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Objectives: Surgical management of aortic arch hypoplasia (AAH) with associated intracardiac anomalies is a challenge in newborns. We reviewed the characteristics and outcomes of neonates and infants who underwent pulmonary artery banding concomitant to arch repair and single-stage total repair at our institution., Methods: Medical records of 60 patients undergoing aortic arch reconstruction for AAH from 2014 to 2019 were retrospectively reviewed. Twenty-five patients were female (41.6%), and the age of the patients ranged from 4 to 120 days (median, 19.5 days). The patients were divided into two groups: Group 1 (23 patients) underwent pulmonary artery banding concomitant to arch repair, and Group 2 (37 patients) underwent single-stage total repair in addition to arch repair. All arch repair procedures consisted of an extended (to the midportion of the ascending aorta) patch aortoplasty., Results: Postoperative early mortality occurred in 12 patients, eight in Group 1 (34.8%) and four in Group 2 (10.8%). There was an early survival advantage in Group 2 (p = .019). Recoarctation occurred in 13 cases (21.6%), and 11 (18.3%) of them required reintervention (balloon angioplasty: 7, reoperation: 4). On univariate analysis, risk factors associated with death were pulmonary artery banding (hazard ratio [HR], 0.44; confidence interval [CI], 0.09-2; p = .019), prematurity (HR, 4.67; CI, 1.34-16.18; p = <.001), preoperative mechanical ventilation support requirement (HR, 0.048; CI, 0.52-6.39; p = .048), and functional single ventricle (HR, 0.43; CI, 0.1-1.86; p = .006). The mean duration of follow-up was 21.9 ± 15.1 months, and there was no late death in either group., Conclusion: Single-stage repair of AAH with intracardiac pathologies has better results than palliation, according to survival rates and postoperative results. The use of the patch augmentation technique in AAH is valid and associated with an acceptable incidence of recurrent arch obstruction., (© 2020 Wiley Periodicals LLC.)

Background and Aim: To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re‐interventions, for the rare instances of transposition physiology with concordant ventriculo‐arterial connections. Methods: We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo‐arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Results: Among reported cases, just over two‐thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four‐fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one‐fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multistaged. Overall mortality was 25%, with one‐fifth of patients requiring pacemakers for surgically‐induced heart block. The majority of survivors were in good functional state. Conclusions: The flow patterns produced by discordant atrioventricular and concordant ventriculo‐arterial connections remain an important, albeit rare, indication for atrial redirection or hemi‐Mustard's procedure with bidirectional Glenn. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long‐term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits. [ABSTRACT FROM AUTHOR]

Retrograde device closure of perimembranous ventricular septal defects (pmVSDs) is an interesting procedure with several technical advantages and encouraging follow‐up results. Nevertheless, aortic valve injuries may occur and require sufficient attention. Herein, we report the first two cases of severe aortic regurgitation that we have linked to the retrograde pmVSD closure with the KONAR‐MF™ VSD occluder. We detail the technical aspects, comprehensively discuss procedural errors and conclude with important learning points. [ABSTRACT FROM AUTHOR]

Background: The interatrial communication, one of the most frequent congenital heart defects, represents an important intracardiac shunt between systemic and pulmonary circulations. Direction and magnitude of the interatrial shunting depends upon several features, including defect size, shape and location, pressure difference between right and left atrium, and difference in right and left ventricular compliance. Methods: In this review article, the presence or absence of interatrial communication, and its role, have been analyzed, as they can have a critical impact on the cardiovascular physiopathology, and the interatrial communication can prove to be either clinically harmful, useful or indispensable. Accordingly, the utility and role of the interatrial communication in modern congenital, pediatric and adult, disease has evolved, with modification of the indications to close, maintain patency, or create an interatrial communication. Results: The interatrial communication and shunting can be manipulated to maximize the oxygen delivery to the tissues, accordingly with the underlying congenital heart defect. While not always relevant to patients with bi‐ventricular circulations, this becomes extremely important in children and adults with complex congenital heart defects. Conclusions: With improving long‐term survival for the vast majority of congenital heart patients, an advanced understanding of the role and utility of the interatrial communication, and of all the possibilities of its manipulation, is essential to improve the patient outcomes. [ABSTRACT FROM AUTHOR]

Background: Absent pulmonary valve (PV) is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot (TOF). Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. Discussion: Here, we describe the case of a 37 year old gentleman who presented with a diagnosis of TOF with absent PV, hugely dilated right and left pulmonary arteries and an aneurysmal right ventricular outflow. Conclusion: TOF with absent PV surviving into adulthood is a rarity. An aneurysmal right ventricular outflow tract in an unrepaired case of TOF has not been described before. Massively dilated left and right pulmonary arteries as in this case can cause significant and even life‐threatening airway compromise, thus necessitating early surgical repair. [ABSTRACT FROM AUTHOR]