Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.

Abnormal congenital aorto-cardiac communications (CACC) are a heterogeneous constellation of anomalies that provide an abnormal connection between the aorta and other cardiac chambers or structures, including the atria, ventricles, the main pulmonary artery, and the coronary sinus. The current terminology of CACC has significant errors and shortcomings including inconsistent and interchangeable use of terms of fistula and tunnel and lack of an inclusive classification with practical information on therapeutic management. The aims of this study were threefold: firstly, to perform a concise narrative review of congenital pathologic connections between the aortic root and cardiac chambers which include rupture of congenital sinus of Valsalva aneurysm, aorto-left ventricular and less commonly right ventricular tunnels, coronary cameral fistulas, and aorto-atrial communications; secondly, to investigate the differentiating features of the so-called aorta right atrial tunnel (ARAT), with and without coronary artery take-off from the tunnel, and coronary cameral fistula (CCF) by applying a differential diagnostic assistance toolbox to two groups of patients with ARAT and CCF; and lastly, to propose a practical and inclusive anatomic-therapeutic classification for CACCs.

Arrhythmogenic calmodulin E105A mutation alters cardiac RyR2 regulation leading to cardiac dysfunction in zebrafish.

Calmodulin (CaM) is a universal calcium (Ca )-binding messenger that regulates many vital cellular events. In cardiac muscle, CaM associates with ryanodine receptor 2 (RyR2) and regulates excitation-contraction coupling. Mutations in human genes CALM1, CALM2, and CALM3 have been associated with life-threatening heart disorders, such as long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia.

A randomized, controlled, multi-center trial of the efficacy and safety of the Occlutech Figulla Flex-II Occluder compared to the Amplatzer Septal Occluder for transcatheter closure of secundum atrial septal defects.

Aims: The aim of this study was to compare the efficacy and safety of the Occlutech Figulla Flex II Occluder (OFFII) with the Amplatzer Septal Occluder (ASO) in patients > 8kg undergoing transcatheter ASD closure.

Methods And Results: Randomized, controlled, multi-center prospective clinical trial with randomization 2:1 in favor of the OFFII. Primary efficacy endpoint was the rate of successful device placement and defect closure without major complications at hospital discharge.

3-Year Outcomes of the Edwards SAPIEN Transcatheter Heart Valve for Conduit Failure in the Pulmonary Position From the COMPASSION Multicenter Clinical Trial.

Objectives: This study provides the 3-year follow-up results of the COMPASSION (Congenital Multicenter Trial of Pulmonic Valve Regurgitation Studying the SAPIEN Transcatheter Heart Valve) trial. Patients with moderate to severe pulmonary regurgitation and/or right ventricular outflow tract conduit obstruction were implanted with the SAPIEN transcatheter heart valve (THV).

Background: Early safety and efficacy of the Edwards SAPIEN THV in the pulmonary position have been established through a multicenter clinical trial.

Pitfalls of stenting coarctation of an angulated right circumflex aortic arch in Goldenhar syndrome.

We report stenting of coarctation of an angulated right circumflex aortic arch (RCAA) using four Cheatham Platinum stents in a child with Goldenhar syndrome. Difficulties in measuring the accurate length of the curved and narrowed transverse aortic arch marked discrepancy between the luminal diameters of the long narrow transverse arch and wide descending thoracic aorta, increased displacement force caused by the 90° bend between the two parts resulted in repeated stent migrations. We discuss the tips to avoid distal stent migration in the setting of an angulated RCAA.

Rare and low-frequency coding variants alter human adult height.

Height is a highly heritable, classic polygenic trait with approximately 700 common associated variants identified through genome-wide association studies so far. Here, we report 83 height-associated coding variants with lower minor-allele frequencies (in the range of 0.1-4.

Percutaneous mitral valve repair: The MitraClip device.

Chronic mitral regurgitation (MR) is the most common cardiac valvular disease with more than 4 million people in the United States alone suffering from moderate or severe MR. Left untreated, chronic MR results in serious consequences. Surgical correction with mitral valve repair or replacement remains the mainstay of therapy for MR.

Patent Foramen Ovale: Stroke and Device Closure.

Patent foramen ovale (PFO) is a common finding in healthy adults and has long been implicated in cryptogenic stroke. The pathogenesis is hypothesized to be caused by microemboli gaining access into the systemic circulation via a PFO. Proposed treatment options include medical therapy and/or PFO closure.

Current management of coarctation of the aorta.

Coarctation of the aorta (C) is the sixth most common lesion in congenital heart disease and represents a spectrum of aortic narrowing that varies from a discrete entity to tubular hypoplasia. This condition was once thought to be a relatively simple lesion that would be "cured" upon repair of the narrowing, however, despite relief of the anatomical obstruction the subsequent risk of early morbidity and death persists. This review outlines the optimal management strategy of this disease from neonatal to adult life and provides insights to approach this straightforward but challenging condition.

TAVR update: Contemporary data from the UK TAVI and US TVT registries.

The introduction of transcatheter aortic valve replacement (TAVR in US - TAVI in Europe) has resulted in a paradigm shift in the treatment of patients with severe aortic stenosis. Although three randomized trials and multiple single-center and multicenter registry studies have been published, the profile and longer-term outcome of patients undergoing transcatheter aortic valve replacement (TAVR) in patients with severe aortic stenosis have been limited. The recently published reports from the United Kingdom (UK)(1) and United States (US)(2) TAVR registries add tremendously to the currently available literature.

Transitional Hemodynamics in Preterm Neonates: Clinical Relevance.

Background: Each newborn enters this world facing tremendous respiratory, hemodynamic and neuroendocrine challenges while going through drastic physiological changes during the process of adaption from fetal to postnatal life. Even though the vast majority of term infants transition smoothly without apparent consequences, this task becomes increasingly arduous for the extremely preterm infant.

Methods & Results: This article reviews the physiology and pathophysiology of cardiovascular adaptation of the very preterm neonate.

Transcatheter pulmonary valve replacement: State of the art.

Background: The number of patients undergoing transcatheter pulmonary valve replacement has been steadily increasing, resulting in many research projects focusing on this patient population. This has highlighted the need to summarize the currently available data.

Methods: A review of the literature was conducted and the results summarized.