The Ross Procedure in Children and Infants: A Systematic Review With Pooled Analyses.

Background: The Ross procedure is a surgical option for congenital aortic stenosis that involves replacing the diseased aortic valve with a pulmonary autograft. Little is known about outcomes in children, particularly those younger than 1 year. Methods: A systematic review with pooled analyses was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria. Inferred individual patient data were extracted from life tables. The primary end points were early (≤30 days) and late (>30 days) mortality rates following the Ross procedure in children. Secondary end points were freedom from reintervention for the right ventricular outflow tract and pulmonary autograft. These end points were assessed in the overall population of children. Sensitivity analyses were performed in subgroups younger than 1 year of age (infants) and in noninfant children. Results: A total of 25 studies on 2737 patients met inclusion criteria. The pooled early survival rate was 96.0% (95% confidence interval [CI]: 95.1%-96.8%) overall and 86.8% (95% CI: 82.1%-90.3%) among infants. Pooled overall 10-year survival, freedom from pulmonary autograft reintervention, and freedom from right ventricular outflow tract reintervention rates were 91.1%, 90.2%, and 79.7%, respectively. Corresponding pooled rates in infants were 79.3%, 87.1%, and 51.2%. Mortality was significantly higher among infants compared with noninfant children (hazard ratio: 3.38, 95% CI: 2.44-4.68; P < 0.001). In metaregression analyses, younger age was strongly associated with poorer survival and higher reintervention rates. Conclusions: Modest survival and autograft reoperation rates were observed following the Ross procedure in children. Surgery in infancy was strongly associated with poorer survival and higher reintervention rates.

Contexte: L'intervention de Ross est une option chirurgicale visant à corriger une sténose aortique congénitale. Elle consiste à remplacer une valve aortique pathologique en utilisant la propre valve pulmonaire du patient (autogreffe pulmonaire). Les résultats de cette intervention ont été peu étudiés chez les enfants, en particulier chez les enfants de moins d'un an. Méthodologie: Une revue systématique avec analyses des données groupées a été menée en respectant les critères PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses). Les données dérivées de patients individuels ont été tirées de tables de survie. Les principaux critères d'évaluation étaient les taux de mortalité précoce (≤ 30 jours) et tardive (> 30 jours) à la suite de l'intervention de Ross réalisée chez des enfants. Les critères d'évaluation secondaires étaient l'absence de nouvelle intervention pour la chambre de chasse du ventricule droit et pour l'autogreffe pulmonaire. Ces paramètres ont été évalués pour l'ensemble de la population pédiatrique. Des analyses de sensibilité ont été réalisées dans des sous-groupes de patients âgés de moins d'un an (nourrissons) et d'enfants plus âgés. Résultats: Au total, 25 études portant sur 2 737 patients répondaient aux critères d'inclusion. Les taux de survie précoce étaient de 96,0 % (intervalle de confiance [IC] à 95 %; 95,1 à 96,8 %) dans l'ensemble des patients et de 86,8 % (IC à 95 %; 82,1 à 90,3 %) chez les nourrissons. Le taux de survie globale à 10 ans, d'absence de nouvelle intervention pour l'autogreffe pulmonaire et d'absence de nouvelle intervention pour la chambre de chasse du ventricule droit étaient respectivement de 91,1 %, de 90,2 % et de 79,7 % (données groupées). Les taux correspondant pour le sous-groupe composé de nourrissons étaient de 79,3 %, de 87,1 % et de 51,2 % (données groupées). La mortalité était significativement plus élevée chez les nourrissons comparativement aux enfants plus âgés (rapport des risques instantanés : 3,38, IC à 95 % : 2,44 à 4,68; p < 0,001). Dans les analyses de métarégression, le jeune âge était fortement corrélé à un taux de survie plus bas et à des taux plus élevés de nouvelles interventions. Conclusions: Des taux modestes de survie et de réopération d'autogreffe ont été observés après la procédure de Ross chez les enfants. La chirgurgie pendant la petite enfance était fortement associée à une survie plus faible et à des taux de réintervention plus élevés.

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.

Considerations in Timing of Surgical Repair in Tetralogy of Fallot.

Certain aspects of the treatment of tetralogy of Fallot (TOF) repair remain controversial. The optimal timing of the elective repair of asymptomatic patients and the ideal strategy for managing symptomatic neonates and infants with TOF are still debated despite years of experience in TOF treatment. In this article, we discuss why a surgical correction at 3-6 months of age is likely the ideal time frame for the elective repair of TOF. We also elaborate on our strategy for managing symptomatic neonates and infants with TOF and why we prefer an early single-stage primary repair.

Certains aspects du traitement de la tétralogie de Fallot (TF) par correction chirurgicale demeurent controversés. Le moment le plus approprié pour l'intervention non urgente chez les patients qui ne présentent pas de symptômes et la meilleure stratégie de prise en charge des nouveau-nés et des nourrissons atteints de TF symptomatique font encore l'objet de débats, même après de nombreuses années d'expérience dans le traitement de la TF. Dans le présent article, nous expliquons pourquoi la période de 3 à 6 mois est probablement idéale pour réaliser une correction chirurgicale non urgente de la TF. Nous expliquons également notre stratégie de prise en charge des nouveau-nés et des nourrissons qui présentent une TF symptomatique et les raisons pour lesquelles nous préférons réaliser une correction primaire en une seule étape à un âge précoce.

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

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.

Pulmonary Valve Replacement for Pulmonary Regurgitation in Adults With Tetralogy of Fallot: A Meta-analysis-A Report for the Writing Committee of the 2019 Update of the Canadian Cardiovascular Society Guidelines for the Management of Adults With Congenital Heart Disease.

BACKGROUND: There is no systematic evidence review of the long-term results of surgical pulmonary valve replacement (PVR) dedicated to adults with repaired tetralogy of Fallot (rTOF) and pulmonary regurgitation. METHODS: Our primary objective was to determine whether PVR reduced long-term mortality in adults with rTOF compared with conservative therapy. Secondary objectives were to determine the postoperative incidence rate of death, the changes in functional capacity and in right ventricular (RV) volumes and ejection fraction after PVR, and the postoperative incidence rate of sustained ventricular arrhythmias. A systematic search of multiple databases for studies was conducted without limits. RESULTS: No eligible randomized controlled trial or cohort study compared outcomes of PVR and conservative therapy in adults with rTOF. We selected 10 cohort studies (total 657 patients) reporting secondary outcomes. After PVR, the pooled incidence rate of death was 1% per year (95% confidence interval [CI] 0-1% per year) and the pooled incidence rate of sustained ventricular arrhythmias was 1% per year (95% CI 1%-2% per year). PVR improved symptoms (odds ratio for postoperative New York Heart Association functional class > II 0.08, 95% CI 0.03-0.24). Indexed RV end-diastolic (-61.29 mL/m2, -43.64 to -78.94 mL/m2) and end-systolic (-37.20 mL/m2, -25.58 to -48.82 mL/m2) volumes decreased after PVR, but RV ejection fraction did not change (0.19%, -2.36% to 2.74%). The effect of PVR on RV volumes remained constant regardless of functional status. CONCLUSION: Studies comparing PVR and conservative therapy exclusively in adults with rTOF are lacking. After PVR, the incidence rates of death and ventricular tachycardia are both 1 per 100 patient-years. Pooled analyses demonstrated an improved functional status and a reduction in RV volumes.

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 tricuspid valve in adult congenital heart disease.

The tricuspid valve is frequently affected in adults with congenital heart disease but is also frequently overlooked. Disease of this valve can occur primarily or develop secondary to changes in the right ventricle caused by other disease states. The embryology and anatomy of the tricuspid valve are important to understanding pathogenesis of valve dysfunction in congenital heart disease. Clinical findings can be subtle. Multimodality imaging may be necessary to fully assess the cause and impact of tricuspid valve lesions. More research is needed in pathophysiology, imaging, and treatment in this area.

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.

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.