Prenatal Congenital Heart Disease —It Takes a Multidisciplinary Village
AbstractPrenatal diagnosis of congenital heart disease (CHD) allows for thoughtful multidisciplinary planning about location, timing, and need for medical interventions at birth. We sought to assess the accuracy of our prenatal cardiac diagnosis, and postnatal needs for patients with CHD utilizing a multidisciplinary approach. We performed a retrospective chart review of fetal CHD patients between 1/1/18 and 4/30/19. Maternal and infant charts were reviewed for delivery planning, subspecialty care needs, genetic evaluation, prenatal and postnatal cardiac diagnoses, need for prostaglandin (PGE) and neonatal cardiac interven...
Source: Mammalian Genome - April 25, 2023 Category: Genetics & Stem Cells Source Type: research

Isosorbide DiNitrate Effect on Hemodynamic Profile, Liver Stiffness, and Exercise Tolerance in Fontan Circulation (The NEET Clinical Trial)
AbstractAfter Fontan operation, decreased venous capacitance and venoconstriction are adaptive mechanisms to maintain venous return and cardiac output. The consequent higher venous pressure may adversely impact end-organ function, exercise capacity and result in worse clinical outcomes. This pilot study evaluated the safety and effect of isosorbide dinitrate (ISDN), a venodilator, on exercise capacity, peripheral venous pressure (PVP), and liver stiffness in patients with Fontan circulation. In this prospective single-arm trial, 15 individuals with Fontan circulation were evaluated at baseline and after 4 weeks of therapeu...
Source: Mammalian Genome - April 21, 2023 Category: Genetics & Stem Cells Source Type: research

Derivation and Validation of a General Predictive Model for Long Term Risks for Mortality and Invasive Interventions in Congenital and Acquired Cardiac Conditions Encountered in the Young
AbstractAccurate prognostic assessment is a key driver of clinical decision making in heart disease in the young (HDY). This investigation aims to derive, validate, and calibrate multivariable predictive models for time to surgical or catheter-mediated intervention (INT) and for time to death in HDY. 4108 unique subjects were prospectively and consecutively enrolled, and randomized to derivation and validation cohorts. Total follow-up was 26,578 patient-years, with 102 deaths and 868 INTs. Accelerated failure time multivariable predictive models for the outcomes, based on primary and secondary diagnoses, pathophysiologic s...
Source: Mammalian Genome - April 17, 2023 Category: Genetics & Stem Cells Source Type: research

Stratifying Severity of Acute Respiratory Failure Severity in Cyanotic Congenital Heart Disease
AbstractHypoxemia is used to stratify severity in acute respiratory failure (ARF) but is less useful in cyanotic congenital heart disease (CCHD) due to an inability to differentiate hypoxemia from lung injury versus cardiac shunting. Therefore, we aimed to determine whether variables related to respiratory mechanics were associated with outcomes to assist in stratifying ARF severity in pediatric CCHD. We performed a retrospective cohort study from a single cardiac intensive care unit enrolling children with CCHD with ARF requiring mechanical ventilation between 2011 and 2019. Time-averaged ventilator settings and oxygenati...
Source: Mammalian Genome - April 15, 2023 Category: Genetics & Stem Cells Source Type: research

Grafts and Patches: Optimized but Not Optimal Materials for Congenital Heart Surgery
This article provides an overview of the most commonly implanted materials in pediatric cardiac surgery.Structural changes can be detected in all available materials. Depending on the age at implantation and the site of implantation, the extent and time course of material degeneration vary. Autologous material is still the gold standard in reconstructive surgery. Biological materials have largely replaced artificial materials in clinical use.The search for the ideal material continues. In pediatric cardiac surgery, there are only optimized but no optimal materials. (Source: Mammalian Genome)
Source: Mammalian Genome - April 10, 2023 Category: Genetics & Stem Cells Source Type: research

Hemodynamic and Echocardiographic Characteristics and the Presence of Pulmonary Hypertension in Patent Ductus Arteriosus Patients who Underwent Transcatheter Closure
AbstractWe investigated the hemodynamic parameters of pediatric PDA patients and focused on the influence of PDA size on pulmonary arterial pressure and the prevalence of pulmonary hypertension. A total of 52 patients aged between 2  months and 20 years who received transcatheter closure of a PDA from January 2018 to June 2022 in our institution were retrospectively recruited. Their hemodynamic parameters collected both by echocardiography and by cardiac catheterization were analyzed to delineate the influence of PDA size on the pulmonary vascular system. The echocardiographic-based ductal size and indexed PDA size were ...
Source: Mammalian Genome - April 8, 2023 Category: Genetics & Stem Cells Source Type: research

Regional Cerebral Oxygen Saturation and Estimated Oxygen Extraction Ratio as Predictive Markers of Major Adverse Events in Infants with Congenital Heart Disease
This study was a two-center, retrospective study of patients at 12  months of age or younger with CHD who underwent cardiac surgery. The primary outcome was a composite of one or more major adverse events (MAEs) after surgery: death from any cause, circulatory collapse that needed cardiopulmonary resuscitation, and requirement for extracorporeal membrane oxygenati on. Based on the assumptions of arterial to venous blood ratio, eO2ER was calculated. A total of 647 cases were included in this study. MAEs occurred in 16 patients (2.5%). There were significant differences in post-bypass ScO2 [46.61 (40.90, 52.05) vs. 58.52 (5...
Source: Mammalian Genome - April 8, 2023 Category: Genetics & Stem Cells Source Type: research

Revealing Mitral Valve Cleft Using Real-Time 3-Dimensional Echocardiography in Children with Mitral Regurgitation
AbstractMitral valve cleft (MVC) is the most common cause of congenital mitral regurgitation (MR). MVC may be located on the anterior or posterior leaflets. We evaluated children with moderate-to-severe MR using 3D transthoracic echocardiography (3DTTE) to diagnose MVC and determine the location, shape and size of MVC. Twenty-one patients under 18  years of age with moderate-to-severe MR without symptoms who were suspected of having MVC were included in the study. The patients’ history and clinical data were obtained from the medical records. 2D and 3D imaging were performed with a high-quality machine (EPIQ CVx). A ven...
Source: Mammalian Genome - April 5, 2023 Category: Genetics & Stem Cells Source Type: research

Outlet Ventricular Septal Defect: Impact of Surgery on the Late Aortic and Pulmonary Valve Functions
This study retrospectively evaluated long-term post-operative aortic and pulmonary valve functions in outlet ventricular septal defects. We evaluated aortic regurgitation and pulmonary regurgitation using pre- and post-operative echocardiograms. Overall, 158 patients who underwent intracardiac repair due to outlet ventricular septal defects with aortic valve deformity or congestive heart failure were included. The median follow-up period was 7  years (interquartile range: 0–17 years), without deaths or pacemaker implantations. The age, weight, ventricular septal defect size, and mild aortic regurgitation at surgery wer...
Source: Mammalian Genome - April 3, 2023 Category: Genetics & Stem Cells Source Type: research

Is There a Role for Alcohol Septal Ablation in Young Patients with Medically Refractory Hypertrophic Obstructive Cardiomyopathy?
AbstractSurgical myectomy is recommended for symptomatic hypertrophic obstructive cardiomyopathy (HOCM) after optimal pharmacological therapy. Percutaneous transluminal septal myocardial ablation (PTSMA) is reserved for high-risk adults. Symptomatic patients below 25  years underwent either surgery or PTSMA after heart-team discussion and informed consent. Echocardiography assessed gradients in surgical group. PTSMA group underwent invasive transseptal hemodynamic assessment, selective coronary angiography and super-selective cannulation of septal perforators u sing microcatheters. Contrast echocardiography through the mi...
Source: Mammalian Genome - March 30, 2023 Category: Genetics & Stem Cells Source Type: research

Safety and Short-Term Outcomes for Infants   & lt;  2.5 kg Undergoing PDA Device Closure: A C3PO Registry Study
AbstractTo evaluate short-term procedural outcomes and safety for infants  <  2.5 kg who underwent catheterization with intended patent ductus arteriosus (PDA) device closure in a multi-center registry, as performance of this procedure becomes widespread. A multi-center retrospective review was performed using data from the Congenital Cardiac Catheterization Project on Ou tcomes (C3PO) registry. Data were collected for all intended cases of PDA closure in infants <  2.5 kg from April 2019 to December 2020 at 13 participating sites. Successful device closure was defined as device placement at the conclusion ...
Source: Mammalian Genome - March 30, 2023 Category: Genetics & Stem Cells Source Type: research

The Association Between Congenital Heart Disease and Autism Spectrum Disorder: A Systematic Review and Meta-Analysis
AbstractCongenital heart disease (CHD) is linked to an increased incidence of neurodevelopmental impairments in young patients. Given the number of published studies on this topic, a synthesis of the literature is timely and needed. We performed a systematic review and meta-analysis of the medical literature to assess the evidence linking CHD to incidence of autism spectrum disorder (ASD). A systematic review of studies on CHD and ASD in PubMed, Cochrane and Institute for Scientific Information (ISI) from 1965 to May 2021 was conducted. Quantitative estimates of association between CHD and ASD were extracted from eligible ...
Source: Mammalian Genome - March 25, 2023 Category: Genetics & Stem Cells Source Type: research

Assessing the Risk of Hypoglycemia Secondary to Propranolol Therapy for the Treatment of Supraventricular Tachycardia in Infants
This study attempts to offer insight into the risk of hypoglycemia associated with propranolol therapy when treating infantile SVT to help inform future glucose screening guidelines. We conducted a retrospective chart review of infants treated with propranolol in our hospital system. Inclusion criteria were infants  <  1 year of age who received propranolol for the treatment of SVT. A total of 63 patients were identified. Data was collected on sex, age, race, diagnosis, gestational age, nutrition source (Total Parenteral Nutrition (TPN) vs oral), weight (kg), weight for length (kg/cm), propranolol dose (mg/kg/ day)...
Source: Mammalian Genome - March 18, 2023 Category: Genetics & Stem Cells Source Type: research

Transcatheter Closure of a Coronary Artery Fistula in a Neonate with Tetralogy of Fallot
We present the case of a 32-week premature boy weighing 1.79  kg with TOF, confluent branch pulmonary arteries, major aortopulmonary collaterals, and right coronary artery to main pulmonary artery fistula. The patient demonstrated evidence of coronary steal into the pulmonary vasculature with an elevation in the troponin level without hemodynamic instability , and subsequently underwent successful transcatheter occlusion of the fistula via right common carotid access using a Medtronic 3Q microvascular plug. This case demonstrates the realistic potential for early coronary steal in this physiology and possibility of transc...
Source: Mammalian Genome - March 13, 2023 Category: Genetics & Stem Cells Source Type: research