Pre-procedural CT imaging aids neonatal PDA stenting for ductal-dependent pulmonary blood flow with reduction in overall procedural morbidity.

Patent ductus arteriosus stenting for ductal-dependent pulmonary blood flow is a technically challenging neonatal procedure to maintain a stable pulmonary circulation. Pre-procedural computed tomography imaging aids in outlining ductal origin, insertion, size, course and curvature. Computed tomography imaging may add value to procedural outcomes and reduce overall procedural morbidity in neonatal patent ductus arteriosus stenting. We conducted a single centre retrospective chart review of neonates with ductal-dependent pulmonary blood flow who underwent patent ductus arteriosus stenting between January 1, 2014 and June 31, 2020. We compared patients variables between patients who underwent pre-procedural computed tomography imaging to those who did not. A total of 64 patients were referred for patent ductus arteriosus stenting with 33 (52%) obtaining pre-procedural computed tomography imaging. Average age [19 days; range 1-242 days (p = 0.85)] and weight [3.3 kg (range 2.2-6.0 kg; p = 0.19)] was not significantly different between the groups. A diagnosis of pulmonary atresia was made in 42 out of 64 (66%) patients prior to patent ductus arteriosus stenting. The cohort with pre-intervention computed tomography imaging had a significant reduction in the total number of access sites (1.2 versus 1.5; p = 0.03), contrast needed (5.9 versus 8.2 ml/kg; p = 0.008), fluoroscopy (20.7 versus 38.8 minutes; p = 0.02) and procedural time (83.4-128.4 minutes; p = 0.002) for the intervention. There was no significant difference in radiation burden between the groups (p = 0.35). Pre-procedural computed tomography imaging adds value by aiding interventional planning for neonatal patent ductus arteriosus stenting. A statistically significant reduction in the number of access sites, contrast exposure, as well as fluoroscopic and procedural time was noted without significantly increasing the cumulative radiation burden.

ACCAPA: anomalous circumflex coronary artery origin from pulmonary artery.

Anomalous circumflex coronary artery origin from pulmonary artery is a very rare congenital heart disease. Misdiagnosis of this condition may lead to inadequate myocardial protection during cardiopulmonary bypass surgery.

Percutaneous device embolization of an aneurysmal pulmonary artery in an infant weighing <3 kilograms.

Pulmonary artery aneurysm (PAA) and pulmonary artery pseudoaneurysm (PAP) are rare diagnoses in the pediatric population and carry a high risk of mortality if rupture occurs. There is currently no standard therapeutic approach to PAAs and PAPs. Reports of surgical intervention describe high mortality. We present a case of an infant with a PAP that was successfully treated with a percutaneous device closure. Our approach included deployment of a 6-mm Amplatzer Vascular Plug 2 (Abbott, St. Paul, MN) in the right lower pulmonary artery segmental branch just proximal to the origin of the pseudoaneurysm. Subsequent imaging 1-month post-procedure demonstrated a >50% reduction in the size of the PAP when compared to original imaging studies and near-complete resolution 14 months following the intervention. Percutaneous device placement to occlude the vessel supplying peripheral PAAs and PAPs may be a reasonable alternative to open surgical resection when treating patients with this rare, but potentially life-threatening vascular anomaly. To our knowledge, this is the first case describing a successful device closure of a PAP in an infant weighing <3 kg.

Relationship Between Time to Left Atrial Decompression and Outcomes in Patients Receiving Venoarterial Extracorporeal Membrane Oxygenation Support: A Multicenter Pediatric Interventional Cardiology Early-Career Society Study.

OBJECTIVES: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort. DESIGN: Multicenter retrospective study. SETTING: Eleven pediatric hospitals within the United States. PATIENTS: Patients less than 18 years on venoarterial extracorporeal membrane oxygenation who underwent left atrial decompression from 2004 to 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 137 patients (median age, 4.7 yr) were included. Cardiomyopathy was the most common diagnosis (47%). Cardiac arrest (39%) and low cardiac output (50%) were the most common extracorporeal membrane oxygenation indications. Median time to left atrial decompression was 6.2 hours (interquartile range, 3.8-17.2 hr) with the optimal cut-point of greater than or equal to 18 hours for late decompression determined by receiver operating characteristic curve. In univariate analysis, late decompression was associated with longer extracorporeal membrane oxygenation duration (median 8.5 vs 5 d; p = 0.02). In multivariable analysis taking into account clinical confounder and center effects, late decompression remained significantly associated with prolonged extracorporeal membrane oxygenation duration (adjusted odds ratio, 4.4; p = 0.002). Late decompression was also associated with longer duration of mechanical ventilation (adjusted odds ratio, 4.8; p = 0.002). Timing of decompression was not associated with in-hospital survival (p = 0.36) or overall survival (p = 0.42) with median follow-up of 3.2 years. CONCLUSIONS: In this multicenter study of pediatric patients receiving venoarterial extracorporeal membrane oxygenation, late left atrial decompression (≥ 18 hr) was associated with longer duration of extracorporeal membrane oxygenation support and mechanical ventilation. Although no survival benefit was demonstrated, the known morbidities associated with prolonged extracorporeal membrane oxygenation use may justify a recommendation for early left atrial decompression.

Use of Near-Infrared Spectroscopy to Monitor Lower Extremity Perfusion in Pediatric Patients Undergoing Cardiac Catheterization.

Acute femoral artery occlusion is common in pediatric patients following cardiac catheterization. A variety of means are utilized to assess lower extremity (LE) perfusion and arterial patency following cardiac catheterization including palpation of pulses, pulse oximetry, subjective assessment of lower extremity color and temperature, and ultrasound. We sought to evaluate the utility of Near-Infrared Spectroscopy (NIRS) to monitor LE perfusion in pediatric patients undergoing cardiac catheterization. INVOS pediatric sensors were placed on bilateral LE in all pediatric patients ≤ 40 kg undergoing cardiac catheterization. Data were recorded continuously from the start of the procedure until 4-6 h after completion of the procedure. NIRS readings were compared between the accessed versus non-accessed LE at baseline before start of case, time of vascular access, arterial sheath exchange when applicable, sheath withdrawal, and Safeguard application, deflation, and removal. 133 patients underwent 152 catheterizations with mean age 2.4 ± 2.3 years and mean weight 12.4 ± 13.2 kg. NIRS oximetry readings were significantly decreased in the LE with arterial access compared to non-accessed LE from time of sheath insertion until removal of the pressure assist device post procedure. A greater difference was noted in smaller patients. NIRS oximetry readings did not correlate with subjective assessment of lower extremity perfusion after arterial sheaths were removed. One patient had pulse loss 4 h post procedure with a decrease in oximetry readings noted at this point on review. Weight-based heparin protocol was initiated, and a gradual improvement in oximetry readings was noted over the next 5 h. Vascular ultrasound 12 h later showed no evidence of arterial thrombus. NIRS may be helpful in identifying patients who are risk for developing arterial thrombus post cardiac catheterization and for monitoring response to therapy; however, further study in these patients is warranted.

Bench and initial preclinical results of a novel 8 mm diameter double opposed helical biodegradable stent.

BACKGROUND: Metallic endovascular stents are utilized off-label in congenital heart disease. Biodegradable stents (BDS) offer potential advantages in a growing child. We have previously reported double opposed helical (DH) BDS up to 6 mm diameter (DH-6). The objectives are to investigate the bench characteristics of larger 8 mm diameter BDS (DH-8) manufactured with increasing strut thicknesses and the inflammatory profile in a porcine model. METHODS: DH-8 were manufactured with strut thicknesses 0.10, 0.12, and 0.18 mm and mechanical testing performed. Stents were deployed into the infrarenal descending aorta (DAO) of nine minipigs. At insertion (nonsurvival = 2), 1 week (n = 2), 1 month (n = 2), and 9 months (n = 3) follow-up angiography, intravascular ultrasound and histopathology were performed. RESULTS: There was superior recoil and collapse pressure with increasing strut thickness, with 0.18 mm having 1.0% elastic recoil and collapse pressure 0.75 Atmospheres. There was good wall apposition at insertion with 5 BDS (4 DH-8 and 1 DH-6) but suboptimal in 4 as the minipigs infrarenal DAO were >8 mm (deployed at iliac bifurcation). Structural integrity was maintained in 8 BDS with 1 DH-8 collapsed at 9 months, secondary to strut damage at insertion. No thrombosis was seen. There was mild inflammation and neointimal proliferation at 1 week and 1 month, but a moderate inflammatory response at 9 months. CONCLUSIONS: DH-8 with increased strut thickness had acceptable mechanical properties at the cost of an increased inflammatory response. Miniaturization to improve delivery and further investigation on the long-term inflammatory profile of thicker struts, including through degradation, is needed. © 2016 Wiley Periodicals, Inc.

Breast Milk Protects Against Gastrointestinal Symptoms in Infants at High Risk for Autism During Early Development.

OBJECTIVES: Parents of children with autism spectrum disorders (ASDs) often report gastrointestinal (GI) dysfunction in their children. The objectives of the present study were to determine whether infants at high risk for developing ASD (ie, siblings of children diagnosed as having ASD) show greater prevalence of GI problems and whether this prevalence is associated with diet and age at weaning from breast milk. METHODS: Using questionnaires, diet history and GI problems were tracked prospectively and retrospectively in 57 high-risk infants and for comparison in 114 low-risk infants (infants from families without ASD history). RESULTS: In low-risk infants, prevalence of GI symptoms, in aggregate, did not vary with diet or age of weaning. By contrast, high-risk infants with GI symptoms were weaned earlier than those without symptoms (P < 0.04), and high-risk infants showed greater prevalence of GI symptoms, in aggregate, on a no breast milk diet than on an exclusive breast milk diet (P < 0.017). Constipation, in particular, was more prevalent in high-risk infants compared with low-risk infants (P = 0.01), especially on a no breast milk diet (P = 0.002). High-risk infants who completed weaning earlier than 6 months showed greater prevalence of constipation (P = 0.001) and abdominal distress (P = 0.004) than those fully weaned after 6 months. CONCLUSIONS: The greater prevalence of GI symptoms in high-risk infants suggests that GI dysfunction during early infant development may be a part of the ASD endophenotype. Late weaning and exclusive breast milk were associated with protection against GI symptoms in high-risk infants.

Use of Amplatzer Vascular Plugs for the treatment of combined extralobar and intralobar pulmonary sequestration in a 5-year-old child.

Pulmonary sequestration is a rare congenital anomaly that can be asymptomatic or present with recurrent infections, respiratory symptoms, or rarely heart failure. Sequestration is classified as intralobar or extralobar on the basis of whether there is separation from normal lung tissue by its own visceral pleura. Classically, patients are treated with surgical resection. We present a case of multivessel, combined intralobar and extralobar pulmonary sequestration treated with transcatheter embolisation.

The use of a three-dimensional print model of an aortic arch to plan a complex percutaneous intervention in a patient with coarctation of the aorta.

Recently, three-dimensional printing of heart models is being used to plan percutaneous and surgical interventions in patients with CHD. We describe a case where we used a three-dimensional print model to plan a complex percutaneous intervention in a patient with coarctation of the aorta.

Device closure of a pseudoaneurysm of the right ventricular outflow tract in an infant with right ventricle-to-pulmonary artery homograft.

Pseudoaneurysm of the right ventricular outflow tract (RVOT) is a rare complication following surgical repair of congenital heart disease involving a homograft or conduit. Traditionally, surgical intervention is indicated due to risk for rupture, thrombosis, compression of adjacent structures, and infection. We describe a case of a RVOT pseudoaneurysm in a 5 kg patient that was palliated with interventional catheterization utilizing an Amplatzer Ductal Occluder with four years follow-up. Interventional catheterization can successfully manage this complication in small patients for the long-term and thus avoids additional surgery and delays conduit replacement.

Heart Transplantation for Pediatric and Congenital Cardiac Disease: A Comparison of Two Eras over 23 Years and 188 Transplants at a Single Institution.

BACKGROUND: To assess changes in patterns of practice and outcomes over time, we reviewed all patients who underwent heart transplantation (HTx) at our institution and compared two consecutive eras with significantly different immunosuppressive protocols (cohort 1 [80 HTx, June 1995-June 2006]; cohort 2 [108 HTx, July 2006-September 2018]). METHODS: Retrospective study of 180 patients undergoing 188 HTx (June 1995-September 2018; 176 first time HTx, 10 second HTx, and 2 third HTx). In 2006, we commenced pre-HTx desensitization for highly sensitized patients and started using tacrolimus as our primary postoperative immunosuppressive agent. The primary outcome was mortality. Survival was modeled by the Kaplan-Meier method. Univariable and multivariable Cox proportional hazard models were created to identify prognostic factors for survival. RESULTS: Our 188 HTx included 18 neonates, 85 infants, 83 children, and 2 adults (>18 years). Median age was 260.0 days (range: 5 days-23.8 years). Median weight was 7.5 kg (range: 2.2-113 kg). Patients in cohort 1 were less likely to have been immunosensitized preoperatively (12.5% vs 28.7%, P = .017). Nevertheless, Kaplan-Meier analysis suggested superior survival in cohort 2 (P = .0045). Patients in cohort 2 were more likely to be alive one year, five years, and ten years after HTx. Multivariable analysis identified the earlier era (hazard ratio [HR] [95% confidence interval] for recent era = 0.32 [0.14-0.73]), transplantation after prior Norwood operation (HR = 4.44 [1.46-13.46]), and number of prior cardiac operations (HR = 1.33 [1.03-1.71]) as risk factors for mortality. CONCLUSIONS: Our analysis of 23 years of pediatric and congenital HTx reveals superior survival in the most recent 12-year era, despite the higher proportion of patients with elevated panel reactive antibody in the most recent era. This improvement was temporally associated with changes in our immunosuppressive strategy.

Risk Factors for Survival After Heart Transplantation in Children and Young Adults: A 22-Year Study of 179 Transplants.

BACKGROUND: This article reviews all patients who underwent heart transplantation (HTx) within a single institution (172 patients underwent 179 HTx [167 first-time HTxs, 10 second HTxs, 2 third HTxs]) to describe diagnostic characteristics, management protocols, and risk factors for mortality. METHODS: Descriptive analysis was performed for the entire cohort using mean, standard deviation, median, interquartile range, and overall range, as appropriate. Univariable and multivariable Cox proportional hazards models were performed to identify prognostic factors for outcomes over time. The primary outcome of interest was mortality, which was modeled by Kaplan-Meier analysis. RESULTS: Median age at HTx was 263 days (range, 5 days to 24 years; mean = 4.63 ± 5.95 years; 18 neonates, 79 infants). Median weight at HTx was 7.5 kg (range, 2.2-113 kg; mean = 19.36 ± 23.54). Diagnostic categories were cardiomyopathy (n = 62), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 33), transplantation after cardiac surgery for HLHS or HLHS-related malformation (n = 17), non-HLHS congenital heart disease (n = 55), and retransplant (n = 12). Operative mortality was 10.1% (18 patients). Cumulative total follow-up is 1,355 years. Late mortality was 18.4% (33 patients). Overall Kaplan-Meier five-year survival was 76.2%. One hundred twenty-one patients are alive with a mean follow-up of 7.61 ± 6.46 years. No survival differences were seen among the five diagnostic subgroups ( P = .064) or between immunosensitized patients (n = 31) and nonimmunosensitized patients (n = 141; P = .422). CONCLUSIONS: Excellent results are expected for children undergoing HTx with comparable results among diagnostic groups. Pretransplant mechanical circulatory support and posttransplant mechanical circulatory support are risk factors for decreased survival. Survival after transplantation for HLHS or HLHS-related malformation is better with primary HTx in comparison to HTx after prior cardiac surgery.

Serum Cystatin C as an Early Marker of Neutrophil Gelatinase-associated Lipocalin-positive Acute Kidney Injury Resulting from Cardiopulmonary Bypass in Infants with Congenital Heart Disease.

OBJECTIVE: Acute kidney injury (AKI) is a common complication resulting from cardiopulmonary bypass in infants. Urinary neutrophil gelatinase-associated lipocalin (NGAL) is a sensitive and specific marker of such injury. In this study, we compared the performance of serum cystatin C (Cys C) and serum creatinine (Cr) as early markers of renal dysfunction in infants undergoing cardiac surgery under bypass. STUDY DESIGN, SETTING, AND PATIENTS: The study was designed as a prospective observational study. The study was conducted in the cardiac intensive care unit (ICU) of a tertiary, academic children's hospital in the United States. Infants (age <1 year) undergoing cardiac surgery under cardiopulmonary bypass were included in the study. OUTCOME MEASURE: Acute kidney injury was defined based on postoperative urinary NGAL. RESULTS: A total of 17 infants were included in the study, and five of them developed AKI. Serum Cys C and Cr levels were measured postoperatively on days 1, 2, and 3, and compared with baseline levels. On postoperative day 2, infants with AKI showed significant change from baseline in serum Cys C levels compared with non-AKI infants (28% vs. -9%, P = .03). The two groups did not show significant differences with respect to rise in serum Cr on any of the 3 postoperative days. Serum Cr on days 1 and 2 showed nonspecific increases in both AKI and non-AKI groups. The area under the receiver operating characteristic curve for day 2 Cys C was 0.87 (95% CI 0.67-1.00) in recognizing NGAL-positive AKI. CONCLUSIONS: Postoperative serum Cys C appears to be a more specific and sensitive biomarker for NGAL-positive AKI resulting from cardiopulmonary bypass surgery in infants undergoing cardiac surgery.