Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome: 2021 vs 2011.

We sought to examine current practices and changes in practice regarding initial counseling for families of patients with hypoplastic left heart syndrome (HLHS) given the evolution of options and outcomes over time. Counseling (Norwood with Blalock-Taussig-Thomas shunt (NW-BTT), NW with right ventricle to pulmonary artery conduit (NW-RVPA), hybrid palliation, heart transplantation, or non-intervention/hospice (NI)) for patients with HLHS were queried via questionnaire of pediatric care professionals in 2021 and compared to identical questionnaire from 2011. Of 322 respondents in 2021 (39% female), 299 respondents were cardiologists (92.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code () is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases.

Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11).

An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many "short list" versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various "short lists".

Embolic Foreign Material in the Central Nervous System of Pediatric Autopsy Patients With Instrumented Heart Disease.

Upon detection of foreign-body embolization to the central nervous system (CNS) following a specific invasive cardiovascular procedure in 1 autopsied child, we undertook a quality assurance analysis to determine whether other patients had had similar events. Autopsies of all infants and children with history of cardiac catheterization, heart surgery on cardiopulmonary bypass, and/or extracorporeal membrane oxygenation over a 5-year period at a single tertiary care institution were reviewed for light-microscopic evidence of foreign material. Of the 24 patients meeting clinical criteria (13 females, 11 males; ages 6 days to 20 years, median age 7.

Modified Senning Procedure for Correction of Atrioventricular Discordance With Total Anomalous Pulmonary Venous Return, Atrial Situs Inversus, Dextrocardia, and Bilateral Superior Venae Cavae.

The Senning and Mustard baffles remain important techniques for the treatment of congenitally corrected transposition (cc-TGA), isolated ventricular inversion, and D-transposition of the great arteries with delayed presentation. We describe the treatment of an 8-month old infant with atrioventricular discordance, ventriculoarterial concordance, and dextrocardia with atrial situs inversus. A modified Senning procedure was performed through the "left-sided" atrium.

Autophagy is essential for cardiac morphogenesis during vertebrate development.

Genetic analyses indicate that autophagy, an evolutionarily conserved lysosomal degradation pathway, is essential for eukaryotic differentiation and development. However, little is known about whether autophagy contributes to morphogenesis during embryogenesis. To address this question, we examined the role of autophagy in the early development of zebrafish, a model organism for studying vertebrate tissue and organ morphogenesis.

Model of magnetically guided fetal cardiac intervention: potential to avoid direct cardiac puncture.

Objective: Fetal cardiac interventions are performed via direct cardiac puncture and are associated with significant fetal morbidity. The feasibility of utilizing magnetic navigation to maneuver a guide wire and balloon across a fetal aortic valve without direct cardiac puncture is tested.

Methods: A fetal heart model was manufactured and placed in a catheterization laboratory equipped with magnetic navigation.

Mortality in hypoplastic left heart syndrome: review of 216 autopsy cases of aortic atresia with attention to coronary artery disease.

Objectives: Aortic atresia (AA) in hypoplastic left heart syndrome (HLHS) has been associated with increased mortality in several prior studies. We reviewed our autopsy series to explore the relationship of coronary abnormalities to anatomic subsets of HLHS with AA.

Methods: We retrospectively reviewed all pathology specimens with AA/MS (mitral stenosis) and AA/MA (mitral atresia) in the Cardiac Registry of Children's Hospital Boston between 1955 and 2009 including autopsy reports, operative notes, and imaging studies.

A prospective phase II trial of vinblastine and methotrexate in multivessel intraluminal pulmonary vein stenosis in infants and children.

Objective: To determine the safety and efficacy of the chemotherapeutic agents vinblastine and methotrexate in the treatment of children with progressive multivessel intraluminal pulmonary vein stenosis (PVS).

Methods: Children received weekly vinblastine and methotrexate for a period of 1 year. Outcomes (for patients receiving ≥1 month of chemotherapy) were classified separately for patients with isolated PVS and PVS with congenital heart disease (CHD).

Aortic stenosis and severe mitral regurgitation in the fetus resulting in giant left atrium and hydrops: pathophysiology, outcomes, and preliminary experience with pre-natal cardiac intervention.

Objectives: The objective of this article is to review anatomic, physiologic, and clinical features of fetuses and neonates with severe mitral regurgitation (MR) in conjunction with aortic stenosis (AS) and left ventricular (LV) and left atrial (LA) dilation and to present preliminary results of pre-natal intervention for this condition.

Background: Severe fetal valvar AS with an abnormal mitral valve (MV) and MR can lead to left heart dilation, with consequent compression of the right ventricle (RV); hydrops and low cardiac output are often associated.

Methods: This is a retrospective review of fetuses diagnosed with AS, severe MR, and LA dilation (2002 to 2009) and neonates with the same combination of abnormalities (1988 to 2009).

Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle.

The purposes of this study were to characterize the morphologic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle (DI-DORV), a rare congenital heart disease, and to compare these findings to those in patients with double-inlet left ventricle (DILV). The clinical records, imaging studies, and heart specimens of 22 consecutive patients with DI-DORV were reviewed. A comparison group of 44 patients with DILV was matched by age at latest follow-up.

Atrial remodeling after the Fontan operation.

The hemodynamics after Fontan surgery are notable for hypertension and dilation of the right atrium (RA). The effect of this stress on atrial cytoarchitecture has not been systematically studied and might be relevant to arrhythmias and their treatment. Morphologic and histopathologic analyses were performed on tissue from the RA and left atrium (LA) from autopsy specimens of Fontan hearts (n = 47).

Physical models aiding in complex congenital heart surgery.

Purpose: Our aim was to improve spatial imagination of complex congenital cardiac abnormalities for subsequent surgical intervention.

Description: Magnetic resonance imaging data of a patient with complex congenital heart malformations was post-processed with software developed at our institution. The resulting virtual surface data sets were printed out three-dimensionally by rapid prototyping techniques.

Endocardial irregularities of the left atrial roof as seen on coronary CT angiography.

Objective: To describe and characterize morphological characteristics of endocardial irregularities in the roof of the left atrium as seen on coronary CT angiography.

Methods: We retrospectively evaluated the left atrium in 50 consecutive coronary CT patients with multiplanar reformatting, volume rendering, and virtual endoscopy.

Results: Twenty-one of the 50 patients had an endocardial irregularity at the roof of the left atrium.

Cardiac risk after craniopharyngioma therapy.

Although long-term survival after craniopharyngioma treatment is excellent in childhood and early adulthood, sudden deaths in two craniopharyngioma survivors with cardiac findings suggest a need to determine whether treated patients exhibit potential substrates for sudden cardiac death. We present a retrospective review of two index patients with cardiac mortality. This motivated a prospective cardiac screening of 12 survivors that identified nearly a third with significant QTc prolongation.

Common aortic arch anomalies: diagnosis and management.

Vascular rings are a rare form of congenital malformation that completely encircle the trachea and esophagus with vascular structures. The two most common forms are double aortic arch and right aortic arch with an aberrant left subclavian artery and a left-sided ligamentum arteriosum. Patients may present with symptoms of airway or esophageal impingement, including "noisy breathing," stridor, respiratory distress, difficulty feeding, or dysphagia.

Late incompetence of the left atrioventricular valve after repair of atrioventricular septal defects: the morphologic perspective.

Objective: The mortality following repair of atrioventricular septal defects has fallen dramatically in the last 4 decades, but reoperation for late regurgitation across the left atrioventricular valve has remained disconcertingly stagnant. Seeking potential structural causes, we compared the morphology of the surgically created septal leaflet of the left valve following repair of atrioventricular septal defects to the aortic leaflet of the normal mitral valve.

Methods: We compared the mitral valves of 92 normal hearts to the left ventricular components of the bridging leaflets of hearts with atrioventricular septal defect with common atrioventricular junction, determining the shape of the leaflets and the arrangement of the subvalvar apparatus.

Echocardiographic diagnosis and management of bilateral coronary ostial atresia in a patient with pulmonary atresia and intact ventricular septum.

Bilateral atresia of the coronary artery ostia in patients with pulmonary atresia and intact ventricular septum is a rare condition with no reported survivors beyond early infancy. We report the first case of diagnosis by echocardiography followed by successful cardiac transplantation.

Pulmonary vein stenosis: expression of receptor tyrosine kinases by lesional cells.

Background: Primary pulmonary vein stenosis (PVS) is a progressive disorder of infants. Although catheter based intervention and chemotherapy are used to manage the disorder, the benefit of these approaches is reduced considerably by restenosis. The nature of the intimal cells causing the occlusive lesions in PVS is poorly understood.

Morphogenesis of the right ventricle requires myocardial expression of Gata4.

Mutations in developmental regulatory genes have been found to be responsible for some cases of congenital heart defects. One such regulatory gene is Gata4, a zinc finger transcription factor. In order to circumvent the early embryonic lethality of Gata4-null embryos and to investigate the role of myocardial Gata4 expression in cardiac development, we used Cre/loxP technology to conditionally delete Gata4 in the myocardium of mice at an early and a late time point in cardiac morphogenesis.

Inability to cannulate the coronary sinus in patients with supraventricular arrhythmias: congenital and acquired coronary sinus atresia.

We report 4 cases of congenital and acquired coronary sinus ostial atresia incidentally found during electrophysiologic assessment for supraventricular arrhythmias. Congenital variants consisted of coronary sinus drainage predominantly via persistent left superior vena cavae and partial coronary sinus unroofing into the left atrium. The acquired variant was inadvertently produced during surgery for cor triatriatum.