Maternal-fetal comorbidities and obstetrical outcomes of fetal single ventricle cardiac defects: 10 years' experience with a multidisciplinary management protocol at a single center.

OBJECTIVES: To describe and compare maternal and fetal comorbidities and obstetrical outcomes in pregnancies with hypoplastic left and right heart (HLHS and HRH) single ventricle cardiac defects (SVCD) from a single center under a multidisciplinary protocol. METHOD: A single center retrospective review of fetal SVCD from 2013 to 2022. Maternal-fetal comorbidities, delivery, and postnatal outcomes were compared between HLHS and HRH using descriptive statistics and univariate and multivariate analyses. RESULTS: Of 181 SVCD pregnancies (131 HLHS; 50 HRH), 9% underwent termination, 4% elected comfort care, 5 died in utero and 147/152 liveborns survived to the first cardiac intervention. Cesarean delivery occurred in 57 cases (37%), planned in 36 and unplanned in 21. Comorbidities, which did not differ between HLHS and HRH, included fetal growth restriction (FGR, 17%), prematurity (14%), maternal hypertension (9%), maternal obesity (50%), fetal extracardiac anomalies and chromosome anomalies (12%, 13%). In multivariate analysis, only earlier gestational age at delivery and oligohydramnios predicted decreased odds of survival at one year. CONCLUSION: Maternal-fetal comorbidities are common in both HLHS and HRH. Earlier gestational age at delivery and oligohydramnios predict lower postnatal survival. FGR, even with severe early onset, did not significantly impact short- or long-term neonatal survival in single ventricle conditions.

Contemporary pregnancy outcomes for women with moderate and severe congenital heart disease.

Background: Women with congenital heart disease (CHD) are surviving into adulthood, with more undergoing pregnancy. Methods: Retrospective review of the Vizient database from 2017-2019 for women 15-44 years old with moderate, severe or no CHD and vaginal delivery or caesarean section. Demographics, hospital outcomes and costs were compared. Results: There were 2,469,117 admissions: 2,467,589 with no CHD, 1277 with moderate and 251 with severe CHD. Both CHD groups were younger than no CHD, there were fewer white race/ethnicity in the no CHD group and more women with Medicare in both CHD groups compared to no CHD. With increasing CHD severity there was an increase in length of stay, ICU admission rates and costs. There were also higher rates of complications, mortality and caesarean section in the CHD groups. Conclusion: Pregnant women with CHD have more problematic pregnancies and understanding this impact is important to improve management and decrease healthcare utilization.

Genetic Characterization of a Model Ciliopathy: Bardet-Biedl Syndrome.

Bardet-Biedl syndrome (BBS) is a rare ciliopathy affecting multiple organ systems. Patients with BBS are usually diagnosed later in childhood when clinical features of the disease become apparent. In this article, we presented a case of BBS discovered by whole genome sequencing in a newborn with heterotaxy, duodenal atresia, and complex congenital heart disease. Early diagnosis is important not only for prognostication but also to explore ways to mitigate the cone-rod dysfunction and for exploring newer therapies. Our case highlights the importance of a high index of suspicion and the utility of advanced genetic testing to provide an early diagnosis for a rare disease.

All the More Reason to Get a Flu Shot: An Instance of Acute Hemorrhagic Leukoencephalitis in a Patient With Influenza A.

Acute hemorrhagic leukoencephalitis (AHLE) is a rare demyelinating disease of the central nervous system that typically follows a viral or bacterial respiratory infection. We report the first described case of AHLE following influenza A (H3N2) in an otherwise healthy 15-year-old girl with no relevant past medical history who initially presented to the emergency department (ED) by emergency medical services (EMS) with decorticate posturing and right gaze deviation after being found unresponsive at home. Subsequent testing for Influenza A H3N2 via viral polymerase chain reaction (PCR) was positive. Clinical correlation and brain MRI confirmed AHLE. At follow-up three months after discharge, she was found to have intellectual functioning in the extremely low range and she still had deficits in motor skills eight months after discharge. While the patient was reportedly up-to-date on her routine scheduled childhood vaccinations, she had not received her annual influenza vaccination that year. Pediatric infectious disease physicians and neurologists should consider the diagnosis of AHLE in unvaccinated, previously healthy patients with new and rapid onset of neurological symptoms following influenza infection.

Intravascular Ultrasound for Pulmonary Vein Stenosis Interventions in Congenital Heart Disease.

OBJECTIVE: Pulmonary vein stenosis (PVS) is aggressive, with high morbidity and mortality. Surgical and catheter interventions yield modest success, at best. Refinements in catheter interventions could potentially improve outcomes in this patient population. The goal of this study was to determine the utility of intravascular ultrasound (IVUS) for patients with congenital heart disease and PVS. METHODS: Single-center, retrospective review of patients with congenital heart disease and PVS undergoing diagnostic or interventional catheterizations from March 2015 to February 2020. IVUS of the pulmonary veins was performed using an Eagle Eye Platinum IVUS catheter (Volcano Corporation). RESULTS: Five patients underwent 6 procedures (2 diagnostic, 4 interventional). Median age was 1.5 years (range, 0.7-47.5 years) and weight was 8.8 kg (range, 7.3-61 kg). For the interventional procedures, mean pulmonary vein gradient was 8.7 mm Hg with reduction to 1.1 mm Hg (P<.001). Four patients had congenital PVS and 1 patient was post repair of Scimitar syndrome with an obstructed pulmonary venous baffle. Use of IVUS allowed confirmation of stent expansion and apposition, interval vessel growth after initial stenting, and detection of long-segment hypoplasia, unlikely to respond to intervention. There were no thrombotic complications related to IVUS use. CONCLUSIONS: IVUS of the pulmonary veins is safe and easy to perform, and provides detailed imaging of PVS to help guide therapy. For those requiring intervention, adequate stent apposition to the pulmonary vein walls, as well as limiting vessel overdilation, may minimize future in-stent stenosis and need for reintervention in this challenging disease.

Increased length of stay and hospital charges in adolescents with type 1 diabetes and psychiatric illness.

OBJECTIVES: Type I diabetes mellitus (T1DM) is one of the most common chronic diseases of childhood. Diabetic ketoacidosis (DKA) in this population contributes to significant healthcare utilization, including emergency room visits, hospitalizations, and ICU care. Comorbid psychiatric illnesses (CPI) are additional risks for increased healthcare utilization. While CPI increased risk for DKA hospitalization and readmission, there are no data evaluating the relationship between CPI and hospital outcomes. We hypothesized that adolescents with T1DM and CPI admitted for DKA have increased length of stay (LOS) and higher charges compared to those without CPI. METHODS: Retrospective review of 2000-2012 Healthcare Cost and Utilization Project's (HCUP) Kids' Inpatient Databases (KID). Patients 10-21 years old admitted with ICD-9 codes for DKA or severe diabetes (250.1-250.33) with and without ICD-9 codes for depression (296-296.99, 311) and anxiety (300-300.9). Comparisons of LOS, mortality, and charges between groups (No CPI, Depression and Anxiety) were made with one way ANOVA with Bonferroni correction, independent samples Kruskal-Wallis test with Bonferroni correction and χ2. RESULTS: There were 79,673 admissions during the study period: 68,573 (86%) No CPI, 8,590 (10.7%) Depression and 12,510 (15.7%) Anxiety. Female patients comprised 58.2% (n=46,343) of total admissions, 66% of the Depression group, and 71% of the Anxiety group. Patients with depression or anxiety were older and had longer LOS and higher mean charges (p<0.001 for both). CONCLUSION: Comorbid depression or anxiety are associated with significantly longer LOS and higher charges in adolescents with T1DM hospitalized for DKA. This study adds to the prior findings of worse outcomes for patients with both T1DM and CPI, emphasizing the importance of identifying and treating these comorbid conditions.

Greater length of stay and hospital charges for severe asthma in children with depression or anxiety.

RATIONALE: Asthma is one of the most common chronic disorders of childhood and is associated with significant healthcare utilization and costs. Comorbid psychiatric illnesses, specifically depression and anxiety, are more prevalent in patients with asthma and associated with worse asthma control, more emergency department visits, and increased hospitalization rates. OBJECTIVES: We aimed to compare hospital outcomes and charges for children with severe asthma with and without comorbid depression and anxiety, hypothesizing that those with depression and anxiety would have longer hospitalizations and higher charges. METHODS: Retrospective review of the 2000-2012 Healthcare Cost and Utilization Project's Kids' Inpatient Databases for admissions of patients aged 10-21 years with an ICD-9 code severe asthma (status asthmaticus or any asthma diagnosis with a procedure code for endotracheal intubation). Depression and Anxiety subgroups were created based on ICD-9 codes. Data collected included demographics, hospital outcomes and charges and comparisons made between groups. RESULTS: There were 52,485 admissions for severe asthma: 45,094 (86%) with No Comorbid Psychiatric Illnesses, 1284 (2.4%) with depression, and 1297 (2.5%) with anxiety. Patients with depression or anxiety were older, had longer hospitalizations, and higher hospital charges (p < .001 for all). CONCLUSIONS: Comorbid depression or anxiety is associated with significantly longer hospitalizations and higher charges for children with severe asthma. These findings add to prior reports of worse outcomes for children with asthma and comorbid depression or anxiety and suggest that improved screening for and management of these conditions in children with asthma could improve hospital outcomes and reduce costs.

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.

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.