Pre- and post-operative cardiovascular CT in Stage I single ventricle palliation.

OBJECTIVES: The aim of this study is to describe clinical utility of low dose cardiac computed tomography (CT) in the evaluation of single ventricle physiology before and after Stage I palliation. BACKGROUND: Despite the increased utilization of CT imaging and advancement of CT technology, there are limited studies describing the routine clinical use of cardiac CT and radiation dose parameters in the single ventricle Stage I palliation. METHODS: This single center, retrospective study included 57 infants with single ventricle physiology who underwent cardiac CT scans between January 1, 2016 and November 30, 2020. Patients' demographic information, diagnosis, indication, total dose length product (DLP), computed tomographic dose index volume (CTDIvol), cardiac CT findings and intraoperative or intraprocedural findings were reviewed. Estimated effective radiation dose was calculated using a previously published conversion rate. RESULTS: The studies were performed using different generations of CT scanners over the 4 years period: Somatom AS 128, Somatom definition edge, Somatom Force (Siemens Medical Solutions). The studies performed with dual source scanner with prospective gated technique have lower radiation dose exposure with median effective radiation dose of 0.32 mSv. CONCLUSION: Pre- and post-operative cardiovascular CT in Stage I single ventricle palliation using newer generation scanners with prospective gated technique can be done with minimal radiation exposure and good image quality. Cardiac CT is a powerful imaging modality for better management planning in this group of patients.

Risk Factors for Increased Post-operative Length of Stay in Children with Coarctation of Aorta.

Coarctation of the aorta is a relatively common congenital heart disease occurring in 0.4-0.6 per 1000 live births with a low mortality rate. This is a retrospective study, with data abstracted from the Pediatric Health Information System database (PHIS). The study sample included pediatric patients less than or equal to 3 months of age discharged from a PHIS participating hospital between January 1, 2004 and December 31, 2018 who underwent surgical repair of isolated COA. The primary outcome for the study was post-operative hospital length of stay (PH-LOS), and the secondary outcome was in-hospital mortality. Patient demographics, comorbidities, procedures, and outcomes were assessed for statistical differences between eras. A total of 5354 patients were included in the study. The study highlights an increasing trend in PH-LOS and NICU hospital length of stay (NICU-LOS) across the investigated eras. Prematurity (before 37 weeks gestation) was an independent risk factor associated with both longer post-operative length of the stay and higher mortality. In addition, congenital anomalies, respiratory and abdominal surgeries have a significant impact on the post-operative hospital stay. In conclusion, this study is the largest published systematic assessment of PH-LOS in patients with isolated COA repair during infancy to date and identifies independent risk factors of increased PH-LOS.

A Fetal Presentation of a Ruptured Right Ventricular Diverticulum.

Ruptured diverticula and ventricular aneurysms are rare in the fetus, with a limited number of case reports published previously. Additional fetal complications secondary to these ventricular wall abnormalities can be seen. Interventional measures can be considered and attempted either in utero or postnatally to improve the chance of survival. We present a case of a ruptured diverticulum in a fetus and the clinical course.

Non-accidental Trauma Work-up: Unusual Retinal Finding Leads to a Rare Diagnosis.

Lipoprotein lipase (LPL) deficiency is an autosomal recessive condition due to absent or decreased activity of LPL enzyme. The LPL deficiency is a rare condition that is mainly diagnosed in children, but there is no standard screening method at this time. In our report, we describe a 6-day-old male infant who was found to have hypertriglyceridemia after lipemia retinalis was diagnosed from a fundoscopic examination for nonaccidental trauma work-up. After dietary modification was done, his triglyceride levels decreased significantly, and there were no complications. When diagnosed later in life, recurrent pancreatitis can be a significant complication.

Vitamin D, Low-Grade Inflammation and Cardiovascular Risk in Young Children: A Pilot Study.

Vitamin D has anti-inflammatory properties, and deficiency is prevalent in children. There is a paucity of data regarding vitamin D status and its correlation with low-grade inflammation and vasculature. We prospectively enrolled 25 children, 9-11 years old (13 male); 21 obese. Eight atherosclerosis-promoting risk factors were scored as categorical variables with the following thresholds defining abnormality: body mass index Z score ≥ 1.5; systolic blood pressure ≥ 95th percentile (for age, sex, and height); triglyceride ≥ 100 mg/dL; low-density lipoprotein cholesterol (LDL-C) ≥ 110 mg/dL; high-density lipoprotein cholesterol ≤ 45 mg/dL; hemoglobin A1C (HBA1C) ≥ 5.5; 25-hydroxyvitamin D [25(OH) D] ≤ 30 ng/mL, and tobacco smoke exposure. High-sensitivity C-reactive protein (hsCRP) was measured to assess low-grade inflammation and classified as low- (<1 mg/L), average- (1-3 mg/L), and high-risk (>3 to <10 mg/L) groups. The proportion of children within each hsCRP group who had above threshold risk factors was calculated. Carotid artery ultrasound was performed to measure carotid artery intima-media thickness (CIMT). Median (range) for 25(OH) D was 24 (17-45) ng/mL. Eighteen were either 25 (OH) D deficient (<20 ng/mL) or insufficient (20-30 ng/mL), and seven were sufficient (>30 ng/mL). hsCRP was 1.7 (0.2-9.1) mg/L, with 11 being <1.0 mg/L, 8 between 1.0-3.0 and 6 > 3.0 to < 10.0 mg/L. Risk factor score was 3.9 ± 1.7 out of eight. 25(OH) D levels did not correlate with hsCRP or CIMT. While vitamin D deficiency, inflammation, and risk factors coexist at a very young age, causative mechanisms remain unclear.

Advanced imaging improves detection of baffle leaks and stenoses after atrial switch compared with transthoracic echocardiography.

Current guidelines for adults with atrial switch repair recommend baseline cardiovascular magnetic resonance (CMR) for assessment of ventricular size and function, systemic and venous baffle obstruction and leaks, and valvular function. It also recommends transthoracic echocardiography (TTE) for outpatient follow up. Many such patients with implanted cardiac devices may need cardiac computed tomography (CCT) when CMR is not feasible. This study reviews and compares CMR, transesophageal echocardiography (TEE), CCT, cardiac catheterization with angiography and TTE in detection of baffle problems in patients after atrial switch operation. The medical records of patients who had at least one imaging study performed after atrial switch operation at our center from 2010 to 2020 were retrospectively reviewed. Results are reported as descriptive statistics for demographics and imaging findings. The principal outcome measure was detection of baffle leak and/or baffle stenosis. Fifty-seven patients had at least one cardiac imaging study after atrial switch operation (36 Senning and 21 Mustard operations) during the study period. Nearly 33% (19/57) had baffle complications of stenosis and/or baffle leaks identified. All 57 patients had TTE performed but baffle problems were noted by TTE in only 8 (14%) patients (7 baffle stenosis and 1 baffle leak). Of the 49 patients without known baffle problems by TTE, 24 had advanced imaging (TEE/CCT/CMR/angiography). Advanced imaging identified baffle problems in nearly half (11/24, 46%) of them (7 baffle leaks and 4 baffle stenosis). Baffle problems were present in (8/23) patients with transvenous cardiac devices. Baffle complications are common after atrial switch operations and in our study occur in 1/3rd of the patients. However, TTE is not sensitive enough to recognize these complications. Advanced imaging for detection of baffle complications should be considered in all patients after atrial switch operation.

Outcomes after prolonged extracorporeal membrane oxygenation support in children with cardiac disease--Extracorporeal Life Support Organization registry study.

OBJECTIVE: Extracorporeal membrane oxygenation remains the mainstay of mechanical circulatory support initiation and maintenance in children with cardiac insufficiency. However, the outcomes after prolonged extracorporeal membrane oxygenation for cardiac insufficiency in children remain ill defined. METHODS: We reviewed the International Extracorporeal Life Support Organization data from January 1, 2000, through December 31, 2011. We defined prolonged extracorporeal membrane oxygenation as uninterrupted support for ≥14 days. RESULTS: A total of 777 children aged <18 years required extracorporeal membrane oxygenation support for ≥14 days. Of these, 176 (23%) survived to hospital discharge. Compared with the nonsurvivors, the survivors were older (median age, 0.64 vs 0.10 years; P < .01), weighed more (median weight, 7.0 kg; range, 2-90; vs median, 4.0; range, 1.4-100 kg; P < .01), had a shorter duration of support (mean, 20 ± 6 vs 22 ± 9 days; P < .01), and a fewer number of organ system complications (mean, 2.8 ± 1.7 vs 3.6 ± 1.6, P < .01). Children with congenital heart disease had worse survival than those with cardiomyopathy and myocarditis (15% vs 42% and 52%, respectively; P < .01), and those with 1-ventricle physiology had worse survival than those with 2-ventricle physiology (10% vs 18%, P = .01). Seven percent (n = 56) reached cardiac transplantation, with 66% surviving to hospital discharge versus 19% of those not transplanted (P < .01). CONCLUSIONS: The attrition is high after prolonged extracorporeal membrane oxygenation support for cardiac insufficiency in children. Cardiac transplantation in this cohort was rarely achieved and was associated with high mortality compared with benchmarks for cardiac transplantation survival. Earlier redirection of care or conversion to other modes of mechanical support as a bridge to transplantation should be considered.