Cardiac Catheterization Interventions in the Right Ventricular Outflow Tract and Branch Pulmonary Arteries Following the Arterial Switch Operation.

The arterial switch operation for d-transposition of the great arteries achieves anatomic repair but creates the potential for right ventricular outflow tract obstruction as a result of the LeCompte maneuver. The resultant right ventricular hypertension is generally well tolerated but a select group are referred for cardiac catheterization. The outcomes of these catheterizations have not been well described. The objective of this study was to describe the degree and nature of right ventricular outflow tract obstruction found during cardiac catheterization among patients following the arterial switch operation as well as determine the rate of intervention and assess the acute impact of any catheter intervention undertaken. We conducted a retrospective study of patients after arterial switch operation with the LeCompte maneuver and subsequent right heart catheterization. Descriptive statistics were reported, and paired sample t tests were used for analysis. 544 children had an arterial switch operation, of which 110 children (20%) had a cardiac catheterization procedure after surgery and 11% had a right heart catheterization. Of the right heart catheterizations, 90% had an intervention (balloon and/or stent). In the interventional group, the right ventricle to systemic pressure ratio decreased modestly, from 2/3 to half systemic, after balloon dilation and/or stent placement (p < 0.01). No serious complications were observed.

Closing the gap between acceptable and ideal in catheterisation for paediatric and congenital heart disease-A global view.

In recent issues of the Journal of the Society for Cardiovascular Angiography and Interventions and the Journal of the American College of Cardiology: Cardiovascular Interventions, Holzer and colleagues presented an Expert Consensus Document titled: "PICS / AEPC / APPCS / CSANZ / SCAI / SOLACI: Expert consensus statement on cardiac catheterization for pediatric patients and adults with congenital heart disease." This Expert Consensus Document is a massively important contribution to the community of paediatric and congenital cardiac care. This document was developed as an Expert Consensus Document by the Pediatric and Congenital Interventional Cardiovascular Society, the Association for European Paediatric and Congenital Cardiology, the Asia-Pacific Pediatric Cardiac Society, the Cardiac Society of Australia and New Zealand, the Society for Cardiovascular Angiography and Interventions, and the Latin American Society of Interventional Cardiology, as well as the Congenital Cardiac Anesthesia Society and the American Association of Physicists in Medicine.As perfectly stated in the Preamble of this Expert Consensus Document, "This expert consensus document is intended to inform practitioners, payors, hospital administrators and other parties as to the opinion of the aforementioned societies about best practices for cardiac catheterisation and transcatheter management of paediatric and adult patients with congenital heart disease, with added accommodations for resource-limited environments." And, the fact that the authorship of this Expert Consensus Document includes global representation is notable, commendable, and important.This Expert Consensus Document has the potential to fill an important gap for this patient population. National guideline documents for specific aspects of interventions in patients with paediatric heart disease, including training guidelines, do exist. However, this current Expert Consensus Document authored by Holzer and colleagues provides truly globally applicable standards on cardiac catheterisation for both paediatric patients and adults with congenital heart disease (CHD).Our current Editorial provides different regional perspectives from senior physicians dedicated to paediatric and congenital cardiac care who are practicing in Europe, the Asia-Pacific region, Latin America, Australia/New Zealand, and North America. Establishing worldwide standards for cardiac catheterisation laboratories for children and adults with CHD is a significant stride towards improving the quality and consistency of care. These standards should not only reflect the current state of medical knowledge but should also be adaptable to future advancements, ultimately fostering better outcomes and enhancing the lives of individuals affected by CHD worldwide.Ensuring that these standards are accessible and adaptable across different healthcare settings globally is a critical step. Given the variability in resources and infrastructure globally, the need exists for flexibility and tailoring to implement recommendations.The potential impact of the Expert Consensus Document and its recommendations is likely significant, but heterogeneity of healthcare systems will pose continuing challenges on healthcare professionals. Indeed, this heterogeneity of healthcare systems will challenge healthcare professionals to finally close the gap between acceptable and ideal in the catheterisation of patients with paediatric and/or congenital heart disease.

Applying Practice Analysis to Develop a New Test Content Outline for the Pediatric Cardiology Certification Examination.

The evolving breadth and complexity of the contemporary pediatric cardiology specialty requires regular, systematic analysis of the practice to ensure that training and certification requirements address the demands of real-world clinical experience. We report the process of the American Board of Pediatrics (ABP) for conducting such a practice analysis and revising the test content outline (TCO) for the pediatric cardiology subspecialty certification exam. A panel of 15 pediatric cardiologists conducted seven 2-h virtual meetings, during which they identified 37 unique tasks that represent the work a pediatric cardiologist may reasonably expect to perform within the first 5 years after training. These tasks were grouped into nine performance domains, similar to the entrustable professional activities (EPA), previously endorsed by the ABP in collaboration with the pediatric cardiology education community, and which represent the critical activities of the profession. The panel then enumerated the knowledge, skills, and abilities necessary to perform each task. These deliberations resulted in two work products: a practice analysis document (PAD) and subspecialty board TCO based on testable knowledge, skills, and abilities. Survey assessments of the panel's work were then distributed to pediatric cardiology fellowship program directors and to practicing pediatric cardiologists for their input, which largely aligned with the panel's recommendations. Survey responses were considered in the final revisions of the PAD and TCO. This approach to practice analysis proved to be an efficient process for describing the work performed by today's pediatric cardiologists and the knowledge, skills, and abilities needed to competently perform that work.

Late gestation predictors of a postnatal biventricular circulation after fetal aortic valvuloplasty.

OBJECTIVES: Fetal aortic valvuloplasty (FAV) for severe aortic stenosis (AS) has shown promise in averting progression to hypoplastic left heart syndrome. After FAV, predicting which fetuses will achieve a biventricular (BiV) circulation after birth remains challenging. Identifying predictors of postnatal circulation on late gestation echocardiography will improve parental counseling. METHODS: Liveborn patients who underwent FAV and had late gestation echocardiography available were included (2000-2017, n = 96). Multivariable logistic regression and classification and regression tree analysis were utilized to identify independent predictors of BiV circulation. RESULTS: Among 96 fetuses, 50 (52.1%) had BiV circulation at the time of neonatal discharge. In multivariable analysis, independent predictors of biventricular circulation included left ventricular (LV) long axis z-score (OR 3.2, 95% CI 1.8-5.7, p < 0.001), LV ejection fraction (OR 1.3, 95% CI 1.0-1.8, p = 0.023), anterograde aortic arch flow (OR 5.0, 95% CI 1.2-20.4, p = 0.024), and bidirectional or right-to-left foramen ovale flow (OR 4.6, 95% CI 1.4-15.8, p = 0.015). CONCLUSION: Several anatomic and physiologic parameters in late gestation were found to be independent predictors of BiV circulation after FAV. Identifying these predictors adds to our understanding of LV growth and hemodynamics after FAV and may improve parental counseling.

Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era.

Scoliosis is common in children with congenital heart disease (CHD) and may have deleterious effects on quality of life and hemodynamics. Relatively little is known about the outcomes of spinal fusion for scoliosis repair in children with complex CHD. We reviewed all cases of children with CHD undergoing first time spinal fusion excluding those with minor CHD between 1995 and 2015. Seventy-eight patients were identified and included in the study. 97.4% of patients included had undergone prior cardiac surgery and sixteen patients had single ventricle circulations. 17.9% of patients experienced a significant perioperative event defined as an aggregate of the presence of any of the following: need for early unanticipated reoperation, neurologic deficit, postoperative bleeding requiring intervention, end organ dysfunction, or death. There were no deaths in our cohort. 38.5% of patients experienced any adverse event, the majority of which were related to perioperative fluid shifts. Larger preoperative Cobb angle and longer length of spinal fusion were associated with increased risk of significant perioperative event while larger preoperative Cobb angle and longer length of spinal fusion, older age at time of surgery, single ventricle circulation, cyanosis and patients taking cardiac medications at the time of surgery were more likely to experience any adverse event. Operative repair of scoliosis in children with complex CHD has been performed without mortality over a 20-year period in a single institution, albeit with a higher rate of perioperative complication than is seen in the general pediatric population. Patients with large preoperative Cobb angles and cyanotic single ventricle circulations appear to be at the highest risk for perioperative complications.

Adjunct Targeted Biologic Inhibition Agents to Treat Aggressive Multivessel Intraluminal Pediatric Pulmonary Vein Stenosis.

OBJECTIVE: To evaluate the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis (PVS). We describe the 48- and 72-week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal PVS. STUDY DESIGN: This single-arm, prospective, open-label US Food and Drug Administration approved trial enrolled patients with ≥2 affected pulmonary veins after surgical or catheter-based relief of obstruction between March 2009 and December 2014. Drug therapy was discontinued at 48 weeks, or after 24 weeks of stabilization, whichever occurred later. RESULTS: Among 48 enrolled patients, 5 had isolated PVS, 26 congenital heart disease, 5 lung disease, and 12 both. After the 72-week follow-up, 16 patients had stabilized, 27 had recurred locally without stabilization, and 5 had progressed. Stabilization was associated with the absence of lung disease (P = .03), a higher percentage of eligible drug doses received (P = .03), and was not associated with age, diagnosis, disease laterality, or number of veins involved. Survival to 72 weeks was 77% (37 of 48). Adverse events were common (n = 1489 total), but only 16 were definitely related to drug treatment, none of which were serious. CONCLUSION: Survival to 72 weeks was 77% in a referral population with multivessel intraluminal PVS undergoing multimodal treatment, including antiproliferative tyrosine kinase blockade. Toxicity specific to tyrosine kinase blockade was minimal.

Early hemodynamic changes after fetal aortic stenosis valvuloplasty predict biventricular circulation at birth.

OBJECTIVE: To describe the early hemodynamic changes after fetal aortic valvuloplasty (FAV) for evolving hypoplastic left heart syndrome due to mid-gestational aortic stenosis and to assess whether these early changes predict biventricular (BiV) circulation at neonatal discharge. METHOD: We retrospectively reviewed all technically successful FAV cases resulting in live birth between 2000 and 2015 (n = 93, 45% BiV circulation at neonatal discharge). Paired testing methods were used to compare pre-intervention and post-intervention measures of left ventricular hemodynamics. Logistic regression was used to determine whether these changes were predictive of post-natal outcome. RESULTS: Measures of left heart physiology were markedly abnormal pre-FAV and improved significantly post-FAV. No subjects had systolic antegrade transverse aortic arch flow pre-FAV and 65% of subjects had antegrade flow post-FAV. The number of subjects with abnormal left-to-right patent foramen ovale flow decreased, and the number with biphasic mitral valve inflow increased. The median left ventricular ejection fraction improved after intervention. Amongst the pre-post changes, gaining partially or exclusively antegrade systolic arch flow was the most significant independent predictor of BiV circulation (OR 9.80 and 19.83, respectively, both P < 0.001). CONCLUSION: Technically successful FAV is associated with immediate improvements in left heart physiology that are predictive of BiV circulation at neonatal discharge.

Echocardiographic predictors of neonatal illness severity in fetuses with critical left heart obstruction with intact or restrictive atrial septum.

BACKGROUND: Neonates with critical left heart obstruction and intact atrial septum (IAS) or restrictive atrial septum (RAS) are at risk for hypoxia within hours of birth and remain a group at high risk for mortality. METHODS: Prenatally diagnosed fetuses with critical left heart obstruction and IAS or RAS with follow-up from January 1, 2005, to February 14, 2017, were included. Primary outcome was a composite measure of severe neonatal illness (pH < 7.15, venous pH < 7.10, bicarbonate < 16 mmol/L, lactic acid > 5 mmol/L, or median oxygen saturation < 60% within 2 hours of birth). RESULTS: Of 68 live born fetuses, 52 (76.5%) had hypoplastic left heart syndrome, 14 (20.5%) had critical aortic stenosis, and two (3%) had complex anatomy with mitral stenosis/atresia. There were 27 (39.7%) fetuses with IAS and 41 (60.3%) with RAS. Severe neonatal illness was present in 36 (52.9%). The strongest discriminators for severe neonatal illness were a pulmonary vein A:R VTI ≤ 2.7 (P < 0.001, AUC 0.93) and larger pulmonary vein diameter (P = 0.025, AUC 0.77). A:R VTI ≤ 2.7 predicted death or transplant (log-rank P = 0.03). CONCLUSIONS: In neonates with hypoplastic left heart syndrome and IAS or RAS, A:R VTI ≤ 2.7 is predictive of severe neonatal instability. This threshold can help guide resource planning, delivery management, and improve fetal intervention criteria.

Transcatheter Intervention of Coronary Obstructions in Infants, Children, and Young Adults.

This study assesses the characteristics of coronary obstructions that underwent transcatheter intervention in a pediatric catheterization laboratory, the procedural details, and patient outcomes. Acute cardiac failure due to coronary obstructions in children is rare. The role of catheter based intervention is largely unreported. Single center retrospective review between January 2000 and December 2016. Thirty-three patients (median age 2y/o [0-38], weighing 9.6 kg [2.2-91]) underwent 39 transcatheter interventions on 39 lesions, mainly left main coronary (16/39; 39%) and right coronary (9/39; 23%) arteries. Most patients had congenital heart disease (29/33; 88%). Cath indications included ventricular dysfunction (17), cardiac arrest (7), failure to wean from cardiopulmonary bypass (5), and other (4). Almost half (18/39; 46%) were performed on ECMO support. Obstructions were post-surgical (16; 4 with coronary manipulation), thrombotic (13; 5 < 30 days from cardiac surgery), and miscellaneous lesions (10). Interventions included 25 bare metal stents implanted in 22 lesions in 17 patients (mainly post-surgical lesions; 3 at Damus-Kaye-Stansel anastomosis), nine balloon angioplasty only, four lytic therapy ± mechanical disruption of thrombus, and four technical failures. There were no procedure-related deaths. Most patients survived to discharge or transplant (24/33; 73%). Six patients who received stents had follow-up catheterization (median 15.5 months [1-106]); all were without restenosis. Most coronary obstructions intervened upon in a pediatric cath lab were on young, critically ill patients with congenital heart disease secondary to surgical manipulation/injury or thrombosis. Transcatheter intervention should be considered a potential treatment strategy in this population.

Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome.

OBJECTIVE: To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment. STUDY DESIGN: Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory. RESULTS: Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P < .001) and similar to published reports in patients with hypoplastic left heart syndrome without fetal intervention; scores in the single ventricular versus biventricular group were 97 ± 19 vs 89 ± 14, respectively (P = .10). On multivariable analysis, independent predictors of a lower General Adaptive Composite score were total hospital duration of stay in the first year of life (P = .001) and, when forced into the model, biventricular status (P = .02). For all other neurodevelopmental questionnaires (Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar. CONCLUSION: Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes.

Transcatheter Pulmonary Valve Replacement and Acute Increase in Diastolic Pressure are Associated with Increases in Both Systolic and Diastolic Pulmonary Artery Dimensions.

Stable positioning of a transcatheter pulmonary valve (TPV) in native outflow tracts depends on a clear understanding of underlying anatomy and outflow tract dimensions. We hypothesized that restoration of pulmonary competence may acutely alter these dimensions. A retrospective single-center review of consecutive patients after TPV placement from 2007 to 2014 was performed. Patients with less than moderate pulmonary regurgitation were excluded. We reviewed acute catheterization data on 46 patients, most with tetralogy of Fallot (70%). Baseline and post-implant (7.5 ± 3 min post-deployment) measurements of central pulmonary arteries (PAs) were determined angiographically. The right PA diameter increased (20 ± 4-24 ± 6 mm systole*, 16 ± 4-21 ± 6 mm diastole*), as did the left PA (20 ± 6-24 ± 8 mm systole*, 16 ± 5-21 ± 7 mm diastole*). PA pressures increased from averages of 29.3/10.6 (17) to 29.8/15.1 (21) mmHg. We noted that pre-implant systolic PA diameter correlated with diastolic PA diameter post-implant (r = 0.9). On follow-up catheterization in seven patients [median 3 years; (1-8)], combined central PA diameter decreased an average of 20% (systole: 20% ± 12, diastole: 18% ± 11) as compared to post-implant measurements. Acute pulmonary valve competence in patients with at least moderate pulmonary regurgitation results in an immediate increase in PA diameter (20% systole and 30% diastole). The cause of this diameter change is unclear. This acute change may have implications for device and patient selection (*p < 0.001).

Is Endomyocardial Biopsy a Safe and Useful Procedure in Children with Suspected Cardiomyopathy?

Endomyocardial biopsy (EMB) is a common procedure used to aid in the diagnosis, prognosis and treatment of suspected pediatric cardiomyopathy. In suspected cardiomyopathy, no multicenter experience has previously reported on the safety and utility of EMBs. Retrospectively, adverse event (AE) and patient and procedural characteristics were obtained at seven institutions participating in the Congenital Cardiac Catheterization Outcomes Project for both a cardiomyopathy (n = 158) and a post-transplant surveillance (n = 2665) cohort. Descriptive information regarding biopsy indication, pathology and clinical management based on EMB findings were retrospectively obtained. High-severity AEs were more common in the cardiomyopathy cohort when compared to the post-transplant surveillance cohort. The cardiomyopathy cohort was younger, more hemodynamically vulnerable and required more cardiorespiratory support during the procedure. The eight high-severity AEs in the cardiomyopathy group included one myocardial perforation, two ECMO cannulations and three deaths following the EMB. Factors associated with high-severity AEs included performing another catheter-based intervention during the EMB and longer fluoroscopy time. Notably, an increased number of biopsy attempts did not increase the risk of an AE. Suspected myocarditis was the most common indication. Diagnostic EMB pathology and thus alteration to clinical management based on pathology occurred more frequently in patients with suspected myocarditis. In conclusion, there is an increased incidence of high-severity AEs in patients undergoing EMB for suspected cardiomyopathy. EMB may be more clinically useful in the management of suspected myocarditis. The increased risk of high-severity AEs when additional interventions are performed highlights the hemodynamic vulnerability in patients with suspected cardiomyopathy.

Systemic rapamycin to prevent in-stent stenosis in peripheral pulmonary arterial disease: early clinical experience.

OBJECTIVES: We have taken a novel approach using oral rapamycin - sirolimus - as a medical adjunct to percutaneous therapy in patients with in-stent stenosis and high risk of right ventricular failure. BACKGROUND: Peripheral pulmonary artery stenosis can result in right ventricular hypertension, dysfunction, and death. Percutaneous pulmonary artery angioplasty and stent placement acutely relieve obstructions, but patients frequently require re-interventions due to re-stenosis. In patients with tetralogy of Fallot or arteriopathy, the problem of in-stent stenosis contributes to the rapidly recurrent disease. METHODS: Rapamycin was administered to 10 patients (1.5-18 years) with peripheral pulmonary stenosis and in-stent stenosis and either right ventricular hypertension, pulmonary blood flow maldistribution, or segmental pulmonary hypertension. Treatment was initiated around the time of catheterisation and continued for 1-3 months. Potential side-effects were monitored by clinical review and blood tests. RESULTS: Target serum rapamycin level (6-10 ng/ml) was accomplished in all patients; eight of the nine patients who returned for clinically indicated catheterisations demonstrated reduction in in-stent stenosis, and eight of the 10 patients experienced no significant side-effects. Among all, one patient developed diarrhoea requiring drug discontinuation, and one patient experienced gastrointestinal bleeding while on therapy that was likely due to an indwelling feeding tube and this patient tolerated rapamycin well following tube removal. CONCLUSIONS: Our initial clinical experience supports that patients with peripheral pulmonary artery stenosis can be safely treated with rapamycin. Systemic rapamycin may provide a novel medical approach to reduce in-stent stenosis.

Monitoring the health of the work environment with a daily assessment tool: the REAL - Relative Environment Assessment Lens - indicator.

BACKGROUND: Evidence shows that the health of the work environment impacts staff satisfaction, interdisciplinary communication, and patient outcomes. Utilising the American Association of Critical-Care Nurses' Healthy Work Environment standards, we developed a daily assessment tool. METHODS: The Relative Environment Assessment Lens (REAL) Indicator was developed using a consensus-based method to evaluate the health of the work environment and to identify opportunities for improvement from the front-line staff. A visual scale using images that resemble emoticons was linked with a written description of feelings about their work environment that day, with the highest number corresponding to the most positive experience. Face validity was established by seeking staff feedback and goals were set. RESULTS: Over 10 months, results from the REAL Indicator in the cardiac catheterisation laboratory indicated an overall good work environment. The goal of 80% of the respondents reporting their work environment to be "Great", "Good", or "Satisfactory" was met each month. During the same time frame, this goal was met four times in the cardiovascular operating room. On average, 72.7% of cardiovascular operating room respondents reported their work environment to be "Satisfactory" or better. CONCLUSION: The REAL Indicator has become a valuable tool in assessing the specific issues of the clinical area and identifying opportunities for improvement. Given the feasibility of and positive response to this tool in the cardiac catheterisation laboratory, it has been adopted in other patient-care areas where staff and leaders believe that they need to understand the health of the environment in a more specific and frequent time frame.

Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: postnatal outcomes of the first 100 patients.

BACKGROUND: Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported. METHODS AND RESULTS: We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank P=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, -1.3 to +8.2), and 80% had normal ejection fraction. CONCLUSIONS: Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted.

Balloon valvuloplasty for congenital aortic stenosis: Multi-center safety and efficacy outcome assessment.

OBJECTIVE: To describe contemporary outcomes of balloon aortic valvuloplasty (BAVP) performed in 22 US centers. BACKGROUND: BAVP constitutes first-line therapy for congenital aortic stenosis (cAS) in many centers. METHODS: We used prospectively-collected data from two active, multi-institutional, pediatric cardiac catheterization registries. Acute procedural success was defined, for purposes of this review, as a residual peak systolic gradient≤35 mm Hg and no more than mild aortic regurgitation (AR) for patients with isolated cAS. For patients with mixed aortic valve disease, a residual peak systolic gradient≤35 mm Hg without worsening of AR was considered successful outcome. RESULTS: In 373 patients with a median age of 8 months (1 day to 40 years of age) peak systolic gradient had a median of 59 [50, 71] mm Hg pre-BAVP and 22 [15, 30] mm Hg post-BAVP (P<0.001). Procedural success was achieved in 160 patients (71%). The factors independently associated with procedural success were: first time intervention (OR=2.0 (1.0, 4.0) P=0.04), not-prostaglandin dependent, (OR=3.5 (1.5, 8.1); P=0.003), and isolated cAS (absence of AR) (OR=2.1 (1.1-3.9); P=0.03). Twenty percent of patients experienced adverse events, half of which were of high severity. There was no procedural mortality. Neonatal status was the only factor associated with increased risk of high severity adverse events (OR 3.7; 95% CI 1.5-9.0). CONCLUSION: In the current era, BAVP results in procedural success (gradient reduction with minimal increase in AR) in 71% of patients treated at US centers where BAVP is considered first-line therapy relative to surgery.

Percutaneous left atrial decompression in patients supported with extracorporeal membrane oxygenation for cardiac disease.

OBJECTIVES: Left atrial decompression using cardiac catheterization techniques has been described at centers with extracorporeal membrane oxygenation programs. Left atrial decompression can decrease cardiogenic edema, minimize ventricular distension, and allow myocardial recovery. We describe Boston Children's Hospital's experience with percutaneous left atrial decompression techniques, acute outcomes, and clinical impact of left atrial decompression in extracorporeal membrane oxygenation patients. SUBJECTS: Patients supported with extracorporeal membrane oxygenation undergoing percutaneous left atrial decompression were identified and assigned to two groups 1) myocarditis/suspected myocarditis or 2) nonmyocarditis cardiac disease. INTERVENTIONS: Three techniques including vent placement, static balloon dilation, and stent implantation were used. MEASUREMENTS AND MAIN RESULTS: Change in left atrial pressure and severity of pulmonary edema on chest radiography pre and post procedure, impact of timing and technique of left atrial decompression on resolution of left atrial hypertension, and extracorporeal membrane oxygenation survival were evaluated. Furthermore, we evaluated the presence of residual atrial septal defect during follow-up. Percutaneous left atrial decompression was performed in 44 of 419 extracorporeal membrane oxygenation cases (10.5%) and was frequently used for myocarditis (22 of 44 patients; 50%). Techniques included 25 vents, 17 static balloon dilations, and two stents. All techniques were equally successful and significantly reduced left atrial pressure and pulmonary edema. Survival to hospital discharge was not associated with extracorporeal membrane oxygenation duration prior to left atrial decompression, change in left atrial pressure, or technique used. Persistent atrial septal defect was noted in five surviving patients (excluding transplant recipients and deceased), two required closure. CONCLUSIONS: Left atrial decompression can be performed effectively in children on extracorporeal membrane oxygenation using various percutaneous techniques. Reduction in pulmonary venous congestion is usually evident by chest radiography within 48 hours of intervention. Persistent atrial septal defect may require closure at the time of extracorporeal membrane oxygenation decannulation or during long-term follow-up.

Systemic Embolic Complications of Pulmonary Vein Angioplasty in Children.

Pulmonary vein stenosis (PVS) carries significant morbidity and mortality for affected children, and its management often requires multiple angioplasty procedures. PVS angioplasty can be complicated by systemic embolic events such as stroke, but incidence and risk factors are poorly understood. We reviewed pediatric catheterizations involving PVS angioplasty and/or stent placement performed at Boston Children's Hospital between July 2005 and February 2014. A total of 406 cases were performed in 144 distinct patients. Patients underwent a median of two catheterizations, at median age 1 year and weight 6.9 kg. Eleven (2.7 %) catheterizations were complicated by clinically apparent systemic embolic events, comprising 10 strokes (one with associated hepatic embolism) and 1 renal infarct. Prevalence of clinically evident stroke among this cohort was 7.6 %. Using a prior (uncomplicated) catheterization to allow each patient to serve as their own control, we sought to identify potentially modifiable risk factors for systemic embolic events. Although this analysis was limited by low power, complicated and uncomplicated angioplasties did not appear to differ in case time, contrast dose, anticoagulation management, use of cutting balloons, number of catheter exchanges, or size of long sheath used. Significant non-embolic adverse events were common, occurring in 25 % of catheterizations. Systemic embolism appears to complicate PVS angioplasty at a rate much higher than that described for other congenital catheterizations. This risk may be inherent to the procedure rather than related to any modifiable or operator-dependent factors.

Left Ventricular Dysfunction Following Neonatal Pulmonary Valve Balloon Dilation for Pulmonary Atresia or Critical Pulmonary Stenosis.

Pulmonary valve (PV) balloon dilation (BD) is the primary therapy for infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS). We observed left ventricular (LV) dysfunction in patients following BD and sought to determine its incidence, clinical course and associated risk factors. Clinical, echocardiographic and catheterization data for all patients who underwent neonatal (<2 weeks age) PV BD for critical PS or PAIVS between January 2000 and February 2014 were retrospectively analyzed (n = 129). Post-procedure LV dysfunction was defined as ejection fraction (EF) <54 %. Median age at PV BD was 1 day. Most (71 %) patients had critical PS. Median PV diameter pre-BD was 6.0 mm with PV z-scores -4.1 to 0.9, median LV EF pre-BD was 58 %. Post-BD LV dysfunction developed in 45 patients (35 %); 15 patients had LV EF ≤40 %. Median time to normalization of LV EF was 10 days (range 2-72). In univariate analysis, diagnosis (critical PS or PAIVS), right ventricle to LV pressure ratio pre-BD, acute procedural complication and post-BD inotropic support were not associated with post-BD LV dysfunction. In multivariable analysis, the predictors of post-procedure LV dysfunction were lower PV z-score (OR 1.81, p 0.04), tricuspid regurgitation pre-BD ≥ moderate (OR 3.73, p 0.008) and larger right ventricular apical area (OR 1.99, p 0.04). LV dysfunction post-neonatal PV BD develops in a significant number of patients (35 %) and can be severe, but resolves. The risk of developing LV dysfunction post-PV BD is highest in patients with larger right ventricles.

Development of a charge adjustment model for cardiac catheterization.

A methodology that would allow for comparison of charges across institutions has not been developed for catheterization in congenital heart disease. A single institution catheterization database with prospectively collected case characteristics was linked to hospital charges related and limited to an episode of care in the catheterization laboratory for fiscal years 2008-2010. Catheterization charge categories (CCC) were developed to group types of catheterization procedures using a combination of empiric data and expert consensus. A multivariable model with outcome charges was created using CCC and additional patient and procedural characteristics. In 3 fiscal years, 3,839 cases were available for analysis. Forty catheterization procedure types were categorized into 7 CCC yielding a grouper variable with an R (2) explanatory value of 72.6%. In the final CCC, the largest proportion of cases was in CCC 2 (34%), which included diagnostic cases without intervention. Biopsy cases were isolated in CCC 1 (12%), and percutaneous pulmonary valve placement alone made up CCC 7 (2%). The final model included CCC, number of interventions, and cardiac diagnosis (R (2) = 74.2%). Additionally, current financial metrics such as APR-DRG severity of illness and case mix index demonstrated a lack of correlation with CCC. We have developed a catheterization procedure type financial grouper that accounts for the diverse case population encountered in catheterization for congenital heart disease. CCC and our multivariable model could be used to understand financial characteristics of a population at a single point in time, longitudinally, and to compare populations.