Prevalence of cardiovascular implantable electronic devices in children with type 1 myotonic dystrophy.

INTRODUCTION/AIMS: Type 1 myotonic dystrophy (DM1) is a neuromuscular disorder of multiple organ systems with important electrophysiologic (EP) manifestations, leading to a cumulative incidence of sudden death of 6.6%. Due to genetic anticipation, there is a pediatric subset of this patient population. However, most EP research on DM1 patients has been in adults, making cardiac care for pediatric patients difficult and directed by adult guidelines which often leads to cardiovascular implantable electronic device (CIED) implants. We sought to investigate the prevalence of CIEDs in the pediatric DM1 population. METHODS: The Vizient® Clinical Data Base was queried from October 2019 to October 2023 for admissions with and without ICD-10 code for myotonic dystrophy (G71.11), with and without codes for presence of a pacemaker or ICD (Z95.0, Z95.810). Patients who were identified were stratified by age: Pediatric (0-21 years) and Adult (22-50 years). RESULTS: Prevalence of CIED in pediatric DM1 was 2.1% and in adult DM1 was 15.8%. When comparing to pediatric and adult patients with CIED and without DM1, the odds ratio for CIED in pediatric DM1 was 48.8, compared to 23.3 for CIED in adult DM1. DISCUSSION: There are pediatric DM1 patients who have received CIED despite a lack of data to inform this decision-making. Further research will be important to ensure appropriate use of CIED in this population and to develop appropriate guidelines to direct management.

Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set.

Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. "Low Performers" were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and "Normal Performers" were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.

Congenital left atrial appendage ostial stenosis in an extremely premature infant diagnosed by transthoracic echocardiography.

Congenital left atrial appendage ostial stenosis is a very rare congenital cardiac condition. We present the case of an extremely premature infant with congenital left atrial appendage ostial stenosis diagnosed by transthoracic echocardiographic imaging.

Health Insurance Status and Access to Healthcare Among Young Adults with Congenital Heart Disease: from the Congenital Heart Survey To Recognize Outcomes, Needs and Well-beinG (CH STRONG), 2016-2019.

Having health insurance is associated with better access to healthcare and lower rates of comorbidity in the general population, but data are limited on insurance's impact on adults with congenital heart disease (ACHD). The Congenital Heart Survey To Recognize Outcomes, Needs and well-beinG (CH STRONG) was conducted among ACHD in three locations from 2016 to 2019. We performed multivariable logistic regression to determine the associations between health insurance and both access to healthcare and presence of comorbidities. We also compared health insurance and comorbidities among ACHD to similarly-aged individuals in the Behavioral Risk Factor Surveillance System (BRFSS) as a proxy for the general population. Of 1354 CH STRONG respondents, the majority were ≤ 30 years old (83.5%), and 8.8% were uninsured versus 17.7% in the BRFSS (p < 0.01). Compared to insured ACHD, uninsured were less likely to report regular medical care (adjusted odds ratio [aOR] 0.2, 95% confidence interval [CI] 0.1-0.3) and visited an emergency room more often (aOR 1.6, CI 1.0-2.3). Among all ACHD reporting disability, uninsured individuals less frequently received benefits (aOR 0.1, CI 0.0-0.3). Depression was common among uninsured ACHD (22.5%), but insured ACHD had lower rates of depression than insured in the BRFSS (13.3% vs. 22.5%, p < 0.01). In conclusion, rates of insurance were higher among ACHD compared to the general population. Nonetheless, uninsured ACHD inconsistently accessed healthcare and benefits. Further studies are needed to determine if insurance ameliorates the risk of morbidity as ACHD age.

Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease.

The aim of the current study is to investigate hospitalization outcomes of COVID-19 positive children and adults with moderate or severe congenital heart disease to children and adults without congenital heart disease. Retrospective review using the Vizient Clinical Data Base for admissions of patients with an ICD-10 code for COVID-19 from April 2020 to March 2021. Admissions with COVID-19 and with and without moderate or severe congenital heart disease (CHD) were stratified into pediatric (< 18 years) and adult (≥ 18 years) and hospital outcomes were compared. There were 9478 pediatric COVID-19 admissions, 160 (1.7%) with CHD, and 658,230 adult COVID-19 admissions, 389 (0.06%) with CHD. Pediatric admissions with COVID-19 and CHD were younger (1 vs 11 years), had longer length of stay (22 vs 6 days), higher complication rates (6.9 vs 1.1%), higher mortality rates (3.8, 0.8%), and higher costs ($54,619 vs 10,731; p < 0.001 for all). Adult admissions with COVID-19 and CHD were younger (53 vs 64 years, p < 0.001), had longer length of stay (12 vs 9 days, p < 0.001), higher complication rates (8 vs 4.8%, p = 0.003), and higher costs ($23,551 vs 13,311, p < 0.001). This appears to be the first study to report the increased hospital morbidities and costs for patients with CHD affected by COVID-19. Our hope is that these findings will help counsel patients moving forward during the pandemic.

Higher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle.

Patients with single ventricle congenital heart disease are at risk of unpredictable protein-losing enteropathy (PLE) after surgical palliation. Based on prior reports of physiologic differences for patients with single morphologic right versus left ventricles, we hypothesized that those with right ventricular morphology would have a higher incidence of PLE. We performed a retrospective review of > 15 million pediatric hospitalizations from the Healthcare Cost and Utilization Project KID 2000-2012 databases for admissions 5-21 years old with ICD-9 codes for hypoplastic left heart syndrome (HLHS) and tricuspid atresia (TA) with and without PLE. Incidence of PLE was compared between those with HLHS and TA. In addition, outcomes and costs were compared between admissions with and without PLE and between HLHS and TA. Of 1623 HLHS admissions, 289 (17.8%) had PLE, and of 926 TA admissions, 58 (5.9%) had PLE (p < 0.001). Admissions with PLE were older compared to those without PLE (12 vs 10 years, p < 0.001) and PLE onset occurred at a younger age for HLHS than TA (11 vs 14 years, p < 0.001). There were no differences in hospital outcomes or costs. Review of this large administrative database suggests a higher incidence of PLE in patients with HLHS and a younger age of onset compared to those with TA. These data suggest that a single systemic right ventricle may be an independent risk factor for developing PLE.

Use of rotational angiography in congenital cardiac catheterisations to generate three-dimensional-printed models.

BACKGROUND: Three-dimensional printing is increasingly utilised for congenital heart defect procedural planning. CT or MR datasets are typically used for printing, but similar datasets can be obtained from three-dimensional rotational angiography. We sought to assess the feasibility and accuracy of printing three-dimensional models of CHD from rotational angiography datasets. METHODS: Retrospective review of CHD catheterisations using rotational angiography was performed, and patient and procedural details were collected. Imaging data from rotational angiography were segmented, cleaned, and printed with polylactic acid on a Dremel® 3D Idea Builder (Dremel, Mount Prospect, IL, USA). Printing time and materials' costs were captured. CT scans of printed models were compared objectively to the original virtual models. Two independent, non-interventional paediatric cardiologists provided subjective ratings of the quality and accuracy of the printed models. RESULTS: Rotational angiography data from 15 catheterisations on vascular structures were printed. Median print time was 3.83 hours, and material costs were $2.84. The CT scans of the printed models highly matched with the original digital models (root mean square for Hausdorff distance 0.013 ± 0.003 mesh units). Independent reviewers correctly described 80 and 87% of the models (p = 0.334) and reported high quality and accuracy (5 versus 5, p = NS; κ = 0.615). CONCLUSION: Imaging data from rotational angiography can be converted into accurate three-dimensional-printed models of CHD. The cost of printing the models was negligible, but the print time was prohibitive for real-time use. As the speed of three-dimensional printing technology increases, novel future applications may allow for printing patient-specific devices based on rotational angiography datasets.

Transcatheter treatment of acquired coronary sinus ostium atresia in a child with complex congenital heart disease.

Congenital coronary sinus ostium atresia is a rare condition that is typically considered benign, as long as there is adequate drainage via another route. However, in children with single ventricle congenital heart disease, adequate drainage may not be assured after complex surgical interventions, putting them at risk for myocardial injury. We present a patient with complex single ventricle congenital heart disease who developed acquired coronary sinus ostium atresia after surgical intervention that was treated by a transcatheter approach to prevent the sequelae of coronary sinus hypertension.

Acute and medium term results of balloon expandable stent placement in the transverse arch-a multicenter pediatric interventional cardiology early career society study.

OBJECTIVES AND BACKGROUND: Coarctation of the aorta represents 5-8% of all congenital heart disease. Although balloon expandable stents provide an established treatment option for native or recurrent coarctation, outcomes from transverse arch (TAO) stenting, including resolution of hypertension have not been well studied. This study aims to evaluate immediate and midterm results of TAO stenting in a multi-center retrospective cohort. METHODS: TAO stenting was defined as stent placement traversing any head and neck vessel, with the primary intention of treating narrowing in the transverse aorta. Procedural details, complications and medications were assessed immediately post procedure, at 6 month follow-up and at most recent follow-up. RESULTS: Fifty-seven subjects, 12 (21%) native, and 45 (79%) surgically repaired aortic arches, from seven centers were included. Median age was 14 years (4 days-42 years), median weight 54 kg (1.1-141 kg). After intervention, the median directly measured arch gradient decreased from 20 mmHg (0-57 mmHg) to 0 mmHg (0-23 mmHg) (p < .001). The narrowest arch diameter increased from 9 mm (1.4-16 mm) to 14 mm (2.9-25 mm) (p < .001), with a median increase of 4.9 mm (1.1-10.1 mm). One or more arch branches were covered by the stent in 55 patients (96%). There were no serious adverse events. Two patients warranted stent repositioning following migration during deployment. There were no late complications. There were 8 reinterventions, 7 planned, and 1 unplanned (6 catheterizations, 2 surgeries). Antihypertensive management was continued in 19 (40%) at a median follow-up of 3.2 years (0.4-7.3 years). CONCLUSIONS: TAO stenting can be useful in selected patients for resolution of stenosis with minimal complications. This subset of patients are likely to continue on antihypertensive medications despite resolution of stenosis.

Early bioprosthetic tricuspid valve stenosis due to size mismatch in Ebstein anomaly-Successful transcatheter treatment.

Patients with congenital heart disease are surviving well into adulthood thanks to advances in medical and clinical care. We present a patient with Ebstein anomaly who underwent surgical tricuspid valve replacement and suffered early valve stenosis due to her unique anatomy. This case highlights the importance of the "unnatural" anatomy that can be encounter in this challenging patient population.

Effect of comorbid neuropsychiatric disorders on children and adolescents undergoing surgery for moderate and severe congenital heart disease.

BACKGROUND: Children and adolescents with congenital heart disease (CHD) are at an increased risk of neuropsychiatric disorders (NPDs). The purpose of this study is to determine how a comorbid NPD affects hospital outcomes and costs for CHD patients undergoing cardiac surgery. METHODS: Retrospective review of the 2000-2012 Healthcare Cost and Utilization Project Kids' Inpatient Databases for admissions 10 to 21 years old with an ICD-9 code for moderate or severe CHD and a procedure code for cardiopulmonary bypass as a marker for cardiac surgery; admissions with syndromes that could be associated with NPD were excluded. Demographics, hospital outcomes, and charges were compared between admissions with and without NPD ICD-9 codes using analysis of variance, independent samples Kruskal-Wallis, and χ2 , as appropriate. RESULTS: There were 4768 admissions with CHD and cardiac surgery: 4285 (90%) with no NPD, 93 (2%) with cognitive deficits, 390 (8%) with mood/behavior deficits. Patients with NPD had a longer length of stay and higher mean charges (P < .001 for both). Patients with mood/behavior deficits were older and patients with cognitive deficits were more likely female (P < .001 for both). CONCLUSIONS: Children and adolescents with moderate or severe CHD and NPD who undergo cardiac surgery incur longer hospital stays and higher charges. Recognizing and addressing the underlying NPDs may be important to improve postoperative progression for children and adolescents with CHD hospitalized for cardiac surgery.

Invasive Cardiac Procedures in Interstage Single Ventricle Patients in Emergent Hospitalizations.

Single ventricle congenital heart disease (SV CHD) patients are at risk of morbidity and mortality between the first and second palliative surgical procedures (interstage). When these patients present acutely they often require invasive intervention. This study sought to compare the outcomes and costs of elective and emergent invasive cardiac procedures for interstage patients. Retrospective review of discharge data from The Vizient Clinical Data Base/Resource Manager™, a national health care analytics platform. The database was queried for admissions from 10/2014 to 12/2017 for children 1-6 months old with ICD-9 or ICD-10 codes for SV CHD who underwent invasive cardiac procedures. Demographics, length of stay (LOS), complication rate, in-hospital mortality and direct costs were compared between elective and emergent admissions using t test or χ2, as appropriate. The three most frequently performed procedures were also compared. 871 admissions identified, with 141 (16%) emergent. Age of emergent admission was younger than elective (2.9 vs. 4 months p < 0.001). Emergent admissions including cardiac catheterization or superior cavo-pulmonary anastomosis had longer LOS (58.7 vs. 25.8 day, p < 0.001 and 54.8 vs .22.6 days, p < 0.001) and higher costs ($134,774 vs. $84,253, p = 0.013 and $158,679 vs. $81,899, p = 0.017). Emergent admissions for interstage SV CHD patients undergoing cardiac catheterization or superior cavo-pulmonary anastomosis are associated with longer LOS and higher direct costs, but with no differences in complications or mortality. These findings support aggressive interstage monitoring to minimize the need for emergent interventions for this fragile patient population.

Anxiety reduction after pre-procedure meetings in patients with CHD.

BACKGROUND: Cardiac catheterisations for CHD produce anxiety for patients and families. Current strategies to mitigate anxiety and explain complex anatomy include pre-procedure meetings and educational tools (cardiac diagrams, echocardiograms, imaging, and angiography). More recently, three-dimensionally printed patient-specific models can be added to the armamentarium. The purpose of this study was to evaluate the efficacy of pre-procedure meetings and of different educational tools to reduce patient and parent anxiety before a catheterisation. METHODS: Prospective study of patients ≥18 and parents of patients <18 scheduled for clinically indicated catheterisations. Patients completed online surveys before and after meeting with the interventional cardiologist, who was blinded to study participation. Both the pre- and post-meeting surveys measured anxiety using the State-Trait Anxiety Inventory. In addition, the post-meeting survey evaluated the subjective value (from 1 to 4) of individual educational tools: physician discussion, cardiac diagrams, echocardiograms, prior imaging, angiograms and three-dimensionally printed cardiac models. Data were compared using paired t-tests. RESULTS: Twenty-three patients consented to participate, 16 had complete data for evaluation. Mean State-Trait Anxiety Inventory scores were abnormally elevated at baseline and decreased into the normal range after the pre-procedure meeting (39.8 versus 31, p = 0.008). Physician discussion, angiograms, and three-dimensional models were reported to be most effective at increasing understanding and reducing anxiety. CONCLUSION: In this pilot study, we have found that pre-catheterisation meetings produce a measurable decrease in patient and family anxiety before a procedure. Discussions of the procedure, angiograms, and three-dimensionally printed cardiac models were the most effective educational tools.

Treatment of tricuspid regurgitation and para-ring leak in tetralogy of Fallot with oversized SAPIEN 3 valve-in-ring implantation.

A 40-year-old female with previously repaired tetralogy of Fallot had recurrent severe tricuspid regurgitation with a para-ring leak after annuloplasty ring placement. Because of multiple prior sternotomies and co-morbidities, she was not felt to be a surgical candidate. Percutaneous placement of an oversized SAPIEN 3 valve-in-ring in the tricuspid position successfully treated the regurgitation and para-ring leak.

Higher Cost of Hospitalizations for Non-cardiac Diagnoses in Adults with Congenital Heart Disease.

Adults with congenital heart disease (CHD) are a rapidly increasing population and their impact on healthcare resources is not fully understood. The purpose of this study was to describe the costs of hospitalizations for non-cardiac disease for adults with CHD. We conducted a retrospective review of hospital discharge data from the University HealthSystem Consortium Clinical Data Base/Resource Manager from January 2011 through December 2013. Patients were ≥ 18 years old at admission with any ICD-9 code for moderate or high severity CHD; cardiac surgical admissions were excluded. The comparison group consisted of patients ≥ 18 years old with no ICD-9 codes for any severity CHD. There were 9,169,700 non-CHD, 28,224 moderate CHD, and 3045 high severity CHD hospital admissions. Total length of stay was longer for acute kidney injury, depressive disorder, esophageal reflux, and obstructive sleep apnea for any severity CHD; ICU admission rates were higher for all diagnoses with any severity CHD. Mean observed direct costs were higher for all diagnoses for moderate CHD and all diagnoses except dehydration, type 2 diabetes, obesity, and obstructive sleep apnea for high severity CHD. This review identified significantly increased hospitalization costs for adults with moderate and high severity CHD who are admitted for non-cardiac medical conditions not associated with concomitant cardiac surgical procedures. Admissions with CHD diagnoses had higher ICU admission rates, longer lengths of stay, and higher mortality for most non-cardiac admission diagnoses. These data will add to our understanding of the economic impact of adults with CHD.

Lower Hospital Charges and Societal Costs for Catheter Device Closure of Atrial Septal Defects.

Atrial septal defects (ASD) are among the most common congenital heart defects. As more ASDs are corrected by interventional catheterization instead of surgery, it is critical to understand the associated clinical and societal costs. The goal of this study was to use a national U.S. database to describe hospital charges and societal costs for surgical and catheter-based (ASD) closure. Retrospective review of hospital discharge data from the Kids' Inpatient Database from January 2010 to December 2012. The database was queried for admissions for <21 years old with ICD-9 procedure codes for surgical (35.51 or 35.61) or catheter (35.52) ASD closure; those with other cardiac conditions and/or additional cardiac procedures were excluded. Age, length of stay (LOS), and hospital charges and lost parental wages (societal costs) were compared between groups using t test or Mann-Whitney U test, as appropriate. Four hundred and eighty-six surgical and 305 catheter ASD closures were identified. LOS, hospital charges, and total societal costs were higher in surgical ASD compared to catheter ASD admissions (3.6 vs. 1.3 days, p < 0.001, $87,465 vs. $64,109, p < 0.001, and $90,000 vs. $64,966, p < 0.001, respectively). In this review of a large national inpatient database, we found that hospital and societal costs for surgical ASD closure are significantly higher than catheter ASD closure in the United States in the current era. Factors that likely contribute to this include longer LOS and longer post-operative recovery. Using "real-world" data, this study demonstrates a substantial cost advantage for catheter ASD closure compared to surgical.

Application and Utility of iPads in Pediatric Tele-echocardiography.

BACKGROUND: Telemedicine is used with increasing frequency to improve patient care in remote areas. The interpretation of medical imaging on iPad(®) (Apple, Cupertino, CA) tablets has been reported to be accurate. There are no studies on the use of iPads for interpretation of pediatric echocardiograms. We compared the quality of echo images, diagnostic accuracy, and review time using three different modalities: remote access on an iPad Air (iPad), remote access via a computer (Remote), and direct access on a computer linked through Ethernet to the server, the "gold standard" (Direct). MATERIALS AND METHODS: Fifty consecutive archived pediatric echocardiograms were interpreted using the three modalities. Studies were analyzed blindly by three pediatric cardiologists; review time, diagnostic accuracy, and image quality were documented. Diagnostic accuracy was assessed by comparing the study diagnoses with the official diagnosis in the patient's chart. Discrepancies between diagnoses were graded as major (more than one grade difference) or minor (one grade difference in severity of lesion). RESULTS: There were no significant differences in accuracy among the three modalities. There was one major discrepancy (size of patent ductus arteriosus); all others were minor, hemodynamically insignificant. Image quality ratings were better for iPad than Remote; Direct had the highest ratings. Review times (mean [standard deviation] minutes) were longest for iPad (5.89 [3.87]) and then Remote (4.72 [2.69]), with Direct having the shortest times (3.52 [1.42]) (p < 0.0001). CONCLUSIONS: Pediatric echocardiograms can be interpreted using convenient, portable devices while preserving accuracy and quality with slightly longer review times (1-2 min). These findings are important in the current era of increasing need for mobile health.

Ventricular morphology is a determinant of diastolic performance in patients with single ventricle physiology undergoing stage 3 palliative surgery.

Patients with single ventricle anatomy undergo staged surgical palliation which results in pulmonary and systemic circulations in series with a single systemic pump. Single right ventricular morphology has been found to be an independent risk factor for worse survival. We sought to compare patients with single left (SLV) and single right (SRV) ventricular morphology to identify hemodynamic differences that may contribute to worse survival in patients with a single right ventricular. Single center, retrospective review of invasive hemodynamic data. All patients with single ventricle anatomy who underwent superior cavo-pulmonary anastomosis (Stage 2 palliation) and/or total cavo-pulmonary anastomosis (Stage 3 palliation) from August 1995 through May 2011 were identified. Patients were compared over time, and SLV and SRV patients were compared. Seventy-nine single ventricle patients (56 % SRV) underwent staged palliation and were analyzed. There was no difference in overall mortality (12 % SLV, 11 % SRV). There was no difference in hemodynamics at pre-Stage 2 catheterization between ventricular morphology, but SRV patients had higher ventricular end-diastolic pressure at pre-Stage 3 catheterization (7.6 vs. 6.4 mmHg, p = 0.026). End-diastolic pressure decreased after Stage 2 surgery for SLV patients, but not SRV patients. Intrinsic differences in morphology, function, and response to performing as the systemic ventricle between single right and left ventricles may lead to an elevated ventricular end-diastolic pressure. This could limit passive flow through the pulmonary circuit and coronary perfusion after Stage 3 palliation and potentially lead to poorer long-term performance for SRV patients.

Patients with single ventricle anatomy may respond better to octreotide therapy for chylothorax after congenital heart surgery.

BACKGROUND AND AIMS: Chylothorax (CTX) occurs in 3% to 6% of children after surgery for congenital heart disease with significant morbidity and mortality. Octreotide has been proposed as therapy, but there are no predictors of response. The objective of this study was to identify possible predictors of response to octreotide. METHODS: Single-center retrospective review of patients who developed CTX after cardiac surgery. Data collected included demographics, cardiac lesion, surgical data, hospital course, CTX volume and duration, and interventions for CTX. Patients who received octreotide as part of their therapy were compared to those who did not. RESULTS: A total of 1150 patients underwent 1455 cardiac surgeries with 67 (4.6%) episodes of CTX. Patients with CTX were younger, lower weight, more likely to undergo cardiopulmonary bypass, and had higher RACHS-1 scores and mortality. Nineteen patients with CTX received octreotide as part of their treatment and six (32%) had at least 50% reduction in CTX volume. Patients who responded to octreotide had lower CTX volume (18 mL/kg/day vs. 55 mL/kg/day, p=0.023) and a higher proportion of patients with single ventricle anatomy (67% vs. 18%, p=0.046). CONCLUSIONS: There is a subset of patients who seem to respond to octreotide, but they have lower CTX volume and may have already been improving before octreotide therapy. Patients with single ventricle anatomy seemed to respond to octreotide and may benefit from its use.