Isolated Coarctation of the Aorta: Current Concepts and Perspectives.

Current management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.

Atrial septostomy in patients supported with venoarterial extracorporeal membrane oxygenation: Analysis of the IMPACT registry data.

BACKGROUND: Patients with severe cardiac dysfunction, supported on extra-corporeal membrane oxygenation (ECMO), develop left atrial hypertension and often warrant atrial decompression. The procedural risks and outcomes, however, are not well established. Objective of this study is to understand the utilization, safety, and outcomes of atrial septostomy in children supported on venoarterial ECMO utilizing the IMPACT registry database. METHODS: Data from 55 independent sites reporting to the registry was used for this descriptive study. Patients supported with cardiac ECMO that underwent percutaneous atrial septostomy (PAS) were included and data analyzed. RESULTS: Between 2011 and 2018, 223 patients underwent atrial septostomy and were reported to the registry. Mean age was 4.65 years with 52% being males. Diagnoses of heart failure, sepsis, arrhythmia, and renal insufficiency were commonly noted prior to ECMO. The procedure was performed urgently or emergently in most cases (80%). PAS was associated with significant procedural complications such as arrhythmia (6.8%), tamponade (5.4%), and unplanned surgery (3.6%) but no procedural mortality. Overall hospital mortality was 46% (103/223) with 46 of these deaths occurring within 7 days of procedure. Regression analysis showed that African American race (vs Caucasian), race other than Caucasian, chronic lung disease and emergent procedure need were significantly associated with early post-procedural mortality (all p < 0.05). CONCLUSION: Using a large multicenter cardiac catheterization registry, we found that left atrial decompression can be performed without procedural mortality but is associated with significant morbidity. Impact of race, chronic lung disease and procedural urgency on early mortality warrant further studies.

Risk Factors for Red Blood Cell Transfusions in Children Undergoing Cardiac Catheterization.

OBJECTIVE: To identify risk factors associated with risk of red blood cell transfusions (RBCTs) following pediatric cardiac catheterizations. STUDY DESIGN: We performed a review of all pediatric cardiac catheterizations from 2012 to 2017. The primary endpoint was RBCT within 72 hours of pediatric cardiac catheterization. Patient and procedural factors were reviewed. Generalized linear modelling was performed to describe interactions among relevant risk factors. RESULTS: In total, 831 RBCTs occurred within 72 hours of 6028 pediatric cardiac catheterizations (13.8%). Univariate analysis revealed that the prevalence of RBCT was highest among infants (37.6% incidence of RBCT) and among those with higher estimated blood loss as a percent of blood volume (P = .03). Among infants, multivariate analysis revealed that weight (OR 0.72; 95% CI 0.63-0.81), complex 2-ventricle (OR 3.14, 95% CI 2.18-4.57), and single ventricle status (OR 5.21, 95% CI 3.42-8.01) were associated with risk of RBCT. Inpatient infants from intensive care (OR 4.74; 95% CI 3.49-6.49) or stepdown units (OR 2.33; 95% CI 1.58-3.46) were at higher risk. Length of procedure (OR 2.57; 95% CI 2.03-3.26) and oxygen saturation (OR 0.98; 95% CI 0.97-0.99; P < .01) were also associated with RBCTs. CONCLUSIONS: Hospitalized infants with single ventricle or complex 2-ventricle anatomy are at highest risk of RBCT. Length of procedure, blood loss, and oxygen saturations are additional risk factors associated with RBCT. Operators should consider these factors when planning pediatric cardiac catheterizations, particularly when exposure to RBCT is undesirable.

Thromboprophylaxis strategies for children with single-ventricle circulations (superior or total cavo-pulmonary connections) after stent implantation.

OBJECTIVE: To define optimal thromboprophylaxis strategy after stent implantation in superior or total cavopulmonary connections. BACKGROUND: Stent thrombosis is a rare complication of intravascular stenting, with a perceived higher risk in single-ventricle patients. METHODS: All patients who underwent stent implantation within superior or total cavopulmonary connections (caval vein, innominate vein, Fontan, or branch pulmonary arteries) were included. Cohort was divided into aspirin therapy alone versus advanced anticoagulation, including warfarin, enoxaparin, heparin, or clopidogrel. Primary endpoint was in-stent or downstream thrombus, and secondary endpoints included bleeding complications. RESULTS: A total of 58 patients with single-ventricle circulation underwent 72 stent implantations. Of them 14 stents (19%) were implanted post-superior cavopulmonary connection and 58 (81%) post-total cavopulmonary connection. Indications for stenting included vessel/conduit stenosis (67%), external compression (18%), and thrombotic occlusion (15%). Advanced anticoagulation was prescribed for 32 (44%) patients and aspirin for 40 (56%) patients. Median follow up was 1.1 (25th-75th percentile, 0.5-2.6) years. Echocardiograms were available in 71 patients (99%), and advanced imaging in 44 patients (61%). Thrombosis was present in two patients on advanced anticoagulation (6.3%) and none noted in patients on aspirin (p = 0.187). Both patients with in-stent thrombus underwent initial stenting due to occlusive left pulmonary artery thrombus acutely post-superior cavopulmonary connection. There were seven (22%) significant bleeding complications for advanced anticoagulation and none for aspirin (p < 0.001). CONCLUSIONS: Antithrombotic strategy does not appear to affect rates of in-stent thrombus in single-ventricle circulations. Aspirin alone may be sufficient for most patients undergoing stent implantation, while pre-existing thrombus may warrant advanced anticoagulation.

IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease.

The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality.

Premounted stents for branch pulmonary artery stenosis in children: A short term solution.

OBJECTIVE: Define outcomes of premounted stent implantation (PMS) for branch pulmonary artery stenosis (BPAS). BACKGROUND: PMS for BPAS in children raises concern of long term viability, with limited maximal expansion. METHODS: We reviewed our cardiac database over an 11-year period ending in 2013. Primary endpoint was need for surgical stent intervention (SSI). Other endpoints included acute results and repeat interventions (RI). RESULTS: 82 PMS were implanted in 60 children for BPAS. Median weight was 6.3 (25th -75th 4.6-9.8) kg. Median stent diameter was 6 (range 4-9) mm. Acutely, vessel diameter improved from 2.0 (25th -75th 1.6-3.4) to 5.0 (25th -75th 4.2-5.9) mm (p < 0.001), pressure gradient decreased from 41 (25th -75th 29-50) to 11 (25th -75th 7-18) mmHg (n = 47, p < 0.001), RV:Ao pressure ratio decreased from 100% (25th -75th 85-110%) to 59% (25th -75th 49-74%) (n = 40, p < 0.001). Freedom from SSI was 81% at 1 year and 35% at 5 years. Freedom from RI was 50% at 1 year and 14% at 5 years. 86% of PMS underwent SSI during a concomitant planned cardiac surgery. 45% patients had stent redilation, improving stent diameter from 4.6 (25th -75th 4.1-5.4) to 5.7 (25th -75th 4.9-7) mm (p < 0.001). 1 stent (3%) was able to be fractured longitudinally. CONCLUSIONS: PMS is an effective short term solution for BPAS in children. PMS is associated with expected early need for transcatheter reintervention to accommodate for growth, but also has high rates of SSI.

Angiographic follow-up of infants and children undergoing percutaneous carotid artery interventions.

OBJECTIVE: The purpose of this study was to review the outcomes following the percutaneous carotid arterial (PCA) approach in infants and children with congenital heart disease. BACKGROUND: PCA access is becoming more commonly adopted following reports demonstrating it is a safe alternative to surgical carotid cutdown and even the femoral arterial route. However, follow-up outcomes after PCA remain unreported. METHODS: We reviewed all cases with PCA access and follow-up catheterizations which included carotid artery (CA) angiography between May 2012 until December 2016. We examined for evidence of CA stenosis at follow-up angiography and assessed any other CA complications associated with vascular access. RESULTS: There were 61 PCA catheterizations performed in 55 unique patients. Follow-up CA imaging with angiography was available in 43 patients (78%, 43/55). There was no vessel stenosis nor lumen irregularity in 28 (65%) patients. In 15 cases (35%), there was a mild degree of irregularity or narrowing by angiography (median 4.1%, range 2.3%-12.5%). Nine patients underwent repeat PCA catheterizations. Seven of these had no visible vascular stenosis on follow-up angiographic imaging, including a patient who was accessed three separate times from the CA. No statistically significant risk factors for developing mild CA stenosis were identified. CONCLUSIONS: PCA access for pediatric interventional catheterization appears to be safe with a very low rate of mild stenosis, and very few complications. Follow-up outcomes in our series are excellent, with a CA patency rate of 100%, even after multiple procedures. Mild CA stenosis was not associated with patient size or sheath introducer caliber. While the acute results from percutaneous CA catheterization have proven safe in recent literature, longer-term outcomes remain unreported. At our institution, the outcomes following percutaneous carotid access are associated with an excellent patency rate of 100%, even after multiple procedures on the same vessel. A low incidence of mild vessel stenosis can be appreciated on follow-up angiography.

Intervention on Surgical Systemic-to-Pulmonary Artery Shunts: Carotid Versus Femoral Access.

OBJECTIVES: The purpose of this study was to compare results between the femoral arterial (FA) and carotid arterial (CA) approaches in catheter-based interventions on Blalock-Taussig shunts (BTS). BACKGROUND: Transcatheter intervention on BTS is often performed in shunt-dependent, hypoxemic infants. The approach to BTS intervention likely has an impact on timeliness and overall success. METHODS: The authors reviewed all cases of catheter intervention for BTS obstruction between 2012 and 2017 for their institution. They sought to compare procedural success rates and time, sheath time, time to arterial access, and time from access to stent implantation between FA and CA approaches. RESULTS: There were 42 BTS interventions between 34 patients. BTS intervention was more successful from the CA approach (p = 0.035). Among the FA cohort, BTS intervention was unsuccessful in 8 cases (25%), 5 of which were converted to CA with subsequent success. The CA cohort had lower procedure time (62 min vs. 104 min; p = 0.01) and anesthesia time (119 min vs. 151 min; p = 0.01). Additionally, CA access was associated with shorter time to arterial access (4.0 min vs. 9.3 min; p < 0.01), time to placement of the guidewire through the BTS (6.5 min vs. 13 min; p < 0.01), and time from the final sheath to BTS stent implantation (9 min vs. 20 min; p < 0.01). CONCLUSIONS: Operators should consider the route of access to the BTS deliberately. The authors' approach has been the carotid artery as an alternative access site-associated with greater procedural success, shorter procedural time, and shorter time to stent implantation.

Reintervention Is Associated With Improved Survival in Pediatric Patients With Pulmonary Vein Stenosis.

OBJECTIVES: The aim of this study was to evaluate survival following catheter intervention in pediatric patients with pulmonary vein stenosis (PVS). BACKGROUND: Despite aggressive surgical and catheter intervention on PVS in children, recurrence and progression of stenosis can lead to right heart failure and death. Clinicians continue to seek effective treatment options for PVS. METHODS: A single-center, retrospective study was performed including all patients <18 years of age who underwent catheter intervention (balloon angioplasty and bare-metal stent and drug-eluting stent insertion) on PVS. Endpoints included death, vein loss, and rate of reintervention. RESULTS: Thirty patients underwent intervention (balloon angioplasty, n = 9; bare-metal stent, n = 5; drug-eluting stent, n = 16) at a median age of 6.4 months (4.3 to 9.9 months). Median follow-up duration was 30.6 months (77 days to 10.5 years). Fourteen patients (47%) died at a median of 2.0 months (0.4 to 3.2 months) following intervention. There was no association between DES placement and survival (p = 0.067). Reintervention (catheter or surgical) was associated with improved survival (p = 0.001), with a 1-year survival rate of 84% compared with 25% for no reintervention. Vein loss occurred in 34 of 58 (59%) veins at a median of 3.3 months (1.0 to 5.0 months). One-year vein survival was higher with DES implantation (p = 0.031) and with reintervention (p < 0.001). CONCLUSIONS: DES implantation at first catheter intervention appears to be associated with improved vein survival but may not result in improved patient survival. However, reintervention appears to be associated with improved patient survival and vein patency, suggesting that despite mode of treatment, frequent surveillance is important in the care of these patients.

Trends in U.S. Cardiovascular Care: 2016 Report From 4 ACC National Cardiovascular Data Registries.

Cardiovascular disease is a leading cause of death and disability in the United States. National programs, such as the National Cardiovascular Data Registry, facilitate assessments of the quality of care and outcomes for broad populations of patients with cardiovascular disease. This report provides data for 2014 from 4 National Cardiovascular Data Registry hospital quality programs: 1) CathPCI (Diagnostic Catheterization and Percutaneous Coronary Intervention) for coronary angiography and percutaneous coronary intervention (667,424 procedures performed in 1,612 hospitals); 2) ICD Registry for implantable cardioverter-defibrillators (158,649 procedures performed in 1,715 hospitals); 3) ACTION-GWTG (Acute Coronary Treatment and Intervention Outcomes Network-Get With The Guidelines) for acute coronary syndromes (182,903 patients admitted to 907 hospitals); and 4) IMPACT (Improving Pediatric and Adult Congenital Treatment) for cardiac catheterization and intervention for pediatric and adult congenital heart disease (20,169 procedures in 76 hospitals). The report provides perspectives on the demographic and clinical characteristics of enrolled patients, characteristics of participating centers, and selected measures of processes and outcomes of care in these programs.

Early catheterization after initiation of extracorporeal membrane oxygenation support in children is associated with improved survival.

OBJECTIVES: The study evaluated the institutional experience with cardiac catheterization on extracorporeal membrane oxygenation (ECMO) support. BACKGROUND: There is scant literature on the outcomes of catheterization on ECMO. METHODS: A retrospective review was performed of all children who underwent catheterization on ECMO from 2003 to 2013. Patients were categorized as cardiomyopathy (CM) or congenital heart disease (CHD). RESULTS: During the study period, 215 children were placed on cardiac ECMO. Of these, 29.8% underwent 75 catheterization procedures while on ECMO support. The median age of the cohort was 1.5 months (range 0 days -16.7 years) and the median weight was 3.9 kg (2.2-63.1 kg). CM patients constituted 18.8% of the cohort and all of them underwent atrial septoplasty (an atrial septal stent in 7/12 and balloon atrial septoplasty or septostomy in 5). The survival to hospital discharge rate was 83% and the transplant-free survival rate was 58.3%. CHD patients constituted 81.2% of the cohort. In this group, transcatheter interventions were performed in 40.4% and subsequent surgical interventions in 40.4%. Survival to hospital discharge rate was 34.6% and transplant free survival rate was 32.7%. Overall, 76.7% underwent transcatheter or surgical interventions. The major catheterization complication rate was 6.7%. The mean ECMO-to-catheterization time was 1.6 days for survivors and 3.5 days for non-survivors (P = 0.034). Survival to discharge was better for the CM group compared to the CHD group (P = 0.01). Among CHD, survival was better with transcatheter interventions compared to no interventions or surgical interventions (P < 0.001). CONCLUSIONS: Cardiac catheterization and transcatheter interventions on ECMO can be performed with low rate of complications. Catheterization was associated with high rate of interventions. Better survival to hospital discharge was associated with transcatheter interventions, earlier performance of catheterization after ECMO and diagnosis of CM. © 2016 Wiley Periodicals, Inc.

Procedural characteristics and adverse events in diagnostic and interventional catheterisations in paediatric and adult CHD: initial report from the IMPACT Registry.

OBJECTIVES: To report procedural characteristics and adverse events on data collected in the registry. BACKGROUND: The IMPACT--IMproving Paediatric and Adult Congenital Treatment--Registry is a catheterisation registry of paediatric and adult patients with CHD undergoing diagnostic and interventional cardiac catheterisation. We are reporting the procedural characteristics and adverse events of patients undergoing diagnostic and interventional catheterisation procedures from January, 2011 to March, 2013. METHODS: Demographic, clinical, procedural, and institutional data elements were collected at the participating centres and entered via either a web-based platform or software provided by American College of Cardiology-certified vendors, and were collected in a secure, centralised database. Centre participation was voluntary. RESULTS: During the time frame of data collection, 19,797 procedures were entered into the IMPACT Registry. Procedures were classified as diagnostic only (35.4%); one of six specific interventions (23.8%); other or multiple interventions (40.7%); and were further broken down into four age groups. Anaesthesia was used in 84.1% of diagnostic procedures and 87.8% of interventional ones. Adverse events occurred in 10.0% of diagnostic and 11.1% of interventional procedures. CONCLUSIONS: The IMPACT Registry is gathering data to set national benchmarks for diagnostic and certain specific interventional procedures. We are seeing little differences in procedural characteristics or adverse events in diagnostic procedures compared with interventional procedures overall, but there is significant variation in adverse events amongst age categories. Risk stratification and patient acuity scores will be required for further analysis of these differences.

Direct physician reporting is associated with reductions in radiation exposure in pediatric cardiac catheterizations.

OBJECTIVE: The objective is to quantify radiation dose in children undergoing cardiac catheterization and determine the impact of increased reporting transparency on total radiation exposure. BACKGROUND: Cardiac catheterization (cath) can result in significant radiation exposure in children. There has been growing interest in quantifying and reducing radiation exposure in pediatric cath procedures. Our center underwent a slight change in practice recently that resulted in direct physician reporting of radiation dose following every case. METHODS: We reviewed cath procedures across three different eras in four cath categories: post-heart transplant annual cath, unilateral pulmonary artery (PA) stent placement, pre-Fontan cath, and pre-Glenn cath. The eras were defined as: Era 1, 1/2009-1/2011; Era 2, 1/2011-9/2013; and Era 3, 9/2013-5/2014. In Era 3, the physician performing the cath was responsible for reporting the radiation data. RESULTS: Across the three eras, there were significant decreases in cumulative air KERMA (mGy) among all four cath categories. From Era 2 to Era 3, the greatest decreases in radiation were noted, particularly in dose area product (cGy·cm2) in the transplant annual evaluation and pre-Glenn cases. In Era 1, 2 cases (1.2%) had a frame rate reduction, while in Era 2, 22 cases (12.0%), and in Era 3, 83 cases (21.6%) had frame rate changes (P<0.01). CONCLUSIONS: Increased physician awareness of radiation exposure is associated with a significant reduction in radiation dose across a variety of cath procedures. This is seen not only by the overall reduction in radiation across case types, but also as the frame rate was more frequently changed during individual cases, indicating an important change in physician behavior and practice.

Transcatheter interventions across fresh suture lines in infants and children: an 8-year experience.

OBJECTIVES: The purpose of this study was to examine the outcomes of catheter dilation interventions in general upon surgical anastomotic sites in the immediate postoperative period. BACKGROUND: Early postoperative cardiac catheter intervention is regarded as high-risk, particularly when a dilation intervention across a fresh suture line is performed. METHODS: All catheter dilation interventions performed upon a fresh suture line within 30 days of congenital heart surgery between August 2005 and December 2013 were reviewed. Values are reported as median and interquartile range (IQR). Primary endpoint was procedural success, which was defined as an increase in vessel diameter of >75% of the adjacent normal vessel or 50% increase over pre-dilation diameter. Secondary endpoints included procedural complications and survival to hospital discharge. RESULTS: Forty-five patients, median age 0.15 years (0.03-0.51 years), weight 4.1 kg (3.1-6.4 kg), underwent 62 interventional procedures on median postoperative day 7 (3-13 days). Among the 62 dilation interventions at surgical anastomotic areas, were 30 stent and 32 balloon angioplasty procedures. There were two major complications including: arterial dissection during stent placement and a procedural mortality due to pulmonary artery avulsion during angioplasty. There were 12 deaths (27%) prior to hospital discharge. The median ratio of maximum stent diameter to stenosis diameter was 2.62 (2.27-3.73). The median ratio of maximum balloon diameter to stenosis diameter was 2.27 (1.84-2.94). Stent placement was more likely to result in procedural success (OR 2.1; 95% confidence interval 1.6-2.8). CONCLUSION: Though caution is paramount, early postoperative catheter dilation intervention across fresh suture lines can be performed safely in small, critically ill children.

Validation of a Prescreening Program for Transcatheter Atrial Septal Defect Closure.

Prior to initiation of a formal screening program, 30 % of patients referred for device closure of an atrial septal defect (ASD) at our institution had atrial septal anatomy which precluded ASD device closure. This resulted in inefficiencies in workflow, staff utilization, and inconvenience for families. Our objective was to report our experience with an ASD prescreening evaluation/protocol and family consultation process. This is a retrospective study of pediatric patients who underwent a comprehensive ASD prescreening evaluation at Children's Healthcare of Atlanta between January 2011 and June 2013. The comprehensive prescreening process included a detailed transthoracic echocardiogram and family consultation. Upon establishing a formal prescreening process for patients with secundum ASDs, 138 patients were screened for appropriateness of ASD device closure. At time of prescreening, 40 patients (29 %) were deemed not to be suitable candidates for device ASD closure. In 9 patients (6.5 %), after discussion with the interventionalist, parents elected to pursue surgical ASD closure. A total of 78 patients (56.5 %) underwent successful transcutaneous ASD device closure. In addition, there was a significant discrepancy in the reported size of the ASD between the outside evaluation and the ASD size discovered during the prescreening process. The addition of a prescreening program for transcatheter ASD closure has decreased the incidence of unsuccessful ASD closure from 30 to 10.3 %. The use of a comprehensive evaluation process for ASD device closure improves the likelihood of successful device implantation and permits adequate preprocedural counseling for the patient and family.