Fatal visceral disseminated varicella zoster virus infection during initial remission induction therapy in a patient with lupus nephritis: a case report and review of the literature.

Visceral disseminated varicella zoster virus (VZV) infection is a severe complication, characterized by a notably high mortality rate. Herein, we present a case of a 36-year-old-man involving visceral disseminated VZV infection that emerged during remission induction therapy involving high-dose prednisolone (PSL), mycophenolate mofetil (MMF), and hydroxychloroquine for lupus nephritis. Two months after starting the immunosuppressive therapy, he experienced a rapid onset of severe upper abdominal pain.

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).

Early Impact of the COVID-19 Pandemic on Congenital Heart Surgery Programs Across the World: Assessment by a Global Multi-Societal Consortium.

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica.

Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease.

Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery.

Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica.

Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease.

Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery.

Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

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.

History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada.

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".

International quality improvement initiatives.

Across the globe, the implementation of quality improvement science and collaborative learning has positively affected the care and outcomes for children born with CHD. These efforts have advanced the collective expertise and performance of inter-professional healthcare teams. In this review, we highlight selected quality improvement initiatives and strategies impacting the field of cardiovascular care and describe implications for future practice and research.

The World Database for Pediatric and Congenital Heart Surgery: The Dawn of a New Era of Global Communication and Quality Improvement in Congenital Heart Disease.

The World Society for Pediatric and Congenital Heart Surgery was founded with the mission to "promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service." Early on, the Society's members realized that a crucial step in meeting this goal was to establish a global database that would collect vital information, allowing cardiac surgical centers worldwide to benchmark their outcomes and improve the quality of congenital heart disease care. With tireless efforts from all corners of the globe and utilizing the vast experience and invaluable input of multiple international experts, such a platform of global information exchange was created: The World Database for Pediatric and Congenital Heart Disease went live on January 1, 2017.

Konno Operation: The 2015 Kyoto Symposium Konno Memorial Lecture.

The Konno operation consists of a prosthetic aortic valve replacement by using an anterior enlargement of the small aortic annulus. The original procedure includes a longitudinal incision in the aortic septum placed near the midpoint between the two coronary ostia, a vertical incision in the outflow tract of the right ventricle to join the septal incision, prosthetic aortic valve replacement, and patch reconstruction of the outflow tracts of both ventricles by means of a fusiform Dacron patch. The concept of this operation has been applied in other complex operations, such as modified Konno operation, Ross-Konno operation, and aortic valve replacement after arterial switch operation.

Pathogenic Function of Herpesvirus Entry Mediator in Experimental Autoimmune Uveitis by Induction of Th1- and Th17-Type T Cell Responses.

Herpesvirus entry mediator (HVEM), a member of the TNFR superfamily, serves as a unique molecular switch to mediate both stimulatory and inhibitory cosignals, depending on its functions as a receptor or ligand interacting with multiple binding partners. In this study, we explored the cosignaling functions of HVEM in experimental autoimmune uveitis (EAU), a mouse model resembling human autoimmune uveitis conditions such as ocular sarcoidosis and Behcet disease. Our studies revealed that EAU severity significantly decreased in HVEM-knockout mice compared with wild-type mice, suggesting that stimulatory cosignals from the HVEM receptor are predominant in EAU.

Ventricular septation for double inlet left ventricle.

Ventricular septation is a biventricular repair for certain types of functionally univentricular hearts. Double inlet left ventricle (DILV) is one type of functionally univentricular heart which in certain instances is amenable to ventricular septation. Thirty-four patients underwent ventricular septation for DILV from 1971 to 2000.

Long-term results of modified Fontan operation for single-ventricle patients associated with atrioventricular valve regurgitation.

Background: The long-term outcome of modified Fontan operation concomitant with a valve operation for atrioventricular valve (AVV) regurgitation is not well described.

Methods: Between 1977 and 2003, 500 children who underwent modified Fontan operation were subdivided into 192 with AVV plasty (group P) and 308 without AVV plasty (group N). Factors associated with patient outcome were investigated retrospectively.

[Ventricular septation using two-patch technique for total inlet-trabecular septal defect].

A 3-year-old girl underwent ventriclar septation using 2 patch technique. Echocardiography at birth revealed single left ventricle with pulmonary hypertension. Pulmonary artery banding was performed at the age of 1 month.

Initial application in the EACTS and STS Congenital Heart Surgery Databases of an empirically derived methodology of complexity adjustment to evaluate surgical case mix and results.

Objectives: Outcomes evaluation is enhanced by assignment of operative procedures to appropriate categories based upon relative average risk. Formal risk modelling is challenging when a large number of operation types exist, including relatively rare procedures. Complexity stratification provides an alternative methodology.

Manouguian double-valve replacement in a 6-year-old girl.

A 6-year-old girl with a diagnosis of aortic regurgitation with stenosis and mitral regurgitation because of short chordae was referred to us for surgery. Echocardiography revealed that the aortic and the mitral annular diameters were 16 and 23 mm, respectively. The Ross procedure and mitral valvuloplasty were scheduled.

Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries.

Objectives: Recently, the double-switch operation for congenitally corrected transposition of the great arteries has become the procedure of choice in our institute; however, the long-term follow-up is uncertain.

Methods: From 1983 to 2010, 90 patients with congenitally corrected transposition of the great arteries underwent the double-switch operation, which comprised of an atrial switch plus intraventricular rerouting (with or without extracardiac conduits) in 72 patients (group I), and an atrial switch plus arterial switch in 18 patients (group II). The mean age at operation was 7.

Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience.

Background: Surgical results for functional univentricular heart with total anomalous pulmonary venous connection (TAPVC) have been unsatisfactory to date.

Methods: During a 25-year period until December 2009, 207 TAPVC patients underwent surgical repair at our institute, including 56 with a univentricular heart. The 10-year survival rate was 51.

Angiopoietin balance in septic shock patients with acute lung injury: effect of direct hemoperfusion with polymyxin B-immobilized fiber.

Acute lung injury (ALI) in sepsis is characterized by an increase in microvascular permeability, resulting in pulmonary edema. Several studies have suggested that angiopoietin-1 and -2 play a contributory role in the pathogenesis of ALI. Polymyxin B-immobilized fiber column hemoperfusion is effective for sepsis-induced ALI.

Twin atrioventricular nodes connecting to sling of conduction tissue in congenitally corrected transposition associated with straddling tricuspid valve.

This report describes a case of reentrant supraventricular tachycardia relating to "twin" atrioventricular nodes in a 6-year-old female patient with congenitally corrected transposition of the great arteries associated with straddling tricuspid valve. Straddling and overriding of the tricuspid valve literally or by definition indicate malalignment of the inlet part of the ventricular septum and atrial septum by an abnormally large ventriculoatrial septal angle. Therefore, anterior atrioventricular conduction tissues are expected in this setting; however, the present report describes an exception to the rule that combination of straddling tricuspid valve is unlikely to be associated with twin atrioventricular nodes/sling.

The conduction system in heterotaxy.

Cardiac malformations associated with the syndrome of atriovisceral heterotaxy are among the most complex forms of congenital heart disease. Accordingly, the disposition of the specialized conduction tissue (the conduction system) is also variable and complex in these particular anomalies. The location of the conduction system and the degree of abnormalities of the conduction system correlate with the severity and location of the associated structural cardiac anomalies.