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.

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.

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.

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). The primary outcome was in-hospital mortality. Statistical analysis was performed using univariable and multivariable logistic regression models. RESULTS: Our study cohort consisted of 12,074 patients (81 DS and 11,993 non-DS). The overall in-hospital mortality rate significantly improved in the recent era (2009-2016): 2.4% to 1.3%, P < .001. The DS group had a higher in-hospital mortality rate (12.3% vs 1.6%, P < .001) with an odds ratio of 8.6 (95% confidence interval, 4.4-17.0). The DS group had a higher 30-day mortality rate, a longer median postoperative length of stay, and a higher incidence of postoperative complications. The multivariable model showed that DS was the strongest predictor of in-hospital mortality, with an odds ratio of 11.6 (95% confidence interval, 5.1-26.4), adjusted for other significant variables including era effect, weight, and primary cardiac diagnosis. CONCLUSIONS: The in-hospital mortality for the Fontan operation significantly improved in the contemporary era. DS was a significant risk factor for in-hospital morbidity and mortality associated with the Fontan operation.

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.

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). Factors associated with functional health status domains were determined using multivariable linear regression analysis. RESULTS: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R2 = 0.10-0.70, adj-R2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution. CONCLUSIONS: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.

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. The coronary sinus was severely dilated due to coronary sinus ostial stenosis. Primary surgical closure of coronary artery fistula was performed with resection of coronary sinus ostial stenosis.

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. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

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.

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 2.6 × 2.4 × 3.9 cm and situated right anterolateral to the ascending aorta and extending into the right atrioventricular groove. Furthermore, the right coronary artery traversed through the center of the mass. Surgical resection, on cardiopulmonary bypass, consisted of excision by skeletonizing the right coronary artery along the length of the mass. The pathology report was consistent with a lymphatic malformation. The postoperative course was uneventful without recurrence at follow-up.

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). Admission, peak, and 48-hour measurements were recorded. Outcomes of interest were prolonged duration of mechanical ventilation and intensive care unit and hospital stays, represented by the upper 25% for all patients. Areas under the receiver-operating characteristic curves (AUC) were determined for all study timepoints and outcome variables. RESULTS: Ninety-two patients were analyzed; their median age was 0.65 (range, 3 days to 17.9 years), and 17 (18%) had single-ventricle anatomy. The VVR measurements outperformed vasoactive-inotrope scores in isolation at all timepoints, with higher AUC values for all outcomes. Of the three timepoints assessed, the 48-hour VVR score most consistently predicted poor outcome, especially with regard to prolonged duration of mechanical ventilation (AUC 0.980) and prolonged intensive care unit stay (AUC 0.919). CONCLUSIONS: In a heterogeneous population of children undergoing cardiac surgery, the 48-hour VVR score was a very strong predictor of outcomes, and outperformed the more traditional vasoactive-inotrope score. The VVR score, therefore, represents a novel and potentially powerful means of predicting clinical outcomes relatively early in the hospital course of these patients.

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.

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.

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.

Use of a novel vasoactive-ventilation-renal score to predict outcomes after paediatric cardiac surgery.

OBJECTIVES: Prior studies have established peak postoperative lactate and the vasoactive-inotrope score (VIS) as modest predictors of outcome following paediatric cardiac surgery. We developed a novel vasoactive-ventilation-renal (VVR) score and aimed to determine if this index, which incorporates postoperative respiratory, cardiovascular and renal function, would more consistently predict outcome in this patient population. METHODS: We performed an Institutional Review Board-approved retrospective analysis of 222 infants at our institution less than 365 days old who underwent surgery for congenital heart disease at our centre from January 2009 to April 2013. The VVR score was calculated as follows: vasoactive-inotrope score + ventilation index + (change in serum creatinine from baseline × 10). For all patients, peak lactate and admission, peak, and 48 h VIS and VVR were recorded. RESULTS: For all outcome measures, areas under the curve for 48-h VVR were greater than its corresponding admission and peak values, VIS alone at all three time points and peak lactate. On multivariate regression, 48-h VVR was strongly associated with prolonged intubation [odds ratio (OR): 39.13, P <0.0001], significantly more so than 48-h VIS (odds ratio: 6.18, P <0.0001) and peak lactate (odds ratio: 2.52, P = 0.017). The 48-h VVR was also more significantly associated with prolonged use of vasoactive infusions, chest tube drainage and ICU and hospital stay when compared with VIS alone and peak lactate. CONCLUSIONS: The novel 48-h VVR was a robust predictor of outcome following paediatric cardiac surgery and outperformed the VIS and peak postoperative lactate.