Although CorMatrix used for valve repair induced an intense inflammatory response, little or no remodeling to form tissue resembling a 3-layered native valve was seen at ≤9 months after implantation.

Background In neonates with single ventricle, smaller ascending aorta diameter is associated with cerebral white matter ( WM ) microstructural abnormalities. We sought to determine whether this association persists into adolescence. Methods and Results Ascending aorta Z scores were obtained from first postnatal echocardiogram. Brain magnetic resonance imaging with diffusion tensor imaging was acquired in adolescence and used to obtain fractional anisotropy, axial diffusivity, radial diffusivity, and mean diffusivity in 33 WM tract regions of interest. Partial Pearson correlation coefficients were evaluated for associations between ascending aorta Z scores and WM microstructure measures, adjusting for sex, age at magnetic resonance imaging, scanner field strength, and Norwood status. Among 42 single ventricle patients aged 10 to 19 years, 31 had undergone the Norwood procedure as neonates. Lower ascending aorta Z scores were associated with lower fractional anisotropy in bilateral pontine crossing tracts ( P =0.02), inferior fronto‐occipital fasciculus ( P =0.02), and inferior longitudinal fasciculus ( P =0.01); left cingulum–cingulate bundle ( P =0.01), superior longitudinal fasciculus ( P =0.04), and superior longitudinal fasciculus–temporal component ( P =0.01); and right cingulum–hippocampal bundle (P =0.009) and inferior cerebellar peduncle ( P =0.01). Lower ascending aorta Z scores were associated with higher radial diffusivity and mean diffusivity in a similar regional pattern but not with axial diffusivity. Conclusions In adolescents with single ventricle, smaller aorta diameter at birth is associated with abnormalities of WM microstructure in a subset of WM tracts, mostly those located in deeper brain regions. Our findings suggest that despite multiple intervening medical or surgical procedures, prenatal cerebral blood flow may have a lasting influence on WM microstructure in single‐ventricle patients.

Pulmonary vein stenosis (PVS) is a rare, frequently lethal disease with heterogeneous phenotypes and an unclear etiology. Limited studies have reported associations between PVS and congenital heart disease (CHD), chronic lung disease (CLD), and/or prematurity; however, to date, there have been no studies that report detailed clinical syndromic phenotypes and the potential role of genetics in PVS. An existing registry of multivessel PVS patients seen at Boston Children’s Hospital (BCH) was queried between August 2006 and January 2017 for all existing genetic testing data on these patients. PVS was defined as an intraluminal pulmonary venous obstruction in ≥2 vessels with mean pressure gradients > 4 mmHg. One-hundred-and-fifty-seven patients (46% female, with a median age at PVS diagnosis of 3 months) formed the cohort. Seventy-one (45%) patients had available genetic testing information. Of the 71 patients, a likely genetic diagnosis was found in 23 (32%) patients: 13 (57%) were diagnosed with Trisomy 21 (T21), five (22%) with Smith–Lemli–Opitz Syndrome, five (22%) had other pathologic genetic disease, and 24 (33%) had variants of unknown significance. The majority of 13 patients with T21 and PVS had common atrioventricular canal (CAVC) (10, 77%) and all had severe pulmonary hypertension (PHTN), which led to their PVS diagnosis. In our study, PVS was associated with T21, the majority of whom also had CAVC and PHTN. Therefore, complete assessment of the pulmonary veins should be considered for all T21 patients, especially those with CAVC presenting with PHTN. Furthermore, prospective standardized genetic testing with detailed clinical phenotyping may prove informative about potential genetic etiologies of PVS.

This case series describe two patients with giant aneurysms of the atrial appendages. This report discusses the clinical symptoms, imaging modalities, indications for surgical intervention, and histology of atrial appendage aneurysms.

The morphology of ALMR as evaluated by echocardiography is heterogeneous and can be classified on the basis of lesion location and leaflet involvement. Intraleaflet morphology is associated with significant mitral stenosis; younger age at diagnosis and short chordae are associated with ALMR resection.

PMI provided most gross anatomic cardiac diagnoses available by autopsy in our series of patients with congenital heart defects and the cost appears to be lower.

Background: Primary hypertension (HTN) in pediatrics is a significant public health concern with increasing prevalence. The risk for adult HTN and end-organ damage begins in childhood. Therefore, evidence-based guidelines were developed by the American Academy of Pediatrics (AAP) in 2017 with detailed recommendations on screening and diagnosis. Ongoing follow-up is essential for diagnosis (at least 3 follow-up visits are needed to diagnose HTN if BP is high). However, gaps in care(GIC) exist in detecting and diagnosing HTN in clinical practice with barriers to accurate HTN detection at the patient, provider, and health system levels. Patient factors such as social determinants of health (SDoH), including the Childhood Opportunity Index (COI), leading to GIC with recommended follow-up needed for HTN diagnosis at primary care visits are poorly understood. Methods: A total of 69,960 patients aged 6-22 years seen at Nemours Children’s Health in DE and PA between August 2017- March 2019 formed the cohort. SDoH, including language, race, ethnicity, and COI, were explored. Data were analyzed using descriptive statistics and logistic regression. Results: Only 596 patients(0.9%) had HTN or were recognized as having a high BP. 73%(432) patients had over a 1-year GIC. GIC was associated with insurance type(P=0.05) and having 2 or fewer caregivers(P=0.004). GIC was not associated with race, ethnicity, language, interpreter use, or COI. Conclusions: Despite evidence-based guidelines, HTN detection remains poor. Patient factors, including insurance and the number of caregivers, likely lead to GIC with follow-up for HTN in pediatric patients. However, GIC for routine HTN follow-up are not broadly associated with SDoH. Other barriers likely exist at the provider and health system level leading to GIC and poor HTN detection.

In this cross-sectional, nationally representative study, Vasudevan et al 1 describe the prevalence of high blood pressure (BP) and other cardiovascular disease (CVD) risk factors in children and adolescents enrolled in the 2016-2018 Comprehensive National Nutrition Survey (CNNS) of all 29 states of India. The analysis shows an alarming prevalence of high BP in Indian youth: 35% of 10-to 12-year-olds and 25% of 13-to 19-year-olds had BP in the stage 1 or 2 hypertension range, defined according to 2017 American Academy of Pediatrics cut points. Moreover, youth with high BP were more likely to have other CVD risk factors, such as overweight or obesity, high blood glucose levels, or lipid abnormalities. Using 2011 Indian census data, this finding translates to approximately 69 million Indian youth with high BP and possibly other CVD risk factors.