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Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure. Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs. [ABSTRACT FROM AUTHOR]

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm 2 /BSAm 2 ) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Coarctation of the aorta is a form of congenital heart disease requiring surgical intervention. If is often associated with other forms of congenital heart disease. Anomalous origin of the left coronary artery (ALCA) from the opposite sinus of Valsalva is implicated in sudden cardiac death. We report the first case in the literature of coarctation of the aorta associated with ALCA from the opposite aortic sinus and describe surgical correction of these two lesions., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The most common modes of medical education for congenital heart disease (CHD) rely heavily on 2-dimensional imaging. Three-dimensional (3D) printing technology allows for the creation of physical cardiac models that can be used for teaching trainees. 3D printed cardiac models were created for the following lesions: pulmonic stenosis, atrial septal defect, tetralogy of Fallot, d-transposition of the great arteries, coarctation of the aorta, and hypoplastic left heart syndrome. Medical students participated in a workshop consisting of different teaching stations. At the 3D printed station, students completed a pre- and post-intervention survey assessing their knowledge of each cardiac lesion on a Likert scale. Students were asked to rank the educational benefit of each modality. Linear regression was utilized to assess the correlation of the mean increase in knowledge with increasing complexity of CHD based on the Aristotle Basic Complexity Level. 45 medical students attended the CHD workshop. Students' knowledge significantly improved for every lesion (p < 0.001). A strong positive correlation was found between mean increase in knowledge and increasing complexity of CHD (R 2  = 0.73, p < 0.05). The 3D printed models, pathology specimens and spoken explanation were found to be the most helpful modalities. Students "strongly agreed" the 3D printed models made them more confident in explaining congenital cardiac anatomy to others (mean = 4.23, ± 0.69), and that they recommend the use of 3D models for future educational sessions (mean = 4.40, ± 0.69). 3D printed cardiac models should be included in medical student education particularly for lesions that require a complex understanding of spatial relationships.

After pulmonary artery (PA) patch augmentation, surgical or catheterization reintervention to address PA stenosis is not uncommon. Multiple patch materials are available and the need for reintervention may be related to characteristics of the patch material. In this retrospective chart review of patients who underwent PA augmentation from 2004 through 2013, we compare outcomes after PA augmentation with standard patch (SP) material and extracellular matrix (ECM) patch material. The primary outcome was reintervention-free survival (surgery or catheterization) using Kaplan-Meier survival analysis with the log-rank test. Risk factors for reintervention were assessed with Cox proportional hazard analyses. Baseline characteristics between groups were similar, except single-ventricle (SV) patients were more likely to have SP, and duration of follow-up was longer in the SP group. Median time to reintervention for all patients was 1099 days (95 % CI 646-1552 days). There was no difference in reintervention-free survival between the patch groups (p = 0.12); however, in multivariable analysis controlling for surgeon and patch, longer reintervention-free survival was associated with SV physiology (HR 0.57 95 % CI 0.34-0.95, p = 0.03) and aortic cross-clamp (XC) use (HR 0.52, 95 % CI 0.32-0.84, p = 0.008). Since there is no difference in median time to reintervention following PA augmentation with SP or ECM, patch choice should be determined by other factors, such as surgeon preference and cost. Longer reintervention-free survival in SV patients may be related to the set schedule of staged palliation. XC use may allow more extensive PA augmentation, thus protecting against the need for reintervention.

We reviewed our experience of surgical repair of Tetralogy of Fallot (TOF) in children weighing less than or equal to 4 kg to compare outcome of early palliation versus complete repair as the initial surgical approach. Seventy-six patients, weighing ≤ 4 kg, with TOF surgery between January 2005 and September 2013 were included in this single-center retrospective study. Twenty-five patients who underwent initial shunt procedure followed by later full repair were compared to 51 patients who had primary full repair for differences in baseline characteristics and outcomes. Shunt group patients had lower body weight, 2.76 ± 0.69 versus 3.11 ± 0.65 (kg), p = 0.03, and lower preoperative oxygen saturations, 82 ± 7 versus 90 ± 6 (%), p = 0.0001, than full repair group. A higher number of surgical procedures per patient was recorded in shunt patients, 2.29 ± 0.59 versus 1.27 ± 0.49, p = 0.00002. Thirteen of 51 patients in the full repair group required a repeat surgery. Catheterization procedures were performed in 12 patients in shunt and in 15 patients in full repair group, with interventional angioplasty in three and 11, respectively, p ≥ 0.05. Two patients, both in the shunt group, died after the surgery. Early full repair had longer hospital stay but significantly less hospitalizations 1.95 ± 1.3 versus 2.5 ± 1.4, p = 0.03. Initial complete repair of TOF in small children yielded favorable outcome with significantly less surgical procedures and subsequent hospitalizations. Cath laboratory re-interventions for residual defects were similar after both surgical approaches, and type of initial surgery does not predict freedom from re-intervention.

Few recent studies have assessed the epidemiology of health care-associated infections (HAIs) in the pediatric population after cardiac surgery. A retrospective cohort study was performed to assess the epidemiology of several types of HAIs in children 18 years of age or younger undergoing cardiac surgery from July 2010 to June 2012. Potential pre-, intra-, and postoperative risk factors, including adherence to the perioperative antibiotic prophylaxis regimen at the authors' hospital, were assessed by multivariable analysis using Poisson regression models. Microorganisms associated with HAIs and their susceptibility patterns were described. Overall, 634 surgeries were performed, 38 (6 %) of which were complicated by an HAI occurring within 90 days after surgery. The HAIs included 7 central line-associated bloodstream infections (CLABSIs), 12 non-CLABSI bacteremias, 6 episodes of early postoperative infective endocarditis (IE), 9 surgical-site infections (SSIs), and 4 ventilator-associated pneumonias (VAPs). Mechanical ventilation (rate ratio [RR] 1.07 per day; 95 % confidence interval [CI] 1.03-1.11; p = 0.0002), postoperative transfusion of blood products (RR 3.12; 95 %, CI 1.38-7.06; p = 0.0062), postoperative steroid use (RR 3.32; 95 % CI 1.56-7.02; p = 0.0018), and continuation of antibiotic prophylaxis longer than 48 h after surgery (RR 2.56; 95 % CI 1.31-5.03; p = 0.0062) were associated with HAIs. Overall, 66.7 % of the pathogens associated with SSIs were susceptible to cefazolin, the perioperative antibiotic prophylaxis used by the authors' hospital. In conclusion, HAIs occurred after 6 % of cardiac surgeries. Bacteremia and CLABSI were the most common. This study identified several potentially modifiable risk factors that suggest interventions. Further studies should assess the role of improving adherence to perioperative antibiotic prophylaxis, the age of transfused red blood cells, and evidence-based guidelines for postoperative steroids.

A significant inverse relationship of surgical institutional and surgeon volumes to outcome has been demonstrated in many high-stakes surgical specialties. By and large, the same results were found in pediatric cardiac surgery, for which a more thorough analysis has shown that this relationship depends on case complexity and type of surgical procedures. Lower-volume programs tend to underperform larger-volume programs as case complexity increases. High-volume pediatric cardiac surgeons also tend to have better results than low-volume surgeons, especially at the more complex end of the surgery spectrum (e.g., the Norwood procedure). Nevertheless, this trend for lower mortality rates at larger centers is not universal. All larger programs do not perform better than all smaller programs. Moreover, surgical volume seems to account for only a small proportion of the overall between-center variation in outcome. Intraoperative technical performance is one of the most important parts, if not the most important part, of the therapeutic process and a critical component of postoperative outcome. Thus, the use of center-specific, risk-adjusted outcome as a tool for quality assessment together with monitoring of technical performance using a specific score may be more reliable than relying on volume alone. However, the relationship between surgical volume and outcome in pediatric cardiac surgery is strong enough that it ought to support adapted and well-balanced health care strategies that take advantage of the positive influence that higher center and surgeon volumes have on outcome.

Advances in the management of hypoplastic left heart syndrome (HLHS) have resulted in improved survival. However, short and long-term mortality in patients with a restrictive atrial septum remains high. All neonates diagnosed with HLHS from 2003 to 2010 at our institution were evaluated. Patients who underwent atrial septostomy within the first 72 h conformed the restrictive atrial septum group (HLHS-RS). Patients with a non-restrictive communication (HLHS-NRS) formed the control group. Outcomes and survival status were determined from review of medical records. Of the 141 newborns diagnosed with HLHS, 20 (14 %) required intervention for a restrictive atrial septum. Procedural success was achieved in 17/20 (85 %) patients. Complications occurred in ten procedures, two of which were life threatening. No procedural deaths occurred. Overall median follow up was 35.5 months (0.4-104). Initial hospitalization survival was 16/20 (80 %) for the HLHS-RS group and 114/121(94 %) for the HLHS-NRS (p = 0.028). Twenty (14 %) patients were lost to follow up and 9 (6 %) underwent heart transplant. Overall survival was 10/16 (62 %) for HLHSRS patients and 77/95 (81 %) for HLHS-NRS (p = 0.1). Survival after initial discharge was 10/12 (83 %) for the HLHS-RS group and 77/88 (87 %) for the HLHS-NRS (p = 0.67). No predictors for HLHS-RS outcome were identified. Mortality at first-stage palliation in HLHS neonates with a restrictive atrial septum remains higher than in those with an unrestrictive communication. However, survival after initial hospital discharge is similar.

Quality-of-care evaluation must take into account variations in "ase mix."This study reviewed the application of two case-mix complexity-adjustment tools in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database: the Aristotle Basic Complexity (ABC) score and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. The 2006 STS Congenital Heart Surgery Database Report, the first STS report to incorporate both methods, included 45,635 operations from 47 centers. Each operation was assigned an ABC score in a range from 1.5 (lowest complexity) to 15 (highest complexity), an ABC level in a range from 1 (lowest complexity) to 4 (highest complexity), and a RACHS-1 category in a range from 1 (lowest risk) to 6 (highest risk). The overall discharge mortality was 3.9% (1,222/31,719 eligible cardiac index operations). Of the eligible cardiac index operations, 85.8% (27,202/31,719) were eligible for analysis by the RACHS-1 method, and 94.0% (29,813/31,719) were eligible for analysis by the ABC approach. With both RACHS-1 and ABC, as complexity increases, discharge mortality also ncreases. The ABC approach allows classification of more operations, whereas the RACHS-1 discriminates better at the higher end of complexity. Complexity stratification is a useful method for analyzing the impact of case mix on pediatric cardiac surgical outcomes. Both the RACHS-1 and ABC methods facilitate complexity stratification in the STS database.

There have been comprehensive screening methods developed to identify unwanted inpatient events. A comprehensive assessment of complication diagnoses during congenital heart surgery admissions has not been performed. We examined the frequency of complications identified by a complication screening method and their relationship to patient characteristics among congenital heart surgery admissions. Data were obtained from the Healthcare Cost and Utilization Project Kids' Inpatient Database 2000. Among congenital heart surgery admissions, age < or =20 years, we identified International Classification of Disease, 9th Revision, Clinical Modification (ICD-9-CM) codes indicating complication. Complication diagnoses were categorized as related to drug/biologic, procedures, devices, implants and grafts, and radiation. We used the Risk Adjustment for Congenital Heart Surgery risk categories (1-6) to examine the association between case complexity and complications. Multivariate analyses estimated the odds for a complication diagnosis by patient characteristics, including age, prematurity, chromosomal anomalies, noncardiac structural anomalies, and surgical risk category. Among 12,717 cases, 4014 (32%) had at least 1 complication diagnosis code. Procedure-related complication diagnoses represented 75% of complication diagnoses; device, implant, or graft represented 21%; drug/biologic represented 4% and radiation represented 0%. Multivariate analyses demonstrated that higher surgical case complexity and older age had a greater risk for a complication diagnosis: Risk Category 2, odds ratio (OR) 1.8; 3 OR 2.9; 4 OR 2.9; 5 OR 5.0; 6 OR 4.1, relative to category 1, all p < 0.01; age > or =12 years, OR 1.3, p < 0.001; <1 year OR 1.1, p = 0.31. Premature cases had decreased odds OR 0.4, p < 0.001. This complication screening method indicates that unwanted patient events occur frequently during congenital heart surgery admissions. Children undergoing complex congenital heart surgery are at greatest risk for these unwanted events. Further study of these events is needed to determine their preventability and severity.