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).

Congenital absence of coronary ostia in a single/common coronary system.

Coronary ostial atresia seen with pulmonary atresia and coronary-cameral fistulae or, more rarely, in isolation manifested as left main coronary artery atresia, is well described. We describe the clinical course and post-mortem findings in a neonate who suffered a fatal cardiac arrest and was found to have congenital absence of both coronary ostia in a single/common coronary system.

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".

Biventricular Repair in Interrupted Aortic Arch Type C With Aortic Atresia.

We report a case of interrupted aortic arch type C with aortic atresia and a ventricular septal defect with two well-developed ventricles, who underwent a successful single-stage biventricular repair with the modified Yasui procedure and arch reconstruction. Angiography done during conduit revision showed bilateral brachiocephalic trunks with high branching. The child is doing well six years after the initial operation.

Pulmonary atresia with intact ventricular septum and coronary cameral fistulae: an unusual finding of a subsystemic right ventricle.

We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.

Identifying Abnormal Ostial Morphology in Anomalous Aortic Origin of a Coronary Artery.

Background: Anomalous origin of a coronary artery from the contralateral sinus of Valsalva is associated with exercise-induced ischemia and sudden death. That is thought to be due to aortic enlargement in patients with an elliptical ostium. We hypothesize that virtual angioscopy can identify abnormal coronary ostial morphology in these patients.

Absent pulmonary valve, tricuspid atresia, and congenital heart block.

We describe management of a patient with a prenatal diagnosis of absent pulmonary valve, tricuspid atresia, ventricular septal defect, and congenital heart block. Initial treatment consisted of temporary pacemaker implantation, and subsequent palliation included a central shunt during the neonatal period and placement of a permanent pacemaker. At seven months of age, a bidirectional Glenn anastomosis was performed.

Cardiac MRI and CT: differentiation of normal ostium and intraseptal course from slitlike ostium and interarterial course in anomalous left coronary artery in children.

Objective: Anomalous left coronary artery from the inappropriate aortic sinus with intraseptal course is generally benign but can be confused on imaging studies with the potentially lethal interarterial, intramural anomalous left coronary artery. The purpose of this study was to assess normal ostial morphologic features and intraseptal course using cardiac MRI and CT in pediatric patients with intraseptal anomalous left coronary artery.

Materials And Methods: A retrospective review was conducted of the medical records of 14 children with the diagnosis of intraseptal anomalous left coronary artery between November 2009 and March 2013.

Hypoplastic left ventricle and scimitar syndrome.

Hypoplastic left ventricle with scimitar syndrome is a rare combination of anatomic lesions. Five patients with this anatomy have been followed up at our institution. Four of these patients are alive: 2 after heart transplant, 1 underwent a Norwood operation, and 1 has survived staged palliation, culminating in a Fontan operation.

Conjoined hearts in thoracopagus twins.

This study aimed to identify the anatomic and pathologic structural cardiac abnormalities in conjoined twins and to focus on those that have prevented the successful separation of conjoined hearts. A retrospective review was undertaken to examine consecutive cases of thoracopagus conjoined twins with conjoined hearts evaluated at The Children's Hospital of Philadelphia from 1 January 1980 through 6 October 2008. The records included autopsy and surgical findings as well as clinical reports.

Mitral valve dysplasia syndrome: a unique form of left-sided heart disease.

Background: Mitral valve dysplasia syndrome is a unique form of left-sided heart disease characterized by aortic outflow hypoplasia, dilated left ventricle, dysplastic/incompetent mitral valve, and a restrictive/intact atrial septum. Patients with this constellation of abnormalities have been managed in a variety of ways with overall poor outcomes.

Methods: We performed a retrospective review of all patients with mitral valve dysplasia syndrome to identify fetal echocardiographic markers predictive of outcomes.

Differential branch pulmonary artery regurgitant fraction is a function of differential pulmonary arterial anatomy and pulmonary vascular resistance.

Objectives: We sought to investigate whether differential branch pulmonary artery (BPA) regurgitation correlates with differences in BPA anatomy and physiology.

Background: Patients with repaired conotruncal anomalies such as Tetralogy of Fallot frequently have residual BPA stenosis or BPA size differences. Previous reports have demonstrated an increased left pulmonary artery (LPA) regurgitant fraction (RF) in these patients.

A novel case of L-transposition with a right-dominant double aortic arch.

We describe a case of congenitally corrected transposition with a double aortic arch. This unique combination of lesions highlights the importance of a complete anatomic assessment prior to referral for surgery.

Pre-Fontan cardiac magnetic resonance predicts post-Fontan length of stay and avoids ionizing radiation.

Objective: Patients frequently undergo cardiac catheterization before the Fontan operation because of the limited echocardiographic windows in the region of the superior cavopulmonary connection and branch pulmonary arteries. Patients with obstruction to pulmonary blood flow are at increased risk for prolonged length of hospital stay after the Fontan operation. Cardiac magnetic resonance has unlimited imaging windows and can quantify both the branch pulmonary artery size and net flow distribution and thereby serve as a method for identifying patients at increased risk for prolonged length of stay.

Heterotaxy syndrome with mirror image anomalies in identical twins.

Heterotaxy syndrome is a term used to describe disorders of lateralization of thoracic and abdominal organs as well as atria. It is commonly associated with complex congenital heart disease as well as abnormalities of the spleen, intestinal malrotation, and abnormalities of liver and stomach position. Most reported cases of heterotaxy syndrome are sporadic, though familial occurrences have also been reported.

Parachute mitral valve: morphologic descriptors, associated lesions, and outcomes after biventricular repair.

Objective: In "true" parachute mitral valve, mitral valve chordae insert into one papillary muscle. In parachute-like asymmetric mitral valve, most or all chordal attachments are to one papillary muscle. This study compared morphologic features, associated lesions, and palliation strategies of the two parachute mitral valve and dominant papillary muscle types and examined interventions and midterm outcomes in patients with biventricular circulation.