion: Patients undergoing abdominal operations during their neonatal cardiac surgery hospitalization represent a highly vulnerable cohort and have more than 3-fold higher odds of mortality.

Objectives: This study aimed to report the incidence of cardiac arrest and in-hospital mortality after pediatric congenital heart surgery in a middle-income country. Methods: This retrospective cohort study was conducted in Thailand. Patients <18 years of age who underwent congenital heart surgery between 2014 and 2019 and experienced cardiac arrest following surgery during the same hospital stay were included. We examined the characteristics of patients who experienced cardiac arrest and its management to determine the mortality-associated factors following cardiac arrest. Results: Overall, 116 cases of cardiac arrest following 1,928 congenital heart surgery operations were included, which resulted in 93/116 (80%) deaths. The incidence of cardiac arrest and in-hospital mortality per 100 patients (95% confidence interval) were 6.0% [116/1,928 (5.0%-7.2%)] and 4.8% [93/1,928 (4.0%-5.9%)], respectively. The incidence of cardiac arrest was higher in neonates (33.6%, 47/140), high-risk STAT 5 surgery (54.8%, 23/43), and emergent/urgent surgery (25.4%, 81/319). Most cardiac arrests occurred within 24 h (66/116, 57%) and in the intensive care unit (90/116, 78%). The most common cause of cardiac arrest was cardiovascular-related (74.1%, 86/116). Multivariable analysis showed the factors associated with mortality (adjusted odds ratio, [95% confidence interval]) included cardiac arrest after 72 h (5.594 [1.073-29.167]), multiple cardiac arrests (10.231 [1.884-55.566]), and every minute increase in cardiopulmonary resuscitation (1.027 [1.005-1.048]). Conclusions: Congenital heart surgery at our middle income cardiac surgical center was associated with relatively high incidence rates of cardiac arrest and in-hospital mortality, and a very high mortality rate following cardiac arrest. The mortality-associated factors after cardiac arrest were cardiac arrest after 72 h, multiple cardiac arrests, and longer duration of cardiopulmonary resuscitation.

Background: In adult patients with transposition of the great arteries (TGA) and systemic right ventricle (sRV), the use of ventricular assist devices (VADs) is uncommon. Methods: We conducted a systematic review of published studies to examine the indications, hemodynamic effects, and outcomes of VADs in this patient population. We reviewed English-language literature for case reports, case series, and reviews that included individual patient data, such as demographics, hemodynamic parameters, types of implanted VADs, and outcomes. Results: We identified 107 patients, 76% (81/107) males, mean age 40.2 ± 10.6 at the time of implantation, 38.3% (41/107) with left TGA (L-TGA), and 61.7% (66/107) with dextro-TGA (D-TGA). The VAD support resulted in hemodynamic improvement, including a decrease in the mean pulmonary arterial pressure (45 ± 15 mm Hg before implantation to 25 ± 9.3 mm Hg afterwards, P < .001), pulmonary vascular resistance (6.3 ± 4.9 Wood units (WU) to 2.4 ± 1.35 WU, P < .001), right atrial pressure (16.4 ± 7.3 mm Hg to 9.5 ± 3.7 mm Hg, P = .009), and pulmonary capillary wedge pressure (25.45 ± 7.12 mm Hg to 14.25 ± 5.6 mm Hg, P < .001). The cardiac index increased from 2.0 ± 0.5 L/min/m² to 2.8 ± 0.6 L/min/m² (P = .004). The 1-year survival rate was 80.5%. Eventually, 31 (29%) underwent heart transplantation, and 48 (54%) remained on VAD at the time of publication. Conclusions: Durable VADs provide hemodynamic improvement and excellent survival in adults with a systemic right ventricle. Expanding the use of VADs for this patient population would be justified.

Pulmonary atresia with intact ventricular septum (PA-IVS) and myocardial dysfunction is a challenging entity to manage. Presented is a patient with PA-IVS who developed myocardial dysfunction and heart failure following an episode of periprocedural hypotension. He was successfully treated with a single ventricle assist device (SVAD) placed without cardiopulmonary bypass and a ductal stent as a bridge to donation after circulatory death heart transplant. This report describes the technique of SVAD placement without cardiopulmonary bypass.

Coral reef aorta (CRA) is a rare, calcified aortic lesion observed primarily in adults and elderly patients. This condition can lead to resistant hypertension and impaired organ perfusion. Although surgical treatment remains the predominant approach, endovascular intervention is emerging as an alternative for suitable cases. We present a 16-year-old male patient with a history of kidney transplantation who was admitted due to hypertension. Imaging revealed severe stenosis at the isthmus level caused by a calcific lesion characteristic of CRA. Given the complexity of the lesion and the surgical challenges, an endovascular approach was chosen. A covered stent was successfully implanted, resulting in significant hemodynamic improvement. At the sixth month follow-up, the patient remained normotensive, and antihypertensive medications were discontinued. To our knowledge, this case represents the first pediatric patient reported to have undergone endovascular treatment for severe aortic coarctation associated with CRA. Our findings suggest that endovascular intervention using a covered stent may provide a viable alternative to surgery in selected pediatric cases, minimizing morbidity and mortality. Further studies are needed to assess the long-term outcomes of this approach.