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Concept: Right atrium


Wilms' tumor (WT) is the most common pediatric renal tumor that often spreads to inferior vena cava and sometimes up to right atrium (RA). We describe successful management of 3-year-old child diagnosed with WT having extension up to RA. He was operated under cardiopulmonary bypass and extubated on postoperative day 2 and discharged. Perioperative anesthesia concerns were shock from dynamic tricuspid valve obstruction, intraoperative massive blood loss, and a higher risk of pulmonary thromboembolism during tumor manipulation.

Concepts: Oncology, Blood, Heart, Vein, Right atrium, Tricuspid valve, Wilms' tumor, Inferior vena cava


OPINION STATEMENT: Ebstein anomaly (EA) is a rare congenital heart defect that may not be detected until late in adolescence or adulthood. Since the original description in a 19-year-old laborer with severe tricuspid valve (TV) regurgitation in 1866, our understanding of this rare condition has increased to the recognition that it is an abnormality not only of the TV, but also of the right ventricle (RV). EA is the result of failure of delamination of the TV leaflets from the interventricular septum, resulting in adherence of the leaflets to the underlying myocardium. This results in a wide variety of abnormalities, including apical and posterior displacement of the dilated TV annulus; dilation of the “atrialized” portion of the RV; and fenestrations, redundancy, and tethering of the anterior leaflet of the TV. The malformed TV is usually regurgitant, but may rarely be stenotic. The clinical manifestations of EA in the adult depend on several factors, including the extent of TV leaflet distortion, degree of tricuspid regurgitation (TR), right atrial pressure, and presence of a right-to-left atrial level shunt. Over the past several decades, advances in diagnostic imaging and surgical techniques have contributed to our current management of this challenging congenital heart defect.

Concepts: Heart, Right ventricle, Ventricle, Right atrium, Tricuspid valve, Congenital heart defect, Congenital heart disease, Ebstein's anomaly


Due to the increased life expectancy and continual improvements in cardiological treatment options, diseases of the tricuspid valve, in particular tricuspid valve insufficiency will become increasingly more recognized as an interventional target. While tricuspid stenosis is rare and can be effectively treated with balloon valvuloplasty, no effective transcatheter approach to tricuspid regurgitation (TR) has yet been established. As the tricuspid annulus is a complex and highly dynamic structure that offers little resistance, orthotopic long-term fixation of transcatheter valves with the current techniques is challenging and has not yet been performed in human patients. Alternative treatment concepts include transcatheter caval valve implantation (CAVI) to address the regurgitation of blood into the caval veins, which has resulted in hemodynamic improvement and is currently undergoing further clinical investigation. Other interventional treatment concepts are aimed at tricuspid valve repair, e.g. by annular plication with the Mitralign™ device or the TriCinch™ system. In the medium-term it can be assumed that percutaneous systems and therapy options will become available for these indications whereby the functional and prognostic effects of these treatment procedures will be corroborated in the appropriate patient groups by corresponding studies.

Concepts: Heart, Right atrium, Tricuspid valve, Annulus, Abstraction, Tricuspid insufficiency, Regurgitation, Valvular heart disease


The EACVI/ASE/Industry Task Force to standardize deformation imaging prepared this consensus document to standardize definitions and techniques for using two-dimensional (2D) speckle tracking echocardiography (STE) to assess left atrial, right ventricular, and right atrial myocardial deformation. This document is intended for both the technical engineering community and the clinical community at large to provide guidance on selecting the functional parameters to measure and how to measure them using 2D STE.This document aims to represent a significant step forward in the collaboration between the scientific societies and the industry since technical specifications of the software packages designed to post-process echocardiographic datasets have been agreed and shared before their actual development. Hopefully, this will lead to more clinically oriented software packages which will be better tailored to clinical needs and will allow industry to save time and resources in their development.

Concepts: Heart, Echocardiography, Left-wing politics, Right atrium, Standardization, Clinical death, 2D computer graphics, Computer software


The acknowledgment of tricuspid regurgitation (TR) as a stand-alone and progressive entity, worsening the prognosis of patients whatever its aetiology, has led to renewed interest in the tricuspid-right ventricular complex. The tricuspid valve (TV) is a complex, dynamic and changing structure. As the TV is not easy to analyse, three-dimensional imaging, cardiac magnetic resonance imaging and computed tomography scans may add to two-dimensional transthoracic and transoesophageal echocardiographic data in the analysis of TR. Not only the severity of TR, but also its mechanisms, the mode of leaflet coaptation, the degree of tricuspid annulus enlargement and tenting, and the haemodynamic consequences for right atrial and right ventricular morphology and function have to be taken into account. TR is functional and is a satellite of left-sided heart disease and/or elevated pulmonary artery pressure most of the time; a particular form is characterized by TR worsening after left-sided valve surgery, which has been shown to impair patient prognosis. A better description of TV anatomy and function by multimodality imaging should help with the appropriate selection of patients who will benefit from either surgical TV repair/replacement or a percutaneous procedure for TR, especially among patients who are to undergo or have undergone primary left-sided valvular surgery.

Concepts: Blood, Heart, Medical imaging, Magnetic resonance imaging, Pulmonary artery, Right ventricle, Right atrium, Tricuspid valve


Nowadays some percutaneous options for tricuspid valve (TV) repair are available: Tricinch (4Tech Cardio, Galway, Ireland) mimicking the Kay procedure, Trialign (Mitralign, Boston, MA, USA) aiming to bicuspidise TV, MitraClip (Abbott Vascular, Abbott Park, Illinois, USA) mimicking Alfieri’s stitch, direct transcatheter annuloplasty with Cardioband (Valtech Cardio, Or Yehuda, Israel) and transcatheter Forma Repair (Edwards Lifesciences, Irvine, California, USA) providing a surface for leaflet coaptation. A multimodality imaging approach is fundamental for defining the pathophysiology of tricuspid regurgitation (TR), preprocedural planning and intraprocedural monitoring. Both 2-dimensional and 3-dimensional (3D) transthoracic echocardiography and transoesophageal echocardiography (TOE) are essential for grading and anatomical characterisation of TR, and evaluation of dimensions and function of right ventricle (RV) and estimation of pulmonary pressure. In particular, 3D echocardiography provides a better anatomical definition of TV apparatus and tricuspid annulus (TA) and additional information about the anatomical relationships of TV and surrounding structures. CT offers complementary information during the preprocedural planning especially for procedures targeting TA such as annular structure and dimensions, quality and amount of annular tissue and its relationship with the right coronary artery, and the sizing of the inferior vena cava. Moreover, appropriate patient selection is crucial. The best candidate seems to be a patient with functional TR due to predominant annular dilatation with modest apical tethering, at least partial preservation of leaflets coaptation, not severe pulmonary hypertension and not advanced RV dilation and dysfunction. An example of intraprocedural multimodality imaging approach with TOE, fluoroscopy, angiography and intracardiac echocardiography is also reported.

Concepts: Cardiology, Heart, Echocardiography, Right ventricle, Right atrium, Tricuspid valve, Inferior vena cava, Tricuspid insufficiency


Ablation of non-pulmonary vein (PV) triggers is an important step to improve outcomes in atrial fibrillation ablation. Non-pulmonary vein triggers typically originates from predictable sites (such as the left atrial posterior wall, superior vena cava, coronary sinus, interatrial septum, and crest terminalis), and these areas can be ablated either empirically or after observing significant ectopy (with or without drug challenge). In this review, we will focus on ablation of non-PV triggers, summarizing the existing evidence and our current approach for their mapping and ablation.

Concepts: Heart, Atrial fibrillation, Cardiovascular system, Right atrium, Inferior vena cava, Superior vena cava, Sinus venosus


Acute decompensated heart failure (ADHF) is often accompanied by liver congestion through increased right atrial pressure (RAP). Liver stiffness (LS) assessed non-invasively using transient elastography is related to increased RAP and liver congestion in patients with general HF. We investigated the relationship of LS with clinical and echocardiographic variables and outcomes in patients with ADHF.Methods and Results:The subjects were 105 patients with ADHF admitted to hospital between October 2016 and June 2017. Patients were divided into 2 groups based on median LS at admission (low LS <8.8 kPa [n=52] vs. high LS ≥8.8 kPa [n=53]). Death from cardiovascular disease and readmission for HF were primary endpoints. Total bilirubin and γ-glutamyl transpeptidase levels, MELD-XI score, diameters of the inferior vena cava and right ventricle, and severity of tricuspid regurgitation were greater in the high LS group (all P<0.05). During a median (interquartile range) follow-up period of 153 (83-231) days, cardiac events occurred in 29 patients (54%) in the high LS group and in 13 (25%) in the low LS group (P=0.001). After adjusting for variables that influence organ congestion, a high LS ≥8.8 kPa was still significantly associated with cardiac events (all P<0.05).

Concepts: Blood, Heart failure, Heart, Ventricle, Right atrium, Tricuspid valve, Inferior vena cava, Acute decompensated heart failure


Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.

Concepts: Blood, Heart, Artery, Vein, Right atrium, Inferior vena cava, Superior vena cava, Sinus venosus


Isolated persistent left superior vena cava (SVC) in the absence of right SVC is a rare congenital variant of thoracic venous drainage with the left subclavian and jugular veins that drain into the right atrium through the coronary sinus. Inferior vena cava interruption with azygos continuation is another congenital anomaly resulting in venous drainage of the lower extremities via a typically dilated azygos vein. Although both variants are generally asymptomatic and incidentally detected, these can have clinical implications in specific circumstances and in particular during device implantation. We report a case of pacemaker implantation in which both anatomical variants were present.

Concepts: Blood, Heart, Vein, Right atrium, Inferior vena cava, Superior vena cava, Small cardiac vein, Veins of the torso