Report of the 2022 Society of Thoracic Surgeons Congenital Heart Surgery Practice Survey.

BACKGROUND: The Society of Thoracic Surgeons Workforce on Congenital Surgery performed a practice survey to analyze contemporary data. METHODS: An electronic survey was sent to congenital heart surgeons in North America. Details on demographics, training paradigm, clinical practice, and work satisfaction were queried, tabulated, and analyzed. RESULTS: Of 312 unique contacts, 201 (64.4%) responded. Of these, 178 (89%) were practicing. The median age was 52 years (interquartile range, 43, 59 years), and 157 (88%) were male. The number of female respondents increased from 12 (7%) in 2015 to 18 (11%) at present. Practice composition was predominantly mixed pediatric and adult (141; 79%), although 15 (8%) surgeons practiced exclusively pediatric surgery. Most surgeons (154; 87%) reported performing the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 5 procedures. One-fourth (42; 24%) reported performing fewer than 50 pediatric cases per year, and 18 (10%) stated that their primary role was as a surgical assistant. Individual surgeon case volume was most commonly 100 to 149 total cases (29%). Although one-half (91; 51%) reported their volume as being "just right," 74 (42%) reported that their case volume was "too small." Seventy-six (43%) reported too many surgeons in their region. Of the 201 practicing surgeons, 30 (14.9%) plan retirement in the next 5 years. Most described career satisfaction, with 102 (57%) being very satisfied and 48 (27%) somewhat satisfied. CONCLUSIONS: Although most congenital heart surgeons in North America are satisfied with their careers, more than 40% believe that their caseload is inadequate and that there are too many surgeons in their region. Further analysis is warranted regarding career dissatisfaction and diversity.

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.

Tracheal surgery for airway anomalies associated with increased mortality in pediatric patients undergoing heart surgery: Society of Thoracic Surgeons Database analysis.

OBJECTIVES: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database. METHODS: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001). CONCLUSIONS: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration.

Courage, Fortitude, and Effective Leadership of Surgical Teams During COVID-19.

The world as we once knew it has been drastically altered secondary to coronavirus disease 2019 (COVID-19). The impact of these changes, particularly for those practicing in the medical profession, extends beyond the physical to the psychological, emotional, and spiritual. We discuss the factors that contribute to these stresses, way to manage them, and how we as leaders of our teams can inspire resilience and help our colleagues endure these most difficult times.

COVID-19: FAQs-Congenital Heart Surgery Recovery and Defining a "New Normal".

As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees' experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.

Sinus of Valsalva Aneurysm in a Patient With Mosaic Trisomy 13: Case Report and Brief Review of the Literature.

This report describes a unique case involving an obese 16-year-old boy with a mosaic form of trisomy 13 and no previous cardiac history who presented with a new murmur, hypertension, pleural effusions, and congestive heart failure in the context of sore throat and fever. Evaluation revealed a diagnosis of ruptured noncoronary sinus of Valsalva (SOV) aneurysm. The diagnosis and surgical management of a ruptured noncoronary SOV aneurysm in a pediatric patient are briefly outlined. An SOV aneurysm is an anatomic dilation of one of the sinuses of the aortic root. Aneurysmal dilation occurs more commonly in the right aortic sinus (70%-80%), compared to the noncoronary sinus (23%-25%), and more rarely the left coronary sinus (5%). Rupture of these aneurysms has been reported to be both spontaneous and secondary to physical exertion, hypertension, or trauma. Signs of rupture include a continuous murmur, patients may present with chest pain or with symptoms of acute congestive heart failure. Diagnosis, in this case, was made by transthoracic echocardiography with careful interpretation of color Doppler images.

Use of a Modified Classic BT Shunt in a Patient With Multiple Conduit Obstructions With Positive Lupus Anticoagulant.

The use of a right ventricle-to-pulmonary artery conduit has re-emerged as a popular alternative to a systemic artery-to-pulmonary artery shunt in the Norwood procedure. Both proximal obstruction secondary to dynamic compression and distal obstruction at the anastomosis site with the pulmonary arteries are well described. In an effort to prevent complications, a technique where in the conduit is placed through the entire full thickness, or dunked, through the RV free wall has been described. We report a case of a patient with HLHS and positive lupus anticoagulant who developed recurrent conduit obstructions. The "Sano" was abandoned in favor of an autologous source of pulmonary blood flow-the modified Blalock-Taussig shunt using the "turn down" of the carotid artery.

Mitral Valve Surgery in the First Year of Life.

Data are limited on outcomes associated with mitral valve surgery in infants. Prior studies report high mortality and increased risk for late cardiac failure particularly for those with mitral stenosis. We sought to evaluate outcomes in patients with mitral stenosis (MS) or regurgitation (MR) who had mitral valvuloplasty or replacement in the first year of life. A retrospective analysis of all patients in a single institution who underwent mitral valvuloplasty or replacement in their first year of life from 2004 to 2016 (n = 25), excluding patients with single ventricle pathology or those undergoing surgery for atrioventricular canal defect, was carried out. Median age and weight at surgery were 76.5 days (range 2-329) and 4.5 kg (range 3.0-10.1), respectively. The primary mitral pathology was MR in 16 and MS in 9 patients. Median follow-up among living patients was 4 years (range 106 days-12.3 years). Overall survival was 96% at 30 days and 87.8% at 1, 5, and 10 years. There were three early deaths (12%), all within 6 weeks of surgery. There were no late deaths. Three patients required valve replacement, 1 of which had a primary mitral valve replacement and died within 30 days of surgery. Re-intervention-free survival (surgical and catheter based) was 83.8%, 73.3%, and 48.9% at 1, 5, and 10 years per Kaplan-Meier estimates. There was no difference in re-intervention-free survival between patients with MR versus MS. No risk factors for death or re-intervention were identified. Mitral valvuloplasty and replacement can be performed in infants under 1 year of age with acceptable survival and need for re-intervention.

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.

The World Database for Pediatric and Congenital Heart Surgery "A Call to Service for North American Congenital Heart Surgery Programs".

The World Society for Pediatric and Congenital Heart Surgery has endorsed the establishment of an international platform for the exchange of knowledge and experience for those that treat patients with a congenital heart defect. On January 1, 2017, the release of the World Database for Pediatric and Congenital Heart Surgery opened a new era in evaluation of treatment with congenital heart defects. The contribution of data from countries with established congenital surgical databases will greatly enhance the efforts to provide the most accurate measure of overall surgical outcomes across the globe.

Intervention for arch obstruction after the Norwood procedure: Prevalence, associated factors, and practice variability.

OBJECTIVE: Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality. METHODS: From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed. RESULTS: Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg). CONCLUSIONS: Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes.

Heterotaxy Syndrome and Intestinal Rotation Abnormalities.

BACKGROUND: Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA. METHODS: Infants ≤6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at the discretion of each participating center. RESULTS: Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants were evaluated routinely. The median age at symptom presentation was 46 days (range: 5-171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a median follow-up of 1.6 years (range: 0.06-4.93 years). CONCLUSIONS: IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.

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.

Two Rare Vascular Rings With Ductal Origin of the Left Pulmonary Artery: A Previously Unrecognized Syndrome?

We report two neonates with distal ductal origin of the left pulmonary artery who also had rare vascular rings comprised of a left aortic arch and right arterial duct with a midline/rightward descending aorta. To the best of our knowledge, this association has not previously been described, although other abnormalities of the left pulmonary artery in the setting of vascular rings have been reported. We review the embryology, utility of imaging, and clinical course.

Persistent Left Superior Vena Cava: Incidence and Management in Patients Undergoing Repair of Partial Anomalous Pulmonary Venous Connection.

INTRODUCTION: A association between partial anomalous pulmonary venous connection (PAPVC) and systemic venous anomalies has been described in the literature, but the true incidence is yet to be elucidated. At our institution, we sought to find the incidence of a persistent left superior vena cava (PLSVC) in patients undergoing an operation for PAPVC. METHODS: A retrospective review of all pediatric and adult patients with PAPVC who underwent surgical repair from February 2006 to February 2016. All clinical, radiographic, and operative data were reviewed. RESULTS: Eighty-five patients underwent surgical repair. A PLSVC was identified in 15 (17.6%) patients. Every PLSVC drained/connected to the coronary sinus. A bridging vein was present in only 26.7% (4 of 15). Intraoperative management of the PLSVC consisted of direct cannulation in nine (60%) patients, temporary occlusion in one (6.7%) patient, and ligation in one (6.7%) patient. In the 15 PLSVC patients, 6 (40%) had a secundum atrial septal defect (ASD), 2 (13.3%) had a patent foramen ovale (PFO), 10 (66.6%) had a sinus venosus type defect, and 3 (20%) had both an ASD and sinus venosus-type defect. Of the 15 patients with PAPVC and PLSVC, 14 (93%) had anomalous drainage of pulmonary vein(s) on the right side, whereas 1 (7%) had veins on both sides with anomalous drainage. CONCLUSION: Persistent left superior vena cava is present in 17.6% of patients undergoing an operation for PAPVC. Awareness of this association as well as the intraoperative management of PLSVC is advised for those that perform operations for PAPVC. As improper myocardial protection and cardiopulmonary bypass strategies in the presence of a PLSVC can result in deleterious outcomes.