Slide tracheoplasty for congenital tracheal stenosis: A systematic review.

INTRODUCTION: Slide tracheoplasty has become the gold standard surgery for congenital tracheal stenosis (CTS). This condition is rare and the surgery can be challenging and is performed by experienced surgeons in tertiary centers. A few reports involving relatively small cohorts have been published. The aim of this review is to evaluate the post-operative mortality and morbidity of pediatric slide tracheoplasty for CTS. METHODS: A systematic literature review was performed according to PRISMA guidelines. The Medline and EMBASE databases were screened using a search strategy defined in collaboration with a librarian. We included articles reporting the post-operative mortality rate of slide tracheoplasties for treatment of CTS in children, when at least 10 patients were included. RESULTS: A total of 932 articles were reviewed, and 15 studies were eligible with a total of 845 patients. The overall post-operative mortality rate was 9.3 %, and most deaths were airway related. The open revision surgery rate after surgery was 2.8 % and the endoscopic revision rate was 27.6 %. DISCUSSION: This study highlights key factors to consider before the surgery and helps anticipate post-operative follow-up considerations for children with CTS. Several factors were identified as predictors of mortality including young age, weight at the time of surgery and association with lung hypoplasia or aplasia. CONCLUSION: Although slide tracheoplasty has gained popularity in recent years due to better outcomes, it remains a major surgery with mortality risk and the need for multidisciplinary management.

Intrapericardial Ectopic Hepatic Tissue Masquerading as a Fetal Pericardial Tumor.

Prenatal diagnosis of pericardial mass, with associated large pericardial effusion, resected postnatally and diagnosed to be ectopic hepatic tissue on pathology.

Long-Term Outcome Following Pulmonary Valve Replacement in Repaired Tetralogy of Fallot.

BACKGROUND: Late pulmonary valve replacement following repair of tetralogy of Fallot may become necessary in patients with chronic pulmonary insufficiency. There is limited information on the long-term outcome of these prostheses, which is the focus of this study. METHODS: We conducted a retrospective study of patients with repaired tetralogy of Fallot who underwent pulmonary valve replacement from 1990 to 2015 in our institution. We investigated imaging and clinical parameters including mortality and late adverse events (reintervention [surgical or transcatheter]), infective endocarditis, or arrhythmias requiring device implantation or ablation. RESULTS: There were 69 patients divided into 3 groups: Carpentier-Edwards (n = 14), Contegra (n = 40), and pulmonary homograft (n = 15). The mean age at the time of pulmonary valve replacement was 21 ± 12 years. The mean follow-up was 8.5 ± 4.7 years. The mean preoperative and postoperative right ventricular end-diastolic volume index was 210 ± 42 and 120 ± 24 mL/m2, respectively. There were no mortalities. Late adverse events were observed in 23 (33%) patients: 15 (22%) reintervention (surgical or transcatheter), 11 (16%) endocarditis, and 11 (16%) arrhythmias. Overall, 1-, 5-, and 10-year freedom from surgical reintervention was 98.5%, 93.6%, and 79.3%, respectively. The Contegra group had significantly higher pulmonary valve gradients, a higher risk of developing late adverse events compared to Carpentier-Edwards (P = .046) and pulmonary homograft (P = .055) in multivariate analysis and increased risk for reintervention in the univariate analysis (hazard ratio: 3.4; 95% CI: 0.92-13; P value.066). CONCLUSION: Pulmonary valve replacement in patients with repaired tetralogy of Fallot has acceptable short- and intermediate-term outcomes. Contegra prosthesis had a higher risk of late adverse events with higher pulmonary valve gradients.

Current Management of Anomalous Aortic Origin of a Coronary Artery: A Pan-Canadian Survey.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is a significant cause of sudden cardiac death (SCD) in children and adolescents. The natural history of AAOCA and the pathophysiology of AAOCA-related SCD are poorly understood. Therefore, the evaluation and management of AAOCA remain controversial. This survey-based study aims to report the current AAOCA management tendencies in Canada. METHODS: We built a 23-question survey on AAOCA. Questions pertained to patient presentation, investigations, morphology of the anomaly, management, and follow-up. We sent the survey to all the Canadian congenital cardiac surgeons, pediatric cardiologists, and adult congenital cardiologists. Data were anonymized and analysis was performed using descriptive statistics. RESULTS: According to our survey participants (N = 47), patient age (94%) and amount of physical activity (60%) are the most influential factors when deciding whether to offer surgical correction. Aborted SCD, exercise-induced syncope, typical chest pain, and left jaw or arm pain are the most important clinical presentations indicating surgery. The most commonly used preoperative investigations are rest echocardiography (75%), electrocardiogram (68%), and exercise stress test (62%). Most respondents favor the unroofing procedure (78%) for surgical correction. For nonsurgical candidates, most physicians choose competitive exercise restriction (64%). CONCLUSION: We found a divergence between current practices and expert consensus guidelines regarding the treatment of asymptomatic left AAOCA with high-risk features. Our survey also revealed a lack of consensus among clinicians regarding the management of asymptomatic patients, very young patients, and those with right-sided AAOCA. Evidence-based criteria derived from sufficiently powered studies remain to be established to standardize AAOCA treatment.

Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. OBJECTIVES: This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission). METHODS: The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression. RESULTS: Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index. CONCLUSIONS: In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.

Successful treatment of mediastinitis in a young child by omental translocation following extracardiac Fontan graft placement.

Mediastinitis after a midline sternotomy can become a serious complication, especially after implantation of prosthetic vascular grafts. We present a case of a three-year-old boy with hypoplastic left heart syndrome who developed mediastinitis following his third-stage palliation (Fontan operation). Rather than following the "traditional" surgical therapy of graft explantation, debridement, and replacement, we chose to preserve the graft and protect it by omental translocation. The relative merits of this therapeutic approach, which is rarely utilized and underappreciated in children, are outlined and discussed.

Aortopulmonary fistula after outflow tract stent in repaired truncus.

We present a case of an iatrogenic aortopulmonary (AP) fistula in a 9-year-old patient with a history of repaired truncus arteriosus without the use of a right ventricle to pulmonary artery conduit and subsequent transcatheter placement of a right ventricular outflow tract (RVOT) stent. Redilation of the stent resulted in a defect in the aortic wall and the creation of an AP fistula with an associated hemodynamically significant left to right shunt. This case demonstrates a previously unreported adverse event of transcatheter RVOT reintervention after truncus arteriosus repair.

Extent, risk factors, and outcome of fluid overload after pediatric heart surgery*.

OBJECTIVES: Fluid overload is associated with poor PICU outcomes in different populations. Little is known about fluid overload in children undergoing cardiac surgery. We described fluid overload after cardiac surgery, identified risk factors of worse fluid overload and also determined if fluid overload predicts longer length of PICU stay, prolonged mechanical ventilation (length of ventilation) and worse lung function as estimated by the oxygenation index. DESIGN: Retrospective cohort study. SETTING: Montreal Children's Hospital PICU, Montreal, Canada. PATIENTS: Patients 18 years or younger undergoing cardiac surgery (2005-2007). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cumulative fluid overload % was calculated as [(total fluid in - out in L)/admission weight (kg) × 100] and expressed as PICU peak cumulative fluid overload % throughout admission and PICU day 2 cumulative fluid overload %. Primary outcomes were length of stay and length of ventilation. The secondary outcome was oxygenation index. Fluid overload risk factors were evaluated using stepwise linear regression. Fluid overload-outcome relations were evaluated using stepwise Cox regression (length of stay, length of ventilation) and generalized estimating equations (daily PICU cumulative fluid overload % and oxygenation index repeated measures). There were 193 eligible surgeries. Peak cumulative fluid overload % was 7.4% ± 11.2%. Fluid overload peaked on PICU day 2. Lack of past cardiac surgery (p = 0.04), cyanotic heart disease (p = 0.03), and early postoperative fluids (p = 0.0001) was independently associated with higher day 2 fluid overload %. Day 2 fluid overload % predicted longer length of stay (adjusted hazard ratio, 0.95; 95% CI, 0.92-0.99; p = 0.009) and length of ventilation (adjusted hazard ratio, 0.97; 95% CI, 0.94-0.99; p = 0.03). In patients without cyanotic heart disease, worse daily fluid overload % predicted worse daily oxygenation index. CONCLUSION: Fluid overload occurs early after cardiac surgery and is associated with prolonged PICU length of stay and ventilation. Future fluid overload avoidance trials may confirm or refute a true fluid overload-outcome causative association.

The challenge of congenital heart disease worldwide: epidemiologic and demographic facts.

Congenital heart disease (CHD) afflicts a large number of children every year. The incidence of CHD is generally considered to be 8 per 1,000 live births. However, this estimate is perhaps inaccurate and does not take into consideration regional differences. A large review of the literature was performed to establish the true incidence of CHD and geographical variations. Data on the incidence of specific lesions and their geographical variation, as well as on mortality from CHD, was also reviewed. Taking into consideration the available data on incidence, mortality, and access to care, the global challenge that CHD represents was analyzed. Insight into how to confront this challenge is given.

Does ABO-incompatible and ABO-compatible neonatal heart transplant have equivalent survival?

A best evidence topic (BET) in cardiac surgery was written according to a structured protocol. The question addressed was whether ABO-incompatible (ABO-I) heart transplant recipients have a similar survival rate as an ABO-compatible (ABO-C) transplant in the pediatric population <1 year of age. Altogether more than 112 papers were found using the reported search, of which 10 represented the best evidence to answer the clinical question. Generally, ABO-I transplantation has been associated with the neonatal population because of the relative immaturity of the immune system for the first year of life. In the BET the search-term 'pediatric' was used as a method to ensure retrieval all relevant papers. However, the vast majority of the patients reviewed were <1 year of age with specific techniques undertaken to modulate preoperative, intraoperative and postoperative isohemagglutinin titer levels with rejection monitoring. Therefore, the BET conclusions should not be applied to the pediatric group as a broad age classification. Two large series are of particular interest. Patel et al. reviewed all primary heart transplantation recipients < or =1 year of age in the United Network for Organ Sharing/Organ Procurement and Transplantation Network (UNOS/OPTN) registry (ABO-I=35 vs. ABO-C=556). There was no difference in 30-day mortality (ABO-I=5.9% vs. ABO-C=8.8%; P=0.55); one-year mortality (ABO-I=16.6% vs. ABO-C=14.7%; P=0.77); graft rejection (ABO-I=1 vs. ABO-C=0); and graft failure (ABO-I=24% vs. ABO-C=24%; P>0.99). Three-year Kaplan-Meier survival was 70% (P=0.85). Propensity score adjusted analysis did not implicate ABO-I as a predictor of mortality [hazard ratio (HR)=3.6, confidence interval (CI): 0.2-49.0; P=0.33]. The ABO-I group demonstrated an increased need for pacemaker (ABO-I=3.1% vs. ABO-C=0.4%; P=0.03) and higher stroke rate (ABO-I=12.9% vs. ABO-C=1.3%; P<0.0001). Dipchand et al. published the results of the Toronto cohort from 1992 to 2006 (ABO-I=16 vs. ABO-C=38). The median age at transplantation (ABO-I=88 days vs. ABO-C=84 days; P=0.82) and the number of neonatal transplant recipients (ABO-I=17% vs. ABO-C=22%; P=0.59) was similar. The freedom from post-transplantation death or retransplantation was equivalent at one year (ABO-I=77% vs. ABO-C=84%) and seven years (ABO-I=74% vs. ABO-C=74%; P=0.87). No significant difference was observed for the five-year freedom from: rejection (ABO-I=60% vs. ABO-C=45%; P=0.41); renal dysfunction (ABO-I=67% vs. ABO-C=72%; P=0.70); allograft vasculopathy (ABO-I=87% vs. ABO-C=78%; P=0.45); and post-transplantation lymphoproliferative disorder (PTLD) (ABO-I=87% vs. ABO-C=86%; P=0.93). We conclude that ABO-I transplantation is comparable to ABO-C transplantation, with several retrospective papers concluding there is no difference in mortality, morbidity or graft failure in the pediatric population <1 year of age.

Repair of atrioventricular canal with double-outlet right ventricle, transposition, or truncus arteriosus.

Atrioventricular canal and conotruncal anomalies are a heterogeneous group of lesions presenting unique challenges for surgical repair. These are the establishment of unobstructed pathways from left ventricle (LV) to aorta and from right ventricle (RV) to pulmonary artery, closure of the inlet ventricular septal defect (VSD) and atrial septal defect (ASD) ostium primum, and the avoidance of significant left and right atrioventricular valve (AV) regurgitation. Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) has been most commonly achieved, either using a single-patch or a 2-patch technique. In patients with CAVC with double-outlet right ventricle (DORV) with subaortic VSD extension, the 2-patch repair is not unlike that of CAVC with TOF. However, biventricular repair is most challenging in patients with CAVC and complete origin of the aorta from the RV, as in CAVC with DORV and noncommitted VSD and those with CAVC with transposition of the great arteries (TGA) and LVOTO. The technique of VSD translocation allows anatomic biventricular repair for these particularly challenging patients. The arterial switch operation with CAVC repair can be used for patients with CAVC with DORV with subpulmonary VSD extension and CAVC with TGA without left ventricular outflow tract obstruction. Biventricular repair is achievable in most patients with balanced complete atrioventricular canal and conotruncal anomaly. The extreme heterogeneity of CAVC with conotruncal anomalies requires a highly individual approach that is tailored to the specific constellation of lesions in each patient.

Pediatric cardiovascular surgery in South america: current status and regional differences.

Very little information is available about the epidemiology of congenital heart disease in developing parts of the world, including South America. This article describes the incidence of congenital cardiac disease, the different treatment rates among countries, and future solutions for achieving improved coverage for the children with cardiac diseases in South America. An incidence of congenital cardiac disease of 8 per 1000 live births appears to be a fair approximation for the population of the world and also the population in South America. Nevertheless, a wide variation exists in the observed incidence of congenital cardiac disease in South American countries, which can be partly explained by inequalities in the access to diagnosis, differences in the diagnostic criteria, and true regional variations. It is estimated that 58,718 children are born yearly with congenital heart disease in South America. Brazil, Colombia, and Argentina have the highest number, followed by Peru, Venezuela, Chile, Ecuador, Bolivia, Paraguay, Uruguay, and Guyana. It is also estimated that in South America, 24,081 children per year with a new diagnosis of congenital cardiac disease do not receive any treatment. This paper provides strategies for improving the access to and quality of pediatric cardiac surgery in South America.

A small post-operative rise in serum creatinine predicts acute kidney injury in children undergoing cardiac surgery.

To predict development of acute kidney injury and its outcome we retrospectively studied children having cardiac surgery. Acute kidney injury (AKI) was defined using the serum creatinine criteria of the pediatric Risk Injury Failure Loss End-Stage (pRIFLE) kidney disease definition. We tested whether a small rise (less than 50%) in creatinine on post-operative days 1 or 2 could predict a greater than 50% increase in serum creatinine within 48 h in 390 children. AKI occurred in 36% of patients, mostly in the first 4 post-operative days. Using logistic regression, significant independent risk factors for AKI were bypass time, longer vasopressor use, and a tendency for younger age. Using Cox regression, AKI was independently associated with longer intensive care unit stay and duration of ventilation. Patients whose serum creatinine did not increase on post-operative days 1 or 2 were unlikely to develop AKI (negative predictive values of 87 and 98%, respectively). Percentage serum creatinine rise on post-operative day 1 predicted AKI within 48 h (area under the curve=0.65). Our study shows that AKI after pediatric heart surgery is common and is a risk factor for poorer outcome. Small post-operative increases in serum creatinine may assist in the early prediction of AKI.

The World Society for Pediatric and Congenital Heart Surgery: its mission and history.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

Retrograde autologous priming and allogeneic blood transfusions: a meta-analysis.

A literature review and meta-analysis were undertaken to assess the clinical effectiveness of retrograde autologous priming of the cardiopulmonary bypass circuit to reduce allogeneic packed red blood transfusions in adult cardiac surgery. Structured searches of Medline, Embase, Cochrane Collaboration Library, Scopus, Cumulative Index to Nursing and Allied Health Literature and Science Direct were performed to identify randomized trials comparing retrograde autologous priming to a prospective control group. A total of 21,643 studies were identified and eighteen trials were retrieved for full-text review. Six trials met eligibility criteria. Pooled estimates demonstrated that retrograde autologous priming significantly reduced the number of patients receiving intraoperative packed red cell transfusions (OR=0.36; 95% CI: 0.13, 0.94; P=0.04, I(2)=47.5%), total hospital stay packed red cell transfusions (OR=0.26; 95% CI: 0.13, 0.52; P=0.0001, I(2)=0%), and the number of units transfused of total hospital stay packed red blood cells (WMD=-0.60; 95% CI: -0.90, -0.31; P=0.0001, I(2)=0%). Retrograde autologous priming, however, did not provide a clinical benefit in reducing the number of units transfused of intraoperative packed red blood cells (WMD=-0.29; 95% CI: -0.59, 0.01; P=0.05). The combined patient population studied in the six trials was mainly primary isolated coronary artery bypass surgery. Assessing the safety of retrograde autologous priming was not possible due to limited data.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery.

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].