Multimodality Approach to a Complex Scimitar Syndrome: How Advanced Diagnostics Can Guide Therapeutic Strategies.

We report an unusual association of scimitar syndrome with right diaphragmatic hernia, left-sided aortic arch with "aberrant right subclavian artery" in a 2-year-old boy who underwent stepwise transcatheter occlusion of a significant aortopulmonary collateral followed by surgical treatment for the repair of the diaphragmatic hernia and esophageal compression. ().

Transcatheter Treatment of "Complex" Aortic Coarctation Guided by Printed 3D Model.

The transcatheter approach is nowadays considered a cost-effective alternative to surgery in adults with "complex" aortic coarctation. The printed 3D model was crucial in planning transcatheter treatment of a complex case of postsurgical aortic re-coarctation, due to coexistence of transverse aortic arch stenosis and pseudoaneurysm as well as aneurysm of the descending aorta. ().

Neo-aortic posterior sinus of Valsalva reduction and closed coronary artery transfer as a method to approach aortopulmonary mismatch in transposition of great arteries.

Objective: In transposition of great arteries (TGA), aortopulmonary mismatch (APM) can determine postoperative neo-aortic insufficiency after arterial switch operation (ASO). The distortion of sinu-tubular junction may be the geometric mechanism responsible. We developed a strategy able to reduce the mismatch at the timing of ASO, and in this study, we aimed to describe our indications and results.

The impact of native Fallot anatomy on future therapeutic requirements and outcomes at follow-up.

Background: In patients with repaired Fallot, subsequent surgical or interventional procedures and adverse cardiac events are frequent. We aimed to evaluate the impact of a simple pre-operative anatomic classification based on the size of the pulmonary valve (PV) annulus and branches on future therapeutic requirements and outcomes.

Method: This is a single-center retrospective analysis of patients operated for Fallot before the age of 2 years, from January 1990.

Pitfalls in surgical repair of Fallot's tetralogy: The "ventricular subaortic pouch".

Fallot-type ventricular septal defect (VSD) is characterized by anterior malalignment of the outlet septum with variable degree of aortic dextroposition. Correct identification of the borders of the defect is crucial to achieve an optimal surgical repair. We describe a particular postsurgical lesion, identified by cardiac magnetic resonance and cardiac computed tomography, which results from an improper surgical closure technique of anterior malaligned VSD.

Cardiac CT angiography: normal and pathological anatomical features-a narrative review.

The normal and pathological anatomy of the heart and coronary arteries are nowadays widely developed topics and constitute a fundamental part of the cultural background of the radiologist. The introduction of cardiac ECG-gated synchronized CT scanners with an ever-increasing number of detectors and with increasingly high structural characteristics (increase in temporal resolution, increase in contrast resolution with dual-source, dual energy scanners) allows the virtual measurement of anatomical structures complying with heart rate with submillimetric precision permitting to clearly depict the normal anatomy and follow the pathologic temporal evolution. Accordingly to these considerations, cardiac computed tomography angiography (CCTA) asserts itself as a gold standard method for the anatomical evaluation of the heart and permits to evaluate, verify, measure and characterize structural pathological alterations of both congenital and acquired degenerative diseases.

The different surgical impact of the superior cavoatrial incision in children and adults.

Introduction: The single- and double-patch repairs are undoubtedly the most commonly used techniques for the surgical management of partial anomalous pulmonary venous connection associated with sinus venosus atrial septal defect. The aim of this study was to retrospectively compare early and long-term surgical outcomes in paediatric and adult patients, focusing in particular on the occurrence of ectopic atrial rhythm.

Material And Methods: Seventy patients (male: 38, 54.

Impact of right atrium dimension on adverse outcome after pulmonary valve replacement in repaired Tetralogy of Fallot patients.

The hemodynamic impact of residual pulmonary regurgitation (PR) in repaired Tetralogy of Fallot (rTOF) has been well demonstrated. However, markers driving the decision making process to indicate the ideal timing of pulmonary valve replacement (PVR) are still uncertain. Furthermore, very few studies have included the right atrium (RA) dilatation as a preoperative risk factor for post-PVR clinical adverse outcome.

Retrosternal Clots After Fontan Surgery by Systematic Evaluation With Transthoracic Ultrasound.

Objectives: Bleeding is a common, serious, and often subtle complication after total cavopulmonary connection surgery. The aim of the present study was to assess the incidence of retrosternal clots after surgery, which were searched for systematically with transthoracic ultrasound.

Design: Retrospective study.

Native Aortic Arch Interruption in Adulthood: Surgical Management.

Aortic coarctations in adults are mainly represented by recurrent critical narrowing at the site of previous surgical correction, or less frequently by native forms of complex obstructive malformations of the distal arch and isthmus. We present our experience with an unusual form of native adult aortic coarctation presenting as a complete interruption of the aortic arch.

Coronary pathology of inherited generalized arterial calcification of infancy: a case report.

Generalized arterial calcification of infancy (GACI), or idiopathic infantile arterial calcification, is a rare autosomal-recessive disease recognized aAs an inherited disorder characterized by severe pathologic calcification of large- and medium-sized arteries accompanied by smooth muscle cell (SMC) hyperplasia leading to vascular obstruction [1]. The prognosis is extremely poor, with 85% of affected infants dying within the first 6 months of life. Loss-of-function mutations in the ectonucleotide pyrophosphatase phosphodiesterase 1 (ENPP1) gene is recognized as the main defect associated with GACI [1].

An alternative cardiopulmonary bypass strategy for intracaval baffle repair of scimitar syndrome.

Intracaval buffle repair of scimitar syndrome is classically performed under deep hypothermic circulatory arrest or using low-flow modalities of cardiopulmonary bypass with peripheral cannulation. We propose an alternative perfusion approach to the procedure using total intrapericardial cannulation under full-flow normothermic cardiopulmonary bypass.

Coronary Artery Involvement of Williams Syndrome in Infants and Surgical Revascularization Strategy.

Williams syndrome (WS) is a genetic disorder due to deficiency of elastin gene expression. It is characterized by typical somatic abnormalities and a wide range of cardiovascular malformations. Coronary artery involvement is a frequent finding of the syndrome, particularly in those patients with severe supravalvular aortic stenosis.

Palliative Arterial Switch for Congenitally Corrected Transposition of the Great Arteries with Ventricular Septal Defect and Subaortic Hypoplasia.

We present a case of palliative arterial switch with aortic arch reconstruction performed as the first stage of anatomical correction in a patient with congenitally corrected transposition of great arteries (ccTGA), ventricular septal defect (VSD), duct-dependent aortic coartation, and hypoplasia of the ascending aorta and subaortic tract.

Complete rightward cardiac luxation caused due to left tension pneumothorax.

We present a 4-year-old patient with severe rightward cardiac dislocation due to left tension pneumothorax after surgical closure of a perimembranous ventricular septal defect. The chest roentgenograms showed the "dextrocardia-like" appearance of the cardiac silhouette. Given the persistent severe cardiac dislocation despite resolution of the pneumothorax, and considering the patient's ongoing hemodynamic instability, we decided to reopen the chest to return the heart to its natural position.

Congenital mitral disease: anomalous mitral arcade in a young man.

We present the case of a 33-year-old man referred to our institution with a diagnosis of severe mitral valvular stenosis and insufficiency. We realized the valvular disease was due to an "anomalous mitral arcade," a rare congenital malformation of the mitral tensor apparatus characterized by enlarged papillary muscles connected to mitral leaflets by a typical fibrous tissue bridge. This arrangement creates a fibrous continuity between valvular and subvalvular apparatus.

Echocardiographic Assessment of Ebstein's Anomaly in a 60-Year-Old Man.

We present an echocardiographic evaluation of an elderly man affected with Ebstein's anomaly. In the natural history of this congenital disease only 5% of patients survive beyond the fifth decade. The patient presented severe right heart failure and he was refered to our institution for heart transplantation.