World journal for pediatric & congenital heart surgery最新文献

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: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.

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.

Aortic dilation following single ventricle palliation is increasingly recognized, although its long-term clinical implications remain unclear. We present the case of a 38-year-old male born with a bicuspid aortic valve, d-transposition of the great arteries, ventricular septal defect, right ventricular hypoplasia with a straddling right atrioventricular valve. He was palliated with Fontan circulation and developed severe neo-aortic root and arch dilation with native aortic valve stenosis, requiring surgical intervention. His neo-aortic root measured 81 × 65 mm, necessitating a Bentall procedure with total arch and native aortic valve replacement. This case underscores the variable progression of aortic dilation in patients with palliated single ventricle physiology and the need for strict surveillance and individualized management. Larger cohort studies are needed to further refine the criteria for surgical intervention in this unique population.

Combining the Impella device and extracorporeal membrane oxygenation (ECMO) support has been suggested to counteract the left ventricular (LV) afterload seen with ECMO, prevent complications during venoarterial-ECMO (VA-ECMO), and facilitate weaning from VA-ECMO. While this technique has been successfully utilized in adults, there are limited efficacy data in patients under 18 years of age.Between July 2016 and April 2024, eight pediatric patients, who were 13 to 18 years of age and weighing 40 to 59 kg, underwent VA-ECMO support with Impella 2.5 or CP implantations at our institution. All patients were hemodynamically unstable at presentation (INTERMACS 1).The indications for implantation were heart failure secondary to myocarditis (four patients), rejection of a prior orthotopic heart transplant (one patient), heart failure after repair of transposition of the great arteries (one patient), metabolic cardiomyopathy (one patient), and arrhythmia-induced cardiomyopathy (one patient). The VA-ECMO cannulation was performed via neck vessels in six patients and groin vessels in two. The median duration of Impella support was eight days (range, 2-20 days). Our practice is to wean the patient off ECMO first while ensuring support in the early stages of ECMO removal with the Impella device. The median duration of ECMO support was seven days (range, 4-32 days). One patient was bridged to transplant, one patient received a HeartMate3, five patients recovered myocardial function and were weaned off mechanical support, and one patient died while on support.In adolescent acute heart failure, a short ECMO run with LV unloading provided by the Impella device is a promising strategy that might facilitate rapid recovery of myocardial function in hemodynamically unstable adolescents.

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: 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: 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.