Prenatal Diagnosis of Congenital Heart Disease in Liveborn Infants in the New England Region.

Prenatal diagnosis of congenital heart disease requiring early cardiac catheterization or surgical intervention enables optimal delivery planning for appropriate postnatal cardiovascular intervention and care. This allows for improved morbidity and mortality. Prior national data reported prenatal diagnosis rates of 32% for congenital heart disease requiring intervention in infants in the first 6 months of life in the New England region.

Echocardiographic image collection and evaluation in infants with CHD: lessons learned from the imaging core lab for the Residual Lesion Score study.

Many factors affect patient outcome after congenital heart surgery, including the complexity of the heart disease, pre-operative status, patient specific factors (prematurity, nutritional status and/or presence of comorbid conditions or genetic syndromes), and post-operative residual lesions. The esidual esion core is a novel tool for assessing whether specific residual cardiac lesions after surgery have a measurable impact on outcome. The goal is to understand which residual lesions can be tolerated and which should be addressed prior to leaving the operating room.

Recommendations for Cardiac Point-of-Care Ultrasound in Children: A Report from the American Society of Echocardiography.

Cardiac point-of-care ultrasound has the potential to improve patient care, but its application to children requires consideration of anatomic and physiologic differences from adult populations, and corresponding technical aspects of performance. This document is the product of an American Society of Echocardiography task force composed of representatives from pediatric cardiology, pediatric critical care medicine, pediatric emergency medicine, pediatric anesthesiology, and others, assembled to provide expert guidance. This diverse group aimed to identify common considerations across disciplines to guide evolution of indications, and to identify common requirements and infrastructure necessary for optimal performance, training, and quality assurance in the practice of cardiac point-of-care ultrasound in children.

Development of the Residual Lesion Score for congenital heart surgery: the RAND Delphi methodology.

Background And Objective: The Residual Lesion Score is a novel tool for assessing the achievement of surgical objectives in congenital heart surgery based on widely available clinical and echocardiographic characteristics. This article describes the methodology used to develop the Residual Lesion Score from the previously developed Technical Performance Score for five common congenital cardiac procedures using the RAND Delphi methodology.

Methods: A panel of 11 experts from the field of paediatric and congenital cardiology and cardiac surgery, 2 co-chairs, and a consultant were assembled to review and comment on validity and feasibility of measuring the sub-components of intraoperative and discharge Residual Lesion Score for five congenital cardiac procedures.

An ensemble of neural networks provides expert-level prenatal detection of complex congenital heart disease.

Congenital heart disease (CHD) is the most common birth defect. Fetal screening ultrasound provides five views of the heart that together can detect 90% of complex CHD, but in practice, sensitivity is as low as 30%. Here, using 107,823 images from 1,326 retrospective echocardiograms and screening ultrasounds from 18- to 24-week fetuses, we trained an ensemble of neural networks to identify recommended cardiac views and distinguish between normal hearts and complex CHD.

Impact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease.

Background: Many factors affect outcomes after congenital cardiac surgery.

Objectives: The RLS (Residual Lesion Score) study explored the impact of severity of residual lesions on post-operative outcomes across operations of varying complexity.

Methods: In a prospective, multicenter, observational study, 17 sites enrolled 1,149 infants undergoing 5 common operations: tetralogy of Fallot repair (n = 250), complete atrioventricular septal defect repair (n = 249), arterial switch operation (n = 251), coarctation or interrupted arch with ventricular septal defect (VSD) repair (n = 150), and Norwood operation (n = 249).

Variants in ADRB1 and CYP2C9: Association with Response to Atenolol and Losartan in Marfan Syndrome.

Objective: To test whether variants in ADRB1 and CYP2C9 genes identify subgroups of individuals with differential response to treatment for Marfan syndrome through analysis of data from a large, randomized trial.

Study Design: In a subset of 250 white, non-Hispanic participants with Marfan syndrome in a prior randomized trial of atenolol vs losartan, the common variants rs1801252 and rs1801253 in ADRB1 and rs1799853 and rs1057910 in CYP2C9 were analyzed. The primary outcome was baseline-adjusted annual rate of change in the maximum aortic root diameter z-score over 3 years, assessed using mixed effects models.

Comparison of echocardiographic measurements to invasive measurements of diastolic function in infants with single ventricle physiology: a report from the Pediatric Heart Network Infant Single Ventricle Trial.

Background: While echocardiographic parameters are used to quantify ventricular function in infants with single ventricle physiology, there are few data comparing these to invasive measurements. This study correlates echocardiographic measures of diastolic function with ventricular end-diastolic pressure in infants with single ventricle physiology prior to superior cavopulmonary anastomosis.

Methods: Data from 173 patients enrolled in the Pediatric Heart Network Infant Single Ventricle enalapril trial were analysed.

Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations.

Background Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences during early childhood after staged surgical palliations using echocardiography. Methods We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt.

The Impact of the Left Ventricle on Right Ventricular Function and Clinical Outcomes in Infants with Single-Right Ventricle Anomalies up to 14 Months of Age.

Background: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies.

Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome.

Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol versus losartan in young patients with MFS. Data were analyzed from the echocardiograms at 0, 12, 24, and 36 months read in the core laboratory of 608 trial subjects, aged 6 months to 25 years, who met original Ghent criteria and had an AoR z-score (AoRz) > 3.

Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome.

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed.

The Effect of the Superior Cavopulmonary Anastomosis on Ventricular Remodeling in Infants with Single Ventricle.

Background: Infants with single ventricular physiology have volume and pressure overload that adversely affect ventricular mechanics. The impact of superior cavopulmonary anastomosis (SCPA) on single left ventricles versus single right ventricles is not known.

Methods: As part of the Pediatric Heart Network placebo-controlled trial of enalapril in infants with single ventricular physiology, echocardiograms were obtained before SCPA and at 14 months and analyzed in a core laboratory.

Translating clinical trials into clinical practice: a survey assessing the potential impact of the Pediatric Heart Network Infant Single Ventricle Trial.

Background: A few studies have evaluated the impact of clinical trial results on practice in paediatric cardiology. The Infant Single Ventricle (ISV) Trial results published in 2010 did not support routine use of the angiotensin-converting enzyme inhibitor enalapril in infants with single-ventricle physiology. We sought to assess the influence of these findings on clinical practice.

Implementation of a Quality Improvement Bundle Improves Echocardiographic Imaging after Congenital Heart Surgery in Children.

Background: Postoperative echocardiography after congenital heart disease surgery is of prognostic importance, but variable image quality is problematic. We implemented a quality improvement bundle comprising of focused imaging protocols, procedural sedation, and sonographer education to improve the rate of optimal imaging (OI).

Methods: Predischarge echocardiograms were evaluated in 116 children (median age, 0.

Impact of Variability in Echocardiographic Interpretation on Assessment of Adequacy of Repair Following Congenital Heart Surgery: A Pilot Study.

Technical Performance Score (TPS) is based largely on the presence and magnitude of residual lesions on postoperative echocardiograms; this score correlates with outcomes following repair of congenital heart defects. We evaluated reader variability for echocardiographic components of TPS for complete repair of tetralogy of Fallot (TOF) and arterial switch operation (ASO) in two centers and measured its effect on TPS. Postoperative echocardiograms were evaluated in 67 children (39 TOF and 28 ASO).

Competency Testing for Pediatric Cardiology Fellows Learning Transthoracic Echocardiography: Implementation, Fellow Experience, and Lessons Learned.

Unlabelled: There is currently great interest in measuring trainee competency at all levels of medical education. In 2007, we implemented a system for assessing cardiology fellows' progress in attaining imaging skills. This paradigm could be adapted for use by other cardiology programs.

Impact of initial norwood shunt type on right ventricular deformation: the single ventricle reconstruction trial.

Background: The Single Ventricle Reconstruction trial demonstrated a transplantation-free survival advantage at 12-month follow-up for patients with right ventricle-pulmonary artery shunts (RVPAS) with the Norwood procedure compared with modified Blalock-Taussig shunts but similar survival and decreased global right ventricular (RV) function on longer term follow-up. The impact of the required ventriculotomy for the RVPAS remains unknown. The aim of this study was to compare echocardiography-derived RV deformation indices after stage 2 procedures in survivors with single RV anomalies enrolled in the Single Ventricle Reconstruction trial.

Impact of initial shunt type on cardiac size and function in children with single right ventricle anomalies before the Fontan procedure: the single ventricle reconstruction extension trial.

Background: In children with single right ventricular (RV) anomalies, changes in RV size and function may be influenced by shunt type chosen at the time of the Norwood procedure.

Objectives: The study sought to identify shunt-related differences in echocardiographic findings at 14 months and ≤6 months pre-Fontan in survivors of the Norwood procedure.

Methods: We compared 2-dimensional and Doppler echocardiographic indices of RV size and function, neo-aortic and tricuspid valve annulus dimensions and function, and aortic size and patency at 14.

Factors associated with serum B-type natriuretic peptide in infants with single ventricles.

Data regarding the value of B-type natriuretic peptide (BNP) measurements in infants with a single-ventricle (SV) physiology are lacking. This analysis aimed to describe the BNP level changes in infants with an SV physiology before and after superior cavopulmonary connection (SCPC) surgery. Levels of BNP were measured by a core laboratory before SCPC (at 5.

Multicenter study comparing shunt type in the norwood procedure for single-ventricle lesions: three-dimensional echocardiographic analysis.

Background: The Pediatric Heart Network's Single Ventricle Reconstruction (SVR) trial randomized infants with single right ventricles (RVs) undergoing a Norwood procedure to a modified Blalock-Taussig or RV-to-pulmonary artery shunt. This report compares RV parameters in the 2 groups using 3-dimensional echocardiography.

Methods And Results: Three-dimensional echocardiography studies were obtained at 10 of 15 SVR centers.

Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy.

Background: The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects.

Methods And Results: Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area-adjusted aortic root diameter z-score >3.