Identifying Factors that Influence Pediatric Echo Lab Image Quality: Development and Validation of a New Assessment Tool.

No method of evaluating transthoracic echocardiograms (TTE) image quality (IQ) has been validated. Furthermore, structural echo lab elements impacting IQ are unknown. We sought to develop and validate a TTE IQ grading tool and determine patient and echo lab features associated with IQ.

Variants Are Associated with Atrial Dysfunction in Neonates with Hypoplastic Left Heart Syndrome.

: variants are the most well-known genetic risk factor (10%) for hypoplastic left heart syndrome (HLHS) and are associated with decreased cardiac transplant-free survival. encodes for α-myosin heavy chain (α-MHC), a contractile protein expressed in the neonatal atria. We therefore assessed atrial function in HLHS patients with variants.

TANGO1 inhibitors reduce collagen secretion and limit tissue scarring.

Uncontrolled secretion of ECM proteins, such as collagen, can lead to excessive scarring and fibrosis and compromise tissue function. Despite the widespread occurrence of fibrotic diseases and scarring, effective therapies are lacking. A promising approach would be to limit the amount of collagen released from hyperactive fibroblasts.

The Effect of Udenafil on Heart Rate and Blood Pressure in Adolescents With the Fontan Circulation.

The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy.

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study.

Objective: In the SVR trial (Single Ventricle Reconstruction), newborns with hypoplastic left heart syndrome were randomly assigned to receive a modified Blalock-Taussig-Thomas shunt (mBTTS) or a right ventricle-to-pulmonary artery shunt (RVPAS) at Norwood operation. Transplant-free survival was superior in the RVPAS group at 1 year, but no longer differed by treatment group at 6 years; both treatment groups had accumulated important morbidities. In the third follow-up of this cohort (SVRIII [Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type]), we measured longitudinal outcomes and their risk factors through 12 years of age.

Impact of Udenafil on Echocardiographic Indices of Single Ventricle Size and Function in FUEL Study Participants.

Background: The FUEL trial (Fontan Udenafil Exercise Longitudinal) demonstrated statistical improvements in exercise capacity following 6 months of treatment with udenafil (87.5 mg po BID). The effect of udenafil on echocardiographic measures of single ventricle function in this cohort has not been studied.

Is native aortic valvuloplasty at time of Norwood operation in infants with hypoplastic left heart syndrome and aortic stenosis safe?

In hypoplastic left heart syndrome, the size and function of the left ventricle vary and are dependent on the patency of the aortic valve. A patent native aortic valve, permitting left ventricular ejection, can augment cardiac output. We performed a retrospective chart review of patients with hypoplastic left heart syndrome and a stenotic aortic valve who underwent native aortic valvuloplasty at the time of Norwood and found that none of the eight patients identified had clinically significant aortic insufficiency.

Comparing Variants of the Cell-Penetrating Peptide sC18 to Design Peptide-Drug Conjugates.

Herein, the design and synthesis of peptide-drug conjugates (PDCs) including different variants of the cell-penetrating peptide sC18 is presented. We first generated a series of novel sequence mutants of sC18 having either amino acid deletions and/or substitutions, and then tested their biological activity. The effects of histidine substituents were found to be not meaningful for sC18 uptake and cell selectivity.

The pediatric heart network's study on long-term outcomes of children with HLHS and the impact of Norwood Shunt type in the single ventricle reconstruction trial cohort (SVRIII): Design and adaptations.

Background: The Single Ventricle Reconstruction (SVR) Trial was the first randomized clinical trial of a surgical approach for treatment of congenital heart disease. Infants with hypoplastic left heart syndrome (HLHS) and other single right ventricle (RV) anomalies were randomized to a modified Blalock Taussig Thomas shunt (mBTTS) or a right-ventricular-to-pulmonary-artery shunt (RVPAS) at the time of the Norwood procedure. The aim of the Long-term Outcomes of Children with HLHS and the Impact of Norwood Shunt Type (SVR III) study is to compare early adolescent outcomes including measures of cardiac function, transplant-free survival, and neurodevelopment, between those who received a mBTTS and those who received an RVPAS.

Intranasal dexmedetomidine for transthoracic echocardiography in infants with shunt-dependent single ventricle heart disease.

Objectives: We investigated the efficacy and complication profile of intranasal dexmedetomidine for transthoracic echocardiography sedation in patients with single ventricle physiology and shunt-dependent pulmonary blood flow during the high-risk interstage period.

Methods: A single-centre, retrospective review identified interstage infants who received dexmedetomidine for echocardiography sedation. Baseline and procedural vitals were reported.

Conal Septal Hypoplasia in Tetralogy of Fallot-Impact on Clinical Course, Treatment Strategies, and Outcomes After Surgical Intervention.

We sought to characterize the clinical course and outcomes of intervention for Tetralogy of Fallot (TOF) with associated conal septal hypoplasia (CSH) compared to those with identifiable conal septum on initial newborn echocardiogram. We performed a retrospective, 1:2 case-control study of children with TOF anatomy, 33 with CSH and 66 with typical TOF, who underwent surgical repair from 1991-2019 at Children's Wisconsin. Data on echocardiographic anatomic features, systemic oxygen saturations, medical therapies, admissions, palliative interventions, operative strategies, and long-term follow-up were compared.

Obstruction of the Anomalous Pulmonary Venous Connection in Scimitar Syndrome: Progression to Spontaneous Anomalous Pulmonary Vein Atresia during Infancy without Intervention.

• Infantile scimitar syndrome can progress from pulmonary venous obstruction to atresia. • Echo evidence of RPA diastolic flow reversal can be a sign of venous obstruction. • Despite development of atresia, this infant was asymptomatic, with normal PA pressure.

Prognostic utility of echocardiographically derived left ventricular strain in assessing neonatal enteroviral myocarditis outcome.

Background: Neonatal enteroviral myocarditis is a rare but potentially fatal illness. We sought to identify echocardiographic markers at diagnosis that could help risk-stratify infants for poor outcome and to characterise late sequelae.

Methods: We reviewed data for infants <30 days of age diagnosed with enteroviral myocarditis between 1999 and 2019 at Children's Wisconsin.

Congenital Left Coronary Atresia: Diagnostic Pearls and Management Challenges in Two Children Presenting with Syncope.

• LMCA ostial atresia has variable clinical presentation. • Diagnosis of LMCA ostial atresia is challenging and often requires multiple modalities. • Coronary revascularization is possible for LMCA ostial atresia by varying surgical techniques.

Native Aortic Root Thrombus in 3-Year-Old Fontan Patient with Hypoplastic Left Heart Syndrome: Presentation and Echocardiographic Findings of This Life-Threatening Complication.

• Native aortic root thrombus is a life-threatening complication in patients with HLHS. • Echo evaluation of the native aortic root is an important consideration. • Echo monitoring is a useful tool for assessment of thrombus response to therapy.

Computational Assessment of Hemodynamic Significance in Patients With Intramural Anomalous Aortic Origin of the Coronary Artery Using Virtually Derived Fractional Flow Reserve and Downstream Microvascular Resistance.

Anomalous aortic origin of a coronary artery (AAOCA) is the second most common cause of sudden cardiac death in young athletes. One of the hypothesized mechanisms of ischemia in these patients is the lateral compression of the anomalous artery with an intramural or interarterial course. The presence of a narrowing in the anomalous artery will cause physiologic changes in downstream resistance that should be included for computational assessment of possible clinical ramifications.

Intranasal dexmedetomidine: the ideal drug for sedation in the pediatric echo lab?

Background: Intranasal dexmedetomidine is an attractive option for procedural sedation in pediatrics due to ease of administration and its relatively short half-life. This study sought to compare the safety and efficacy of intranasal dexmedetomidine to a historical cohort of pediatric patients sedated using chloral hydrate in a pediatric echo lab.

Methods: Chart review was performed to compare patients sedated between September, 2017 and October, 2019 using chloral hydrate and intranasal dexmedetomidine.

Height Versus Body Surface Area to Normalize Cardiovascular Measurements in Children Using the Pediatric Heart Network Echocardiographic Z-Score Database.

Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18 years of age enrolled in the Pediatric Heart Network Echo Z-Score Project.

Impact of Valve Type (Ross vs. Mechanical) on Health-Related Quality of Life in Children and Young Adults with Surgical Aortic Valve Replacement.

Background: The impact of aortic valve replacement (AVR) type on health-related quality of life (HRQOL) in adolescents and young adults is unclear, but may vary depending on need for anticoagulation or re-intervention. We sought to determine the differences in HRQOL following AVR with either the Ross procedure or mechanical AVR in this young population.

Methods: Patients 14-35 years old and at least 1 year post-AVR were included.

Pediatric Heart Network Echocardiographic Z Scores: Comparison with Other Published Models.

Background: Different methods have resulted in variable Z scores for echocardiographic measurements. Using the measurements from 3,215 healthy North American children in the Pediatric Heart Network (PHN) echocardiographic Z score database, the authors compared the PHN model with previously published Z score models.

Methods: Z scores were derived for cardiovascular measurements using four models (PHN, Boston, Italy, and Detroit).

Role of echocardiographic scoring systems in predicting successful biventricular versus univentricular palliation in neonates with critical aortic stenosis.

Background: There are several published echo-derived scores to help predict successful biventricular versus univentricular palliation in neonates with critical aortic stenosis. This study aims to determine whether any published scoring system accurately predicted outcomes in these neonates.

Methods: Single centre, retrospective cohort study including neonates who underwent aortic valve intervention (surgical valvotomy or balloon valvuloplasty) with the intention of biventricular circulation.

Patient-Specific Numerical Analysis of Coronary Flow in Children With Intramural Anomalous Aortic Origin of Coronary Arteries.

Unroofing surgery for anomalous aortic origin of a coronary artery (AAOCA) alters coronary anatomy by opening the intramural segment so that the anomalous coronary orifice arises perpendicularly from appropriate aortic sinus. Computational fluid dynamics modeling (CFD) allows for quantification of hemodynamics linked to morbidity such as wall shear stress (WSS), relative to patient-specific features like the angle of origin (AO). We hypothesize that CFD will reveal abnormal WSS indices in unroofed arteries that are related to AO.

Echocardiographic Identification of Pulmonary Artery Flow Reversal: An Indicator of Adverse Outcomes in Single Ventricle Physiology.

Individuals with single ventricle congenital heart disease (CHD) undergo multiple staged surgical palliations. Staged single ventricle palliation with a superior cavopulmonary connection (SCPC) in infancy followed by a Fontan in early childhood relies on passive, unobstructed pulmonary blood flow and normal pulmonary vasculature. We hypothesized that patients with echocardiographic identification of retrograde flow in a branch pulmonary artery (PA) after SCPC or Fontan are at increased risk for adverse outcomes.

Limited utility of repeated vital sign monitoring during initiation of oral propranolol for complicated infantile hemangioma.

Background: Initial propranolol recommendations for infantile hemangioma published in 2013 were intended as provisional best practices to be updated as evidence-based data emerged.

Methods: A retrospective multicenter study was performed to evaluate utility of prolonged monitoring after first propranolol dose and escalation(s). Inclusion criteria included diagnosis of hemangioma requiring propranolol of greater than or equal to 0.

Challenges and lessons learned from the Pediatric Heart Network Normal Echocardiogram Database study.

Background: The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies.

Methods: Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations.