Outcomes of Patients With Sickle Cell Disease and Trait After Congenital Heart Disease Surgery.

Background: Sickle cell disease (SCD) is a hemoglobinopathy that can cause multiorgan dysfunction. This study assessed the perioperative outcomes of patients undergoing operations for congenital heart disease who had SCD or sickle cell trait (SCT).

Methods: We performed a retrospective review of patients with SCD or SCT who had records in The Society of Thoracic Surgeons Congenital Heart Surgery Database between 2014 and 2019.

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).

Repair of a mixed form of supracardiac total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a rare congenital heart defect. We report an infant with a mixed form of supracardiac TAPVC, in whom all pulmonary veins, except the right upper, entered a pulmonary venous confluence that is connected to a vertical vein and drained into the superior vena caval-right atrial junction. Several segmental right upper pulmonary veins entered the superior vena cava, superior to the entry of the vertical vein.

Significant Improvements in Mortality After the Fontan Operation in Children With Down Syndrome.

Background: Down syndrome (DS) is considered a risk factor for mortality associated with the Fontan operation. The objective was to show the contemporary short-term outcome of the Fontan operation for a functionally univentricular heart in patients with DS and non-DS, along with an analysis of significant predictors for in-hospital mortality.

Methods: This was a retrospective study using The Society of Thoracic Surgeons Congenital Database to assess in-hospital mortality and its predictors in patients with DS and non-DS undergoing the Fontan operation over 16 years (2001-2016).

Cardiac Paraganglioma in a 14-Year-Old.

Cardiac paraganglioma (PGL) is a rare catecholamine-secreting tumor forming 1% to 3% of cardiac tumors. Although most PGL occur sporadically, evidence exists that 40% of them may be related to familial cancer predisposition syndromes. We present a unique case of a 14-year-old female who presented with persistent hypertension and was found to have a cardiac PGL.

Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons' Society Study.

Objectives: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch.

Methods: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141).

Utilisation of a three-dimensional printed model for the management of coronary-pulmonary artery fistula from left main coronary artery.

Coronary-pulmonary artery fistula is a rare anomaly in which an aortopulmonary collateral artery arises from a coronary artery, often seen in patients with pulmonary atresia with ventricular septal defect. In the presented case, a coronary-pulmonary artery fistula arose from the left main coronary artery and supplied blood flow to a left upper lobe segment. The life-sized three-dimensional printed model was helpful in pre-surgical planning for unifocalisation of the aortopulmonary collateral arteries.

Coronary Sinus Stenosis With Right Coronary Artery to Coronary Sinus Fistula.

Coronary artery fistula is a rare congenital cardiac anomaly. We report a 34-year-old woman who presented with a recurrent large pericardial effusion during pregnancy. She was found to have a right coronary artery to coronary sinus fistula.

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.

Purulent Pericarditis Due to Paronychia in a 16-Month-Old Child: A Nail-Biting Story.

Purulent pericarditis is a rare infectious disease with significant mortality, even in the modern antibiotic era. The presenting signs can often be subtle and patients can deteriorate rapidly with cardiac tamponade. We report a previously healthy 16-month-old female who developed purulent pericarditis associated with paronychia and sepsis caused by methicillin-sensitive .

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".

Acute kidney injury following first-stage palliation in hypoplastic left heart syndrome: hybrid versus Norwood palliation.

Objective: The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes. Design and patients This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure.

Cardiac Lymphangioma Encasing Right Coronary Artery in an Infant.

Cardiac lymphangioma is a rare primary benign tumor of the heart. We report a 3-year-old with cystic lymphangioma encasing the right coronary artery. Cardiac magnetic resonance imaging confirmed a intra-pericardial heterogeneous mass measuring 2.

Morphological and growth responses of vascular smooth muscle and endothelial cells cultured on immobilized heparin and dextran sulfate surfaces.

Heparin has shown promise as a component of various biomaterial formulations, but its variable properties and inhibitory effects on some cell types have raised interest in use of dextran sulfate as an alternative. In this study, we characterized the interactions of vascular smooth muscle (SMC) and endothelial cells (EC) with heparin and dextran sulfate immobilized onto chitosan-based films. Films were modified by blending chitosan with type I collagen and covalently attaching heparin or dextran sulfate at various levels.

Prospective Validation of a Novel Vasoactive-Ventilation-Renal Score as a Predictor of Outcomes After Pediatric Cardiac Surgery.

Background: We sought to further validate the novel vasoactive-ventilation-renal (VVR) score in a prospective study of a heterogeneous cohort of children undergoing cardiac surgery that includes patients with single-ventricle anatomy and residual mixing lesions.

Methods: We prospectively performed an observational study of all children less than 18 years of age who underwent surgery for congenital heart disease at our center from November 2013 to June 2014. We calculated VVR score as follows: vasoactive-inotrope score + ventilation index + (change in serum creatinine from baseline × 10).

Vasoactive Inotropic Score (VIS) as Biomarker of Short-Term Outcomes in Adolescents after Cardiothoracic Surgery.

Our aim was to evaluate the Vasoactive Inotropic Score (VIS) as a prognostic marker in adolescents following surgery for congenital heart disease. This single-center retrospective chart review included patients 10-18 years of age, who underwent cardiac surgery from 2009 to 2014. Hourly VIS was calculated for the initial 48 postoperative hours using standard formulae and incorporating doses of six pressors.

Effect of Hybrid stage 1 procedure on ventricular function in infants with hypoplastic left heart syndrome.

Objective: The effect of Hybrid stage 1 palliation for hypoplastic left heart syndrome on right ventricular function is unknown. We sought to compare right ventricular function in normal neonates and those with hypoplastic left heart syndrome before Hybrid palliation and to assess the effect of Hybrid palliation on right ventricular function, using the right ventricular myocardial performance index and the ratio of systolic and diastolic durations.

Methods: We carried out a retrospective review of echocardiographic data on 23 infants with hypoplastic left heart syndrome who underwent Hybrid palliation and 35 normal controls.

Risk Factors for Extubation Failure Following Neonatal Cardiac Surgery.

Objective: Extubation failure after neonatal cardiac surgery has been associated with considerable postoperative morbidity, although data identifying risk factors for its occurrence are sparse. We aimed to determine risk factors for extubation failure in our neonatal cardiac surgical population.

Design: Retrospective chart review.

BNP in children with congenital cardiac disease: is there now sufficient evidence for its routine use?

Interest in brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) in the management of children with CHD has increased. There are, however, no current guidelines for their routine use. The aim of this review article is to provide an update on the data regarding the use of BNP/NT-proBNP in the evaluation and surgical treatment of children with CHD.