Endovascular Repair of a Descending Thoracic Aortic Aneurysm in a Pediatric Patient with Tuberous Sclerosis: A Case Report and Review of the Literature.

Aortic aneurysm in children is rare, but has been described in the tuberous sclerosis complex (TSC) population. While surgical repair has been utilized as the primary means of intervention, we present the first known case reporting exclusion of a descending thoracic aortic aneurysm with percutaneous covered stent implantation in a pediatric patient with TSC. A review of the literature is also included herein.

Percutaneous Common Carotid Artery Access for Cardiac Interventions in Infants Does Not Acutely Change Cerebral Perfusion.

Pediatric cardiac interventions via percutaneous common carotid artery (CCA) access have been shown to be safe and effective. However, the impact of placement of a sheath in the carotid artery for interventions on cerebral perfusion is unknown. In this study we used cerebral near-infrared spectroscopy (NIRS) to analyze the effects of percutaneous CCA access for cardiac interventions on cerebral perfusion. This study is a retrospective chart review carried out at a tertiary care center on all pediatric patients who underwent percutaneous cardiac catheterization via carotid artery access from January 2010 to January 2020. All patients who had ipsilateral NIRS recorded on the side of carotid artery access were included. Patients with only partial or no ipsilateral NIRS data were excluded. The primary outcome measure was the change in NIRS upon CCA access; the mean NIRS for 15 min before obtaining access was compared to the mean NIRS during the procedure and to the mean NIRS 15 min after removal of the CCA sheath. We hypothesized that there would be a significant drop in NIRS values on the side of CCA access. There were 48 catheterizations in the study period where percutaneous CCA was accessed. Of those, 21 catheterizations had complete data and were included in the study. 13 (62%) were of males. The median age was 23 days (IQR 7-79). The indications for CCA access were patent ductus arteriosus stent implantation (n = 13; 62%), aortic valvuloplasty (n = 5; 24%), balloon angioplasty of coarctation of aorta (n = 2; 10%), and renal artery angioplasty (n = 1; 4%). In 16 patients (72%), the left common carotid artery was accessed. The median weight of the patients was 3.3 kg (IQR 2.8-2.9). The most common sheath size used was 4F, in 16 patients (72%). The mean NIRS prior to the procedure was 67 ± 15%, during the procedure was 68 ± 20%, and after removal of sheath was 68 ± 21%. Paired t test of cerebral NIRS before, during, and after the procedure showed no significant change with CCA access (P = 0.08). No patient in the series had a documented neurologic deficit following the procedure. Percutaneous CCA access was not associated with a decrease in NIRS on the side of the access during the procedure, suggesting there was no significant acute change in cerebral perfusion with CCA access.

Acute Rheumatic Fever.

The incidence of acute rheumatic fever (ARF) is 8 to 51 per 100,000 people worldwide. It most commonly affects children 5 to 15 years of age after a group A streptococcal infection. Overcrowding and poor socioeconomic conditions are directly proportional to the incidence of ARF. Rheumatic carditis is a manifestation of ARF that may lead to rheumatic heart disease (RHD). Timely treatment of group A streptococcal infection can prevent ARF, and penicillin prophylaxis can prevent recurrence of ARF. Prevention of recurrent ARF is the most effective way to prevent RHD. ARF is diagnosed using the 2015 modified Jones criteria. There is no gold standard laboratory test. Therefore, clinicians need to be aware of the clinical signs and symptoms of ARF to include in their differential diagnosis when seeing such patients. Secondary prophylaxis with benzathine penicillin G has been shown to decrease the incidence of RHD and is key to RHD control. Clinicians need to understand the implications of secondary prophylaxis for ARF. There is also a need to improve ARF diagnosis, to find novel therapies to reduce the incidence of ARF, and to reduce the prevalence of RHD. RHD research is neglected and underfunded. Thus, there is also a need for RHD advocacy and public health awareness to increase research on RHD.

Trends in Infant Mortality After TAPVR Repair over 18 Years in Texas and Impact of Hospital Surgical Volume.

For some congenital heart lesions, higher institutional surgical volume has been associated with better survival than in lower volume centers. The relationship between institutional surgical volume and mortality in infants after total anomalous pulmonary vein return (TAPVR) repair has not been well explored. The Texas Inpatient Public Use Data File was queried for hospitalizations including TAPVR repair in infants between January 1, 1999 and December 31, 2016. We first evaluated the change in mortality over the study period. We then evaluated associations between institutional TAPVR surgical volume and mortality using univariable analysis and multivariable analysis accounting for center effects. For secondary analyses, we evaluated the association between volume and mortality among non-mutually exclusive TAPVR subsets, including isolated TAPVR, TAPVR with other congenital heart disease (CHD), TAPVR with heterotaxy, and TAPVR with single ventricle anatomy. Of 971 surgical hospitalizations that met inclusion criteria, 62% were male. Mortality after TAPVR repair decreased over the study period from 15.1% (1999-2004) to 7.6% (2012-2016) with an odds ratio per increasing year of 0.96 (95% CI 0.92-0.99, p = 0.030). By univariable analysis, earlier era, preterm birth, lower institutional surgical volume, heterotaxy, and additional CHD were associated with increased mortality. Institutional surgical volume remained significant in multivariate analysis with an odds ratio per increase in surgical volume of every 10 patients of 0.93 (95% CI 0.90-0.96, p < 0.001). When examining by subgroup, isolated TAPVR had the lowest mortality (n = 606, mortality = 6%), compared to TAPVR with other CHD (n = 359, mortality = 20%), TAPVR with heterotaxy (n = 135, mortality = 21%), and TAPVR with single ventricle (n = 128, mortality = 23%). In all groups except those with single ventricle, higher surgical volume was associated with lower mortality in multivariate analyses (isolated TAPVR p = 0.001, TAPVR with other CHD p = 0.009, TAPVR with heterotaxy p < 0.001, TAPVR with single ventricle p = 0.161). This is the first study to demonstrate an association between institutional surgical volume and mortality after TAPVR repair. Higher volume centers are associated with lower hospital mortality after TAPVR repair, including TAPVR with other CHD.

Venous duct stenting in a 940-gram infant with infradiaphragmatic total anomalous pulmonary venous return.

An extremely low birthweight infant (940 grams) with a rare variant of obstructed infracardiac total anomalous pulmonary venous return underwent stenting of the venous duct as bridge to later surgical intervention. While technically challenging, this procedure represents a bridge to surgery for infants who might otherwise not be surgical candidates.

Single tertiary center experience using Gore Cardioform Atrial Septal Defect Occluder for secundum atrial septal defect closure with a focus on deficient rims.

BACKGROUND: The data on the use of Gore Cardioform Septal Occluder (GCA; W. L. Gore and Associates, Inc.) for atrial septal defect (ASD) with deficient rims is limited. METHODS: All patients evaluated by transesophageal echocardiogram (TEE) for ASD occlusion were included. TEE planes at 35°, 0°, and 90° were assessed for anterior-superior (AS) and posterior (P), anterior-inferior (AI) and posterior-superior (PS), as well as superior (S) and inferior (I) rims. ASD size >20 mm, and rims less than 5 mm were defined as large and deficient, respectively. We included patients who had a procedural failure along with the patients in whom the procedure was not attempted after echocardiogram in the unsuccessful group. RESULTS: In 148 patients, the median weight, age, and ASD size were 36 kg (range, 8-60 kg), 11.8 years (range, 1-60 years), and 14.2 ± 8.28 mm, respectively. One or more deficient rims were noted in 112 of 148 (75.7%): 99 (67%) AS, 36 (24%) P, 17 (11%) AI, 30 (20%) PS, 26 (18%) S, and 33 (22%) I. ASD closure was performed in 115 (78%) patients. The procedure was successful in 111 (96.5%) patients with procedural failure in 4 (3.4%) patients. Multiple deficient rims were associated with reduced procedural success (OR 0.36, 95% CI, 0.25-0.56). On multivariate analysis deficient P, PS, and I rims were associated with an unsuccessful group (P = .001, .046, and .005, respectively). Complications included 1 device embolization, 1 vascular injury, and 5 arrhythmias. CONCLUSIONS: Transcatheter closure of ASDs with deficient rims is feasible using GCA. Large ASDs with deficient P, PS, and I rims were associated with unsuccessful closure. Risk stratification and comprehensive evaluation of ASD rims is vital for the use of GCA.

Does Cardiac Catheterization Facilitate Hemodynamic Optimization of Pediatric Patients on Continuous-Flow Ventricular Assist Devices?

Understanding optimal ventricular assist device (VAD) parameters for pediatric patients is valuable given the inherent issue of patient-device size mismatch and heterogeneous cardiac anatomy in children. We evaluated our center's experience of continuous-flow VAD (CF-VAD) optimization using cardiac catheterization. We performed a retrospective analysis of all patients on CF-VAD support who underwent hemodynamic heart catheterization from 2013 to 2018. Fifteen patients had 16 hemodynamic catheterizations performed. The indications for hemodynamic optimization by catheterization included clinical signs of heart failure while on CF-VAD (9 of 16, 56%), pretransplant evaluation of pulmonary hypertension (2 of 16, 13%), or assessment of myocardial recovery (5 of 16, 31%). The median age at catheterization was 12 years (interquartile range: 8-16). Median baseline speed of device was 2333 ± 253 rotations per minute. The goal was to find the speed at which optimal hemodynamics were achieved, defined by low wedge pressure with an acceptable central venous pressure. Of the 16 catheterizations, there were 9 (56%) speed increases to achieve optimal hemodynamics and 5 (33%) speed decreases for hemodynamic optimization or for potential explant. The speed was not changed in 2 (13%) catheterizations as the patients were determined to be at an optimal hemodynamic state. Overall, VAD settings were optimized in 75% (14 of 16) of hemodynamic catheterizations. There were no adverse events related to catheterization. Thus, we conclude that catheterization-based hemodynamic assessment is safe and effective for optimizing VAD speed and provides guidance on medical management in children supported on CF-VAD.

Mullins-Sheath Facilitated Delivery of Gore Cardioform ASD Occluder Devices for Closure of Large or Challenging Secundum Atrial Septal Defects.

OBJECTIVES: To describe a deployment technique of the Gore Cardioform atrial septal defect (ASD) occluder (W.L. Gore and Associates) for large secundum ASDs and ASDs with challenging anatomy. BACKGROUND: The Gore Cardioform ASD occluder has recently been approved for closure of secundum ASDs; however, there are limitations to its delivery system. METHODS: A retrospective study was conducted on the use of a Mullins sheath (Cook Medical) to facilitate Gore Cardioform ASD occluder delivery for secundum ASD closure in the cardiac catheterization laboratory from June, 2017 to December, 2019 at Texas Children's Hospital/Baylor College of Medicine. RESULTS: Out of 98 patients who underwent an attempt at ASD closure using the Gore Cardioform ASD occluder, a Mullins sheath was used in 52 patients (median age, 8 years [interquartile range, 4-13 years] and weight 27.2 kg [interquartile range, 17.9-51.2 kg]), with a successful implant in 46/52 patients (88%). The Mullins sheath was primarily used to deliver large devices (>32 mm) in 38/46 successful implants (83%). There were 2 major adverse events (atrial fibrillation requiring cardioversion). At a median follow-up of 43 days (interquartile range, 1-374 days), no patient had more than a mild residual shunt. The ASD size, maximum sheath size, and device size were larger in patients in whom the Mullins sheath was used as compared with those patients in whom a Mullins sheath was not used. CONCLUSIONS: The Mullins sheath-facilitated delivery of the Gore Cardioform ASD occluder device may be a useful adjunct technique for closure of large secundum ASDs and secundum ASDs with challenging anatomy.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management. Geocoding was used to determine a census tract-based socioeconomic deprivation index. Descriptive statistics of patient characteristics and regression analysis of RHD classification, disease severity, and initial antibiotic prescription according to community deprivation were obtained. Data for 947 cases showed median age at diagnosis of 9 years; 51% and 56% identified as male and non-White, respectively. Most (89%) had health insurance and were first diagnosed in the United States (82%). Only 13% reported travel to an endemic region before diagnosis. Although 96% of patients were prescribed secondary prophylaxis, only 58% were prescribed intramuscular benzathine penicillin G. Higher deprivation was associated with increasing disease severity (odds ratio, 1.25; 95% CI, 1.08-1.46). Conclusions The majority of recent US cases of ARF and RHD are endemic rather than the result of foreign exposure. Children who live in more deprived communities are at risk for more severe disease. This study demonstrates a need to improve guideline-based treatment for ARF/RHD with respect to secondary prophylaxis and to increase research efforts to better understand ARF and RHD in the United States.

Ultrasound-guided intraoperative trans-epicardial needle biopsy of an intracardiac tumor.

We describe the use of a novel interventional approach to the histopathologic diagnosis of a ventricular septal tumor using intraoperative ultrasound-guided trans-epicardial biopsy without the need for cardiopulmonary bypass in a 2-year-old child. This novel approach has not been previously reported. Multidisciplinary collaboration between cardiothoracic surgery, cardiology, cardiac imaging, and interventional radiology provided the ability to perform cardiac biopsy. This technique may be used in specific cases of cardiac tumors where tissue diagnosis is important, but surgical resection is deemed excessively risky or impossible.

Genetic abnormalities/syndromes significantly impact perioperative outcomes of conotruncal heart defects.

OBJECTIVES: The main objective of the study is to characterize the effects of genetic abnormalities/syndromes (GA/S) on perioperative outcomes of cardiac surgeries involving repair of conotruncal heart defects (CTHD). DESIGN: The study involves a single-center retrospective analysis of patients who underwent complete repair of CTHDs (tetralogy of Fallot [TOF], truncus arteriosus, interrupted aortic arch, and ventricular septal defect with coarctation) between January 2000 and December 2015. The primary outcome was the post operative length of stay (PLOS). The secondary outcomes were mortality, cardiac complications, hematologic complications, infections, and number of medications-at-discharge. SETTING: Cardiac intensive care unit in a tertiary pediatric hospital in South Florida that performs around 300 open-heart surgeries a year. SUBJECTS: A total of 177 patients with CTHDs who underwent cardiac surgeries in the stated time period were included in the final study cohort. MEASUREMENTS AND MAIN RESULTS: Majority of patients had TOF (72.5%) and 46 (26%) had GA/S. The most common GA/S was 22q11 deletion (37%). PLOS was significantly increased in patients with GA/S (P < 0.05). Patients with GA/S were 4.5 times more likely to have a postoperative cardiac complication, 4.2 times more likely to have a postoperative infection, and received 1.6 times more medications at discharge than those without GA/S. However, GA/S was not associated with increased perioperative mortality. Black patients were three times more likely to have a longer PLOS than White patients. CONCLUSIONS: Perioperative outcomes in patients with GA/S suggested an increased residual cardiovascular disease and increased resource usage. Notably, this is the first study demonstrating the effect of race and ethnicity on PLOS in CTHD patients.

Cavernous Sinus Thrombosis Secondary to Retropharyngeal Abscess.

This is a case of bilateral cavernous sinus thrombosis in a five-and-a-half-year-old boy who presented with bilateral proptosis with difficulty in swallowing and excessive salivation. Clinical features along with contrast-enhanced computed tomography and hemogram diagnosed the case as bilateral cavernous sinus thrombosis and retropharyngeal abscess with third cranial nerve palsy. The patient was treated with standard regimen successfully. Cavernous sinus thrombosis is a very rare complication of retropharyngeal abscess.

A case of male goltz syndrome.

We present the case of a boy with a clinical diagnosis of Goltz syndrome (focal dermal hypoplasia), a rare genodermatosis characterized by widespread dysplasia of mesodermal and ectodermal tissues. A 9-year-old male patient with Goltz syndrome presented with typical skin lesions along with progressive dimness of vision and mental retardation since birth. It is inherited in an X-linked dominant fashion and is normally lethal in male patients, and so very few male patients, like the index case, have been reported.

A case of antley-bixler syndrome.

Antley-Bixler syndrome (ABS) is rare form of craniosynostosis of both autosomal dominant and autosomal recessive inheritance. We are reporting a female term appropriate for gestational age newborn with clinical features of frontal bossing, brachycephaly, proptosis, synostosis of radioulnar joints, hemangioma over nose and philtrum, hydrocephalus suggestive of ABS.

Changes in biochemical contents of expressed breast milk on refrigerator storage.

To determine the biochemical integrity of refrigerated breast milk for 96 hours at 4C, a longitudinal observational study done with fresh milk samples. It is found that there were significant changes in pH, serum albumin and lactose concentrations in breast milk though within normal range.