Standardizing Prostaglandin Initiation in Prenatally Diagnosed Ductal-Dependent Neonates; A Quality Initiative.

Prostaglandin E1 (PGE) is used in patients with ductal-dependent congenital heart disease (CHD). Side effects of apnea and fever are often dose dependent and occur within 48 h after initiation. We initiated a standardized approach to PGE initiation after our institution recognized a high incidence of side effects and a wide variety of starting doses of PGE. Neonates with prenatally diagnosed ductal-dependent CHD were identified, started on a standardized protocol that started PGE at 0.01 mcg/kg/min, and evaluated for PGE related side effects. Compliance, outcomes and dose adjustments during the first 48 h post-PGE initiation were evaluated. Fifty patients were identified (25 pre-intervention; 25 post-intervention). After intervention, compliance with the protocol was 96%, and apnea or fever occurred in 28% (compared to 63% pre-intervention, p = 0.015). Dose adjustments (either increase or decrease) prior to cardiac surgery were similar in both cohorts (60%, 52%, p = 0.569). There were no mortalities or emergent procedures performed due to ductus arteriosus closure. Standardizing a protocol for initiating PGE in prenatally diagnosed ductal-dependent CHD was successful and reduced the incidence of apnea, fever, and sepsis evaluations. A starting dose of 0.01 mcg/kg/min did not cause increased adverse effects.

Gastrostomy tube placement in congenital cardiac surgery: a multi-institutional database study.

INTRODUCTION: Neonates and infants who undergo congenital cardiac surgery frequently have difficulty with feeding. The factors that predispose these patients to require a gastrostomy tube have not been well defined. We aimed to report the incidence and describe hospital outcomes and characteristics in neonates and infants undergoing congenital cardiac surgery who required gastrostomy tube placement. MATERIALS AND METHOD: A retrospective review was performed on patients undergoing congenital cardiac surgery between October 2015 and December 2020. Patients were identified by International Classification of Diseases 10th Revision codes, utilising the performance improvement database Vizient® Clinical Data Base, and stratified by age at admission: neonates (<1 month) and infants (1-12 months). Outcomes were compared and comparative analysis performed between admissions with and without gastrostomy tube placement. RESULTS: There were 11,793 admissions, 3519 (29.8%) neonates and 8274 (70.2%) infants. We found an increased incidence of gastrostomy tube placement in neonates as compared to infants following congenital cardiac surgery (23.1% versus 6%, p = <0.001). Outcomes in neonates and infants were similar with increased length of stay and cost in those requiring a gastrostomy tube. Gastrostomy tube placement was noted to be more likely in neonates and infants with upper airway anomalies, congenital abnormalities, hospital infections, and genetic abnormalities. DISCUSSION: Age at hospitalisation for congenital cardiac surgery is a definable risk factor for gastrostomy tube requirement. Additional factors contribute to gastrostomy tube placement and should be used when counselling families regarding the potential requirement of a gastrostomy tube.

Cardiac Concerns in the Pediatric Athlete.

Cardiovascular disease remains the number one cause of death in Americans. It is no secret that exercise mitigates this risk. Exercise and regular physical activity are beneficial for physical health including aerobic conditioning, endurance, strength, mental health, and overall improved quality of life. Unfortunately, today many children and adolescents are sedentary, lacking the recommended daily amount of physical activity, leading to higher rates of obesity, cardiovascular disease, stroke, diabetes, anxiety, and depression. Given this rising concern, the World Health Organization launched a 12-year plan to improve physical activity in children and adolescents by reducing the inactivity rate by 15% in the world. How does this apply to children and adolescents with acquired or congenital heart disease?.

Increased risk of intraventricular hemorrhage in low birth weight infants with aortic coarctation.

Objective: To determine if there is an association between aortic coarctation (CoA) and intraventricular hemorrhage (IVH) in low birth weight (LBW) infants that may justify earlier intervention. While there is an increasing number of reports of successful early CoA intervention in LBW infants, there are no data to justify this approach.Material and methods: Retrospective review of the University Health System Consortium Clinical Data Base/Resource Manager, a national hospital discharge database. LBW (≤2 kg) infants, with and without IVH and isolated CoA were identified; IVH was stratified into low (grade 1 or 2) and high (grade 3 or 4) severity. Odds ratios were calculated for any, low and high-grade IVH with CoA.Results: Forty-six thousand and twenty LBW infants were identified; 3716 (8.1%) had IVH, 3001 (81%) with low, and 724 (19%) with high severity. Sixty-four infants had CoA, 13 had associated IVH (10 with low and three with high severity). The odds ratio for any IVH with CoA was 2.91 (95% CI 1.58-5.35), low severity 2.77 (95% CI 1.41-5.46) and high severity 3.45 (95% CI 1.07-11.07).Conclusions: In conclusion, this large retrospective database review found that LBW infants with CoA may be at increased risk of IVH. Further study is needed to determine if earlier catheter- or surgical-based intervention for CoA could reduce the risk of IVH in this population.

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.

Utility of three-dimensional models in resident education on simple and complex intracardiac congenital heart defects.

OBJECTIVE: Applications of three-dimensional (3D) printed models in medicine include preprocedure planning, patient education, and clinical training. Reproducing complex anatomy as a 3D printed model can be useful for understanding congenital heart defects (CHD). We hypothesized that using 3D printed models during didactic sessions with resident physicians will improve trainees' understanding of CHD. DESIGN AND INTERVENTION: We performed a prospective, randomized educational intervention for teaching pediatric and pediatric/emergency medicine residents about simple (ventricular septal defect [VSD]) and moderately complex (tetralogy of Fallot [ToF]) CHD. Residents were divided into two groups: intervention and control. Each group completed a subjective survey about their comfort with the anatomy, evaluation, and treatment of VSD and ToF and took an objective test on VSD and ToF. They separately received the same 20 min lecture, including projected two-dimensional digital images of VSD and ToF; the intervention group was given 3D printed models created using the same imaging data. After the lecture, the groups repeated the survey and test questions. RESULTS: Twenty-six residents participated in the VSD session, 34 in the ToF. There were no differences in demographics between control and intervention groups. All residents had higher subjective comfort with VSD and ToF after the lectures. There was no difference in baseline test scores for VSD or ToF groups. The control group scored higher on the VSD postlecture test. The intervention group scored higher on the ToF postlecture test. CONCLUSION: Incorporation of 3D printed models into lectures about CHD imparts a greater acute level of understanding, both subjective and objective, for pediatric and combined pediatric/emergency medicine residents. There does not seem to be an added benefit for understanding ventricular septal defects, but there is for tetralogy of Fallot, likely due to increased complexity of the lesion and difficulty visualizing spatial relationships in CHD with multiple components.

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.

Malrotation is not associated with adverse outcomes after cardiac surgery in patients with heterotaxy syndrome.

BACKGROUND: Patients with heterotaxy syndrome (HS) often have asymptomatic malrotation. There is a lack of consensus regarding the management of these patients, particularly in patients with complex congenital heart disease (CHD). We sought to describe the prevalence of malrotation and incidence of volvulus in a population of patients with complex CHD and to identify the impact of malrotation on morbidity and mortality following cardiac surgery. METHODS: We performed a retrospective review of all patients with HS and complex CHD who required cardiac surgery in the first year of life at a single center between October 1995 and September 2015. Malrotation was diagnosed by abdominal imaging or by direct inspection during abdominal surgery. Demographic data was collected along with details of hospitalization following cardiac and GI surgeries. Descriptive analysis along with appropriate hypothesis testing was conducted to evaluate the results. RESULTS: We identified 49 patients with HS, 42 with single ventricle anatomy and 7 with biventricular anatomy. Of the 49 patients, 29 (59%) were diagnosed with malrotation, 6 (12%) had normal intestinal rotation, and 14 (29%) had no evaluation of intestinal rotation. The prevalence of malrotation in the population who underwent abdominal imaging was 29 out of 35 (83%). There was no difference in survival following cardiac surgery between patients with malrotation and those with unknown or normal intestinal anatomy. Comparing patients with malrotation and patients with normal or unknown intestinal rotation, there was also no difference in surrogate markers of morbidity. Of the 29 patients with known malrotation, only 2 patients (7%) underwent therapeutic Ladd procedures and 19 (65%) underwent prophylactic Ladd procedures. CONCLUSIONS: We conclude that the outcomes following cardiac surgery for patients with HS are not impacted by the presence of malrotation. Furthermore, we also found that the incidence of volvulus in the studied group is low. Given these findings, and the understanding that patients with HS and significant CHD are frequently tenuous and high risk surgical candidates, we do not believe performing prophylactic Ladd procedures is warranted. LEVEL OF EVIDENCE: III.

Cardiovascular Considerations in the Female Athlete.

Over several decades, much has been learned about the diverse physical impacts of exercise. Those who excel, such as elite athletes, have physiologic differences compared with the general population. There is a growing body of data suggesting that gender may play a role in these adaptations. Further, certain cardiac conditions may exhibit a gender predilection. This article explains the particular cardiac nuances of the female athlete.

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.