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Objectives We sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA). Background To date there is no robust ri...

Mitral annular disjunction (MAD) is an abnormal insertion of the hinge line of the posterior mitral leaflet on the atrial wall: the mitral annulus shows a separation or "disjunction" between the leaflet-atrial wall junction and the crest of the left ventricle myocardium. This anomaly is often observed in patients with myxomatous mitral valve prolapse. The anatomical substrate of MAD remains unclear for the following reasons: (1) most studies are focused on the association between MAD and arrhythmias, rather than on pathomorphological aspects of MAD; and (2) the complex anatomic architecture of the posterior mitral annulus is often simply described as the posterior segment of a fibrous ring. The aims of this paper are to review the pertinent normal anatomy of the mitral valve and to propose new hypotheses on the morphological nature of MAD.

Hearts with double outlet ventricles and concordant atrioventricular connections account for about 1%–3% of all cases of congenital heart disease. We review hearts with two ventricles and concordant atrioventricular connections with double outlet right ventricle (DORV), double outlet left ventricle (DOLV) and double outlet both ventricles (DOBV) from the morphological and clinical imaging perspectives. These hearts are a heterogeneous group of congenital cardiac malformations with a wide range of pathophysiologies that require an individualised surgical approach based on a precise understanding of the complex cardiovascular anatomy. Owing to their differing temporal, spatial and contrast resolutions, we propose that multimodality imaging provides optimal characterisation of various intracardiac morphological features of double outlet hearts. This approach aids clinical diagnosis for optimising treatment options across these malformations.

Bei haufiger rechtsventrikularer apikaler Schrittmacherstimulation kann eine Einschrankung der linksventrikularen Pumpfunktion mit dem klinischen Bild einer Herzinsuffizienz resultieren, mit zusatzlichen moglichen Konsequenzen bis hin zu erhohter Mortalitat. Dies fuhrte zu dem Bestreben, alternative Stimulationsorte zu identifizieren, die eine physiologischere Stimulation erlauben. Als attraktive Alternative wird heutzutage das His-Bundel(HB)-Pacing diskutiert, bei dem durch direkte Stimulation das physiologische Reizleitungssystem genutzt werden kann und so eine intrinsische Uberleitung mit physiologischer Kammerkontraktion moglich wird. Das Aufsuchen und Identifizieren des His-Bundels stellt fur den Implanteur eine Herausforderung dar. Erforderlich fur das endokardiale Mapping ist eine exakte Kenntnis der anatomischen Orientierungspunkte sowie die Fahigkeit zur Beurteilung lokaler Elektrogramme. Ziel dieses Ubersichtsartikels ist die Beschreibung der anatomischen Landmarken, die helfen sollen, diese wichtigen Zielstrukturen aufzufinden.

For many years, the left atrial appendage (LAA) was considered a dormant embryological remnant; however, it is a structurally complex and functional organ that contributes to cardiac haemodynamic changes and volume homeostasis through both its contractile properties and neurohormonal peptide secretion. When dysfunctional, the LAA contributes to thrombogenesis and subsequent increased predisposition to cardioembolic events. Consequently, the LAA has gained much attention as a therapeutic target to lower this risk. In addition, attention has focused on the LAA in its role as an electrical trigger for atrial tachycardia and atrial fibrillation with ablation of the LAA to achieve electrical isolation showing promising results in the maintenance of sinus rhythm. This in-depth review explores the structure, physiology and pathophysiology of the LAA, as well as LAA intervention and their sequelae.

The mitral annulus (MA) is not a continuous ring of connective tissue from which are suspended mitral leaflets. Instead, it is a much more complex structure made up of a mix of fibrous, muscular, and adipose tissues. MA is a key structure in any type of mitral valve repair and recently it has been targeted for transcutaneous devices. Thus, a deep understanding of MA anatomy has never been more important. Traditionally, cardiac anatomy has been described using anatomic specimens. Currently, sophisticated non-invasive techniques allow imaging of MA with a richness of anatomical details unimaginable only two decades ago. The aim of this review is to provide a better understanding of the peculiar aspects of MA as they are revealed through these imaging techniques and discuss clinical implications related to this complex structure.

Idiopathic ventricular arrhythmias occur in the absence of underlying structural heart disease and less commonly in the presence of coexistent, but mechanistically unrelated, myocardial scar. These arrhythmias originate from several anatomical sites in both ventricles, with a predilection in outflow tract structures. The 12-lead surface ECG is the initial mapping tool, which is widely used to identify their origin. Specific features can predict the site of idiopathic ventricular arrhythmias, thus differentiating right from left ventricular, as well as endocardial from epicardial origins. In this review, we aim to analyse electrocardiographic landmarks for determination of idiopathic ventricular arrhythmia sources, with specific emphasis on pertinent caveats and anatomical relationships.

Interest in the anatomy of the interatrial septum (IAS) and its adjoining atrioventricular (AV) junction has risen enormously in the past two decades with the simultaneous evolution of left-sided percutaneous structural heart disease and complex electrophysiologic procedures. These procedures require, in fact, a direct route to the left atrium through the IAS. Thus, a thorough understanding of the complex anatomy of the IAS and AV junction is essential for performing a safe and effective transseptal puncture. There is a large amount of literature carefully describing the anatomy of the IAS and AV junction. These studies are based almost exclusively on anatomic specimens. Conversely, in this review the authors emphasize the role of noninvasive imaging techniques, in particular cardiac magnetic resonance, two- and three-dimensional transesophageal echocardiography, and computed tomography in visualizing specific aspects of the normal IAS and AV junction. Where appropriate, the authors present images side by side, with corresponding anatomic specimens.

Aims Inadvertent puncture of the aortic root (AR) is a well-known complication of transseptal puncture (TSP). Strategies for handling of this potentially lethal complication have not been identified yet. In this study, we present typical anatomical locations and clinical management of aortic root puncture (ARP) due to TSP. Methods and results All patients with ARP were retrospectively collected from seven hospitals. Aortic root puncture was identified and classified regarding angiographical and intraoperative findings in cardiac surgery: (i) TSP from the right atrium (RA) to the non-coronary sinus (NCS), (ii) TSP from RA to the non-coronary sinutubular junction (STJ), and (iii) TSP from RA to the ascending aorta (AA). A total of 24 patients with inadvertent ARP were identified. In 19 patients, penetration of the aorta was accomplished by the inner dilator, in 5 patients by the complete sheath. Previous cardiac surgery had been performed in six patients. There were 13 RA-to-NCS punctures, 2 RA-to-STJ punctures, and 9 RA-to-AA punctures. No cardiac tamponade (CT) occurred in patients with RA-to-NCS and RA-to-STJ punctures. In 8 of 9 patients with RA-to-AA puncture, CT occurred immediately requiring urgent pericardiocentesis and surgical repair. Two patients died after surgical repair. In the 16 patients without surgical therapy, no shunt from the AR to the RA was observed 3 months after the procedure. Conclusion Aortic root puncture due to mislead TSP via NCS or STJ is usually not associated with a severe clinical course while ARP into the AA via the epicardial space generally leads to CT requiring surgical repair.

Surgery for tetralogy of Fallot has a long history, which may be described as both a success story and a failure story. It is a success story because prognosis without surgery is very poor, but surgery makes it possible for affected babies to reach adulthood and lead productive lives. It is a failure story, however, since we still cannot cure this condition; we can only palliate it as illustrated in the sobering long-term outcome of affected patients. In this review article, we aim to explore the reason for this failure. This may be summed up in terms of the nature of the obstruction to the right ventricular outflow tract, which characterizes this malformation and must be relieved. This obstruction has several possible components, but none may be eliminated without harming the ventricle. There seems to be no 'extra' muscle band in tetralogy of Fallot that may be dispensed with without undermining ventricular function; every muscle band that is there should be there, just like in the normal heart, except that these are thicker than normal and somewhat displaced in tetralogy of Fallot, thus narrowing the right ventricular outflow tract. Consequently, ventricular function deteriorates with every muscle band that is cut, just like in the normal heart. We have to harm the heart in order to repair it. Every repaired Fallot is inevitably a damaged heart. Consequently, repair of this condition cannot be curative at present; it is palliative surgery.

Transcatheter procedures offer an alternative to cardiac surgery in select patients with structural heart disease (SHD). Unfortunately, inadvertent disruption of electrical pathways and subsequent development of new onset conduction disturbances can occur in up to 5–70% of percutaneous interventions, result in pacemaker implantation, and confer a worse prognosis. The physical proximity between the conduction system (atrioventricular node, bundle of His, and bundle branches) and the site of percutaneous repair is increasingly recognized as a key factor influencing new onset conduction disturbance development in procedures located near the conduction system. This review covers the incidence, clinical significance, and mechanisms of new onset conduction disturbances and discusses current and emerging strategies to address this complication in these populations.

Transcatheter techniques for the treatment of tricuspid regurgitation (TR) are being more frequently used and several new devices are in development. Since 90% of patients with TR have secondary TR, catheter based systems which reduce the dilated tricuspid annulus area are of particular interest. In order to perform an annuloplasty procedure effectively and safely, knowledge about the anatomy of the tricuspid valve apparatus and especially of the annulus in relation to the important neighboring structures such as the aortic root, the RCA, the electrical pathways and the CS is fundamental. In addition, comprehensive understanding of the device itself, the delivery system, its maneuverability and the individual procedural steps is required. Furthermore, the use of multi-modality imaging is important. For each step of the procedure the appropriate imaging modality as well as the optimal; imaging planes are crucial to provide the necessary information to best guide the individual procedural step.

Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown aetiology. We aimed to better understand familial recurrence pattern. Methods An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, leading to investigate 1043 unrelated ccTGA probands. Results Atrioventricular block at diagnosis and laterality defects were observed in 35.4% and 29.9%, respectively. ccTGA associated with primary ciliary dyskinesia in 10 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (d-TGA: 28.4%), laterality defects (13.6%) and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and d-TGA and 9 familial co-segregation of ccTGA and laterality defects. In one family there was co-segregation of ccTGA, d-TGA and heterotaxy syndrome in 3 distinct relatives. Conclusion ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, d-TGA, laterality defects and in some cases primary ciliary dyskinesia strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

Background: Adults with repaired tetralogy of Fallot die prematurely from ventricular tachycardia (VT) and sudden cardiac death. Inducible VT predicts mortality. Ventricular scar, the key substrate for VT, can be noninvasively defined with late gadolinium enhancement (LGE) cardiovascular magnetic resonance but whether this relates to inducible VT is unknown. Methods: Sixty-nine consecutive repaired tetralogy of Fallot patients (43 male, mean 40±15 years) clinically scheduled for invasive programmed VT-stimulation were prospectively recruited for prior 3-dimensional LGE cardiovascular magnetic resonance. Ventricular LGE was segmented and merged with reconstructed cardiac chambers and LGE volume measured. Results: VT was induced in 22 (31%) patients. Univariable predictors of inducible VT included increased RV LGE (odds ratio [OR], 1.15; P =0.001 per cm 3 ), increased nonapical vent LV LGE (OR, 1.09; P =0.008 per cm 3 ), older age (OR, 1.6; P =0.01 per decile), QRS duration ≥180 ms (OR, 3.5; P =0.02), history of nonsustained VT (OR, 3.5; P =0.02), and previous clinical sustained VT (OR, 12.8; P =0.003); only prior sustained VT (OR, 8.02; P =0.02) remained independent in bivariable analyses after controlling for RV LGE volume (OR, 1.14; P =0.003). An RV LGE volume of 25 cm 3 had 72% sensitivity and 81% specificity for predicting inducible VT (area under the curve, 0.81; P 10 cm 3 was 100% sensitive and >36 cm 3 was 100% specific for predicting inducible VT. Conclusions: Three-dimensional LGE cardiovascular magnetic resonance-defined scar burden is independently associated with inducible VT and may help refine patient selection for programmed VT-stimulation when applied to an at least intermediate clinical risk cohort.

The detailed anatomy of the interatrial septum (IAS) and mitral annulus (MA) as observed on cardiac magnetic resonance, computed tomography and two-dimensional/three-dimensional transthoracic and transesophageal echocardiography is reviewed. The IAS comprises of two components: the septum primum that is membrane-like forming the floor of the fossa ovalis (FO) and the septum secundum that is a muscular rim that surrounds the FO. The latter is an enfolding of atrial wall forming an interatrial groove. Named Waterston’s groove, it is filled with adipose tissue on the epicardial side. Thus, the safest area for transseptal puncture (TSP) is within the limits of the FO floor, which provides direct interatrial access. While crossing an intact septum is a well-established procedure, TSP is a more complex and time-consuming procedure in the presence of patent foramen ovalis, aneurysmal FO or atrial septal defect closure devices. MA comprises two distinctive segments: an anterior-straight and a posterior-curved segment. The posterior MA is a thin, discontinuous fibrous ‘string’, interspersed with adipose tissue, where four components converge: the atrial and ventricular musculature, epicardial adipose tissue and the leaflet’s hinge line. In parts of where this fibrous string is deficient or absent, the posterior leaflet is inserted directly on ventricular and atrial myocardium rendering the MA less robust and producing an ‘asymmetric’ dilation. The marked vulnerability of posterior MA to calcifications might be due to its insertion on the crest of ventricular myocardium being subject to friction injury due to the contraction and relaxation of LV.

Tricuspid regurgitation (TR) is a frequent and complex problem, commonly combined with left-sided heart disease, such as mitral regurgitation. Significant TR is associated with increased mortality if left untreated or recurrent after therapy. Tricuspid regurgitation was historically often disregarded and remained undertreated. Surgery is currently the only Class I Guideline recommended therapy for TR, in the form of annuloplasty, leaflet repair, or valve replacement. As growing experience of transcatheter therapy in structural heart disease, many dedicated transcatheter tricuspid repair or replacement devices, which mimic well-established surgical techniques, are currently under development. Nevertheless, many aspects of TR are little understood, including the disease process, surgical or interventional risk stratification, and predictors of successful therapy. The optimal treatment timing and the choice of proper surgical or interventional technique for significant TR remain to be elucidated. In this context, we aim to highlight the current evidence, underline major controversial issues in this field and present a future roadmap for TR therapy.

The mitral valve (MV) is a complex and intricate structure. With the development of transesophageal echocardiography in the 1990s, it became possible to evaluate MV anatomy and function in real time during surgical procedures. Subsequently, new surgical and percutaneous techniques for MV repair as well as replacement have evolved. Development of 3-dimensional and intracardiac echocardiography, as well as computed tomography, cardiac resonance imaging, and most recently fusion imaging, have paved the way for a more comprehensive evaluation of the MV as well as for the planning of percutaneous MV procedures such as balloon valvuloplasty, paravalvular mitral leak closure, percutaneous edge-to-edge repair, transcatheter MV annuloplasty, artificial chord implantation, and transcatheter MV replacement. The applicability and use of the various imaging modalities for the assessment and guidance of therapy for MV disorders is discussed in this paper.

The left atrial appendage (LAA) has received increasing attention in recent years because of thrombi formation in patients with atrial fibrillation, which increases the risk of stroke. In patients who have contraindications for long-term oral anticoagulation therapy, percutaneous procedures are used to occlude the LAA and there are now several devices available for implantation, both endocardially and epicardially. Despite the high-resolution imaging techniques on hand today, limitations remain in providing information about wall thickness and neighboring structures; therefore, in-depth knowledge of the normal atrial anatomy is mandatory when considering such interventions. Here, the anatomy of the right and left atria is reviewed with relevance to interventional procedures required for LAA occlusion. The components of the atria, particularly the LAA as well as the atrial septum, are described with emphasis on their spatial relationships to neighboring cardiac and extracardiac structures. Sound knowledge of the atrial anatomy including endocardial and epicardial aspects is necessary. This will help interventionists take full advantage of imaging techniques when assessing the suitability of the LAA anatomy for closure, selecting the optimal device types and sizes, and guiding the LAA closure procedure, thereby reducing potential complications and increasing procedural success.

Aims The aim of this study is to characterize the arrhythmogenic substrate for peri-mitral atrial flutter (PMAFL), thereby determining a personalized ablation strategy to treat PMAFL. Methods and results Thirty-six consecutive PMAFL patients (mean age: 63.8 ± 11.3, 23 males) underwent detailed three-dimensional electroanatomic mapping in left atrium (LA). The LA was divided into septal-anterior wall (SAW), posterior inferior wall (PIW), and mitral isthmus (MI) region, respectively. Ablation strategy was determined based on the endocardial bipolar voltage map. Based on electrophysiological substrates, 10, 17, and 9 cases were classified into iatrogenic, spontaneous, and no-substrate PMAFL, respectively. The mean voltage in SAW was significantly lower in spontaneous PMAFL (iatrogenic: 1.07 ± 0.66 mV; spontaneous: 0.65 ± 0.44 mV; no-substrate: 1.60 ± 0.53 mV, P

This review provides an update on rheumatic mitral stenosis. Acute rheumatic fever (RF), the sequela of group A β-hemolytic streptococcal infection, is the major etiology for mitral stenosis (MS). While the incidence of acute RF in the Western world had substantially declined over the past five decades, this trend is reversing due to immigration from non-industrialized countries where rheumatic heart disease (RHD) is higher. Pre-procedural evaluation for treatment of MS using a multimodality approach with 2D and 3D transthoracic and transesophageal echo, stress echo, cardiac CT scanning, and cardiac MRI as well as hemodynamic assessment by cardiac catheterization is discussed. The current methods of percutaneous mitral balloon commissurotomy (PMBC) and surgery are also discussed. New data on long-term follow-up after PMBC is also presented. For severe rheumatic MS, medical therapy is ineffective and definitive therapy entails PMBC in patients with suitable morphological mitral valve (MV) characteristics, or surgery. As procedural outcomes depend heavily on appropriate case selection, definitive imaging and interpretation are crucial. It is also important to understand the indications as well as morphological MV characteristics to identify the appropriate treatment with PMBC or surgery.

The epicardial adipose tissue (EAT) refers to the deposition of adipose tissue fully enclosed by the pericardial sac. EAT has a complex mixture of adipocytes, nervous tissue, as well as inflammatory, stromal and immune cells secreting bioactive molecules. This heterogeneous composition reveals that it is not a simply fat storage depot, but rather a biologically active organ that appears playing a "dichotomous" role, either protective or proinflammatory and proatherogenic. The cardiac magnetic resonance (CMR) allows a clear visualization of EAT using a specific pulse sequence called steady-state free precession. When abundant, the EAT assumes a pervasive presence not only covering the entire epicardial surface but also invading spaces that usually are almost virtual and separating walls that usually are so close each other to resemble a single wall. To the best of our knowledge, this aspect of cardiac anatomy has never been described before. In this pictorial review, we therefore focus our attention on certain cardiac areas in which EAT, when abundant, is particularly intrusive. In particular, we describe the presence of EAT into: (a) the interatrial groove, the atrioventricular septum, and the inferior pyramidal space, (b) the left lateral ridge, (c) the atrioventricular grooves, and (d) the transverse pericardial sinus. To confirm the reliability in depicting the EAT distribution, we present CMR images side-by-side with corresponding anatomic specimens.

Objectives The morphologically right and left ventricles are distinguished from each other based on their internal anatomical features, because their external (epicardial) surfaces do not appear to have any distinguishing mark for such ventricular identification. Nevertheless, ventricular identification based on epicardial characteristics, if these were possible, would be interesting to surgeons, because this would enable them to identify each ventricle rapidly upon opening the chest. This made us curious as to whether or not the two ventricles may be distinguished based on their epicardial coronary arterial patterns, because this is the most obvious epicardial ventricular feature. Methods This idea led us to formulate the following 2 hypotheses: (i) The morphologically left ventricle is always the one that receives the higher number of the marginal arteries as compared to the morphologically right ventricle. (ii) Only the morphologically left ventricle receives the diagonal arteries from the anterior and posterior interventricular arteries. These hypotheses were tested in this anatomical observational study by examination of 98 normal and 398 congenitally malformed formaldehyde-preserved hearts encompassing most malformations, including rare ones and hearts in which 1 ventricle is hypoplastic. Results These examinations show that both hypotheses are false. Conclusions The two ventricles cannot be distinguished from each other based on the number of marginal arteries that they receive or which one receives diagonal arteries; both ventricles may receive diagonal arteries from either or both interventricular arteries.

Atrial arrhythmias are frequently encountered in patients with congenital heart disease (CHD) with or without corrective surgery and respond to pharmacological therapies with only limited success. This review describes the technologies currently available for performing successful ablation procedures in this very complex patient cohort. In addition to an understanding of the underlying anatomy, which can be supplemented by 3D imaging with the aid of magnetic resonance imaging (MRI) or computed tomography (CT), the choice between the different 3D mapping systems (sequential versus simultaneous) is presented. Finally, conventional manual navigation is compared with magnetic navigation and then discussed with regard to the respective arrhythmias encountered with the different forms of CHD.

Background Our aim is to identify the pathognomonic anatomical markers and the best terminology to describe the cardiac malformations associated with absent or multiple spleens, which are known as asplenia or polysplenia syndromes or isomerism. Materials and methods We have reviewed 65 hearts with isomerism of atrial appendages of the Anatomical Collections of Congenital Heart Disease, Institute of Pathological Anatomy of the University of Padua consisting of 1800 specimens. All the hearts were classified according to sequential segmental classification. Results The incidence of isomerism was 3.6%. Of the total, 45 hearts with isomerism of right atrial appendages showed bilateral trilobed lungs, short bronchi, and absent spleen. The atrioventricular junction was univentricular in 49% of cases with a common atrioventricular valve in 91%. Pulmonary atresia and double outlet right ventricle were present in 40% and 47% of cases, respectively. Total anomalous pulmonary venous drainage and absent coronary sinus were always present. In 20 hearts with isomerism of left atrial appendages, bilateral bilobed lungs with long bilateral bronchi and multiple spleens were always found. The biventricular atrioventricular connection was present in 65% with a common valve in 30% of the hearts. The ventriculoarterial connection was concordant in 45% of cases, and aortic atresia and pulmonary atresia were both noted in 15% of each. An anomalous symmetric pulmonary venous drainage was observed in 65% of the hearts and interruption of inferior vena cava was found in 75% of cases. Conclusions We believe that the appropriate terminology is based on the symmetrical morphology of the atrial appendages. The absence of the coronary sinus and the total anomalous pulmonary venous drainage are the markers of isomerism of the right atrial appendages. Symmetric pulmonary venous drainage and interruption of inferior vena cava are the markers of isomerism of left atrial appendages. In recent years, thanks to the improvement of clinical diagnosis and of surgical techniques these patients have the possibility to survive to adult age.

Interest on tricuspid valve (TV) (and hence in TV anatomy) has increased in the last two decades with the awareness that functional tricuspid regurgitation (FTR) is an insidious disease progressively leading to untreatable right heart failure and eventually to death. Medical therapy may alleviate symptoms, while surgical therapy may improve outcome but it is associated with high mortality and recurrence of significant regurgitation. Nowadays, an increasing number of left valve diseases are successfully treated through a percutaneous transcatheter approach. The negative impact that the untreated FTR may have in these patients has highlighted the necessity of developing transcatheter solutions also for FTR and numerous catheter devices for treating FTR are currently under evaluation. The essential pre-requisite for an effective and safe surgical or transcatheter therapy is a deep knowledge of the normal TV anatomy. In this review, we describe the anatomy of TV and surrounding structures as revealed by computed tomography, cardiac magnetic resonance, 2D/3D transthoracic echocardiography, and 2D/3D transoesophageal echocardiography emphasizing strengths and weaknesses of each of these imaging tools. To confirm the anatomical fidelity of these imaging modalities, where appropriate, the non-invasive images where presented, side-by-side, with corresponding images from anatomic specimens.

Two-dimensional (2D) transthoracic echocardiography is one of the most used diagnostic tools in clinical cardiology. Similarly, 2D transesophageal echocardiography is considered an indispensable tool for cardiologists and cardiac anesthesiologists worldwide. However, because of their tomographic nature, both techniques display only thin cut planes of a given area of the heart, which are far from representing the “anatomic reality.” It is widely accepted that experienced echocardiographers are able to reconstruct mentally a three-dimensional image of any cardiac structure on the basis of their interpretation of multiple tomographic slices. However, this may not be the case with less experienced echocardiographers. In particular, the authors noticed that less experienced echocardiographers are almost totally unaware of which structures lie “nearby” a given 2D tomographic plane, that is, what is adjacent in the elevation plane. In this article, the authors report the use of three-dimensional transesophageal echocardiographic images to discover which structures are located nearby (i.e., “behind” and “in front”) the corresponding 2D cross-sections. The authors believe that this novel use of three-dimensional echocardiography is a unique aid to disclose what cannot be seen in a given 2D cross-section, thereby expanding our understanding of 2D echocardiographic anatomy. This may be an effective method to encourage all to “think” in three dimensions, even when they use 2D echocardiography.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculoarterial connections.1 The incidence has been reported to be around 1/33 000 live births, accounting for ≈0.05% of congenital heart malformations.2 Although a familial recurrence of heart defects in subjects with ccTGA has been reported,3 the etiology of this malformation is not currently known.1, 4 The morphological right atrium is connected to the morphological left ventricle across the mitral valve, with the left ventricle then connected to the pulmonary trunk; the morphological left atrium is connected to the morphological right ventricle across the tricuspid valve, with the morphological right ventricle connected to the aorta. Because of this “double discordance,” the systemic venous return is pumped to the lungs, while the pulmonary venous return is directed to the body. In the context of usual atrial arrangement (situs solitus), the morphological left ventricle is thus positioned to the right and the aorta, arising from the morphological right ventricle, is more commonly left‐sided. In mirror‐image atrial arrangement (situs inversus), the morphological left ventricle is left‐sided, and the aorta is usually positioned to the right. Most patients have at least 1 associated cardiac anomaly, mainly ventricular septal defect (VSD), subvalvular and/or valvular pulmonary stenosis, or abnormalities of the systemic (tricuspid) atrioventricular (AV) valve with coexisting accessory pathways.4 Because of the unusual position of the atrioventricular node and course of the atrioventricular conduction bundle, cardiac conduction disorders are frequent and markedly alter the natural history and management of the malformation.5, 6, 7 This article provides a comprehensive, up‐to‐date description of the characteristics of the cardiac conduction system in patients with ccTGA, their clinical sequelae, and their contemporary management.

The aim of this study is to review the key features of anatomy of the sinus node of the human sinus node and its pathological variations as reported in the literature and supplemented with our observations. The sinus node is located adjacent to important structures such as the right phrenic nerve and the nodal artery. Although intimately related to its matrix of fibrous tissue, the nodal cells are in contact with atrial myocardium through nodal extensions. Nodal cell numbers decrease with age as part of the normal aging process. Pathological changes include diminished size of node and nodal cell replacement with fibro-fatty tissues. The location of the normal sinus node is well recognised. There is variation in the relationship of the node and its arterial supply to the terminal crest and the cavo-atrial junction.

Percutaneous left atrial appendage (LAA) exclusion is an evolving treatment to prevent embolic events in patients with nonvalvular atrial fibrillation. In the past few years multiple percutaneous devices have been developed to exclude the LAA from the body of the left atrium and thus from the systemic circulation. Two- and 3-dimensional transesophageal echocardiography (TEE) is used to assess the LAA anatomy and its suitability for percutaneous closure to select the type and size of the closure device and to guide the device implantation procedure in conjunction with fluoroscopy. In addition, 2- and 3-dimensional TEE is also used to assess the effectiveness of device implantation acutely and on subsequent follow-up examination. Knowledge of the implantation options that are currently available along with their specific characteristics is essential for choosing the appropriate device for a given patient with a specific LAA anatomy. We present the currently available LAA exclusion devices and the echocardiographic imaging approaches for evaluation of the LAA before, during, and after LAA occlusion.

Persistence of the embryonic “fifth aortic arch” in postnatal life is a rare, enigmatic – and at times controversial – condition, with variable anatomical forms and physiological consequences. First described in humans over 40 years ago by Van Praagh, the condition was labelled the “great pretender” by Gerlis 25 years later, because of its apparent propensity to mimic anatomically similar structures. Despite many subsequent case reports citing the condition, the true developmental origin of these structures remains unresolved, and has been the subject of debate among embryologists for more than a century. A persistent fifth aortic arch has been defined as an extrapericardial structure, arising from the ascending aorta opposite or proximal to the brachiocephalic artery, and terminating in the dorsal aorta or pulmonary arteries via a persistently patent arterial duct. This description may therefore encompass various anatomical forms, such as a unilateral double-lumen aortic arch, an unrestrictive aortopulmonary shunt, or a critical vascular channel for either the systemic or pulmonary circulation. The physiological properties of these vessels, such as their response to prostaglandins, may also be unpredictable. In this article, we demonstrate a number of cases that fulfil the contemporary definition of “persistent fifth aortic arch” while acknowledging the embryological controversies associated with this term. We also outline the key diagnostic features, particularly with respect to the use of new cross-sectional imaging techniques.

The pericardial space is a fairly new portal for interventional cardiac electrophysiologists to access the heart and requires an understanding of its anatomic peculiarities to maneuver safely around the epicardial surfaces of the heart and great veins. Subxiphoid puncture should allow the diaphragm and fibrous pericardium to be crossed without entering the right ventricle, which is the most anteriorly situated cardiac chamber. The phrenic nerves descend on the fibrous pericardium bilaterally. The right nerve is related to the superior caval vein and the superior right pulmonary vein, whereas the left nerve takes variable courses over the left atrial appendage and the left ventricle. Related to the fibrous pericardium overlying the posterior wall of the left atrium is the esophagus.

Background— Ablation of para-Hisian accessory pathway (AP) poses high risks of atrioventricular block. We developed a pacing technique to differentiate the near-field (NF) from far-field His activations to avoid the complication. Methods and Results— Three-dimensional mapping of the right ventricle was performed in 15 mongrel dogs and 23 patients with para-Hisian AP. Using different pacing outputs, the NF- and far-field His activation was identified on the ventricular aspect. Radiofrequency application was delivered at the NF His site in 8 (group 1) and the far-field His site in 7 dogs (group 2), followed by pathologic examination after 14 days. NF His activation was captured with 5 mA/1 ms in 10 and 10 mA/1 ms in 5 dogs. In group 1, radiofrequency delivery resulted in complete atrioventricular block in 3, right bundle branch block with HV (His-to-ventricular) interval prolongation in 1, and only right bundle branch block in 2 dogs, whereas no changes occurred in group 2. Pathologic examination in group-1 dogs showed complete or partial necrosis of the His bundle in 4 and complete necrosis of the right bundle branch in 5 dogs. In group 2, partial necrosis in the right bundle branch was found only in 1 dog. Using this pacing technique, the APs were 5.7±1.2 mm away from the His bundle located superiorly in 20 or inferiorly in 3 patients. All APs were successfully eliminated with 1 to 3 radiofrequency applications. No complications and recurrence occurred during a follow-up of 11.8±1.4 months. Conclusions— Differentiating the NF His from far-field His activations led to a high ablation success without atrioventricular block in para-Hisian AP patients.

The aim of this study was to estimate the incidence and to analyze the anatomy of double inlet-double outlet right ventricle complex and its associated cardiac anomalies in our autopsy series. Among the 1640 hearts with congenital heart disease of our Anatomical Collection, we reviewed the specimens with double inlet-double outlet right ventricle, according to the sequential-segmental analysis, identifying associated cardiac anomalies and examining lung histology to assess the presence of pulmonary vascular disease. We identified 14 hearts with double inlet-double outlet right ventricle (0.85%). Right atrial isomerism was observed in 10 hearts, situs solitus in 3 and left atrial isomerism in one. Regarding the mode of atrioventricular connection, all hearts but one had a common atrioventricular valve. Systemic or pulmonary venous abnormalities were noted in all patients with atrial isomerism. In nine patients a valvular or subvalvular pulmonary stenosis was present. Among the functionally "univentricular hearts", double inlet- double outlet right ventricle represents a peculiar entity, mostly in association with right atrial isomerism. Multiple cardiac anomalies are associated and may complicate surgical repair.

We report on a patient with a significantly enlarged right heart caused by a rare right ventricle to right atrium fistula. Cardiac magnetic resonance revealed diagnosis and delineated detailed anatomy of the defect. The shunt was successfully closed with an implantation of Amplatzer Vascular Plug II. We discuss possible causes of this anomaly and its treatment. © 2013 Wiley Periodicals, Inc.

Background The impact of overload duration and age on remodelling process in human atria remains controversial. We analysed histological markers of right atrial (RA) remodelling in CHD patients in order to provide some insights into impacts of age and overload duration on substrates of atrial arrhythmias. Methods Sixty-five CHD patients who underwent initial cardiac surgery were enrolled (median age 18years). Patients with dominant left atrial overload were excluded. RA tissues resected from CHD patients during surgery were examined by means of histology and immunohistochemistry and compared with RA tissues from age-matched control hearts from post-mortem (n=22, median age 22years). Patient histories of preoperative SVTs and echocardiography were also examined. Results The greatest extent of fibrosis, the largest myocyte diameter and the longest capillary distance and the most CD45-positive cell infiltration was observed in CHD samples with SVT, followed by CHD without SVT and then control samples. All histological changes were correlated with age in CHD samples (r=0.462, 0.718, 0.529 and 0.447, respectively) with relatively steep and continuous manner, whereas only myocyte diameter was correlated with age in control samples (r=0.576). RA dimensions and area obtained from echocardiography also correlated with histological markers in CHD patients. Conclusions Dominant and chronic RA overload due to CHD resulted in time-course related RA structural remodelling, which could provide the background for atrial arrhythmia. The overload duration, rather than age, seems to be a key factor for atrial histological degeneration in the cohort of CHD.

Pulmonary arterial hypertension (PAH) is considered primarily a disease of the distal pulmonary arteries whereas little is known on the effect of long-standing pulmonary hypertension on the larger proximal pulmonary arteries. This study aims to investigate the structural changes in the great arteries of adults who developed PAH in association with congenital heart disease (CHD), with severe cases termed Eisenmenger syndrome.We performed macroscopic and light microscopy analyses on the great arteries of 10 formalin-fixed human hearts from patients with PAH/CHD and compared them to age-matched healthy controls. A detailed histology grading score was used to assess the severity of medial wall abnormalities.Severe atherosclerotic lesions were found macroscopically in the elastic pulmonary arteries of 4 PAH/CHD specimens and organised thrombi in 3; none were present in the controls. Significant medial wall abnormalities were present in the pulmonary trunk (PT), including fibrosis (80%), and atypical elastic pattern (80%). Cyst-like formations were present in less than one third of patients and were severe in a single case leading to wall rupture. The cumulative PT histology grading score was significantly higher in PAH/CHD cases compared to controls (p0.0001) and correlated positively with larger PT diameters (ρ=0.812, p0.0001) and the degree of medial wall hypertrophy (ρ=0.749, p0.0001).Chronic PAH in association with CHD results in marked macroscopic and histological abnormalities in the large pulmonary arteries. These abnormalities are likely to affect haemodynamics and contribute to morbidity and mortality in this cohort.

Nowadays computed tomography, cardiac magnetic resonance imaging, and tridimensional transoesophageal echocardiography provide anatomic images of right-atrial structures with an impressive richness of anatomical details. It is therefore surprising that these techniques are not routinely used as complementary tools in teaching anatomy. This review aims to fill this gap showing the normal anatomy of right atrium as displayed by these sophisticated imaging techniques. A better understanding of right-atrial anatomy is crucial for the treatment of primary right-atrium electrical disorders as well as for catheter-based interventions for structural heart disease. The success of these procedures is, in fact, related to an accurate anatomical pre-procedural assessment. In this review, we describe the normal anatomy and variants of those right-atrial structures relevant for both ablationists and interventionalists.

Background— Remote magnetic navigation–guided ablation with 3-dimensional (3D)-image integration could provide maximum benefit in patients with complex anatomy. We reviewed supraventricular tachycardia (SVT) ablation in adult patients with congenital heart disease to assess the contribution of these technologies. Methods and Results— One hundred fifty-four SVT ablation procedures (228 SVTs) using a 3D-electroanatomic mapping system in 116 adult patients with congenital heart disease (mean age, 41; 76 male) were classified into 3 groups: Group A, manual mapping/ablation (n=60 procedures); Group B, remote magnetic navigation–guided mapping/ablation with normal femoral vein access (49); and Group C, remote magnetic navigation–guided mapping/ablation with difficult access (45). Group A included simple anomalies with less SVTs. Group B comprised predominantly Fontan patients with more SVTs. Group C included more complex defects, such as intra-atrial baffle or interrupted inferior venous access, in which retrograde aortic and superior venous accesses were used exclusively with more frequent use of image integration (97.8%; P P =0.370). In group C, fluoroscopy time was the shortest (median, 4.2 min; P P P =0.787) during a mean 20-months follow-up period. Conclusions— The combination of remote magnetic navigation, 3D-image integration, and electroanatomic mapping system facilitated safe and feasible ablation with very low fluoroscopy exposure even in patients with complex anomalies.

THE COMPLEX SHAPE OF THE RIGHT VENTRICLE MAKES IT CHALLENGING FOR MOST IMAGING MODALITIES. This is especially true for the right ventricle outflow tract (RVOT), which is important for many congenital diseases, but has an even more complex anatomy and function. Cardiac computed tomography and cardiac

In patients with transposition of the great arteries, the identification of coronary anatomy is fundamental to optimal surgical outcome. A number of classifications describing the coronary vessels' origin and course in transposition of the great arteries have been published. However, all are limited to operative or pathological case series. They are often alphanumeric classifications that do not lend themselves to clinical practice; they do not consider certain important anatomical variations that may increase surgical morbidity and mortality, nor do they fully delineate coronary anatomy or define the relationship to adjacent structures seen with cardiovascular computed tomography. Using cardiovascular computed tomography for illustrative purposes, we propose and validate a universal sequential descriptive classification and an associated alphanumeric classification that may be used for all coronary anomalies with or without associated congenital heart disease.

Catheter ablation of cardiac arrhythmias has rapidly evolved from a highly experimental procedure to a standard form of therapy for various tachyarrhythmias. The advances in this field have included, first, the development of techniques of catheter ablation that often requires the precise destruction of minute amounts of arrhythmogenic tissues and, second, techniques of resynchronization therapy that require pacing different parts of the ventricles. A detailed prepocedural knowledge of cardiac anatomy can improve the safety of the procedure and its rate success. It helps the electrophysiologist to choose the appropiate region for ablation, shortening the procedural time. The atrial anatomy structures are usually localized before ablation by different imaging techniques such as fluoroscopy, electroanatomic mapping, intracardiac echocardiography or multidetector computed tomography. In this review, we describe the normal anatomy of the atria, highlighting the landmarks of interest to intervencional cardiologist, stressing their relationship to other structures. This article is part of a JCTR special issue on Cardiac Anatomy. 2.691 JCR (2013) Q2, 50/125 Cardiac & cardiovascular systems, 51/122 Medicine, research & experimental

Aims Chronic inflammation in the atrial myocardium was shown to play an important role in the development of atrial fibrosis in patients with atrial fibrillation (AF). However, it is not clear to what extent atrial inflammatory reaction associated with AF extends on the ventricular myocardium. Our aim was to assess the extent of fibrosis and lymphomononuclear infiltration in human ventricular myocardium and explore its association with AF. Methods and results Medical records from consecutive autopsies were checked for presence of AF. Heart specimens from 30 patients died from cardiovascular causes (64 ± 12 years, 17 men) were collected in three equal groups: no AF, paroxysmal AF, and permanent AF. Tissue samples were taken from the Bachmann’s bundle, crista terminalis, posterior left atrium, left ventricle and right ventricle free walls and stained with Masson’s trichrome for analysis of fibrosis extent. Immunohistochemistry was performed using antibodies against CD3- and CD45-antigens and quantified as number of antigen-positive cells per 1 mm2. Fibrosis extent, CD3+ and CD45+ cell counts were elevated in AF patients at all sites ( P

International audience; Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac surgery due to their association with myocardial ischaemia and sudden death. Such anomalies are detectable by imaging modalities and, according to various definitions, their prevalence ranges from 0.21 to 5.79%. This consensus document from the Development, Anatomy and Pathology Working Group of the European Society of Cardiology aims to provide: (i) a definition of normality that refers to essential anatomical and embryological features of coronary vessels, based on the integrated analysis of studies of normal and abnormal coronary embryogenesis and pathophysiology; (ii) an animal model-based systematic survey of the molecular and cellular mechanisms that regulate coronary blood vessel development; (iii) an organization of the wide spectrum of coronary artery anomalies, according to a comprehensive anatomical and embryological classification scheme; (iv) current knowledge of the pathophysiological mechanisms underlying symptoms and signs of coronary artery anomalies, with diagnostic and therapeutic implications. This document identifies the mosaic-like embryonic development of the coronary vascular system, as coronary cell types differentiate from multiple cell sources through an intricate network of molecular signals and haemodynamic cues, as the necessary framework for understanding the complex spectrum of coronary artery anomalies observed in human patients.

real-time 3-dimensioanl transesophageal echocardiography (RT3DTEE) provides high quality images of the posterior structures of the heart ([1][1]) and has been used for guiding several catheter-based procedures ([2][2]). The electrical isolation of pulmonary veins (PVs) has become a highly effective

Recent decades have seen rapid developments in arrhythmia treatment, especially the use of catheter ablation. Although the substrates of atrial fibrillation, its initiation and maintenance, remain to be fully elucidated, catheter ablation in the left atrium has become a therapeutic option for patients with this arrhythmia. With ablation techniques, various isolation lines and focal targets are deployed; the majority of these are anatomic approaches. It has been over a decade since we published our first article on the anatomy of the left atrium relevant to interventional electrophysiologists.1 Our aim then, as now, was to increase awareness of anatomic structures inside the left atrium. In this review of anatomy, we revisit the left atrium, inside as well as outside, for a better understanding of the atrial component parts and the spatial relationships of specific structures. ### Location and Atrial Walls Viewed from the frontal aspect of the chest, the left atrium is the most posteriorly situated of the cardiac chambers. Owing to the obliquity of the plane of the atrial septum and the different levels of the orifices of the mitral and tricuspid valves, the left atrial chamber is more posteriorly and superiorly situated relative to the right atrial chamber. The pulmonary veins enter the posterior part of the left atrium with the left veins located more superior than the right veins. The transverse pericardial sinus lies anterior to the left atrium, and in front of the sinus is the root of the aorta. The tracheal bifurcation, the esophagus, and descending thoracic aorta are immediately behind the pericardium overlying the posterior wall of the left atrium. Further behind is the vertebral column. Following the direction of blood flow, the atrial chamber begins at the pulmonary veno-atrial junctions and terminates at the fibro-fatty tissue plane that marks the atrioventricular junction at the mitral orifice. The walls …

"Partial forms of Shone complex are rare. Surgical outcomes of the complete forms have generally been poor, whereas there is a lack of data on long-term follow-up of surgically treated adult partial complex. Between 2001 and 2011, nine patients (age: 38 ± 8 years; six males, 67%) were referred for valvular heart disease. Transthoracic and transoesophageal echocardiography was performed. Data were confirmed by intra-operative findings and reports. Patients were diagnosed as partial Shone complex and presented with mitral stenosis (MS) (45%) or mitral regurgitation (22%) or aortic regurgitation (22%). All but one patient (89%) reported previous surgery: coarctation of the aorta repair (87.5%) and aortic valvulotomy (12.5%). Redo intervention included: mitral valve replacement (25%), mitral repair (25%), aortic valve replacement (37.5%) and subvalvular aortic ridge resection (25%). One patient refused surgery. Patients surgically treated before the age of 5 (87.5%) showed favourable outcome (survival rate: 100%) and a 23.6 (± 4.6)-year follow-up free from events. The patient who underwent first intervention at the age of 50 and the patient with MS who refused surgery showed a 45 (± 7)-year follow-up free from major morbidity. Patients with partial Shone complex, properly diagnosed and treated, show favourable surgical outcome free from major clinical events.. . "

OBJECTIVES: The purpose of this study was to assess the association between structural changes in human atria, age, and history of atrial fibrillation (AF). BACKGROUND: Development of fibrosis in atrial walls is associated with deterioration of atrial conduction and predisposes to AF in experiment. Human data, however, are scarce, and whether fibrosis is a cause or consequence of AF is not known. METHODS: Medical records for consecutive autopsies were checked for AF history and duration. Atrial specimens from 30 patients (ages 64 ± 12 years) were collected in 3 equal age-matched groups as patients without AF history, with paroxysmal AF, or with permanent AF. Tissue samples were obtained at the level of superior pulmonary veins, inferior pulmonary veins, center of posterior left atrial wall, terminal crest, and Bachmann's bundle. Histology sections were assessed for extent of fibrosis, fatty tissues, and inflammatory infiltration at each location. RESULTS: No correlation was observed between age and fibrosis at any location. Fibrosis extent and fatty infiltration were twofold to threefold higher at all locations in patients with history of AF and correlated with lymphomononuclear infiltration. Patients with permanent AF had greater fibrosis extent than did patients with paroxysmal AF. CONCLUSIONS: In post-mortem material, structural changes in the atria were not associated with age, but were significantly correlated with presence of AF and its severity. Our findings suggest that age-related changes per se are unlikely to be the sole cause of advanced fibrosis underlying AF.

The anatomy of the left atrium is reviewed with relevance to various interventional transcatheter procedures requiring manoeuvres within or passage through the left atrium. The component parts of the atrium--the atrial body with a vestibule, appendage, venous component, and the atrial septum--are described with emphasis on their spatial relationships to neighbouring cardiac and extra-cardiac structures. Normal variations are discussed for a better understanding of the anatomy so as to reduce the risk of potential complications during procedures.

Extraordinary advances in technology have made possible percutaneous catheter-based treatment of a wide spectrum of specific pediatric and adult "structural" heart diseases. Many of these percutaneous interventional procedures require access to the left heart via transseptal catheterization. Being able to see the anatomy can be a considerable advantage. However, septal anatomy is more complex than perceived at first sight. The true interatrial septum comprises a valvelike flap forming the floor of the fossa ovalis. On the right atrial aspect, the muscular rim surrounding the fossa is an infolding of the atrial wall. Hence, the target area for safe crossing, without exiting the heart, is the fossa floor and its immediate margin of the rim. Real-time (RT) three-dimensional (3D) transesophageal echocardiography is a recently developed technique that provides 3D images of the heart. Because of lack of interference from bone and lung and the closer proximity of the transducer to the posterior structures of the heart, this technique provides 3D RT images of atrial structures of unprecedented quality. In this review, the authors describe two key areas: a step-by-step approach for acquiring and processing RT 3D transesophageal echocardiographic images of the interatrial septum and, second, septal anatomy as it is visualized by RT 3D transesophageal echocardiography. To demonstrate their consistency with actual anatomy, several RT 3D transesophageal echocardiographic images are matched to equivalent anatomic specimens.