Assessment of Disaster Preparedness at the Household Level in a Pediatric Cardiology Clinic Population.

Natural and human-provoked disasters pose serious health risks to children, particularly children and youth with special healthcare needs, including many cardiology patients. The American Academy of Pediatrics (AAP) provides preparedness recommendations for families, but little is known about recommendation adherence. Caregivers of children seen in a pediatric cardiology clinic network were recruited to complete an electronic survey.

Computation of a probabilistic and anisotropic failure metric on the aortic wall using a machine learning-based surrogate model.

Scalar-valued failure metrics are commonly used to assess the risk of aortic aneurysm rupture and dissection, which occurs under hypertensive blood pressures brought on by extreme emotional or physical stress. To compute failure metrics under an elevated blood pressure, a classical patient-specific computer model consists of multiple computation steps involving inverse and forward analyses. These classical procedures may be impractical for time-sensitive clinical applications that require prompt feedback to clinicians.

Racial and ethnic differences in response to treatment for Marfan syndrome.

Objective: To determine whether racial/ethnic differences exist for the treatment of Marfan syndrome aortopathy. The 2014 Pediatric Heart Network randomised trial of losartan versus atenolol in Marfan syndrome paediatric and young adult patients showed no treatment differences in the rate of aortic root growth over 3 years; however, they did not examine racial/ethnic differences, and recent data suggest that angiotensin receptor blockers may have different pharmacologic effects in different racial/ethnic populations.

Methods: We performed a secondary analysis of public-use data from the Pediatric Heart Network randomised trial comparing the differences by race/ethnicity (non-Hispanic White, non-Hispanic Black, and Hispanic patients) amongst the treatment groups for the primary outcome of rate of aortic root enlargement by z score and secondary outcome of rate of change of absolute diameter of aortic root, z score and absolute diameter of ascending aorta, and blood pressure changes.

Heterogeneous Pulmonary Phenotypes in Filamin A Mutation-Related Lung Disease.

Interstitial lung disease (ILD) has been recently reported in a few patients with pathogenic variants in the Filamin A () gene with variable presentation and prognosis. This study evaluated the respiratory manifestations and clinical features in children with disease. We conducted a retrospective review of pediatric patients with variants in in a tertiary children's hospital.

Treatment of attention deficit/hyperactivity disorder in children with CHD.

Introduction: The prevalence of attention deficit/hyperactivity disorder in the general population is common and is now diagnosed in 4%-12% of children. Children with CHD have been shown to be at increased risk for attention deficit/hyperactivity disorder. Case reports have led to concern regarding the use of attention deficit/hyperactivity disorder medications in children with underlying CHD.

Simultaneous Presentation of Crohn's Disease and Takayasu Arteritis in a Teenage Patient.

A 14-year-old female with no significant medical history presented with hypertensive urgency, in the setting of 4 to 6 weeks of diarrhea, abdominal pain, headaches, anemia, weight loss, and high blood pressures. Her evaluation revealed signs of a systemic inflammatory process that was most suspicious for inflammatory bowel disease. However, when her hypertension was evaluated with a renal Doppler ultrasound, there were signs of narrowing, stenosis, and hypoplasia that led to a diagnostic angiogram of the abdominal aorta.

A Novel Anisotropic Failure Criterion With Dispersed Fiber Orientations for Aortic Tissues.

Accurate failure criteria play a fundamental role in biomechanical analyses of aortic wall rupture and dissection. Experimental investigations have demonstrated a significant difference of aortic wall strengths in the circumferential and axial directions. Therefore, the isotropic von Mises stress and maximum principal stress, commonly used in computational analysis of the aortic wall, are inadequate for modeling of anisotropic failure properties.

Identification of in vivo nonlinear anisotropic mechanical properties of ascending thoracic aortic aneurysm from patient-specific CT scans.

Accurate identification of in vivo nonlinear, anisotropic mechanical properties of the aortic wall of individual patients remains to be one of the critical challenges in the field of cardiovascular biomechanics. Since only the physiologically loaded states of the aorta are given from in vivo clinical images, inverse approaches, which take into account of the unloaded configuration, are needed for in vivo material parameter identification. Existing inverse methods are computationally expensive, which take days to weeks to complete for a single patient, inhibiting fast feedback for clinicians.

Myocarditis in the pediatric population: A review.

Myocarditis has a variable clinical presentation and there is still debate regarding accurate diagnostic criteria. Adding to the controversy surrounding this diagnosis, there is no clear consensus for the treatment or ongoing follow-up of patients with myocarditis. All of this makes the diagnosis and management of myocarditis a particular challenge in the pediatric population.

Clinical history and management recommendations of the smooth muscle dysfunction syndrome due to ACTA2 arginine 179 alterations.

Purpose: Smooth muscle dysfunction syndrome (SMDS) due to heterozygous ACTA2 arginine 179 alterations is characterized by patent ductus arteriosus, vasculopathy (aneurysm and occlusive lesions), pulmonary arterial hypertension, and other complications in smooth muscle-dependent organs. We sought to define the clinical history of SMDS to develop recommendations for evaluation and management.

Methods: Medical records of 33 patients with SMDS (median age 12 years) were abstracted and analyzed.

Biomechanical properties of the thoracic aorta in Marfan patients.

Background: Marfan syndrome (MFS), a genetic disorder of the connective tissue, has been strongly linked to dilation of the thoracic aorta, among other cardiovascular complications. As a result, MFS patients frequently suffer from aortic dissection and rupture, contributing to the high rate of mortality and morbidity among MFS patients. Despite the significant effort devoted to the investigation of mechanical and structural properties of aneurysmal tissue, studies on Marfan aneurysmal biomechanics are scarce.

Systolic ejection click versus split first heart sound: Are our ears deceiving us?

Objective: Bicuspid aortic valve (BAV) disease is associated with potential lifetime complications, but auscultation of a BAV click is commonly missed or mistaken for a benign split first heart sound. Our objective was to determine whether pediatric cardiologists could reliably distinguish between BAV clicks and benign split first heart sounds.

Design: Quality evaluation project using de-identified recordings from an outpatient pediatric cardiology clinic.

Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children.

Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia.

Exome sequencing of patients with histiocytoid cardiomyopathy reveals a de novo NDUFB11 mutation that plays a role in the pathogenesis of histiocytoid cardiomyopathy.

Histiocytoid cardiomyopathy (Histiocytoid CM) is a rare form of cardiomyopathy observed predominantly in newborn females that is fatal unless treated early in life. We have performed whole exome sequencing on five parent-proband trios and identified nuclear-encoded mitochondrial protein mutations in three cases. The molecular genetic basis of Histiocytoid CM remains unknown despite several hypotheses in medical literature.

Lower extremity edema in a child due to pectus excavatum.

A previously healthy 11-year-old girl was referred for pediatric cardiology evaluation because of the development of progressive bilateral lower extremity swelling over the course of 2 years. Her prior workup had included a negative result for proteinuria and a negative ultrasound for deep venous thrombosis. On physical examination, in addition to her edema, she was found to have a severe pectus excavatum deformity, which prompted cardiac magnetic resonance imaging.

Necrotizing enterocolitis in infants with congenital heart disease: the role of red blood cell transfusions.

Necrotizing enterocolitis (NEC) is a rare but catastrophic complication that may occur in newborns with congenital heart disease (CHD). In the preterm population, transfusion of red blood cells (RBCs) and use of RBCs with longer storage time have been independently associated with the development of NEC. To date, it is not known whether similar associations exist for the term newborn with CHD.

The relationship between in-house attending coverage and nighttime extubation following congenital heart surgery*.

Objectives: Many cardiac ICUs have instituted 24/7 attending physician in-house coverage, which theoretically may allow for more expeditious weaning from ventilation and extubation. We aimed to determine whether this staffing strategy impacts rates of nighttime extubation and duration of mechanical ventilation.

Design: National data were obtained from the Virtual PICU System database for all patients admitted to the cardiac ICU following congenital heart surgery in 2011 who required postoperative mechanical ventilation.

Hemolytic anemia after placement of an intraluminal pulmonary artery band.

A newborn with double outlet right ventricle, malposed great vessels, hypoplastic aortic arch, and coarctation of the aorta with no restriction to pulmonary blood flow underwent palliation with arch augmentation and placement of an intraluminal Dacron patch pulmonary artery band (PAB). She subsequently presented in shock because of profound hemolytic anemia. Her intraluminal PAB was taken down and replaced with a traditional extraluminal band with resolution of her hemolysis.

Coarctation of the aorta in the setting of tetralogy of Fallot: an uncommon cause of myocardial dysfunction.

The combination of both right and left heart obstruction has only rarely been described in the medical literature. We present three cases of coarctation of the aorta in patients with variants of tetralogy of Fallot and hypothesise that this condition may be more common than previously suspected and could represent a hidden cause of morbidity in patients with pulmonary atresia/ventricular septal defect.

Necrotising enterocolitis in infants with congenital heart disease: the role of enteral feeds.

Objective: Necrotising enterocolitis is a rare, though catastrophic complication that may occur in term newborns with congenital heart disease. There is considerable controversy regarding the factors that lead to necrotising enterocolitis in this population. We sought to determine the incidence of necrotising enterocolitis among term and near-term newborns with congenital heart disease, focusing on the relationship of enteral feeding to this complication.