World journal for pediatric & congenital heart surgery最新文献

Objective: Tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months. Thus, we sought to evaluate surgical outcomes according to age at repair.

Methods: We performed a retrospective review of patients who underwent unifocalization from age 3 to 12 months. These patients had not undergone prior surgery at our institution or elsewhere and were also not protocoled into early surgery. Patients were divided in the following groups: 3.0 to 4.9 (n = 61), 5.0 to 5.9 (n = 56), 6.0 to 7.9 (n = 56), and 8.0 to 11.9 (n = 47) months. Competing risk regression analyses were performed.

Results: We included 220 patients from 2001 to 2020. Baseline characteristics were not significantly different among the four groups. Overall, single-stage complete repair with bilateral unifocalization, ventricular septal defect closure, atrial septal defect closure, and right ventricular-pulmonary artery (PA) conduit placement was achieved at the index operation in 174 (79%) patients and did not differ across age groups. Early mortality was 4% (n = 9). At one year, 91% (200/220) of the entire cohort was fully septated. Comparing with group 1, group 4 was significantly less likely to undergo any PA reinterventions [hazard ratio (HR) 0.44, 95% CI 0.21-0.92, P = .028] or surgical PA reinterventions [HR 0.12, 95% CI 0.02-0.95, P = .044] following complete repair.

Conclusions: Given the excellent outcomes across all ages, surgical timing for single-stage unifocalization should be dictated by clinical and anatomic details, with potential advantage in select clinically appropriate older infants who appear to be at a lower probability of PA reinterventions following full septation.

Objective: The authors sought to evaluate the role of nutritional indices such as Onodera's prognostic nutrition index (PNI), World Health Organization (WHO)-based anthropometric measurements such as weight for age (w/a), height for age, weight for height, and perioperative serum albumin levels in the determination of postoperative clinical outcomes in pediatric patients who undergo surgery for congenital cardiac defects and surgical complexity (risk-adjusted congenital heart surgery score) and its correlation with postoperative course.

Material and methods: In this prospective observational study, 108 post-pediatric cardiac surgery patients under the age of 18 months were enrolled between January 2023 and August 2023. Through receiver operating characteristic curve analysis we have found the cutoff value for PNI is ≤66.5 and >66.5. The above mentioned parameters were analyzed for postoperative clinical outcomes such as length of intensive care unit (ICU) stay, length of hospital stay, and duration of mechanical ventilation.

Results: Significant negative correlation was found between length of ICU stay and hospital stay with lower PNI (P = .019 and <.001, respectively.). Analysis of low versus high PNI groups was suggestive of a remarkable increase in mechanical ventilation time (P = .03), length of ICU stay (0.01), and hospital stay (P ≤ .001) in the low PNI group. Lower WHO-based w/a Z score was found to be significantly associated with low PNI (<66.5), after adjusting for preoperative albumin, postoperative albumin drop, and C-reactive protein (odds ratio = 1.411 per unit 0.28 increment in W/azs, P = .004).

Conclusion: Preoperative Onoderas PNI is an effective and efficient tool for predicting postoperative clinical morbidity in pediatric patients undergoing congenital heart surgery.

Objective: The aim of this study was to assess the short- and long-term outcomes of patients who underwent the arterial switch operation (ASO) at Siriraj Hospital in Thailand, and to identify postoperative complications and factors that significantly affect patient survival.

Materials and methods: We retrospectively studied all patients with dextro-transposition of the great arteries and anatomic variants who underwent the ASO from January 1995 to December 2020. Twenty-year overall survival and 15-year freedom from reoperation/reintervention were estimated using the Kaplan-Meier method. Univariate and multivariate Cox regression analyses were used to identify factors independently associated with 30-day mortality.

Results: Of the 171 patients included, there were 116 males (67.84%). The median (minimum, maximum) age at operation was 33.5 (3-4,499) days, and the median (minimum, maximum) bodyweight was 3.4 (2.2-44.9) kg. Most patients (115/171, 67.25%) had an intact ventricular septum, whereas 48/171 (28.07%), had ventricular septal defects. The typical coronary anatomic pattern (1LCx-2R) was found in 120 patients (70.1%). Early mortality was 8.7% ± 4.4%, and there was no late death up to 20 years postoperatively with a median follow-up of five years. Fifteen-year freedom from reoperation or reintervention was 85.7%. Atypical coronary pattern was an independent factor for survival with an adjusted hazard ratio of 5.1 (95% confidence interval: 1.22-21.4; P = .026).

Conclusions: The results of this study revealed excellent short- and long-term outcomes of the ASO at our center. Atypical coronary anatomy was found to be the only factor that independently predicted 30-day mortality.

The authors present an exceptionally rare case of a newborn in whom total anomalous pulmonary venous return (TAPVR) had been diagnosed prenatally and in whom genetic testing after neonatal cardiac repair confirmed a missense variant of the FBN1 gene consistent with Marfan syndrome as well as a PRKDC gene mutation associated with severe combined immunodeficiency. To the authors' knowledge, this is the first reported case with this unique combination. Neonatal TAPVR repair was undertaken with a good postoperative outcome and survival until the last follow-up.

Background: Survival beyond one month of age is rare in children born with obstructed infracardiac total anomalous pulmonary venous connection (TAPVC). There are limited data available on surgical outcomes of the same subset. We conducted this retrospective study to identify risk factors associated with surgical outcomes in this patient population.

Method: We present a retrospective study of patients presenting at our institute with obstructed infracardiac TAPVC surviving beyond one month of age during the period from January 2015 to August 2019 (n = 14). Data reviewed included demographic parameters, preoperative laboratory results, echocardiogram images, and computed tomography if performed. Intraoperative as well as postoperative data including type of surgery, use of deep hypothermic arrest, mortality, and morbidity were noted. Follow-up clinical data were also collected from institutional record or telephonically. We have also collected echocardiographic data on follow up.

Results: The mean age of our study cohort was 2.14 ± 0.68 months. Out of 14 total patients 3 patients eventually died. Risk factors that were significantly associated with mortality were low weight (odds ratio [OR] = 7.8, P = .005), preoperative bilirubin level (OR = 7.4, P = .007), use of deep hypothermic circulatory arrest (OR = 4.6, P = .031), tricuspid regurgitation grade (OR = 3.8, P = .050), and pulmonary artery hypertension (OR = 10.5, P = .001). Mean follow up for the study cohort was 2189 ± 538 days. One patient has mild flow acceleration across the anastomosis with a gradient of 5/3 mm Hg without any symptoms. The remainder of the patients are alive with excellent clinical as well as echocardiographic outcome.

Conclusion: The presentation of infants with obstructed infracardiac TAPVC beyond one month of age is very rare. However, timely intervention can provide acceptable early and excellent late outcomes in this rare patient cohort.

Leadership in pediatric and congenital heart surgery requires a distinctive blend of clinical mastery, scientific innovation, and an unwavering commitment to education and mentorship. The individuals who rise to the top of this demanding field exhibit not only surgical prowess but also the ability to foster progress through impactful research, publications, and training the next generation of surgeons. These qualities are exemplified by the six past presidents of the World Society for Pediatric and Congenital Heart Surgery (WSPCHS), whose leadership has been instrumental in advancing both the Society and the broader field of pediatric cardiac care. This article pays tribute to these visionary leaders, highlighting their contributions to the Society and their global impact on congenital heart surgery. Their leadership has profoundly influenced the direction of the WSPCHS, contributing to its success and shaping the future of the specialty.

Background: Double outlet right ventricle (DORV) is a challenging congenital cardiac lesion to surgically master. We utilize computed tomography-guided-three-dimensional (3D) modeling/printing and novel in-house software to delineate anatomical relationships providing operative insight into the surgical approach. Our intent is to highlight this and showcase our technology.

Methods: We have created a repository of 3D heart reconstructions allowing for review of DORV patients. 3D intraoperative software anatomical manipulation and physical 3D prints were used to gain insight into DORV anatomy with the assistance of an on-site 3D Lab. The software used (Arc 3D Model Viewer) was designed in-house by our 3D Lab, tested and refined through ongoing use by our cardiothoracic surgery team. It allows for the subtraction and addition of anatomical structures and rotation in all axes. Clinicians can pan into the heart and determine specific anatomical boundaries and relationships.

Results: From 2010 to 2023, our program operated on 71 patients with DORV and our 3D lab has reconstructed 29 3D-hearts. Reconstructions were analyzed using Arc 3D Model Viewer. 3D reconstructions were viewed in our care conferences and intraoperatively allowing for discussion and determination of the optimal operative approach. Overall survival for DORV patients was 96% (68/71) with two mortalities in patients who did not receive 3D reconstructions.

Conclusion: 3D reconstruction has allowed decision-making to be moved out of the operating room into the preoperative planning phase. 3D reconstruction is now standard for all DORV patients in our surgical service. We hope to demonstrate this technology with our newly developed Arc 3D Model Viewer and summarize our clinical results.

Background: Impella 5.5 ventricular assist device (VAD) insertion is typically done via the axillary artery or directly through the aorta; however, an axillary artery must be ≥6 mm in diameter, which excludes many pediatric patients who do not meet this criterion. The innominate artery is a larger vessel that can better accommodate the Impella VAD in pediatric patients. Outcomes of this technique were compared with other pediatric patients undergoing the standard axillary artery cannulation.

Methods: The Heart Center at Le Bonheur Children's Hospital recently adopted the trans-innominate cervical approach for insertion of the Impella 5.5 VAD. We performed a retrospective cohort study comparing these cases to data collected from cases using an axillary approach found in the Advanced Cardiac Therapies Improving Outcomes Network database. Comparisons between preoperative characteristics, postoperative course, adverse events, anticoagulation, and clinical outcomes for these two groups were analyzed.

Results: Thirty-nine patients were implanted via the axillary approach; seven patients were implanted via the transinnominate cervical approach at our institution. Patients inserted via the trans-innominate approach had fewer days on the device (median, 8 [range, 3-24]), postimplant intensive care unit days (16 [3-24]), and hospital length of stay (14 [3-28]) compared with axillary insertion patients (12.5 [6.75-29.2], 58.5 [12.5-43.5], and 32 [19.7-83.5], respectively). Trans-innominate patients had fewer adverse events than axillary patients.

Conclusions: Trans-innominate cervical approach for insertion of the Impella 5.5 VAD is safe with low rates of adverse events in pediatric patients. As miniaturization of VAD therapy continues to evolve, this technique may provide greater application of these devices for even smaller children.

Transposition of the great arteries (TGA) with a rare form of ventricular septal defect (VSD) needs careful attention when choosing the optimal strategy for repair. A neonate with TGA and an atypical VSD, which extended from the perimembranous to the juxta-arterial regions, underwent the arterial switch procedure. The large VSD was successfully closed via a trans-tricuspid combined with a trans-aortic valve approach concomitantly with the arterial switch maneuver. Surgeons need to be aware aware that the types of VSD are diverse in TGA.