Here today, gone tomorrow: Outcomes of residual leak following secundum atrial septal defect closure with the GORE CARDIOFORM Septal Occluder.

OBJECTIVE: To assess the outcomes and potential predictors of residual leak after implantation of the GORE CARDIOFORM Septal Occluder (GSO) in secundum atrial septal defects (ASDs). BACKGROUND: The non-self-centering design of the GSO could lead to residual leak at the edge of the device. Outcomes of residual leak are poorly understood. METHODS: Retrospective, multicenter review of patients treated with the GSO for the pivotal and continued access study. Procedural, echocardiographic, and follow up data were reviewed. Multivariate analysis was performed to determine predictors of leak at implant and outcomes of these leaks. RESULTS: There were 370 total ASD device closures with the GSO, 65 (17.56%) of which were found to have residual leak at implantation. Patients with residual leak had larger defects (10.33 ± 3.05 mm vs. 9.13 ± 2.89 mm, p = .006) and smaller aortic rims (4.87 ± 3.33 mm vs. 6.17 ± 3.78 mm, p = .019), as compared to those without leak. Larger devices were implanted (p = .008) and more devices per case (p = .007) were utilized in the residual leak cohort as compared to those without leak. Overall, among patients with residual leak, there was a significant decrease in leak size over 1 year, from 1.55 ± 0.75 mm to 0.25 ± 0.74 mm (p < .001), with the majority (87.6%) disappearing by 12-month follow-up. CONCLUSIONS: Residual leak at implantation can be seen after ASD device closure with the GSO, more commonly in larger defects with smaller retroaortic rims. The vast majority of these leaks completely resolve by 1 year follow up.

Results of the combined U.S. multicenter postapproval study of the Nit-Occlud PDA device for percutaneous closure of patent ductus arteriosus.

OBJECTIVES: To report the results of the Nit-Occlud PDA prospective postapproval study (PAS) along with a comparison to the results of the pivotal and continued access trials. BACKGROUND: The Nit-Occlud PDA (PFM Medical, Cologne, Germany), a nitinol coil patent ductus arteriosus (PDA) occluder, was approved by the Food and Drug Administration in 2013. METHODS: The PAS enrolled a total of 184 subjects greater than 6 months of age, weighing at least 5 kg, with PDAs less than 4 mm by angiography at 11 centers. Patients were followed prospectively at 2 months, 12 months, and 24 months postprocedure. These outcomes were compared to the 357 subjects enrolled in the pivotal and continued access protocols. Efficacy and safety data were reported. RESULTS: Among 184 subjects enrolled for the PAS between 2014 and 2017, 180 (97.8%) had successful device implantation. After 12 months, 98.7% (150/152) had trivial or no residual shunt by echocardiography and two subjects had only small residual shunts. There were three device embolizations that were all retrieved by snare without clinical consequence. Together with the pivotal and continued access study, 97.4% (449/461) had complete echocardiographic closure at 12 months in 541 enrolled subjects. The composite success was 94.4%. There were no mortalities and no serious device-related adverse events. CONCLUSIONS: The Nit-Occlud PDA is a safe and effective device for closure of a small to moderate sized PDA. There were no serious device-related adverse events in a large cohort of three clinical trials.

Variation in Anticoagulation Practices in the Congenital Cardiac Catheterization Lab: Results of a Multinational PICES Survey.

The complex nature of congenital heart disease (CHD) has hindered the establishment of management standards for peri-catheterization anticoagulation. We sought to describe anticoagulation practice variability among providers performing cardiac catheterization in children and adults with CHD. A web-based survey ( http://www.surveymonkey.com ) was distributed to pediatric and congenital interventional cardiologists. Respondents were queried on their training, practice setting, years in practice, and case volume. Clinical questions focused on general anticoagulation strategies and on five common clinical scenarios: two diagnostic (biventricular circulation, single ventricle physiology) and three interventional cardiac catheterizations (atrial septal defect closure, pulmonary artery stenting in Fontan circulation, stent placement for coarctation of aorta). Seventy-seven pediatric and congenital interventional cardiologists responded to the survey (81% in the United States). Twenty-six (36%) worked in a public medical institution; 57% worked in a free-standing children's hospital. Twenty-six percent had been in practice for < 5 years and 32% for > 15 years; 75% completed additional training in interventional congenital cardiology. The median number of cases performed was 200/year (IQR 110); median number of interventional cases was 100/year (IQR 100). Responses to general queries and specific clinical scenarios suggested significant variation in anticoagulation practices, including monitoring of anticoagulation during catheterization, protamine use, and outpatient anticoagulation after catheterization. Practices not only varied between providers but also between different clinical scenarios. Practice patterns did not correlate with provider experience or case volume. Management of anticoagulation in the congenital cardiac catheterization lab varies from operator to operator. Our study may provide some initial insight and context for discussion regarding anticoagulation in a field of increasingly heterogeneous interventional techniques and patient substrates. Future studies would be helpful to better define "best practices" for peri-procedural thromboprophylaxis in patients with congenital heart disease.

Elective left pulmonary artery embolisation for pulmonary arteriovenous malformations secondary to cavopulmonary anastomoses not responsive to heart transplantation: a case report.

A 10-year-old female with heterotaxy-asplenia and complex CHD developed pulmonary arteriovenous malformations with associated cyanosis after Fontan completion. She underwent orthotopic heart transplantation, but her pulmonary arteriovenous malformations persisted with progressive worsening cyanosis. Elective transcatheter left pulmonary artery embolisation was performed 2 years post-transplant, which successfully normalised her oxygen saturation without a significant increase in pulmonary artery pressure.

Serial Versus Direct Dilation of Small Diameter Stents Results in a More Predictable and Complete Intentional Transcatheter Stent Fracture: A PICES Bench Testing Study.

Balloon-expandable stents, implanted in infants and children with congenital heart disease (CHD), often require redilation to match somatic growth. Small diameter stents may eventually require longitudinal surgical transection to prevent iatrogenic vascular stenosis. Intentional transcatheter stent fracture (TSF) is an emerging alternative approach to stent transection, but little is known about the optimal stent substrate and best protocol to improve the likelihood of successful TSF. Bench testing was performed with a stent dilation protocol. After recording baseline characteristics, stents were serially or directly dilated using ultra-high-pressure balloons (UHPB) until fracture occurred or further stent dilation was not possible. Stent characteristics recorded were as follows: cell design, metallurgy, mechanism, and uniformity of fracture. Stents tested included bare-metal coronary stents, premounted small diameter stents, and ePTFE-covered small diameter premounted stents. Ninety-four stents representing 9 distinct models were maximally dilated, with 80 (85%) demonstrating evidence of fracture. Comprehensive fracture details were recorded in 64 stents: linear and complete in 34/64 stents (53.1%), linear and incomplete in 9/64 stents (14.1%), transverse/complex and complete in 6/64 stents (9.4%), and transverse/complex and incomplete in 15/64 stents (23.4%). Stent fracture was not accomplished in some stent models secondary to significant shortening, i.e., "napkin-ring" formation. Serial dilation resulted in evidence of fracture in 62/67 (92.5%) stents compared with 18/27 (66.7%) stents in the direct dilation group (p = 0.003). Intentional TSF is feasible in an ex vivo model. Serial dilation more reliably expanded the stent and allowed for ultimate stent fracture, whereas direct large diameter dilation of stents was more likely to generate a "napkin-ring" configuration, which may be more resistant to fracture. In vivo animal and human testing is necessary to better understand the response to attempted TSF for newly developed stents as well as those currently in use.

Functional assessment of anomalous right coronary artery using fractional flow reserve: An Innovative Modality to Guide Patient Management.

Anomalous origin of a coronary artery is a recognized cause of sudden cardiac death (SCD). To date, there is no standard test to predict which patients are at increased risk for SCD. Fractional flow reserve (FFR) is an invasive technique used to qualify focal obstructive coronary lesions. We present a case where FFR was used to guide therapy in a young patient with anomalous right coronary artery (ARCA) when standard noninvasive testing showed ischemic discrepancy. © 2016 Wiley Periodicals, Inc.

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

OBJECTIVES: To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). BACKGROUND: Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. METHODS: This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). RESULTS: Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. CONCLUSIONS: Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

A case report of percutaneous MitraClip implantation in an adult with a double-outlet right ventricle.

Background: Atrioventricular valve regurgitation (AVVR) is present in up to 75% of Fontan patients, and it is associated with an increased risk of Fontan circulation failure, morbidity, and mortality. Traditional treatment options include surgical repair vs. surgical replacement. We present, to the best of our knowledge, one of the first cases of successful trans-catheter repair of severe common AVVR using the MitraClip device. Case summary: A 20-year-old male with a history of double-outlet right ventricle (DORV) with unbalanced common atrioventricular canal to the right ventricle, severely hypoplastic left ventricle, and total anomalous pulmonary venous return status post-Fontan procedure presented with progressively worsening dyspnoea on exertion. Transoesophageal echocardiogram demonstrated severe common AVVR. After discussion of the case during the adult congenital heart disease multidisciplinary conference, patient underwent successful placement of two MitraClip devices, reducing the regurgitation from torrential to moderate. Discussion: MitraClip therapy can be used to alleviate symptoms in patients deemed as high risk for surgery. However, careful attention must be paid to haemodynamics before and after clip placement, which may predict short-term clinical outcomes.

Closure of perimembranous ventricular septal defects with aneurysmal tissue using the Amplazter Duct Occluder I: lessons learned and medium term follow up.

OBJECTIVES: The aim of this report is to describe our experience with closure of perimembranous ventricular septal defects (PMVSD) with aneurysm using the Amplatzer Duct Occluder I (ADO I). BACKGROUND: Atrio-ventricular block (AVB) remains the most troublesome complication of device closure of PMVSDs. Many PMVSDs are associated with an aneurysm that protrudes into the right ventricle. We believe that devices implanted entirely within the VSD aneurysm avoid the risk of AVB. METHODS: A retrospective analysis of patients with PMVSD and aneurysm who underwent attempted ADO I device closure between 3/2004 and 8/2010 [Rady Children's Hospital-San Diego (n = 16), Mattel Children's Hospital at UCLA (n = 4), and Straub Hospital in Honolulu (n = 1)]. RESULTS: Twenty-one patients (16 male) with PMVSD and aneurysm underwent cardiac catheterization for the purpose of VSD closure using an ADO I. Nineteen patients had successful ADO I implantation with the following devices: 10/8 (n = 8), 8/6 (n = 7), 6/4 (n = 3) and 12/10 (n = 2). The procedure was aborted in 2 cases due to temporary AVB and ventricular ectopy during catheterization. One patient developed hemolysis due device malposition and had surgical device retrieval and VSD closure. One patient had new trivial tricuspid regurgitation and in 5/19 the tricuspid regurgitation improved post device closure. Transient accelerated junctional rhythm occurred within 24 hr in 4 patients and nonsustained slow ventricular tachycardia occurred in one patient. At latest follow-up (up to 6 years, median 1.9 years) 11/18 had no residual shunt, 4/18 had trivial and 3/18 small residual shunt. No AVB has been observed. CONCLUSIONS: Percutaneous closure of PMVSD with aneurysm using the ADO I appears to have acceptable efficacy and risk. Atrio-ventricular block in up to mid-term follow up was not encountered.

Transcatheter Systemic AV Valve-in-Valve Implantation in a Patient With LTGA and Ebstein Anomaly.

We present the first in-human transcatheter systemic atrioventricular valve-in-valve implantation in a 37-year-old patient with Ebstein anomaly, levo-transposition of the great arteries, and prior systemic valve replacement. She had severe bioprosthetic valve regurgitation and reduced systolic function. She had high surgical risk and was planned for transcatheter intervention. (Level of Difficulty: Advanced.).

Pediatric Surgical Pulmonary Valve Replacement Outcomes After Implementation of a Clinical Pathway.

BACKGROUND: Standardization of perioperative care can reduce resource utilization while improving patient outcomes. We sought to describe our outcomes after the implementation of a perioperative clinical pathway for pediatric patients undergoing elective surgical pulmonary valve replacement and compare these results to previously published national benchmarks. METHODS: A retrospective single-center descriptive study was conducted of all pediatric patients who underwent surgical pulmonary valve replacement from 2017 through 2020, after the implementation of a clinical pathway. Outcomes included hospital length of stay and 30-day reintervention, readmission, and mortality. RESULTS: Thirty-three patients (55% female, median age 11 [7, 13] years, 32 [23, 44] kg) were included in the study. Most common diagnosis and indication for surgery was Tetralogy of Fallot (61%) with pulmonary valve insufficiency (88%). All patients had prior cardiac surgery. Median hospital length of stay was 2 [2, 2] days, and longest length of stay was three days. There were no 30-day readmissions, reinterventions, or mortalities. Median follow-up time was 19 [9, 31] months. CONCLUSIONS: Formalization of a perioperative surgical pulmonary valve replacement clinical pathway can safely promote short hospital length of stay without any short-term readmissions or reinterventions, especially when compared with previously published benchmarks. Such formalization enables the dissemination of best practices to other institutions to reduce hospital length of stay and limit costs.

Uhl's Anomaly With Left Ventricular Noncompaction: Role of Multimodality Imaging in a Rare Association.

Uhl's anomaly is a rare congenital heart disease characterized by partial or complete absence of the right ventricle myocardium. We report the first case, in a 21-year-old man, of Uhl's anomaly-associated left ventricular noncompaction. This association represents a unique clinical entity and has important implications for management strategies. (Level of Difficulty: Intermediate.).

Occlusion of Sano conduit with the Amplatzer vascular plug: a reliable method for staged elimination of accessory pulmonary blood flow in single ventricle palliation.

BACKGROUND: Staged palliative surgery is often required to regulate pulmonary and systemic blood flow in children with complex congenital heart disease. Accessory sources of pulmonary blood flow have been utilized to provide satisfactory pulmonary blood flow after Glenn anastomosis in select patients. Prolonged exposure to increased flow can lead to elevated pulmonary arterial pressures and volume loading of the ventricle, potentially limiting candidacy for Fontan completion. OBJECTIVE: To review the feasibility, technique, and outcome of transcatheter closure of the Sano conduit utilizing the Amplatzer Vascular Plug (AVP). METHODS: All patients who underwent device closure of the Sano conduit with an AVP were reviewed retrospectively. Patient demographics, indications, procedural details, and patient outcomes were reviewed. Hemodynamic changes before and after occlusion of the Sano conduit were compared. A P value < 0.05 was considered significant. RESULTS: Between 2005 and 2009, 11 male patients underwent Sano occlusion with an AVP. Average weight was 8.8 ± 2.2 kg. The Sano was occluded from both antegrade (n = 6) and retrograde (n = 5) approaches. Arterial saturation and mean pulmonary artery pressure both decreased significantly after Sano occlusion [86.9 ± 4.9% to 82.6 ± 4.9% (P < 0.001) and 14.1 ± 5 mm Hg to 12.8 ± 4 mm Hg (P < 0.05), respectively]. Conduit diameter averaged 3.6 mm (range 3.1-4.4) 13 months after Stage I palliation. The conduit was closed using a 6 mm AVP in nine patients and a 4 mm AVP in the other two. The ratio of AVP size to conduit diameter was 1.5 ± 0.27. There were no procedural complications and all patients at least 2 years of age have undergone Fontan completion. CONCLUSIONS: The Sano conduit can be left open in select patients who do not tolerate closure after Glenn anastomosis. It can be closed safely and effectively using the AVP. © 2010 Wiley-Liss, Inc.

Treatment of pulmonary vein stenosis with expanded polytetrafluoroethylene covered stents.

Atrium expanded polytetrafluoroethylene covered stents were implanted in the stenotic pulmonary veins (n = 5) of three patients. Use of this device was feasible in infants and the procedure was well-tolerated without major complications. The majority (4/5) of covered stents remained patent for at least 3 months after implantation. Stents remain patent in the two surviving patients 6 months after deployment.

Novel Multidisciplinary Management of Acute Kidney Injury After Infant Orthotopic Heart Transplantation.

Acute kidney injury following orthotopic heart transplantation in pediatric recipients is often multifactorial, requiring balance of immune suppression, nephrotoxic medication exposure, nutrition, and fluid status. Therapeutic options are often limited by patient size and hemodynamic stability. We describe a four-month, 4.9-kg female bridged by mechanical circulatory support to transplant after failed stage 1 palliation secondary to recurrent aortic stenosis and severe ventricular dysfunction. Posttransplant, kidney injury was managed by transcatheter relief of central obstruction from an anastomotic stricture and continuous renal replacement therapy, allowing uninterrupted immune suppression, medication, and nutrition delivery until sufficient recovery of renal function.

Resource Allocation and Decision Making for Pediatric and Congenital Cardiac Catheterization During the Novel Coronavirus SARS-CoV-2 (COVID-19) Pandemic: A U.S. Multi-Institutional Perspective.

BACKGROUND: The novel coronavirus (COVID-19) pandemic has placed severe stress on healthcare systems around the world. There is limited information on current practices in pediatric cardiac catheterization laboratories in the United States (US). OBJECTIVES: To describe current practice patterns and make recommendations regarding potential resource allocation for congenital cardiac catheterization during the COVID-19 pandemic. METHODS: A web-based survey was distributed regarding case candidacy and catheterization laboratory preparedness. Centers were categorized based on the current degree of disease burden in that community (as of April 1, 2020). Data and consensus opinion were utilized to develop recommendations. RESULTS: Respondents belonged to 56 unique US centers, with 27 (48.2%) located in counties with a high number of COVID-19 cases. All centers have canceled elective procedures. There was relative uniformity (>88% agreement) among centers as to which procedures were considered elective. To date, only three centers have performed a catheterization on a confirmed COVID-19 positive patient. Centers located in areas with a higher number of COVID-9 cases have been more involved in a simulation of donning and doffing personal protective equipment (PPE) than low-prevalence centers (46.7% vs 10.3%, respectively; P<.001). Currently, only a small fraction of operators has been reassigned to provide clinical services outside their scope of practice. CONCLUSIONS: At this stage in the COVID-19 pandemic, pediatric/congenital catheterization laboratories have dramatically reduced case volumes. This document serves to define current patterns and provides guidance and recommendations on the preservation and repurposing of resources to help pediatric cardiac programs develop strategies for patient care during this unprecedented crisis.

Electrosurgical energy in combination with a transseptal needle: a novel method for the creation of an atrial communication in hypoplastic left heart syndrome with intact atrial septum.

Neonates with hypoplastic left heart syndrome (HLHS) and intact atrial septum (IAS) usually present with severe acidosis and hypoxemia that necessitate immediate intervention to create an atrial septal defect (ASD). Transcatheter creation of an ASD in these patients requires transseptal puncture of a thickened atrial septum in the setting of a very small left atrium. We report on a novel method of perforating the atrial septum using radio-frequency energy in combination with a transseptal needle to facilitate transseptal puncture and subsequent stent placement in a very thick atrial septum of a newborn with HLHS/IAS and a small left atrium.

Decreasing number of deaths of infants with hypoplastic left heart syndrome.

OBJECTIVE: To assess mortality rates and demographic characteristics for infants with hypoplastic left heart syndrome (HLHS) in California. STUDY DESIGN: We used California death registry files from 1990 to 2004 to compare overall mortality and demographic characteristics between infants with HLHS (n = 856) who received surgical intervention and those who received comfort care. The California discharge database was used to calculate the annual incidence of disease and survival rates for infants with HLHS undergoing surgery between 1995 and 1999. RESULTS: The annual number of deaths for infant with HLHS decreased by nearly 50% over the study period, even though the incidence of the disease remained constant during this period. For all deaths, the proportion of infants receiving comfort care decreased significantly over time compared with those infants who underwent surgery. Although the total number of deaths in infants with HLHS who underwent surgical intervention increased, the mortality rate for this cohort decreased. Interstage unexpected mortality and the median age at death both increased in the infants who underwent surgery. CONCLUSIONS: Over the study period of 1990 to 2004 in California, fewer families chose comfort care for infants diagnosed with HLHS, and the number of deaths for those infants who underwent surgical intervention increased. These changes likely reflect improved treatment outcomes and an increased number of families desiring surgical intervention in higher-risk infants.