Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome: 2021 vs 2011.

We sought to examine current practices and changes in practice regarding initial counseling for families of patients with hypoplastic left heart syndrome (HLHS) given the evolution of options and outcomes over time. Counseling (Norwood with Blalock-Taussig-Thomas shunt (NW-BTT), NW with right ventricle to pulmonary artery conduit (NW-RVPA), hybrid palliation, heart transplantation, or non-intervention/hospice (NI)) for patients with HLHS were queried via questionnaire of pediatric care professionals in 2021 and compared to identical questionnaire from 2011. Of 322 respondents in 2021 (39% female), 299 respondents were cardiologists (92.9%), 17cardiothoracic surgeons (5.3%), and 6 were nurse practitioners (1.9%). Respondents were largely from North America (96.9%). In 2021, NW-RVPA procedure was the preferred palliation for standard risk HLHS patient (61%) and was preferred across all US regions (p < 0.001). NI was offered as an option by 71.4% of respondents for standard risk patients and was the predominant strategy for patients with end-organ dysfunction, chromosomal abnormality, and prematurity (52%, 44%, and 45%, respectively). The hybrid procedure was preferred for low birth-weight infants (51%). In comparison to the identical 2011 questionnaire (n = 200), the NW-RVPA was endorsed more in 2021 (61% vs 52%, p = 0.04). For low birth-weight infants, hybrid procedure was more recommended than in 2011 (51% vs 21%, p < 0.001). The NW-RVPA operation is the most recommended strategy throughout the US for infants with HLHS. The hybrid procedure for low birth-weight infants is increasingly recommended. NI continues to be offered even in standard risk patients with HLHS.

Coronavirus Disease 2019 (COVID-19) Pandemic Implications in Pediatric and Adult Congenital Heart Disease.

The corona virus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given the increased risk for severe COVID-19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID-19 include myocarditis, arrhythmia and myocardial infarction. Importantly, the pandemic has stretched health care systems and many care team members are at risk for contracting and possibly transmitting the disease which may further impact the care of patients with cardiovascular disease. In this review, we describe the effects of COVID-19 in the pediatric and young adult population and review the cardiovascular involvement in COVID-19 focusing on implications for patients with congenital heart disease in particular.

Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results.

INTRODUCTION: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring. METHODS: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues. RESULTS: Comprehensive Single Ventricle Team in 2014 was begun, to standardize care for children with single ventricle heart defects from diagnosis to adulthood within our institution. The team is a multidisciplinary group of providers committed to improving outcomes and quality of life for children with single ventricle heart defects, all functioning within the medical home of our heart center. Standards of care were developed and implemented in five target areas to standardize medical management and patient and family support. Under the team 100 patients have been cared for. Since 2014 a decrease in interstage mortality for HLHS were seen. Using a team approach and the tools of Quality Improvement they have been successful in reaching high protocol compliance for each of these areas. CONCLUSIONS: This article describes the process of building a successful Single Ventricle team, our initial results, and lessons learned. Additional study is ongoing to demonstrate the effects of these interventions on patient outcomes.

Defining pediatric inpatient cardiology care delivery models: A survey of pediatric cardiology programs in the USA and Canada.

BACKGROUND: The treatment of children with cardiac disease is one of the most prevalent and costly pediatric inpatient conditions. The design of inpatient medical services for children admitted to and discharged from noncritical cardiology care units, however, is undefined. North American Pediatric Cardiology Programs were surveyed to define noncritical cardiac care unit models in current practice. METHOD: An online survey that explored institutional and functional domains for noncritical cardiac care unit was crafted. All questions were multi-choice with comment boxes for further explanation. The survey was distributed by email four times over a 5-month period. RESULTS: Most programs (n = 45, 60%) exist in free-standing children's hospitals. Most programs cohort cardiac patients on noncritical cardiac care units that are restricted to cardiac patients in 39 (54%) programs or restricted to cardiac and other subspecialty patients in 23 (32%) programs. The most common frontline providers are categorical pediatric residents (n = 58, 81%) and nurse practitioners (n = 48, 67%). However, nurse practitioners are autonomous providers in only 21 (29%) programs. Only 33% of programs use a postoperative fast-track protocol. When transitioning care to referring physicians, most programs (n = 53, 72%) use facsimile to deliver pertinent patient information. Twenty-two programs (31%) use email to transition care, and eighteen (25%) programs use verbal communication. CONCLUSION: Most programs exist in free-standing children's hospitals in which the noncritical cardiac care units are in some form restricted to cardiac patients. While nurse practitioners are used on most noncritical cardiac care units, they rarely function as autonomous providers. The majority of programs in this survey do not incorporate any postoperative fast-track protocols in their practice. Given the current era of focused handoffs within hospital systems, relatively few programs utilize verbal handoffs to the referring pediatric cardiologist/pediatrician.

Echocardiographic evaluation of the single right ventricle in congenital heart disease: results of new techniques.

Right ventricular (RV) function is increasingly recognized as having prognostic significance in various disease processes. The current gold standard for noninvasive measurement of RV function is cardiac magnetic resonance imaging; however, because of practical considerations, echocardiography remains the most often used modality for evaluating the RV. In the past, because of its complex morphology, echocardiographic assessment of the RV was usually qualitative in nature. Current advances in echocardiographic techniques have been able to overcome some of the previous limitations and thus quantification of RV function is increasingly being performed. In addition, recent echocardiographic guidelines for evaluating the RV have been published to aid in standardizing practice. The evaluation of RV function almost certainly has no greater importance than in the congenital heart population, especially in those patients that have a single RV acting as the systemic ventricle. As this complex population continues to increase in number, accurate and precise evaluation of RV function will be a major issue in determining clinical care.

Initial counseling prior to palliation for hypoplastic left heart syndrome.

OBJECTIVE: Multiple surgical approaches to the initial palliation of patients with hypoplastic left heart syndrome (HLHS) have been advocated throughout the years. We sought to examine what procedure, if any, is recommended for HLHS management in regard to physician preference, anatomical variations, and concomitant medical issues. PATIENTS AND METHODS: A Web-based survey of pediatric cardiologist subscribed to PediHeart was conducted. OUTCOME MEASURES: The types of palliation recommended (Norwood palliation, Sano modification, hybrid palliation, primary cardiac transplantation, or hospice care) for patients with HLHS with anatomic or comorbid variants were queried. Counseling provided by the physicians to families was also documented as regards survival and outcomes. RESULTS: Two hundred physicians (21% female) who averaged 12.3 years removed from training responded to the survey. US East Coast and Midwest respondents were more likely to recommend Norwood palliation (54% and 60%, respectively) and the US South and West respondents preferred Sano modification (73% and 82%, respectively). Norwood or Sano palliation was recommended over hospice care, hybrid palliation, or cardiac transplant for patients with an intact atrial septum (P < 0.05), moderate to severe tricuspid regurgitation (P < 0.05), or low birth weight defined as less than 2 kg (P < 0.05). Hospice was preferred in low-birth-weight infants over hybrid palliation or cardiac transplantation (P < 0.05). Hospice was recommended over any other palliation for premature infants (less than 30 weeks gestation), chromosomal abnormalities, or end-organ dysfunction (P < 0.05). CONCLUSIONS: This survey demonstrates that different palliative options are primarily recommended by caretakers based upon institutional location and patient characteristics. Prospective comparative trials may force a rethinking of this approach over time.

A randomized controlled trial of motavizumab versus palivizumab for the prophylaxis of serious respiratory syncytial virus disease in children with hemodynamically significant congenital heart disease.

Children with hemodynamically significant congenital heart disease (CHD) are at risk for serious respiratory syncytial virus (RSV) disease. This study was designed to assess the safety and tolerability of motavizumab versus palivizumab in children with CHD and was not powered for efficacy. Patients (n = 1236) aged ≤24 mo were randomized to receive five monthly doses (15 mg/kg) of motavizumab or palivizumab during the RSV season. Adverse events (AEs) and serious AEs (SAEs) were recorded through 30 d after the last dose. RSV hospitalizations and RSV outpatient medically attended lower respiratory tract infections (MALRI; season 2) were summarized. Approximately 93 and 50% of patients reported an AE or SAE, respectively. Skin events occurred in 19.3% of motavizumab recipients and 16.2% of palivizumab recipients. Rates of hospitalizations and RSV MALRI were similar between treatment groups [relative risk (RR): 0.75; 95% CI, 0.34-1.59 and RR: 0.49; 95% CI, 0.10-1.99, respectively; both p > 0.05]. Motavizumab and palivizumab had similar safety profiles in children with hemodynamically significantly CHD; with the exception of skin events which were increased in motavizumab recipients. Safety and efficacy were consistent with another study comparing motavizumab with palivizumab in premature infants without CHD.

Quality outcomes of ACHD patients undergoing cardiovascular procedures and hospital admissions in a free-standing children's hospital.

OBJECTIVES: Describe quality outcomes of adults with congenital heart disease (ACHD) undergoing cardiovascular procedures and admissions in a free-standing children's hospital with a multi-disciplinary ACHD program and compared with pediatric outcomes. BACKGROUND: A challenge for the U.S. healthcare system is where to treat the >1 million ACHD patients (pts): adult hospitals without CHD care, or pediatric hospitals without adult services. METHODS: We reviewed all CHD pts ≥ 18 yrs of age from 2002-2007. Procedural and hospital related morbidity and mortality were recorded. ANOVA and t-test compared adult with pediatric pts. RESULTS: Overall, 782 pts, mean age of 29.8 ± 9.9 yrs, encountered 1490 procedures/admissions. For 178 cardiac surgeries (72% reoperations), mortality was 1.8% and complication rate was 7.3%. There was 0% mortality for 412 cardiac catheterizations, 311 electrophysiological procedures, 401 transesophageal echocardiograms (TEE), 244 exercise tests (ETT) and 54 medical admissions. Major adverse event rate was 0.6% for cardiac catheterization and electrophysiological procedures. No adverse events occurred during TEE and ETT. Only 4 pts required transfer to an adult institution (0.25%). There was no significant difference in mortality or adverse events between pediatric and adult CHD pts, p>0.05. CONCLUSIONS: The optimal setting to provide ACHD care remains a complex issue. Our study is the first to demonstrate 1) a low incidence of morbidity and mortality for ACHD pts undergoing cardiovascular procedures or admissions at a free-standing children's hospital, 2) outcomes comparable to pediatric CHD pts. Future models incorporating ACHD programs within pediatric heart centers should be considered to care for this complex population.

Palivizumab and the prevention of respiratory syncytial virus illness in pediatric patients with congenital heart disease.

Respiratory syncytial virus (RSV) is a significant pathogen for infants and children with congenital heart disease. Non-sustained immunity and failure to develop an effective vaccine has steered RSV management toward a passive immunotherapy strategy in at-risk children. Palivizumab is a humanized murine monoclonal antibody targeting the RSV envelope F glycoprotein. In a Phase III clinical trial palivizumab significantly reduced RSV hospitalization in children with significant congenital heart disease and was proven to be safe. Palivizumab is one of the first monoclonal antibodies to significantly impact a pediatric disease.

Hepatic necrosis following repair of totally anomalous pulmonary venous connection.

We report a case of an infant with asplenia syndrome, isomerism of the right atrial appendages, and totally anomalous pulmonary venous connection who experienced hepatic failure following surgical correction of the anomalous pulmonary venous connection. We describe associated anomalies of the portal venous system.

Increased calcium supplementation is associated with morbidity and mortality in the infant postoperative cardiac patient.

OBJECTIVE: The purpose of this study was to assess the association of calcium replacement therapy with morbidity and mortality in infants after cardiac surgery involving cardiopulmonary bypass. DESIGN: Retrospective chart review. SETTING: The cardiac intensive care unit at a tertiary care children's hospital. PATIENTS: Infants undergoing cardiac surgery involving cardiopulmonary bypass between October 2002 and August 2004. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Total calcium replacement (mg/kg calcium chloride given) for the first 72 postoperative hours was measured. Morbidity and mortality data were collected. The total volume of blood products given during the first 72 hrs was recorded. Infants with confirmed chromosomal deletions at the 22q11 locus were noted. Correlation and logistic regression analyses were used to generate odds ratios and 95% confidence intervals, with p < .05 being significant. One hundred seventy-one infants met inclusion criteria. Age was 4 +/- 3 months and weight was 4.9 +/- 1.7 kg at surgery. Six infants had deletions of chromosome 22q11. Infants who weighed less required more calcium replacement (r = -.28, p < .001). Greater calcium replacement correlated with a longer intensive care unit length of stay (r = .27, p < .001) and a longer total hospital length of stay (r = .23, p = .002). Greater calcium replacement was significantly associated with morbidity (liver dysfunction [odds ratio, 3.9; confidence interval, 2.1-7.3; p < .001], central nervous system complication [odds ratio, 1.8; confidence interval, 1.1-3.0; p = .02], infection [odds ratio, 1.5; confidence interval, 1.0-2.2; p < .04], extracorporeal membrane oxygenation [odds ratio, 5.0; confidence interval, 2.3-10.6; p < .001]) and mortality (odds ratio, 5.8; confidence interval, 5.8-5.9; p < .001). Greater calcium replacement was not associated with renal insufficiency (odds ratio, 1.5; confidence interval, 0.9-2.3; p = .07). Infants with >1 sd above the mean of total calcium replacement received on average fewer blood products than the total study population. CONCLUSIONS: Greater calcium replacement is associated with increasing morbidity and mortality. Further investigation of the etiology and therapy of hypocalcemia in this population is warranted.

Identification, imaging, functional assessment and management of congenital coronary arterial abnormalities in children.

The coronary arteries, the vessels through which both substrate and oxygen are provided to the cardiac muscle, normally arise from paired stems, right and left, each arising from a separate and distinct sinus of the aortic valve. The right coronary artery runs through the right atrioventricular groove, terminating in the majority of instances in the inferior interventricular groove. The main stem of the left coronary artery bifurcates into the anterior descending, or interventricular, and the circumflex branches. Origin of the anterior descending and circumflex arteries from separate orifices from the left sinus of Valsalva occurs in about 1% of the population, while it is also frequent to find the infundibular artery arising as a separate branch from the right sinus of Valsalva. Anomalies of the coronary arteries can result from rudimentary persistence of an embryologic coronary arterial structure, failure of normal development or normal atrophy as part of development, or misplacement of connection of a an otherwise normal coronary artery. Anomalies, therefore, can be summarized in terms of abnormal origin or course, abnormal number of coronary arteries, lack of patency of the orifice of coronary artery, or abnormal connections of the arteries. Anomalous origin of the left coronary artery from the pulmonary trunk occurs with an incidence of approximately 1 in 300,000 children. The degree of left ventricular dysfunction produced likely relates to the development of collateral vessels that arise from the right coronary artery, and provide flow into the left system. Anomalous origin of either the right or the left coronary artery from the opposite sinus of Valsalva can be relatively innocuous, but if the anomalous artery takes an interarterial course between the pulmonary trunk and the aorta, this can underlie sudden death, almost invariably during or immediately following strenuous exercise or competitive sporting events. Distal anomalies of the coronary arteries most commonly involve abnormal connections, or fistulas, between the right or left coronary arterial systems and a chamber or vessel. We discuss the current techniques available for imaging these various lesions, along with their functional assessment, concluding with a summary of current strategies for management.

Hyperglycemia is a marker for poor outcome in the postoperative pediatric cardiac patient.

OBJECTIVE: Hyperglycemia in critical care populations has been shown to be a risk factor for increased morbidity and mortality. Minimal data exist in postoperative pediatric cardiac patients. The goal of this study was to determine whether hyperglycemia in the postoperative period was associated with increased morbidity or mortality. DESIGN: Retrospective chart review. SETTING: Tertiary care, free-standing pediatric medical center with a dedicated cardiac intensive care unit. PATIENTS: We included 184 patients <1 yr of age who underwent cardiac surgery requiring cardiopulmonary bypass from October 2002 to August 2004. Patients with a weight <2 kg, a preoperative diagnosis of diabetes, preoperative extracorporeal membrane oxygenation support, solid organ transplant recipients, and preoperative renal or liver insufficiency were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Age was 4.3 +/- 3.2 months and weight was 4.9 +/- 1.7 kg at surgery. Duration of hyperglycemia was significantly longer in patients with renal insufficiency (p = .029), liver insufficiency (p = .006), infection (p < .002), central nervous system event (p = .038), extracorporeal membrane oxygenation use (p < .001), and death (p < .002). Duration of hyperglycemia was also significantly associated with increased intensive care (p < .001) and hospital (p < .001) stay and longer ventilator use (p < .001). Peak glucose levels were significantly different in patients with renal insufficiency (p < .001), infection (p = .002), central nervous system event (p = .01), and mortality (p < .001). CONCLUSIONS: Hyperglycemia in the postoperative period was associated with increased morbidity and mortality in postoperative pediatric cardiac patient. Strict glycemic control may improve outcomes in this patient population.

Improved interstage mortality with the modified Norwood procedure: a meta-analysis.

BACKGROUND: Modification of the Norwood procedure has been reported to improve immediate postoperative mortality compared with the classic Norwood. Interstage mortality has not been shown to be improved with the modified Norwood probably because of the small number of patients from each institution. The goal of this study was to determine if meta-analysis would provide sufficient data to prove statistical difference in interstage mortality for the modified Norwood procedure. METHODS: PubMed was searched using six different terms individually for articles from January 2003 to October 2004. Manuscripts that compared the classic to modified Norwood were reviewed. Mantel-Haenszel analysis was used to evaluate the relationship between treatment method and mortality stratified across hospitals. The Breslow-Day procedure tested homogeneity of odds ratio across hospitals. Separate analyses were performed for inpatient and interstage periods. RESULTS: A total of 4,545 citations was screened. Five manuscripts met the criteria. Seventy-two patients undergoing classic Norwood and 84 patients undergoing modified Norwood survived to initial hospital discharge. The Breslow-Day statistic supported homogeneity of odds ratios for survival across hospitals (chi2 = 2.09, df = 4, p = 0.72). Odds of interstage death was 11.6 times greater (2.2 to 62.1, 95% CI) for the classic Norwood compared with the modified Norwood procedure. This difference was statistically significant by the Mantel-Haenszel chi2 (11.0, p = 0.001). The Breslow-Day statistic supported homogeneity of the odds ratios across hospitals (chi2 = 3.1, df = 4, p = 0.53). CONCLUSIONS: The modified Norwood procedure has a significantly lower interstage mortality compared with the classic Norwood procedure. A large randomized study is needed to determine whether these results remain consistent.