Right aortic arch with aberrant left subclavian artery (RAA-ALSA) is one of the most common complete vascular rings encountered in pediatric cardiovascular practice. The associated Kommerell diverticulum (KD), a remnant of the primitive fourth dorsal aortic arch from which the aberrant subclavian artery arises, forms part of the posterior ring and may contribute importantly to persistent airway and esophageal compression. Historically, repair consisted of division of the ligamentum arteriosum. Recognition that posterior compression can persist after ligamentum division has led many surgeons to add diverticulum resection and transfer or reimplantation of the aberrant subclavian artery. The optimal strategy remains controversial. Advocates of routine KD resection cite persistent compression after simple ring division, the low morbidity of resection in experienced centers, and abnormal diverticular histopathology that may imply future aneurysmal risk. Opponents emphasize that most children do well after less extensive repair that persistent symptoms frequently reflect associated airway disease rather than residual vascular compression, and that evidence proving superiority of universal resection is lacking. This review summarizes the embryology, anatomy, pathophysiology, and surgical management of KD in RAA-ALSA; critically examines evidence for routine and selective resection; and proposes a practical anatomy-driven approach.
Background Total anomalous pulmonary venous connection (TAPVC) remains a complex congenital cardiac malformation. Despite improved survival, infracardiac and mixed variants continue to carry disproportionate risks of early mortality, postoperative pulmonary venous obstruction (PVO), and reintervention. Methods This narrative review examines the anatomic and technical challenges of infracardiac and mixed TAPVC, mechanisms and consequences of postoperative PVO, and contemporary strategies to reduce residual or recurrent obstruction, with particular emphasis on primary sutureless repair and anatomy-specific surgical approaches. Results Contemporary operative mortality for TAPVC is approximately 3–10% but remains higher in patients with preoperative obstruction. Postoperative PVO occurs in approximately 5–15% and is an important determinant of reintervention and late mortality. In infracardiac TAPVC, obstruction may occur along with the descending vertical vein, producing severe pulmonary venous hypertension and physiologic instability. Mixed TAPVC presents additional challenges from heterogeneous drainage, multiple or small confluences, isolated veins, and complex geometry. Primary sutureless repair has evolved from treatment of recurrent PVO to a preventive strategy in high-risk primary TAPVC by avoiding pulmonary venous suture lines and facilitating wide, unobstructed drainage. Conclusions Management of infracardiac and mixed TAPVC requires an anatomy-specific approach focused on preventing postoperative PVO. Important strategies include selective preoperative stabilization, primary sutureless reconstruction in high-risk anatomy, a no-touch approach to selected isolated veins in 3 + 1 mixed TAPVC, and complete reconstruction of the cardiac component when appropriate.
Ebstein anomaly is a rare congenital malformation of the tricuspid valve and right ventricle characterized by marked heterogeneity in anatomy, physiology, and clinical presentation. This broad phenotypic spectrum strongly influences the natural history of the disease, the timing and type of intervention, and ultimately long-term outcomes. Patients range from critically ill neonates with severe hemodynamic compromise and circular shunts to asymptomatic children and adults with progressive tricuspid regurgitation, right ventricular dysfunction, or arrhythmias, as well as survivors of previous surgical interventions. Accordingly, long-term outcomes are best understood within the context of the presenting phenotype and its associated management pathway rather than as a single aggregate measure of survival. Contemporary management therefore requires phenotype-specific decision-making. This review summarizes long-term outcomes across the major clinical and therapeutic subgroups, including critically ill neonates managed with the Starnes procedure, stable neonates and infants undergoing biventricular repair, patients treated with historical repair techniques, contemporary cone reconstruction, and tricuspid valve replacement. Particular emphasis is placed on transplant-free survival, valve durability, ventricular remodeling, arrhythmia burden, functional status, quality of life, and the need for reintervention.
BackgroundPostoperative pulmonary complications (PPCs) are common after thoracic surgery performed with one-lung ventilation (OLV). Although risk scores such as ARISCAT, ASA physical status, and LAS VEGAS are used to predict PPC in general surgery, their performance in thoracic surgery remains unclear. This study aimed to evaluate the ability of these scores to predict PPCs.MethodsThis prospective observational study included 115 adult patients undergoing noncardiac thoracic surgery requiring OLV. The primary outcome was the incidence of in-hospital PPCs within 30 days. We assessed the discriminatory ability of risk scores using receiver operating characteristic curve analysis (area under the curve (AUC)) and identified independent predictors with multivariable logistic regression.ResultsPPCs occurred in 43 patients (37.4%). The LAS VEGAS score demonstrated the best discriminatory performance (AUC = 0.648, p = .005), followed by ARISCAT (AUC = 0.598, p = .067) and ASA (AUC = 0.562, p = .123). Independent predictors of PPCs were preoperative hemoglobin (OR 0.77, 95% CI 0.62-0.95) and intraoperative noradrenaline use (OR 5.93, 95% CI 2.36-15.95). A combined model including these two variables achieved an AUC of 0.772, which was significantly superior to the individual scores.ConclusionsThe ARISCAT, ASA, and LAS VEGAS scores showed only weak-to-moderate predictive performance for PPCs after thoracic surgery with OLV. These findings do not support their routine use in this setting and highlight the need for thoracic surgery-specific risk prediction tools.
BackgroundAortic stenosis is traditionally categorised as degenerative calcific, congenital bicuspid, or rheumatic. Contemporary molecular biology, multimodality imaging, and transcatheter valve therapy demonstrate that aortic stenosis represents a spectrum of mechanistically distinct diseases converging upon a shared haemodynamic endpoint. A four-category pathobiologic classification is proposed linking disease mechanism to operative strategy and procedural risk stratification.MethodsA narrative mechanistic review was performed using contemporary international guidelines, foundational biological studies, and key clinical series. Pathophysiology, characteristic imaging phenotype, and procedural implications were integrated into a clinically applicable framework for use by multidisciplinary heart valve teams.ResultsAortic stenosis arises via common downstream pathways - valve interstitial cell activation, inflammation, extracellular matrix remodelling, and mineralisation - through mechanistically distinct initiating processes. Four phenotypes are identified: (a) congenital structural-biomechanical disease; (b) fibro-calcific inflammatory-osteogenic disease; (c) autoimmune/inflammatory valvulitis; and (d) metabolic/mineralisation-associated disease. Each demonstrates characteristic anatomical morphology, rate of haemodynamic progression, and differential suitability for transcatheter or surgical intervention.ConclusionsAortic stenosis is a final common phenotype of multiple distinct biological processes. A pathobiologic classification improves procedural planning, risk stratification, and therapeutic selection in the era of transcatheter valve therapy, and supports precision-based individualised patient management.
