World Journal for Pediatric and Congenital Heart Surgery最新文献

Background: The use of extracorporeal membrane oxygenation (ECMO) in the postoperative cardiac critical care setting is evolving. Anticoagulation monitoring is among the most challenging aspects of pediatrics. However, there is no consensus on the optimal dosing and monitoring of unfractionated heparin in this setting. To address this, we developed an anti-Xa assay-based protocol derived from the best available clinical and anecdotal evidence of ECMO use and assessed its effectiveness in achieving the anti-Xa assay therapeutic target.

Methods: This prospective single-arm study was conducted in the pediatric carcardiac-surgery intensive care unit of a large tertiary hospital. We used two different anti-Xa assay intensity levels based on the patients' bleeding status.

Results: The median patient age was 7 (interquartile range [IQR]: 5-11.25) months, and the median weight was 5.7 (IQR: 3.8-13.82) kg. The median ECMO duration was 6 (IQR: 4.5-7.5) days. The bleeding protocol was used for most patients. Seventy percent achieved the anti-Xa assay therapeutic target during the study period (median: 75.5 h, IQR: 60.5-117.5 h). Hemorrhagic complications were reported in 40% of the patients, and thrombotic complications were reported in 25%. The median length of stay was 37 (IQR: 22-43) days, with a survival-to-discharge rate of 75%.

Conclusions: Despite a failure to achieve the anti-Xa assay target within the first ECMO days, most patients achieved the target by the median ECMO duration. Moreover, using two different anti-Xa assay levels reduced thrombotic complications.

Background: Mortality associated with the correction of congenital heart disease has decreased to approximately 2% in developed countries and major adverse events are uncommon. Outcomes in developing countries are less well defined. The World Database for Pediatric and Congenital Heart Surgery was utilized to compare mortality and adverse events in developed and developing countries.

Methods: A total of 16,040 primary procedures were identified over a two-year period. Centers that submitted procedures were dichotomized to low/middle income (LMI) and high income (HI) by the Gross National Income per capita categorization. Mortality was defined as any death following the primary procedure to discharge or 90 days inpatient. Multiple logistic regression models were utilized to identify independent predictors of mortality.

Results: Of the total number of procedures analyzed, 83% (n  =  13,294) were from LMI centers. Among all centers, the mean age at operation was 2.2 years, with 36% (n  =  5,743) less than six months; 85% (n  =  11,307) of procedures were STAT I/II for LMI centers compared with 77% (n = 2127) for HI centers (P < .0001). Overall mortality across the cohort was 2.27%. There was a statistical difference in mortality between HI centers (0.55%) versus LMI centers (2.64%) (P < .0001). After adjustment for other risk factors, the risk of death remained significantly higher in LMI centers (odds ratio: 2.36, 95% confidence interval: 1.707-3.27).

Conclusion: Although surgical expertise has increased across the globe, there remains a disparity with some outcomes associated with the correction of congenital heart disease between developing and developed countries. Further studies are needed to identify specific opportunities for improvement.

We describe the hybrid Norwood as first-stage palliation for a patient with hypoplastic left heart syndrome, right aortic arch, right descending aorta, bilateral ductus arteriosus, and left innominate artery arising from the left ductus.

A previously healthy nine-year-old boy with anomalous aortic origin of the left coronary artery (AAOLCA) with high-risk anatomy demonstrated negative stress on magnetic resonance imaging. Invasive cardiac catheterization for intracoronary flow measurement was performed and demonstrated compromised coronary flow during pharmacologic stress and significant stenosis on angiography. The patient underwent surgical intervention with normalization of coronary flow upon postoperative evaluation. Invasive intracoronary flow determination with angiography under provocative stress is emerging as a critical data point for risk stratification and management decision-making in high-risk AAOLCA patients with negative noninvasive perfusion studies.

Objective: The surgical treatment of malformed semilunar valves in congenital heart defects is challenging in terms of providing both longevity and the potential to grow with the recipient. We investigated a new surgical technique "Trileaflet Semilunar Valve Reconstruction" in an acute porcine model, a technique with geometrical properties that could remain sufficient and allow for some growth with the child. Methods: An acute 60-kg porcine model was used. With echocardiography, baseline pulmonary valvular geometry and hemodynamics were investigated. On cardiopulmonary bypass, the pulmonary leaflets were explanted, and the Trileaflet Semilunar Valve Reconstruction was performed with customized homograft-treated pericardial neo-leaflets. Off bypass, hemodynamics was reassessed. Results: Twelve animals were investigated. The neo-valves were found sufficient in ten animals and with minimal regurgitation in two animals. The neo-valve had a peak gradient of 3 ± 2 mm Hg with a peak velocity of 0.8 ± 0.2 m/s. The coaptation in the neo-valve had a mean increase of 4 ± 3 mm, P < .001. The neo-valve had a windmill shape in the echocardiographic short-axis view, and the neo-leaflets billowed at the annular plane in the long-axis view. Conclusions: In this acute porcine model, the neo-valve had no clinically significant regurgitation or stenosis. The neo-valve had an increased coaptation, a windmill shape, and leaflets that billowed at the annular plane. These geometric findings may allow for sustained sufficiency as the annular and pulmonary artery dimension increase with the child's growth. Further long-term studies should be performed to evaluate the efficacy and the growth potential.

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Congenitally corrected transposition of great arteries is an uncommon congenital heart disease characterized by discordance at both the atrioventricular and ventriculoarterial connections. A rare subgroup of patients with congenitally corrected transposition of the great arteries also has a criss-cross heart. The morphology of these patients represents a diagnostic challenge that requires critical analysis to perform a satisfactory surgical procedure. We present a case of a 12-year-old patient with the above mentioned anatomy who underwent physiologic repair.

Background: Left (LAAA) and right atrial appendage aneurysms (RAAA) are rare entities, and their natural history, management, and long-term outcomes are not well studied.

Methods: This retrospective review includes all patients from 2000 to 2021 with atrial appendage aneurysms at our institution identified using an electronic search tool. LAAA and RAAA were confirmed using multimodality imaging and intraoperative findings.

Results: We identified 13 (87%) patients with LAAA and 2 (13%) with RAAA. At diagnosis, 11 (73%) were female, mean age was 51.4  ±  18.8 years, and ejection fraction 56.5  ±  13.1%. Three (20%) patients had congenital heart disease including 2 (13%) with atrioventricular septal defects and 1 (7%) with congenitally corrected transposition. LAAA/RAAA was diagnosed due to new-onset atrial fibrillation (AF) in 6 (40%) patients and embolic stroke in 2 (13%). Ten patients had preexisting AF diagnosed 2.9  ±  1.4 years earlier at a mean age of 50.2  ±  15.5 years. Thrombus within the aneurysm was identified in 2 (15%) LAAA patients. All patients were on anticoagulation and follow-up of the cohort from diagnosis was 7.1  ±  6.2 years. Eleven (73%) patients were surgically managed with 7 (64%) lesions excised, 1 (9%) stapled, and 3 (27%) ligated. Postoperative complications occurred in 2 (18%) patients with 1 (7%) developing tricuspid regurgitation and another with pericardial effusion and tamponade.

Conclusions: Atrial appendage aneurysm is a rare disease and nearly half of patients present with AF. Surgical treatment with concomitant AF ablation is a reasonable and safe management option.

Background: Pediatric cardiac surgery is associated with abnormal coagulation, bleeding, and nearly ubiquitous transfusions. With the popularization of patient blood management, attempts are being made to decrease liberal transfusions by administering prothrombin complex concentrates (PCCs). The safety and efficacy of PCCs in adult cardiac surgery has been studied extensively, but only few reports address this in children. We performed an observational study focused on transfusion requirements after off-label use of activated PCC Factor Eight Inhibitor Bypassing Activity (FEIBA) as an adjunct to post-cardiopulmonary bypass (CPB) hemostatic protocol.

Methods: We reviewed the medical records of children ≤15 kg undergoing cardiac operations with CPB between May 2018 and March 2022. A propensity score (PS) analysis was performed to identify matched pairs of patients who did and did not receive FEIBA.

Results: Out of 210 patients who met the inclusion criteria, 44 patients received FEIBA. Propensity score-based analysis identified 40 matched pairs of patients with similar baseline characteristics. There was no statistically significant difference in the primary outcome-the volume of transfusion after CPB, which included all allogeneic blood products and salvaged washed red cells administered after protamine. Specifically, FEIBA patients were transfused 28 (22-34) mL/kg and controls were transfused 22 (11-49) mL/kg, P = .989. Upon arrival to ICU, the FEIBA group averaged an 8% lower international normalized ratio, compared with the controls (P = .009) and a 1.08 g/dL higher hemoglobin (P = .050). Neither difference remained significant on POD 1.

Conclusions: In this exploratory study, we found no change in transfusion requirements after CPB despite FEIBA administration.