搜索结果：Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual

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.

Technical skill development in congenital heart surgery (CHS) is challenging due to numerous factors which potentially limit the hands-on operative exposure in surgical training. These challenges have stimulated the growth of simulation-based training through the development of 3D-printed models, providing hands-on surgical training (HOST). From its inception in 2015, the models used in the HOST program have constantly improved, and now include valvar/subvalvar apparatus and better materials that mimic real tissue. Evidence shows that deliberate, regular simulation practice can improve a surgeon's technical skills across the spectrum of CHS. Furthermore, surgical trainees who undergo simulation training are able to translate this improved performance into the operative environment with improved patient outcomes. Despite evidence to support the incorporation of simulation methods into congenital training, its widespread adoption into training curricula remains low. This is due to numerous factors including funding, lack of dedicated time or proctorship and access to models-all of which can be overcome with the newer generation of models and committed trainers. Training programs should consider incorporating simulation-methods as a routine component of congenital training programs.

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.

Congenital coronary artery anomalies remain a leading cause of sudden cardiac death in the young. Within these, anomalous aortic origin of a coronary artery represents the largest group, with anomalies of the left coronary artery carrying higher risk for sudden cardiac arrest and clinical manifestations of myocardial ischemia. In contrast, anomalies of origin of the right coronary artery are more common and generally have a more benign clinical course, though rarely also associated with sudden events and myocardial ischemia. Risk stratification to guide management decisions remains to be well defined, though substantial advances have occurred in the last few years, with assessment of myocardial perfusion under provocative stress being an integral part of the evaluation in the young. Discussion and counseling on exercise behavior is essential to foster healthy lifestyle for these patients, acknowledging shared decision-making should be practiced in lieu of many unanswered questions as to outcomes long-term.

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.

Patients with many forms of congenital heart disease (CHD) and hypertrophic cardiomyopathy undergo surgical intervention to relieve left ventricular outflow tract obstruction (LVOTO). Cardiovascular Computed Tomography (CCT) defines the complex pathway from the ventricle to the outflow tract and can be visualized in 2D, 3D, and 4D (3D in motion) to help define the mechanism and physiologic significance of obstruction. Advanced cardiac visualization may aid in surgical planning to relieve obstruction in the left ventricular outflow tract, aortic or neo-aortic valve and the supravalvular space. CCT scanner technology has advanced to achieve submillimeter, isotropic spatial resolution, temporal resolution as low as 66 msec allowing high-resolution imaging even at the fast heart rates and small cardiac structures of pediatric patients ECG gating techniques allow radiation exposure to be targeted to a minimal portion of the cardiac cycle for anatomic imaging, and pulse modulation allows cine imaging with a fraction of radiation given during most of the cardiac cycle, thus reducing radiation dose. Scanning is performed in a single heartbeat or breath hold, minimizing the need for anesthesia or sedation, for which CHD patents are highest risk for an adverse event. Examples of visualization of complex left ventricular outflow tract obstruction in the subaortic, valvar and supravalvular space will be highlighted, illustrating the novel applications of CCT in this patient subset.

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 multidisciplinary perspective within this evolving and nuanced aspect of care for patients with SVCHD.

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.

The lack of donors that are age and size appropriate has compromised pediatric heart transplantation. There may be poor outcomes following mechanical circulatory support as a bridge to heart transplant in certain younger patient cohorts, which combined with the scarcity of donors, increases the morbidity and mortality in children waiting for a heart transplant. Transplantation of hearts donated after circulatory determination of death (DCDD) has been utilized to increase the pool of pediatric donor allografts. The original approach included direct procurement and perfusion in the recipient (DPP), and recently ex-situ normothermic machine perfusion (NMP). However, NMP is expensive, and this methodology does not allow for a functional assessment in a loaded state of the donor organ prior to transplantation. NMP is also not available to the smallest pediatric patients. An alternative method, normothermic regional perfusion (NRP), can overcome the limitations of NMP. NRP is a thoraco-abdominal dynamic in-situ organ assessment method and is instituted following declaration after determination of circulatory death. In-situ tissue perfusion is established to the transplantable organs using a bypass circuit based on strict, prespecified guidelines that do not violate the "dead donor rule." The first pediatric DCDD heart transplant using NRP was performed in 2019, and since then, several centers have reported immense success. Ethical concerns for cerebral reperfusion have not been substantiated by clinical data. Utilization of DCDD donors can vastly expand the donor pool in congenital heart disease (CHD).

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.

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 postsurgical, 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 multidisciplinary 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.

Mitral valve repair is often preferred over replacement due to the preservation of native valve function, allowing for somatic growth and resulting in fewer complications and reoperations. Although there are no randomized trials comparing repair and replacement specifically in this age group, retrospective studies still indicate better long-term survival and lower reoperation rates with repair. While neonates and infants experience higher mortality rates compared to older children, freedom from re-intervention is durable in the survivors and matches that of older children. Especially compression suture annuloplasty and chordal replacement using Gore-Tex sutures have shown favorable outcomes, but the rarity of the condition makes it challenging to train new surgeons. Utilizing detailed video documentation could help bridge this educational gap. While advancements in materials for valve repair in neonates and infants have been slow, emerging options like new materials that grow with the patient offer potential for future innovation. In complex cases such as Shone's syndrome, new techniques, including an-bloc replacement of the aorta and mitral valve, may improve outcomes.

The borderline left ventricle (LV) encompasses a heterogenous group of cardiac defects that result in underdevelopment of the left heart. Data supporting decision making is difficult to interpret because borderline LV hypoplasia is a relatively rare disease comprising of a heterogenous morphologic spectrum with data originating from single-institution retrospective studies that have all used varying inclusion criteria and imaging modalities/analysis methods, whilst further confounded by heterogenous institutional practice patterns and era effects. Long-term data is lacking. This invited expert review offers a perspective on how to interpret and use some of the preoperative imaging parameters commonly proposed to decide between primary biventricular repair (BVR) versus LV recruitment. The need to integrate functional cardiovascular magnetic resonance imaging (CMR) parameters is emphasized. Current approaches and a broad framework with imaging criteria are presented.

The relationship between surgical volume and clinical outcomes in congenital heart surgery has been frequently studied and interpreted to indicate that higher volume programs are associated with superior outcomes after congenital heart surgery. Volume-based assumptions have been used to support the notion that volume-specific stratification of case mix would improve overall outcomes in the United States with specific attention to programs with annualized volumes of 75-200 STS index cases/year. Although not intended, some have perceived these recommendations to indicate that programs performing 75-200 annualized index cases/year offer surgical outcomes of lesser quality than higher volume programs. Nevertheless, some programs performing 75-200 annualized index cases/year consistently perform exceptionally well. Because the inherent advantages - and challenges - associated with being a program in this size range have not been frequently examined, this manuscript focuses on describing these attributes. We hypothesize that better understanding of the determinants of high (or low) performance in this size range could have significant impact on delivery of care in the United States.

The so-called Commando procedure, initially described by David and colleagues, consists in the reconstruction of the mitro-aortic fibrous lamina by a patch that enlarges both annuli. Its use has been described to upsize the aortic and mitral annulus for double valve replacement in adolescents. We describe a modified technique of this reconstruction of the fibrous skeleton of the heart, combined with Konno procedure to further enlarge the aortic annulus. In modified Commando procedure, following the reconstruction of aortomitral continuity with a bovine pericardium CardioCel patch (Admedus Regen Pty Ltd, Perth, WA, Australia), an aortic valved conduit that was made on the bench in order to have bottom skirt that enabled the suturing of the composite conduit far inside the left ventricle outflow tract. Coronary buttons were implanted at the supra-commissural level. The advantages of this modified Commando procedure are (1) the creation of a new aortic annulus when the integrity of this annulus has been compromised, (2) the upsizing of both annuli to any possible size of aortic and mitral prostheses, and (3) the relief of any residual left ventricular outflow tract obstruction.

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.

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.

The Ross procedure is an excellent option for aortic valve replacement resulting in outstanding hemodynamic performance and the ability to avoid systemic anticoagulation. The long-term durability of the autograft is generally good but concerns for later aortic root dilation with ensuing neoaortic insufficiency have prompted efforts to stabilize the autograft, root, sinuses and Sino-tubular junction in order to delay or entirely avoid late reinterventions on the neoaortic root. We have employed an inclusion technique, supporting the Auto-graft in a Terumo Gelweave™ Valsalva graft. We performed a retrospective study of all 129 patients undergoing the Ross procedure from 1992 to 2019 at Children's Wisconsin. Fifty-one underwent the supported Ross (SR) and 78 underwent unsupported Ross (UR). Structured clinical data was collected and echocardiograms were reviewed. Median follow-up was 4.9 years (up to 22.6 years) for UR patients and 3.6 years (up to 11.4 years) for SR patients. In order to provide a fair comparison, we sub -analyzed patients aged 10 to 18 years who underwent the Ross procedure, 16 who underwent the UR and 18 patients who underwent the SR. Change in aortic annulus diameter (P = 0.002), aortic sinus diameter (P = 0.001) change in left ventricular function (P = 0.039) and change in aortic insufficiency (P = 0.008) were all worse in UR. The SR is simple, reproducible, and predictable. It seems to prevent change in annulus diameter, sinus diameter and to reduce late neoaortic insufficiency. Longer follow-up with a larger group of patients is required to draw definitive conclusions.

Septal myectomy is indicated in patients with obstructive hypertrophic cardiomyopathy (HCM) who have persistent symptoms despite medical therapy, intolerance of medication side effects, or severe resting or provocable gradients. Septal myectomy at high volume centers is safe, with low operative mortality (1%) and low rates of complications such as complete heart block or ventricular septal defect (3% and 0.5%, respectively). Additionally, improved survival following myectomy has been observed when compared to patients with obstructive HCM managed medically or those with nonobstructive HCM. As a longstanding, quaternary referral center for septal myectomy, our institution has built significant experience and expertise in the surgical and medical management of HCM, including atypical HCM, defined as preadolescent patients, those with mitral valve disease, and those with isolated midventricular obstruction. The most important factor of septal myectomy in achieving complete resolution of obstruction and avoiding recurrence is the apical extent of the myectomy trough, which must extend to the septum opposite the papillary muscles. If this cannot be fully achieved via a transaortic exposure, especially in preadolescents and patients with midventricular obstruction, then a transapical approach may be needed. Mitral valve repair is rarely necessary as SAM-mediated MR resolves with adequate myectomy alone, but mitral repair is performed in cases of intrinsic valvular disease. In this manuscript we provide a summary of current operative techniques and outcomes data from our institution on the management of these various categories of HCM.