Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare but highly heterogeneous congenital heart lesion defined by absence of continuity between the right ventricle (RV) and pulmonary arteries, a morphologically variable tricuspid valve (TV), RV hypoplasia and frequent right ventricle to coronary connections. Management has evolved from uniform surgical palliation toward nuanced, morphology-driven individualized strategies. Current approaches include transcatheter perforation, surgical valvotomy, staged ventricular recruitment, and single-ventricle or 1.5-ventricle palliation, with transplantation reserved for select patients. Recent publication reported a successful procedure to palliate coronary RV dependency with an aortic to TV conduit. Long-term outcomes hinge on RV growth potential, coronary anatomy, and institutional expertise.
Complete atrioventricular canal defect (CAVC) is the seventh most common congenital heart defect, affecting 1 in 1712 live births. Embryologically CAVC defect arises from failure of normal fusion of the endocardial cushions resulting in a spectrum of abnormalities that involve the atrial septum, atrioventricular (AV) valve, subvalvar apparatus, and the ventricular septum. This article will discuss historic timelines, evolution of management strategies, and outcomes following current surgical approaches in well-balanced CAVC. Recent advances in genetics and imaging techniques will also be discussed.
Arterial switch operation (ASO) has been the standard anatomic repair for D-transposition of the great arteries (d-TGA) since Jatene et al. first described it in 1975. While survival is excellent, some patients develop late complications requiring reoperation, including neo-aortic regurgitation (neo-AR) and root dilatation (ARD). Mechanisms of neo-AR are multifactorial, and management depends on whether the pathology is leaflet- or root-driven. When neo-AR is secondary to ARD, options include valve-sparing root replacement (VSRR) or composite root replacement (Bentall procedure) using either a mechanical or a bioprosthetic valve. In cases of isolated neo-AR without significant ARD, valve repair with annular stabilization can be considered. Alternatively, such cases may be managed with the Ross procedure, first described as 'Switch-Back Ross' by Hazekamp et al., although this approach remains less commonly employed. The specific surgical choice should integrate anatomy, physiology, and patient-specific factors within a shared decision-making framework. This review focuses on the Ross procedure after ASO, with emphasis on operative pearls, expected complications, and durability considerations. We illustrate these principles through the case of a 19-year-old man, late after ASO, who presented with severe neo-AR and mild ARD. He underwent a 'supported' Ross with external annuloplasty and recovered uneventfully. This case underscores the feasibility of the Ross procedure when valve repair is nondurable and emphasizes annular stabilization as key to long-term success, contributing to the limited literature on this approach in post-ASO patients.
Aerodigestive compression syndromes of vascular origin can be broadly classified based on congenital and acquired causes. Congenital causes include various types of arch anomalies (vascular rings including double arches, right aortic arch with aberrant left subclavian artery, circumflex aorta, etc), arch branching anomalies (Left aortic arch with aberrant right subclavian artery, innominate artery related compression), aneurysms of either the aorta or main pulmonary artery or branch pulmonary arteries and any combinations of these. Acquired causes of aerodigestive compression are mostly post-surgical which is a distinct and challenging entity usually arising from arch reconstructive procedures, root amalgamation procedures, procedures involving the LeCompte maneuver and procedures involving PDA stenting. When there are associated thoracic cage and vertebral anomalies, an additional layer of complexity is added to an already challenging problem. At Boston Children’s Hospital, these patients are evaluated with a multi-disciplinary team using diagnostic studies including echocardiography, esophagram, computed tomographic angiography and three-phase dynamic bronchoscopy for a thorough understanding of anatomic and physiologic complexities. Surgical correction to relieve compression requires an individualized plan for each patient and 3D models are routinely employed. Posterior thoracotomy, sternotomy and video assisted thorocoscopy (VATs) are approaches to correct these problems with additional concomitant usage of aortopexy, tracheopexy and localized or complete esophageal mobilization. Intraoperative bronchoscopy is widely used both for surgical planning and for confirmation of relief of compression.
Pulmonary vein stenosis (PVS) is a rare and aggressive condition in infants and children, characterized by progressive neointimal proliferation, multivessel involvement, and high early mortality. Despite historically poor outcomes, a growing body of evidence supports the use of catheter-based interventions as a cornerstone of modern multimodal treatment strategies. Balloon angioplasty, and bare-metal or drug-eluting stents are used to restore vessel patency, often as part of serial procedures. However, recurrent stenosis remains common, necessitating frequent reintervention. Reintervention is associated with improved survival, particularly when paired with early diagnosis, aggressive surveillance, and systemic medical therapies. When transcatheter interventions are applied as part of a multidisciplinary approach, they contribute to meaningful improvements in survival and quality of life. Ongoing innovation in device technology and biologic modulation will be critical in further advancing outcomes for this high-risk population.
Infants with complex congenital heart disease represent a vulnerable subset of pediatric patients who experience unacceptably high mortality while awaiting cardiac allotransplantation. The limited availability of size-matched donor organs and the unsuitability of existing mechanical circulatory support in this patient population compels the development of alternative bridging strategies. Cardiac xenotransplantation using genetically modified porcine donors represents a potential solution to this complex clinical issue. In this review, we describe the scientific and clinical landscape of pediatric cardiac xenotransplantation, summarize the key findings from a preclinical baboon model of orthotopic cardiac xenotransplantation as a bridge to allotransplantation, and discuss future directions required to bring this concept to clinical implementation.
Tracheobronchomalacia is classified as a benign condition characterized by airway collapse resulting from weakening of the airway walls. Current treatment options range from simpler measures, such as the use of a continuous positive airway pressure machine, to more complex surgeries. Airway stents provide a middle ground in terms of treatment invasiveness and post-procedure recovery. Unfortunately, long-term implantation of stents is not clinically viable due to complications of excessive granulation tissue formation and high impedance to mucociliary clearance. Existing stent systems are not optimally designed for benign airway collapse and do not directly address these complications. Optimally designed helical stents, fabricated from both metallic and non-metallic materials, demonstrate potential to address these complications and offer a system suitable for long-term implantation. While a variety of strategies are being investigated to develop viable stent systems, there is a lack of standardized and precise stent-testing platforms. Recent progress in ex vivo tissue-based platforms shows promise of making meaningful progress in airway stent development.
Complex tracheal anomalies are life threatening without intervention and often require surgery in the neonatal period to establish a stable airway. Variations of slide tracheoplasty using all autologous tissue are the gold standard in tracheal reconstruction. We have encountered 9 patients whose complex tracheal anomalies required significant variations to slide tracheoplasty. Six of these patients presented with bronchus suis with extreme long segment tracheobronchial stenosis which were effectively repaired using a side-to-side bronchus suis tracheobronchoplasty. This technique has the advantage of providing an all-autologous reconstruction with significantly less tension. Recently, we have encountered 3 patients with even more radical anatomies consisting of long segment tracheobronchial stenosis in the context of bilateral bronchus suis or Christmas-tree trachea. We employed bilateral side-to-side bronchus suis tracheoplasty in one and anterior-posterior bilateral bronchus suis tracheoplasty in the remaining 2 cases. These techniques have proven extremely effective in stabilizing the airway while preserving growth potential. Early management of these patients remains challenging, and complications are exponentially magnified by the interplay between complex cardiac physiology and complex tracheal reconstruction.
Management of this complex condition has evolved considerably over the past 30 years, transforming the prognosis for this rare and heterogeneous condition. Central to this evolution has been the concept of achieving "unifocalisation" together with the combined approaches of MAPCA recruitment and vascular rehabilitation. Improvements in imaging modalities and applications have transformed the anatomical assessment and understanding of the pulmonary vasculature, which in turn, has facilitated refined surgical techniques and approaches to achieve these improved outcomes. Early assessment enables clinicians to create a precise "road-map" of the pulmonary vasculature, and allow for meticulous planning of the surgical procedures. This article breaks down the management into 6 key decisions that need to be taken in order to achieve the best outcomes across all the variants of the condition.
Pulmonary vein stenosis (PVS) is a severe vascular obstructive lesion historically associated with poor survival. As outcomes have improved in the current era, PVS has become a chronic disease requiring frequent surgeries, catheter-based interventions, and experimental therapies to prevent progression and recurrence. Despite this, PVS survival remains low and has pushed experimental research into new therapies into the forefront. In this paper, we briefly examine the pathophysiology and etiology of PVS and provide a more comprehensive review of computational and animal modeling of PVS and therapies derived from these studies, both current and on the horizon.
The criss-cross heart with double outlet right ventricle (DORV) and combined atrioventricular (AV) and ventriculoarterial (VA) discordance presents one of the most formidable challenges in congenital cardiac surgery. The unique ventricular topology and complex inflow-outflow relationships often preclude standard approaches to biventricular repair, and single-ventricle palliation is frequently selected. However, in appropriately selected patients, anatomical repair through a double switch procedure may offer superior long-term outcomes by preserving systemic ventricular function. This report describes a surgical strategy for addressing a criss-cross heart with DORV and AV/VA discordance using a modified Senning procedure as part of a double switch operation. Preoperative imaging—including transthoracic echocardiography, computed tomography angiography, and cardiac catheterization—is critical for assessing operability. Particular attention must be paid to pulmonary vascular resistance, Qp/Qs, morphologic left ventricular end-diastolic pressure, and coronary anatomy. The surgical technique involves ventricular septal defect baffle closure to direct left ventricular output to the pulmonary artery, an arterial switch operation, and a modified Senning atrial switch. The Senning component is performed using a pedicled autologous pericardial baffle augmented with a large bovine pericardial patch to prevent pulmonary venous pathway narrowing, a frequent concern in patients with criss-cross anatomy. This approach allows for biventricular repair even in cases with severe atrial and ventricular malalignment. The technique aims to achieve unobstructed systemic and pulmonary venous pathways, minimize arrhythmia risk, and establish long-term left ventricular systemic circulation. Careful patient selection and meticulous surgical execution are essential for optimizing outcomes in this complex subgroup of congenital heart disease.
Single ventricle congenital heart disease (SVCHD) remains a high-risk pathology for transplantation-free survival and quality of life. While medical and surgical advancements have significantly improved attrition through palliation and ultimately survival following the Fontan operation, Fontan candidacy evaluation remains a critical component to insuring optimal outcomes for patients with SVCHD. While the initial 10 Commandments proposed by Choussat and colleagues provided pioneering insights and supported early success of the Fontan operation, advancements in care have shifted the focus of Fontan candidacy determination to be patient- and center-specific, seeking to maintain both Fontan and transplantation candidacy across all risk categories. Herein, we present a focused review of the current state of Fontan candidacy determination while also offering a multi-disciplinary perspective within this evolving and nuanced aspect of care for patients with SVCHD.
Repair of atrioventricular septal defect (AVSD) remains one of the most technically demanding procedures in congenital cardiac surgery. In patients with balanced anatomy, biventricular repair provides excellent long-term survival; however, borderline and unbalanced AVSD continue to pose major challenges, particularly in children with Down syndrome. Contemporary practice emphasizes that balance is not fixed but can be influenced by surgical planning, ventricular recruitment, and meticulous management of the left atrioventricular valve (LAVV). LAVV regurgitation remains the principal cause of early failure and late reoperation after AVSD repair, and its impact is especially pronounced in the setting of repeat operations, where annular dilation, leaflet deficiency, and prior surgical manipulation complicate repair.Institutional experience demonstrates that immediate moderate or greater LAVV regurgitation after repair strongly predicts both mortality and reoperation, highlighting the importance of rigorous intraoperative echocardiographic assessment and close collaboration between surgeons and cardiologists. Mild to moderate regurgitation represents a surgical “trap,” as it frequently worsens during early follow-up. Longitudinal data show that nearly half of patients with postoperative moderate regurgitation deteriorate to severe, while almost one third improve spontaneously. Successful redo repair requires mechanism-driven strategies, including cleft closure or patch plasty, annuloplasty, papillary muscle splitting, and leaflet augmentation, tailored to residual pathology.Although advances in imaging, surgical techniques, and experience have improved outcomes, recurrent LAVV regurgitation continues to limit survival and freedom from reoperation. Early recognition and aggressive management remain essential, and the surgical threshold for reintervention should be low to optimize long-term results in this complex population.
Cardiac transplant represents the only definitive strategy for children with end-stage heart failure. Pediatric patients face significant wait-list times and resultant mortality due to a worsening shortage of hearts available for transplant. Donation after circulatory death (DCD) represents an opportunity to increase the number of hearts available to pediatric recipients by 30%. DCD donation has been historically limited in application due to logistical challenges and ethical concerns. A systematic review was carried out using the Cochrane Handbook. Eligible studies were identified using MEDLINE (via OVID), Embase (Elsevier), Cochrane Library/Cochrane Central Register of Controlled Trials (CENTRAL; Wiley), and Web of Science Core Collection (Clarivate). This search was conducted by a professional medical librarian in consultation with the author team and validated against a set of pre-selected articles. This resulted in 438 articles, which were screened based on title and abstract by 2 independent authors. Full-text review was subsequently performed, resulting in 21 articles. Agreement for inclusion included pediatric DCD, published in English, and complete manuscript availability. Four principal techniques were identified for pediatric DCD: rapid procurement and static cold storage, organ care systems, normothermic regional perfusion, and on-table reanimation. These all portend unique advantages and disadvantages and have largely evolved due to limitations of prior techniques. Providing a comprehensive approach to pediatric cardiac DCD is the most effective method to ensure organ stewardship, minimize wait list mortality, and mitigate ethical concerns associated with pediatric DCD.
In Fontan circulatory failure, heart or combined heart-liver transplantation (CHLT) is considered a final therapeutic option. Fontan patients present unique challenges for the cardiac and liver transplant teams. Improving outcomes of CHLT in Fontan patients requires a multidisciplinary team approach to patient selection, meticulous surgical planning, intraoperative and postoperative management, and continuous learning.
While the Ross procedure provides optimal clinical outcomes for young patients with aortic valve disease, the presence of preoperative aortic regurgitation (AR) with a dilated aortic annulus have long been recognized as independent predictors of early autograft failure. While this had led many to abandon the Ross procedure in this setting, we sought to specifically address the clinical and anatomic features that are unique to patients with severe AR, namely a dilated aortic annulus, aortic/pulmonary annular mismatch, ascending aortic dilatation and persistent hypertension early after surgery. Using a tailored surgical approach along with strict blood pressure control postoperatively, we believe that many of the concerns with the Ross procedure in this setting can be successfully mitigated. Importantly, these adjuncts do not compromise the dynamism of the autograft root, an important feature for the long-term benefits of the Ross procedure, especially in young and adolescent populations. In this article, we describe in detail our Ross technique in the setting of young patients with bicuspid AR and a dilated aortic annulus.
We explore the innovative use of atrial appendage tissue for creating pulmonary valves in children, focusing on its application in Tetralogy of Fallot repair. We discuss the background, preclinical studies, surgical approach, and early clinical outcomes, demonstrating the potential of this technique in reducing pulmonary insufficiency and improving surgical outcomes.
The complex heterogeneity inherent to reparative procedures for congenital heart defects and their relatively small volumes makes comprehensive analyses of outcomes particularly challenging. That said, an incisive understanding of the impact of patient- and disease-specific factors is crucial to improving overall outcomes. Datasets that reflect 'real world' contemporary practice of congenital cardiac care and provide collective outcomes data across age groups and institutions in various parts of the world play a vital role in filling this gap. Risk-adjusted benchmark outcomes data offer opportunities to improve the quality and value of care provided at the level of an individual patient, an institution or program, and the overall specialty. On-going enhancements to data collected and their analyses will ensure that the care we provide continues to evolve. Future efforts should be aimed at integrating multiple datasets to ensure access to longitudinal follow-up and effective analysis of long-term outcomes.
Congenital aortic and truncal valve diseases pose significant challenges due to their complex anatomy, diverse clinical presentations, and impact on long-term outcomes. Surgical management requires a tailored approach, balancing the need for immediate functional improvement with strategies to minimize reintervention, especially in growing children. This article outlines different surgical options to treat this pathology. Despite multiple choices, surgical decision-making remains complex, influenced by patient age, valve morphology, and comorbidities. Long-term outcomes depend on achieving a durable repair while preserving ventricular function and minimizing complications, emphasizing the need for multidisciplinary expertise in managing these challenging conditions.
