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. Surgical repair consisted of the Warden procedure combined with direct anastomosis of the vertical vein to the left atrium. Separate pulmonary venous drainage pathways decreased the risk of post-operative pulmonary venous obstruction. Our patient had an uneventful post-operative course and encouraging 2-month follow-up echocardiography. Careful follow-up is warranted to detect post-operative complications, including obstruction of the pulmonary venous and cavoatrial anastomoses.

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). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

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.

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. Main results Our cohort (n=48) underwent surgical palliation at a mean (SD) age of 12 (11) days. Postoperative acute kidney injury was diagnosed in 14 (29%) patients. The prevalence of acute kidney injury in the Hybrid group was 16% and 53% in the Norwood group. Infants who developed acute kidney injury underwent surgery at younger ages [6 (5-10) versus 10 (8-16) days, p=0.016], and had a higher peak lactate level in the initial 24 hours [5.9 (4.2-9.1) versus 3.4 (2.4-6.7), p=0.007]. Norwood procedure was significantly associated with acute kidney injury [odds ratio 11.7 (95% confidence interval 1.3-101.9), p=0.03]. ICU stay [38 (21-84) versus 16 (6-45) days, p=0.038] and time to extubation [204 (120-606) versus 72 (26-234) hours, p=0.014] were longer in those with acute kidney injury. The two patients who developed early postoperative renal failure as per pRIFLE died before discharge from associated comorbidities. CONCLUSIONS: Acute kidney injury occurs in a third of the patients with single ventricle after surgical palliation but is mostly transient. Norwood, compared with Hybrid procedure, is a risk factor for postoperative acute kidney injury, which, in turn, is associated with longer ICU stay and time to extubation.

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". In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the "short list" for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.

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. The composite adverse outcome was defined as any one of death, resuscitation or mechanical support, arrhythmia, infection requiring antibacterial therapy, acute kidney injury or neurologic injury. Surgeries were risk-stratified by the type of surgical repair using the validated STAT score. Statistical analysis (SPSS 19.0) included Mann-Whitney U test, Chi-square test, ROC curves, and binary regression analysis. Our cohort (n = 149) had a mean (SD) age of 13.9 (2.4) years and included 97 (65.1 %) males. Maximal VIS at 24 and 48 h following surgery was significantly higher in subjects (n = 27) who suffered an adverse outcome. Subjects with adverse outcome had longer bypass and cross-clamp times, durations of stay in the hospital, and a higher rate of acute kidney injury, compared to those (n = 122) without postoperative adverse outcomes. The area under the ROC for maximum VIS at 24-48 h after surgery was 0.76, with sensitivity, specificity, and positive and negative predictive values with 95 % CI of 67 (48-82) %, 74 (70-77) %, and 36 (26-44) % and 91 (86-95) %, respectively, at a cutoff >4.75. On binary logistic regression, maximum VIS on second postoperative day remained significantly associated with adverse outcome (OR 1.35; 95 % CI> 1.12-1.64, p = 0.002). Maximal VIS at 24 and 48 h correlated significantly with length of stay and time to extubation. Maximal VIS on the second postoperative day predicts adverse outcome in adolescents following cardiac surgery. This simple yet robust prognostic indicator may aid in risk stratification and targeted interventions in this population.

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. Data were acquired before Hybrid and after Hybrid palliation - post 1, 0-4 days; post 2, 1 week; post 3, 2-3 weeks; post 4, 1-1.5 months following Hybrid palliation. RESULTS: Myocardial performance index and ratio of systolic and diastolic durations were higher in the pre-Hybrid hypoplastic left heart syndrome group (n=23) - 0.47±0.16 versus 0.25±0.07, p<0.001; 1.59±0.44 versus 1.09±0.14, p<0.0001 - compared with controls (n=35). There was no significant change in the myocardial performance index at any of the post-Hybrid time points. Ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid - post 3: 2.08±0.62 and post 4: 2.21±0.45 versus pre: 1.59±0.44, p=0.043 and 0.003. There were no significant differences in parameters between sub-groups of infants who died (n=10) and survivors (n=13). CONCLUSIONS: Right ventricular myocardial performance index and ratio of systolic and diastolic durations were significantly higher in infants with hypoplastic left heart syndrome before intervention compared with controls. The ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid palliation. Our data suggest that infants with hypoplastic left heart syndrome have right ventricular dysfunction before intervention, which worsens over 2 weeks after Hybrid palliation.

Prevalence and risk factors for upper airway obstruction after pediatric cardiac surgery.

OBJECTIVE: To determine the prevalence of and risk factors for extrathoracic upper-airway obstruction after pediatric cardiac surgery. STUDY DESIGN: A retrospective chart review was performed on 213 patients younger than 18 years of age who recovered from cardiac surgery in our multidisciplinary intensive care unit in 2012. Clinically significant upper-airway obstruction was defined as postextubation stridor with at least one of the following: receiving more than 2 corticosteroid doses, receiving helium-oxygen therapy, or reintubation. Multivariate logistic regression analysis was performed to determine independent risk factors for this complication. RESULTS: Thirty-five patients (16%) with extrathoracic upper-airway obstruction were identified. On bivariate analysis, patients with upper-airway obstruction had greater surgical complexity, greater vasoactive medication requirements, and longer postoperative durations of endotracheal intubation. They also were more difficult to calm while on mechanical ventilation, as indicated by greater infusion doses of narcotics and greater likelihood to receive dexmedetomidine or vecuronium. On multivariable analysis, adjunctive use of dexmedetomedine or vecuronium (OR 3.4, 95% CI 1.4-8) remained independently associated with upper-airway obstruction. CONCLUSION: Extrathoracic upper-airway obstruction is relatively common after pediatric cardiac surgery, especially in children who are difficult to calm during endotracheal intubation. Postoperative upper-airway obstruction could be an important outcome measure in future studies of sedation practices in this patient population.

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. SETTING: Urban tertiary care free-standing children's hospital. PATIENTS: Neonates (0-30 d) who underwent cardiac surgery at our institution between January 2009 and December 2012 was performed. INTERVENTIONS: Extubation failure was defined as reintubation within 72 hours after extubation from mechanical ventilation. Multivariate logistic regression analysis was performed to determine independent risk factors for extubation failure. MEASUREMENTS AND MAIN RESULTS: We included 120 neonates, of whom 21 (17.5%) experienced extubation failure. On univariate analysis, patients who failed extubation were more likely to have genetic abnormalities (24% vs 6%; p = 0.023), hypoplastic left heart (43% vs 17%; p = 0.009), delayed sternal closure (38% vs 12%; p = 0.004), postoperative infection prior to extubation (38% vs 11%; p = 0.002), and longer duration of mechanical ventilation (median, 142 vs 58 hr; p = 0.009]. On multivariate analysis, genetic abnormalities, hypoplastic left heart, and postoperative infection remained independently associated with extubation failure. Furthermore, patients with infection who failed extubation tended to receive fewer days of antibiotics prior to their first extubation attempt when compared with patients with infection who did not fail extubation (4.9 ± 2.6 vs 7.3 ± 3; p = 0.073). CONCLUSIONS: Neonates with underlying genetic abnormalities, hypoplastic left heart, or postoperative infection were at increased risk for extubation failure. A more conservative approach in these patients, including longer pre-extubation duration of antibiotic therapy for postoperative infections, may be warranted.

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. BNP/NT-proBNP levels in children with CHD vary substantially according to age, laboratory assay methods, and the specific haemodynamics associated with the individual congenital heart lesion. The accuracy of BNP/NT-proBNP as supplemental markers in the integrated screening, diagnosis, management, and follow-up of CHD has been established. In particular, the use of BNP/NT-proBNP as a prognostic indicator in paediatric cardiac surgery has been widely demonstrated, as well as its role in the subsequent follow-up of surgical patients. Most of the data, however, are derived from single-centre retrospective studies using multivariable analysis; prospective, randomised clinical trials designed to evaluate the clinical utility and cost-effectiveness of routine BNP/NT-proBNP use in CHD are lacking. The results of well-designed, prospective clinical trials should assist in formulating guidelines and expert consensus recommendations for its use in patients with CHD. Finally, the use of new point-of-care testing methods that use less invasive sampling techniques - capillary blood specimens - may contribute to a more widespread use of the BNP assay, especially in neonates and infants, as well as contribute to the development of screening programmes for CHD using this biomarker.

Berlin Heart EXCOR pediatric ventricular assist device for bridge to heart transplantation in US children.

BACKGROUND: Recent data suggest that the Berlin Heart EXCOR Pediatric ventricular assist device is superior to extracorporeal membrane oxygenation for bridge to heart transplantation. Published data are limited to 1 in 4 children who received the device as part of the US clinical trial. We analyzed outcomes for all US children who received the EXCOR to characterize device outcomes in an unselected cohort and to identify risk factors for mortality to facilitate patient selection. METHODS AND RESULTS: This multicenter, prospective cohort study involved all children implanted with the Berlin Heart EXCOR Pediatric ventricular assist device at 47 centers from May 2007 through December 2010. Multiphase nonproportional hazards modeling was used to identify risk factors for early (<2 months) and late mortality. Of 204 children supported with the EXCOR, the median duration of support was 40 days (range, 1-435 days). Survival at 12 months was 75%, including 64% who reached transplantation, 6% who recovered, and 5% who were alive on the device. Multivariable analysis identified lower weight, biventricular assist device support, and elevated bilirubin as risk factors for early mortality and bilirubin extremes and renal dysfunction as risk factors for late mortality. Neurological dysfunction occurred in 29% and was the leading cause of death. CONCLUSIONS: Use of the Berlin Heart EXCOR has risen dramatically over the past decade. The EXCOR has emerged as a new treatment standard in the United States for pediatric bridge to transplantation. Three-quarters of children survived to transplantation or recovery; an important fraction experienced neurological dysfunction. Smaller patient size, renal dysfunction, hepatic dysfunction, and biventricular assist device use were associated with mortality, whereas extracorporeal membrane oxygenation before implantation and congenital heart disease were not.

Association between anemia and packed cell transfusion and outcomes of ventricular septal defect and atrioventricular canal repair in children.

Data on the prevalence and impact of anemia and packed red blood cell (PRBC) transfusions in children with congenital heart disease are limited. Our objectives were to determine the prevalence of anemia and its impact and the impact of PRBC transfusion in the initial 5 days after surgical repair on postoperative outcomes in infants with ventricular septal defect (VSD) and atrioventricular (AV) canal. Retrospective chart review of infants (1 year old) (n = 195) with AV canal or VSD who underwent surgical repair at Children's Hospital of Michigan during a 10-year period. Statistical analyses (SPSS 17.0) included Chi square and Student t test as well as regression analysis with significance set at p = 0.05. Preoperative anemia was diagnosed in 45 of 195 (23%) children. Anemic infants had VSD more frequently (80%), significantly shorter bypass and cross-clamp durations, and higher red cell distribution widths. Postoperative outcomes and PRBC transfusions were similar in the groups. On regression analysis, AV canal was associated with a significantly lower (odds ratio 0.21; 95% confidence interval 0.07-0.68, p = 0.009) risk of anemia. Infants who received a PRBC transfusion (n = 42) had significantly lower birth weights as well as weights at surgery and longer postoperative durations of pressor use, ventilation, oxygen supplementation, and length of stay than those who did not (n = 153) receive transfusions. PRBC transfusion was independently associated with longer postoperative length of stay, oxygen, pressor use, and ventilator duration. Approximately 23% of infants with AV canal or VSD are anemic. PRBC transfusions in the initial 5 postoperative days, but not anemia, are independently associated with adverse postoperative outcomes. Further studies to evaluate conservative transfusion strategies in this population are needed.

Safety and utility of passive peritoneal drainage following Fontan palliation.

OBJECTIVE: Placement of peritoneal drainage catheters intra-operatively has been shown to help prevent fluid overload in children recovering from surgery for two-ventricle heart disease. We aimed to determine whether this practice is also helpful in children recovering from Fontan palliation. MATERIAL AND METHODS: A retrospective review was performed on children with single-ventricle anatomy undergoing Fontan palliation at our institution from 2007 to 2011. Variables in those with peritoneal drainage were compared with those without using t-tests, Mann-Whitney U-tests, chi-square tests, or analysis of variance for repeated measures as appropriate. Data were represented as mean with standard deviation unless otherwise noted. RESULTS: A total of 43 children were reviewed, 21 (49%) with peritoneal drainage catheters. No complications from catheter placement occurred. The groups did not differ with regard to cardiopulmonary bypass duration, dominant ventricle, pre-operative haemodynamic data, fenestration use, and initial intensive care unit ventilation index. Central venous pressures, vasoactive medication use, and diuretic use during the first 48 hours were also not statistically different. At 48 hours, the median fluid balance was -9 (interquartile range : -50, +20) in those with peritoneal drainage and +77 cc/kg (interquartile range : +22, +96) in those without (p < 0.001), yet median duration of mechanical ventilation was 40 hours (range: 19-326) in those with peritoneal drainage and 23 hours (range: 9-92) in those without, p = 0.01. CONCLUSION: Patients with peritoneal drainage recovering from Fontan palliation achieved negative fluid balance as compared with those without peritoneal drainage, although this difference was associated with a longer duration of mechanical ventilation.

Hyponatremia during arginine vasopressin therapy in children following cardiac surgery.

OBJECTIVE: To describe the incidence and severity of hyponatremia after initiation of arginine vasopressin therapy in children recovering from cardiothoracic surgery, and to compare these patients with a control group with similar disease complexity and severity who did not receive arginine vasopressin. DESIGN: Retrospective chart review. SETTING: PICU at a tertiary care university hospital. PATIENTS: Twenty-nine patients who received arginine vasopressin for at least 6 hours during the first 48 postoperative hours following cardiothoracic surgery were compared with 47 patients who did not receive arginine vasopressin. After surgery, all patients received intravenous fluids consisting of dextrose and 0.22% saline for daily fluid requirements as well as isotonic colloid and blood products as needed for additional resuscitation. RESULTS: Mean initial postoperative serum sodium did not differ between groups, 144.6 ± 3.4 in those patients who received arginine vasopressin and 144.5 ± 3.7 in those who did not, p = 0.969. Mean lowest sodium in the first 72 hours, however, was 134.7 ± 3.8 in those who received arginine vasopressin as compared with 137.1 ± 4.3 in the control group, p = 0.019. Hyponatremia occurred in 14 of the patients (48%) who received arginine vasopressin but only in 8 of the patients (17%) in the control group, p = 0.004. Mean age, weight, sex, Aristotle score, and duration of cardiopulmonary bypass were not statistically different between groups. Mean volumes of hypotonic fluids administered and cumulative diuretic dosing during the first 72 hours post-surgery were also not statistically different between groups. CONCLUSIONS: Hyponatremia occurred in nearly half of the infants and children receiving arginine vasopressin therapy in this study. Clinicians should be aware of this association, monitor serum sodium values closely, and consider providing less free water to these patients before hyponatremia occurs.

Clinical response to arginine vasopressin therapy after paediatric cardiac surgery.

OBJECTIVE: To describe the haemodynamic response of children who receive arginine vasopressin for haemodynamic instability after cardiac surgery and to identify clinical variables associated with a favourable response. Materials and Methods We reviewed patients less than or equal to 6 years undergoing open heart surgery in our institution between January, 2009 and July, 2010 who received arginine vasopressin during the first 7 days post operation. Favourable responders were defined as those in whom blood pressure was increased or maintained and catecholamine score was decreased, or blood pressure was increased by greater than or equal to 10% of baseline and catecholamine score was unchanged at 6 hours following arginine vasopressin initiation. RESULTS: Of the 34 patients identified, 17 (50%) patients responded favourably to arginine vasopressin. At 6 hours, the mean blood pressure was increased by 32.2% in responders as compared with 4.6% in non-responders, with a p-value less than 0.001. The mean catecholamine score decreased by 30.1% in responders and increased by 7.6% in non-responders, with a p-value less than 0.001. Anthropometric, demographic, and intra-operative variables were similar in both groups, as was maximum dose of arginine vasopressin. The median time after arrival to the intensive care unit at which arginine vasopressin was initiated, however, was later in those who responded, 20 hours as compared with those who did not, 6 hours, with a p-value equal to 0.032. CONCLUSIONS: Arginine vasopressin therapy led to haemodynamic improvement in only half of the children in this study, and improvement was more likely to occur if arginine vasopressin was initiated after the post-operative night.

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. The primary outcome was operative mortality. Secondary outcomes included postoperative complications. One-to-one propensity score matching was performed between the SCD and SCT groups and the control group for further analysis. RESULTS: Our study population consisted of 73, 411, and 36 501 patients in the SCD, SCT, and control groups, respectively. Median (25%-75% interquartile range) age at surgery was 2.8 (0.4-9.7), 0.60 (0.2-3.1), and 0.70 (0.2-6.4) years in the SCD, SCT, and control cohorts, respectively. Operative mortality, surgery duration, cardiopulmonary bypass time, and cross-clamp time were not significantly different among the 3 groups. The SCD group had a higher rate of postsurgical cardiac arrest than its propensity score-matched control group (5.5% vs 0%, P < .05); otherwise, there were no statistically significant differences in the outcomes between the SCD and SCT groups and their respective matched control groups. CONCLUSIONS: Operative mortality after cardiothoracic procedures in patients with SCD or SCT appeared similar to our control patients. While these patients may require unique perioperative management, they can undergo cardiac surgery without an observed increase in mortality.

Hypoplastic left heart syndrome, restrictive patent foramen ovale, and pulmonary valve stenosis: an unusual combination.

An infant presented with a rare combination of hypoplastic left heart syndrome, restricted patent foramen ovale, and moderately dysplastic pulmonary valve. In preparation for heart transplantation, bilateral pulmonary artery banding was performed to protect the pulmonary vasculature.

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. During surgical resection, even though the main right coronary was preserved, the sinoatrial (SA) nodal artery was surrounded by the tumor and required resection with the tumor. The patient subsequently developed SA node dysfunction and is currently being evaluated for placement of a permanent pacemaker.

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). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

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 Staphylococcus aureus. In addition to antibiotic treatment, she required emergent pericardiocentesis for cardiac tamponade, followed by two surgical interventions including full median sternotomy incision and partial pericardiectomy. At 4-month follow-up, she did well with no evidence of constrictive pericarditis on echocardiogram.