Large bilateral adrenal haemorrhages in a newborn with unrepaired cyanotic CHD.

Management of newborns with cyanotic CHD and bilateral adrenal haemorrhages has not previously been described in the literature. These abnormalities present unique challenges due to the potential for haemodynamic instability, need for open heart surgery and associated systemic anticoagulation in the newborn period, and the risk of catastrophic bleeding.

Melody pulmonary valve bacterial endocarditis: experience in four pediatric patients and a review of the literature.

OBJECTIVES: The objectives of this manuscript are two-fold: (a) to describe the clinical characteristics and management of four pediatric patients with bacterial endocarditis (BE) after Melody pulmonary valve implantation (MPVI); and (b) to review the literature regarding Melody pulmonary valve endocarditis. BACKGROUND: There are several reports of BE following MPVI. The clinical course, BE management and outcome remain poorly defined. METHODS: This is a multi-center report of four pediatric patients with repaired tetralogy of Fallot (TOF) and BE after MPVI. Clinical presentation, echocardiogram findings, infecting organism, BE management, and follow-up assessment are described. We review available literature on Melody pulmonary valve endocarditis and discuss the prognosis and challenges in the management of these patients. RESULTS: Of our four BE patients, two had documented vegetations and three showed worsening pulmonary stenosis. All patients remain asymptomatic after medical treatment (4) and surgical prosthesis replacement (3) at follow-up of 17 to 40 months. Analysis of published data shows that over half of patients undergo bioprosthesis explantation and that there is a 13% overall mortality. The most common BE pathogens are the Staphylococcus and Streptococcus species. CONCLUSIONS: Our case series of four pediatric patients with repaired TOF confirms a risk for BE after MPVI. A high index of suspicion for BE should be observed after MPVI. All patients should be advised to follow lifelong BE prophylaxis after MPVI. In case of BE, surgery should be considered for valve dysfunction or no clinical improvement in spite of medical treatment.

Extracorporeal membrane oxygenation versus counterpulsatile, pulsatile, and continuous left ventricular unloading for pediatric mechanical circulatory support.

OBJECTIVES: Despite progress with adult ventricular assist devices, limited options exist to support pediatric patients with life-threatening heart disease. Extracorporeal membrane oxygenation remains the clinical standard. To characterize (patho)physiologic responses to different modes of mechanical unloading of the failing pediatric heart, extracorporeal membrane oxygenation was compared to intra-aortic balloon pump, pulsatile-flow ventricular assist device, or continuous-flow ventricular assist device support in a pediatric heart failure model. DESIGN: Experimental. SETTING: Large animal laboratory operating room. SUBJECTS: Yorkshire piglets (n = 47; 11.7 ± 2.6 kg). INTERVENTIONS: In piglets with coronary ligation-induced cardiac dysfunction, mechanical circulatory support devices were implanted and studied during maximum support. MEASUREMENTS AND MAIN RESULTS: Left ventricular, right ventricular, coronary, carotid, systemic arterial, and pulmonary arterial hemodynamics were measured with pressure and flow transducers. Myocardial oxygen consumption and total-body oxygen consumption were calculated from arterial, venous, and coronary sinus blood sampling. Blood flow was measured in 17 organs with microspheres. Paired Student t tests compared baseline and heart failure conditions. One-way repeated-measures analysis of variance compared heart failure, device support mode(s), and extracorporeal membrane oxygenation. Statistically significant (p < 0.05) findings included 1) an improved left ventricular blood supply/demand ratio during pulsatile-flow ventricular assist device, continuous-flow ventricular assist device, and extracorporeal membrane oxygenation but not intra-aortic balloon pump support, 2) an improved global myocardial blood supply/demand ratio during pulsatile-flow ventricular assist device and continuous-flow ventricular assist device but not intra-aortic balloon pump or extracorporeal membrane oxygenation support, and 3) diminished pulsatility during extracorporeal membrane oxygenation and continuous-flow ventricular assist device but not intra-aortic balloon pump and pulsatile-flow ventricular assist device support. A profile of systems-based responses was established for each type of support. CONCLUSIONS: Each type of pediatric ventricular assist device provided hemodynamic support by unloading the heart with a different mechanism that created a unique profile of physiological changes. These data contribute novel, clinically relevant insight into pediatric mechanical circulatory support and establish an important resource for pediatric device development and patient selection.

Early and midterm outcomes following surgery for acute type A aortic dissection.

OBJECTIVE: Surgical repair of acute Type A aortic dissection (AADA) is still associated with high in-hospital mortality. We evaluated the impact of perioperative risk factors on early and midterm survival. METHODS: Retrospective (2002-2011) database analysis at a single institution of 132 consecutive AADA patients (88 male, age 59.8 ± 13.6). All but five patients underwent repair with open distal anastomoses and hypothermic circulatory arrest: aortic valve replacement/root replacement (n=44, 33.3%) and valve re-suspension/repair (n=88, 66.7%). Ascending aorta, hemi-arch, and total arch repairs were performed in 11, 113, and eight patients, respectively. Antegrade and retrograde cerebral perfusion were used in all but six patients. RESULTS: Overall in-hospital mortality was 17.4% (n=23). Actuarial survival at one, five, and eight years was 82%, 72%, and 62%, respectively. Perfusion time (cardiopulmonary bypass) (226.5 ± 71.3 vs. 177.5 ± 51.7, p=0.0002), aortic cross-clamp time (min) (132.8 ± 45.7 vs. 109.8 ± 41.2, p=0.01), aortic arch (T2) tear (31% vs. 14%, p=0.03), instability (26% vs. 11%, p=0.02), postoperative stroke (38% vs. 14%, p=0.009), and low cardiac output (50% vs. 15%, p=0.04) all correlated with increased perioperative mortality. A Cox proportional hazard model showed perfusion time (hazard ratio [HR]=1.01), postoperative stroke (HR=2.73), age (HR=1.03), and unstability (HR=1.8) as significant risk factors (p<0.05) affecting the overall survival. CONCLUSION: There is a modern trend towards improving overall perioperative outcomes after surgical repair of AADA; however, early mortality and morbidity remain high even in aortic surgery referral centers.

The Academic Impact of Congenital Heart Surgeons' Society (CHSS) Studies.

PURPOSE: We reviewed all 64 articles ever published by The Congenital Heart Surgeons' Society (CHSS) Data Center to estimate the academic impact of these peer-reviewed articles. MATERIALS AND METHODS: The Congenital Heart Surgeons' Society has performed research based on 12 Diagnostic Inception Cohorts. The first cohort (Transposition) began enrolling patients on January 1, 1985. We queried PubMed to determine the number of publications that referenced each of the 64 journal articles generated by the datasets of the 12 Diagnostic Inception Cohorts that comprise the CHSS Database. Descriptive summaries of the data were tabulated using mean with standard deviation and median with range. RESULTS: Sixty-four peer-reviewed papers have been published based on the CHSS Database. Fifty-nine peer-reviewed articles have been published based on the 12 Diagnostic Inception Cohorts, and five additional articles have been published based on Data Science. Excluding the recently established Diagnostic Inception Cohort for patients with Ebstein malformation of tricuspid valve, the number of papers published per cohort ranged from 1 for coarctation to 11 for transposition of the great arteries. The 11 articles generated from the CHSS Transposition Cohort were referenced by a total of 111 articles (median number of references per journal article = 9 [range = 0-22, mean = 10.1]). Overall, individual articles were cited by an average of 11 (mean), and a maximum of 41 PubMed-listed publications. Overall, these 64 peer-reviewed articles based on the CHSS Database were cited 692 times in PubMed-listed publications. The first CHSS peer-reviewed article was published in 1987, and during the 35 years from 1987 to 2022, inclusive, the annual number of CHSS publications has ranged from 0 to 7, with a mean of 1.8 publications per year (median = 1, mode = 1). CONCLUSION: Congenital Heart Surgeons' Society studies are widely referenced in the pediatric cardiac surgical literature, with over 10 citations per published article. These cohorts provide unique information unavailable in other sources of data. A tool to access this analysis is available at: [https://data-center.chss.org/multimedia/files/2022/CAI.pdf].

Severe calcification of the aorta (porcelain aorta) associated with sarcoidosis in a pediatric heart transplant recipient.

We report a unique case of severe calcification of the aorta, bilateral coronary ostial stenoses and calcification of the mitral valve and left ventricle due to sarcoidosis. The patient underwent neonatal orthotopic heart transplantation secondary to hypoplastic left heart syndrome and developed hypercalcemia with other features of sarcoidosis at 10 yr of age. The mechanism for severe extra-renal calcification localized to the allograft is poorly understood, but may involve discordant local immune modulation and/or calcification-regulation between graft and host tissues.

Early Outcomes of Patients Undergoing Neoaortic Valve Repair Incorporating Geometric Ring Annuloplasty.

OBJECTIVES: During congenital heart surgery, the pulmonary valve and root may be placed into the systemic position, yielding a "neoaortic" valve. With the stress of systemic pressure, the pulmonary roots can dilate, creating aneurysms and/or neoaortic insufficiency (neoAI). This report analyzes the early outcomes of patients undergoing neoaortic valve repair incorporating geometric ring annuloplasty. METHODS: Twenty-one patients underwent intended repair at six centers and formed the study cohort. Thirteen had previous Ross procedures, five had arterial switch operations, and three Fontan physiology. Average age was 21.7 ± 12.8 years (mean ± SD), 80% were male, and 11 (55%) had symptomatic heart failure. Preoperative neoAI Grade was 3.1 ± 1.1, and annular diameter was 30.7 ± 6.5 mm. RESULTS: Valve repair was accomplished in 20/21, using geometric annuloplasty rings and leaflet plication (n = 13) and/or nodular release (n = 7). Fourteen had neoaortic aneurysm replacement (13 with root remodeling). Two underwent bicuspid valve repair. Six had pulmonary conduit changes, one insertion of an artificial Nodulus Arantius, and one resection of a subaortic membrane. Ring size averaged 21.9 ± 2.3 mm, and aortic clamp time was 171 ± 54 minutes. No operative mortality or major morbidity occurred, and postoperative hospitalization was 4.3 ± 1.4 days. At discharge, neoAI grade was 0.2 ± 0.4 (P < .0001), and valve mean gradient was ≤20 mm Hg. At average 18.0 ± 9.1 months of follow-up, all patients were asymptomatic with stable valve function. CONCLUSIONS: Neoaortic aneurysms and neoAI are occasionally seen late following Ross, arterial switch, or Fontan procedures. Neoaortic valve repair using geometric ring annuloplasty, leaflet reconstruction, and root remodeling provides a patient-specific approach with favorable early outcomes.

The World Society for Pediatric and Congenital Heart Surgery: 2021 Update of the World Database for Pediatric and Congenital Heart Surgery.

BACKGROUND: The World Database for Pediatric and Congenital Heart Surgery (WDPCHS), sponsored by the World Society for Pediatric and Congenital Heart Surgery (WSPCHS), provides complex programmatic outcomes analyses for all members of the WSPCHS. METHODS: The Data center, currently at Kirklin Institute for Research in Surgical Outcomes (KIRSO), University of Alabama, Birmingham (USA), provides biannual reports to all active members of the database. This report presents a descriptive analysis of these procedures submitted from January 1, 2017 to December 31, 2020. RESULTS: A total of 37,386 procedures were submitted with an overall mortality of 4.3%. The majority of submissions were from Asian countries. The majority of cases submitted from these countries were of Society of Thoracic Surgeons (STS)-European Association for Cardio-Thoracic Surgery (STAT) Mortality Categories I and II. CONCLUSIONS: The WSPCHS accomplished one of its missions in 2017 when the WDPCHS began accepting data from pediatric and congenital heart surgery programs across the globe. In doing so, it became one of the first organizations to create a platform for the exchange of knowledge and experience, regardless of the socioeconomic status of the particular program or country.

Pleural effusions following the Fontan procedure.

PURPOSE OF REVIEW: The Fontan procedure is the final common pathway in the surgical palliation of many single ventricle heart defects. Mortality has improved but morbidity remains significant. Pleural effusions continue to present challenges after this operation and account for increased length of stay and increased time with draining tube thoracostomies. This review examines recent publications addressing this problem. RECENT FINDINGS: Four papers in 2008-2009 addressed effusions after the Fontan procedure. Off-pump Fontan procedures did not decrease time until chest tube removal. Pulmonary vascular compliance, derived from an electrical circuit model, predicted chest tube indwelling time. A retrospective study identified mean pulmonary artery pressure as a risk factor for effusions lasting more than 14 days after the Fontan procedure. One prospective, randomized trial evaluated the effects of lisinopril on pleural effusions after the Fontan procedure. There was no difference in the length of pleural drainage between the two groups. SUMMARY: Pleural effusions after the Fontan procedure continue to be a challenge. The cause is likely multifactorial and could explain why the literature has no clear message. One randomized, prospective trial suggests that fenestration reduces effusions. Many other reviews report no benefit to fenestration. Sildenafil after the Fontan procedure should be studied in a randomized, prospective fashion.

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

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

Refining The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model With Enhanced Risk Adjustment for Chromosomal Abnormalities, Syndromes, and Noncardiac Congenital Anatomic Abnormalities.

BACKGROUND: The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model adjusts not only for procedure and age group pairings but also for additional patient factors, including the binary presence or absence of a chromosomal abnormality (CA), syndrome (S), and/or a noncardiac congenital anatomic abnormality (NCAA). This analysis refines case-mix adjustment by adding more granular adjustment for individual conditions (CA, S, and NCAA), consistent with a hypothesis that associated risk of mortality differs between individual conditions. METHODS: CA/S corresponding to the same condition were merged to a single condition code. Odds ratios were estimated for all CA/S. For CA/S associated with at least 10 deaths in neonates and infants and at least 10 deaths in children and adults, odds ratios were estimated for the effect of the CA/S separately in neonates/infants and in children/adults. In addition to these condition/age interactions, condition/age/procedure interactions were explored (eg, effect of Down syndrome was estimated based on age and procedure subgroups, including atrioventricular canal repair and single-ventricle palliation). Bayesian modeling was used to create 5 maximally homogeneous groups of CA/S from 81 candidate CA/S variables. A standard logistic regression model then incorporated indicator variables for the 5 categories of CAs/Ss, 7 unique NCAAs, and all other covariates in the previously published Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Model. RESULTS: Analysis included 107,062 operations in 100 centers (2010 to 2015). Operative Mortality was 3,629 (3.4%). In the development sample, the C statistics of the original nonaugmented model and the augmented model were 0.872 and 0.875, respectively. CONCLUSIONS: The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model has been augmented by addition of covariates representing individual CAs, Ss, and NCAAs.

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.

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.

Hands-on surgical training of congenital heart surgery using 3-dimensional print models.

OBJECTIVE: Patient-based congenital heart surgery (CHS) training is opportunity-based and difficult. Three-dimensional (3D) print models of the heart were used for hands-on surgical training (HOST) at the 2015 AATS and subsequently in 2 local institutions. We aim to introduce the process of 3D printing for surgical simulation and to present the attendee's responses. METHODS: Using CT or MR angiograms, the models of congenital heart disease were created and printed with flexible rubberlike material. Altogether, 81 established surgeons or trainees performed simulated surgical procedures with the expert surgeons' guidance and supervision. At the completion of the session, 50 of 81 attendees participated in the questionnaire assessment of the program. RESULTS: All responders found the course helpful in improving their surgical skills. All would consider including HOST sessions in the training programs. All found that the models showed the necessary pathologic findings. Most found that the consistency and elasticity of the model material were different from those of the human myocardium. However, the responders thought that the quality of the models was acceptable (88%) or manageable (12%) for surgical practice. The major weaknesses listed were related to the print material and poor representation of the cardiac valves. CONCLUSIONS: HOST using 3D print heart models is achievable and allows surgical practice on pathological hearts without patients' risk. HOST is a highly applicable surgical simulation format for CHS. Incorporation of HOST in training programs could change the traditional opportunity-based education to the requirement-based standardized education.

Comparison of Blood Viscoelasticity in Pediatric and Adult Cardiac Patients.

Evidence is accumulating that blood flow patterns in the cardiovascular system and in cardiovascular devices do, in some instances, depend on blood viscoelasticity. Thus, to better understand the challenges to providing circulatory support and surgical therapies for pediatric and adult patients, viscous and elastic components of complex blood viscoelasticity of 31 pediatric patients were compared to those of 29 adult patients with a Vilastic-3 rheometer. A random effects model with categorical age covariates found statistically significant differences between pediatric and adult patients for log viscosity (p = 0.005). Log strain (p < 0.0001) and hematocrit (p < 0.0001) effects were also significant, as were the hematocrit-by-log-strain (p = 0.0006) and age-by-log strain (p = 0.001) interactions. The hematocrit-by-age interaction was not significant. For log elasticity, age differences were insignificant (p = 0.39). The model for log elasticity had significant log strain (p < 0.0001), log strain squared (p < 0.0001) and hematocrit (p < 0.0001) effects, as well as hematocrit-by-log-strain and hematocrit-by-log-strain-squared interactions (p = 0.014). A model for log viscosity with continuous age was also fit to the data, which can be used to refine cardiovascular device design and operation to the age of the patient. We conclude that there are distinct differences between pediatric and adult blood viscosity, as well as substantial variation within the pediatric population, that may impact the performance of devices and procedures.

The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2017 Update on Outcomes and Quality.

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%).