Miniseries 2-Septal and paraseptal accessory pathways-Part IV: Inferior paraseptal accessory pathways-lessons from surgical and catheter ablation.

Surgeons and electrophysiologists performing accessory pathway ablation procedures have used the term 'posteroseptal' region. This area, however, is neither septal nor posterior, but paraseptal and inferior; paraseptal because it includes the fibro-adipose tissues filling the pyramidal space and not the muscular septum itself and inferior because it is part of the heart adjacent to the diaphragm. It should properly be described, therefore, as being inferior and paraseptal. Pathways in this region can be ablated at three areas, which we term right inferior, mid-inferior, and left inferior paraseptal. The right- and left inferior paraseptal pathways connect the right and left atrial vestibules with the right and left paraseptal segments of the parietal ventricular walls. The mid-inferior paraseptal pathways take a subepicardial course from the myocardial sleeves surrounding the coronary sinus and its tributaries. Our review addresses the evolution of the anatomical concept of the inferior paraseptal region derived from surgical and catheter ablation procedures. We also highlight the limitations of the 12-lead electrocardiogram in identifying, without catheter electrode mapping, which are the pathways that can be ablated without a coronary sinus, or left heart approach.

Miniseries 2-Septal and paraseptal accessory pathways-Part I: The anatomic basis for the understanding of para-Hisian accessory atrioventricular pathways.

AIMS: Although the anatomy of the atrioventricular conduction axis was well described over a century ago, the precise arrangement in the regions surrounding its transition from the atrioventricular node to the so-called bundle of His remain uncertain. We aimed to clarify these relationships. METHODS AND RESULTS: We have used our various datasets to examine the development and anatomical arrangement of the atrioventricular conduction axis, paying particular attention to the regions surrounding the point of penetration of the bundle of His. It is the areas directly adjacent to the transition of the atrioventricular conduction axis from the atrioventricular node to the non-branching atrioventricular bundle that constitute the para-Hisian areas. The atrioventricular conduction axis itself traverses the membranous part of the ventricular septum as it extends from the node to become the bundle, but the para-Hisian areas themselves are paraseptal. This is because they incorporate the fibrofatty tissues of the inferior pyramidal space and the superior atrioventricular groove. In this initial overarching review, we summarize the developmental and anatomical features of these areas along with the location and landmarks of the atrioventricular conduction axis. We emphasize the relationships between the inferior pyramidal space and the infero-septal recess of the subaortic outflow tract. The details are then explored in greater detail in the additional reviews provided within our miniseries. CONCLUSION: Our anatomical findings, described here, provide the basis for our concomitant clinical review of the so-called para-Hisian arrhythmias. The findings also provide the basis for understanding the other variants of ventricular pre-excitation.

Miniseries 2-Septal and paraseptal accessory pathways-Part III: Mid-paraseptal accessory pathways-revisiting bypass tracts crossing the pyramidal space.

The mid-paraseptal region corresponds to the portion of the pyramidal space whose right atrial aspect is known as the triangle of Koch. The superior area of this mid-paraseptal region is also para-Hisian, and is close to the compact atrioventricular node and the His bundle. The inferior sector of the mid-paraseptal area is unrelated to the normal atrioventricular conduction pathways. It is, therefore, a safe zone in which, if necessary, to perform catheter ablation. The middle part of the mid-paraseptal zone may, however, in some patients, house components of the compact atrioventricular node. This suggests the need for adopting a prudent attitude when considering catheter ablation in this area. The inferior extensions of the atrioventricular node, which may represent the substrate for the slow atrioventricular nodal pathway, take their course through the middle, and even the inferior, sectors of the mid-paraseptal region. In this review, we contend that the middle and inferior areas of the mid-paraseptal region correspond to what, in the past, was labelled by most groups as the 'midseptal' zone. We describe the electrocardiographic patterns observed during pre-excitation and orthodromic reciprocating tachycardia in patients with pathways ablated in the middle or inferior sectors of the region. We discuss the modification of the ventriculo-atrial conduction times during tachycardia after the development of bundle branch block aberrancy. We conclude that the so-called 'intermediate septal' pathways, as described in the era of surgical ablation, were insufficiently characterized. They should not be considered the surrogate of the 'midseptal' pathways defined using endocardial catheter electrode mapping.

Miniseries 2-Septal and paraseptal accessory pathways-Part II: Para-Hisian accessory pathways-so-called anteroseptal pathways revisited.

Surgeons, when dividing bypass tracts adjacent to the His bundle, considered them to be 'anteroseptal'. The area was subsequently recognized to be superior and paraseptal, although this description is not entirely accurate anatomically, and conveys little about the potential risk during catheter interventions. We now describe the area as being para-Hisian, and it harbours two types of accessory pathways. The first variant crosses the membranous septum to insert into the muscular ventricular septum without exiting the heart, and hence being truly septal. The second variant inserts distally in the paraseptal components of the supraventricular crest, and consequently is crestal. The site of ventricular insertion determines the electrocardiographic expression of pre-excitation during sinus rhythm, with the two types producing distinct patterns. In both instances, the QRS and the delta wave are positive in leads I, II, and aVF. In crestal pathways, however, the QRS is ≥ 140 ms, and exhibits an rS configuration in V1-2. The delta wave in V1-2 precedes by 20-50 ms the apparent onset of the QRS in I, II, III, and aVF. In the true septal pathways, the QRS complex occupies ∼120 ms, presenting a QS, W-shaped, morphology in V1-2. The delta wave has a simultaneous onset in all leads. Our proposed terminology facilitates the understanding of the electrocardiographic manifestations of both types of para-Hisian pathways during pre-excitation and orthodromic tachycardia, and informs on the level of risk during catheter ablation.

Peri-infarction block, a marker for VT risk?

We present a monomorphic ventricular tachycardia (VT) in a patient with a history of myocardial infarction and syncope. The ECG shows an acceleration-dependent peri-infarction block inferiorly. The authors suggest that this old (but forgotten) ECG-entity may serve as a marker for assessing the risk of post-infarction VT.

Narrow QRS complexes within left bundle branche block in a patient with chest pain.

We present the case of a patient with chest pain, palpitations, atrial fibrillation, left bundle branch block (LBBB) and occasional early narrow QRS complexes. An intracardiac study revealed rate-dependent LBBB with additional hidden rate-dependent slowing in the contralateral bundle branch, resulting in normalization of the early conducted QRS complexes.

Syncope due to unexpected paroxysmal sinus arrest.

We present two cases with unexpected long sinus arrest resulting in syncope. There were no signs or symptoms of either sick sinus syndrome or increased vagal tone before the occurrence of pauses in these patients. The fact that these patients remained asymptomatic for quite long shows striking resemblance to paroxysmal sub-AV nodal block. We would therefore prefer to classify these cases as paroxysmal sinus arrest as a possible manifestation of the sick sinus syndrome.

An unusual ectopic ventricular rhythm in a young woman.

A case of a 22-year-old young pregnant woman with palpitations and near syncope is presented. Holter monitoring showed very frequent premature beats and runs of wide complex tachycardia, refractory to antiarrhythmic drugs. Electrophysiologic evaluation disclosed spontaneous automatism arising in an atriofascicular pathway. Differential diagnosis is discussed.

Unusual variants of pre-excitation: From anatomy to ablation: Part III-Clinical presentation, electrophysiologic characteristics, when and how to ablate nodoventricular, nodofascicular, fasciculoventricular pathways, along with considerations of permanent junctional reciprocating tachycardia.

The recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, but frequently a difficult, challenge for the clinical cardiac arrhythmologist. In this third part of our series of reviews, we discuss the different steps required to come to the correct diagnosis and management decision in patients with nodofascicular, nodoventricular, and fasciculo-ventricular pathways. We also discuss the concealed accessory atrioventricular pathways with the properties of decremental retrograde conduction that are associated with the so-called permanent form of junctional reciprocating tachycardia. Careful analysis of the 12-lead electrocardiogram during sinus rhythm and tachycardias should always precede the investigation in the catheterization room. When using programmed electrical stimulation of the heart from different intracardiac locations, combined with activation mapping, it should be possible to localize both the proximal and distal ends of the accessory connections. This, in turn, should then permit the determination of their electrophysiologic properties, providing the answer to the question "are they incorporated in a tachycardia circuit?". It is this information that is essential for decision-making with regard to the need for catheter ablation, and if necessary, its appropriate site.

Unusual variants of pre-excitation: From anatomy to ablation: Part I-Understanding the anatomy of the variants of ventricular pre-excitation.

The famous quotation of Winston Churchill, made in his radio broadcast of 1939 regarding Russia's next move, specifically "A riddle wrapped up in a mystery, inside an enigma," perfectly fits the current understanding of unusual accessory atrioventricular pathways, including the variants producing ventricular pre-excitation. It was many decades after their original descriptions that we came better to begin to understand most of their structure-function relationships. Their mysterious pathophysiology was sometimes unveiled after invasive treatments, such as surgical ablation of the atrioventricular conduction axis instead of the accessory pathway itself. Speculations made on this basis have largely been validated by subsequent clinical experience. Most of the names suggested for description of the pathways have stood well the test of time. For some of them, however, this is not the case, with the initial names becoming confusing. In a series of reviews, we re-visit those accessory pathways producing ventricular pre-excitation other than classical Wolff-Parkinson-White syndrome. To set the scene, in this initial review, we describe the development and anatomy of the normal atrioventricular conduction axis, along with the insulating tissues of the atrioventricular junctions. We have sought to illustrate our explanations by using virtual dissection of computerized tomographic datasets, since they retain the intact heart within the setting of the body. These images illustrate well the value of attitudinally appropriate terminology. Thereafter, we discuss the electrophysiological manifestations of the abnormal anatomical pathways which provide the potential for both accessory atrioventricular and intraventricular conduction.

Part II-Clinical presentation, electrophysiologic characteristics, and when and how to ablate atriofascicular pathways and long and short decrementally conducting accessory pathways.

Recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, frequently difficult, challenge for the clinical cardiac arrhythmologist. In this second part of our series of reviews relative to this topic, we discuss the steps required to achieve the correct diagnosis and appropriate management in patients with the so-called "Mahaim" variants of pre-excitation. We indicate that, nowadays, it is recognized that these abnormal rhythms are manifest because of the presence of atriofascicular pathways. These anatomical substrates, however, need to be distinguished from the other long and short accessory pathways which produce decremental atrioventricular conduction. The atriofascicular pathways, along with the long decrementally conducting pathways, have their atrial components located within the vestibule of the tricuspid valve. The short decremental pathways, in contrast, can originate in the vestibules of either the mitral or tricuspid valves. As a starting point, careful analysis of the 12-lead electrocardiogram, taken during both sinus rhythm and tachycardias, should precede any investigation in the catheterization room. When assessing the patient in the electrophysiological laboratory, the use of programmed electrical stimulation from different intracardiac locations, combined with entrainment technique and activation mapping, should permit the establishment of the properties of the accessory pathways, and localization of its proximal and distal ends. This should provide the answer to the question "is the pathway incorporated into the circuit underlying the clinical tachycardia". That information is essential for decision-making with regard to need, and localization of the proper site, for catheter ablation.

Atypical bypass tracts: can they be recognized during sinus rhythm?

Atypical bypass tracts or variants of ventricular pre-excitation are rare anatomic structures often with rate-dependent slowing in conduction, called decremental conduction. During sinus rhythm, electrocardiographic recognition of those structures may be difficult because unlike in the Wolff-Parkinson-White syndrome where usually overt ventricular pre-excitation is present, the electrocardiogram (ECG) often shows a subtle pre-excitation pattern because of less contribution to ventricular activation over the slow and decrementally conducting bypass. Following the structure described by Ivan Mahaim and Benatt corresponding to a fasciculoventricular pathway, several other new variants of ventricular pre-excitation were reported. In this review, we aim to discuss the electrocardiographic pattern of the different subtypes of variants of ventricular pre-excitation, including the atriofascicular pathway, long and short decrementally conducting atrioventricular pathways, fasciculoventricular pathway, the atrio-Hisian bypass tract, and nodoventricular and nodofascicular fibres. Emphasis will be on the ECG findings during sinus rhythm.

When and how does a single ventricular premature beat initiate and terminate supraventricular tachycardia?

BACKGROUND: The differential diagnosis of a supraventricular tachycardia (SVT) is accomplished using a number of pacing maneuvers. The incidence and mechanism of a single ventricular premature beat (VPB) on initiation and termination of tachycardia were evaluated during programmed electrical stimulation (PES) of the heart in patients with the two most common regular SVTs: atrioventricular re-entrant tachycardia (AVNRT) and orthodromic atrioventricular tachycardia (AVRT). METHODS: Three hundred and thirty-seven consecutive patients aged above 18 years with an inducible sustained AVNRT or AVRT were prospectively enrolled. Patients with more than one tachyarrhythmia mechanism were excluded. Two hundred and seventeen patients (64.4%) had typical slow/fast AVNRT and 120 (35.6%) had an orthodromic AVRT using a rapidly conducting accessory pathway for V-A conduction. In this cross-sectional study, we specifically report the analysis of tachycardia induction and termination by a single VPB. RESULTS: Tachycardia induction with a single VPB during sinus rhythm was seen in 7 of 120 AVRT and in only one of the 217 patients with AVNRT, (5.8% vs. 0.3%, p < 0.05). When a single VPB was delivered during basic ventricular pacing these values were 28% versus 4%, respectively, (p < 0.001). Termination of tachycardia by a single VPB was observed in nine (4.1%) patients with AVNRT and in 57 (47.5%) with AVRT (p < 0.001). CONCLUSION: Initiation of SVT by a single VPB during sinus rhythm was uncommon and favored AVRT. Termination of SVT by a single VPB was commonly seen in AVRT but rarely in AVNRT. These findings can be of help when interpreting a noninvasive arrhythmia event recording.

Reappraisal and new observations on atrial tachycardia ablated from the non-coronary aortic sinus of Valsalva.

Aims: The clinical characteristics of atrial tachycardias (AT) ablated from the non-coronary aortic sinus (NCS) are thus far described only in small series. We aimed to outline, in a large cohort of patients, the clinical, electrocardiographic, electrophysiological characteristics of this tachycardia. Methods and results: We analysed data pertaining to clinical, electrocardiographic, and electrophysiological characteristics of 43 consecutive patients from an overall cohort of 441 with AT who were successfully ablated from the NCS. The tachycardias ablated from the NCS were paroxysmal (98%) and adenosine sensitive (35/35). The patients were aged 54.6 ± 12.4 years, showing female preponderance (74%). No P wave pattern was predictive of the location of ablation. Electrophysiological findings suggested a possible micro-reentrant mechanism. During tachycardia, atrial electrograms recorded in the NCS preceded the A in the His region by 10.9 ± 7.4 ms. Fractionated atrial electrograms were noted at the site of ablation in 42 patients during the tachycardia. Radiofrequency ablation terminated the tachycardia within 5 s in 88%, with thermal automaticity seen only in 3 patients. The site of ablation at the base of the NCS was adjacent to the presumed site of the retroaortic node, a remnant of the initial atrio-ventricular canal musculature. Conclusions: AT ablated from the NCS is a paroxysmal arrhythmia in middle-aged women, with distinct electrocardiographic and electrophysiological characteristics. We suspect the retroaortic node to be involved in the tachycardia circuit.

The Maastricht-Duke bridge: An era of mentoring in clinical research - A model for mentoring in clinical research - A tribute to Dr. Galen Wagner.

OBJECTIVE: With the passing of Dr. Galen Wagner, an exceptional collaboration between Maastricht University Medical Center, The Netherlands, and Duke Clinical Research Institute, USA, has come to an end. This article focuses on the background of what Galen coined the Maastricht-Duke bridge (MD-bridge), its merits, limitations and development throughout the years, and his special role. METHODS: Between 2004 and 2015, 23 Maastricht University medical students and post-graduate students were enrolled in the 4-month research elective, mentored by Galen and the Maastricht co-mentor. They were asked to complete a survey about their MD-bridge experience. RESULTS: Sixteen out of the 23 students responded. None but 1 participant had prior research experience. Following their MD bridge-program most participants published 1 or more manuscripts and/or presented their research in an international setting. They felt they had full responsibility as a leader of their project with all participants developing meaningful skills useful in their current job. Fourteen out of 16 would recommend the MD-bridge experience to others. Participants considered the program of great value for their personal growth and independence, giving a feeling of achievement. In addition, for some participants it led to careers in foreign countries including medical practice and research, or obtaining PhDs. CONCLUSIONS: With Galen's impressive career of mentoring students, including the 23 MD-bridge participants, he has left behind an amazing concept of self-development in research and personal life. The successes of the MD-bridge prove that it is possible for students to be young investigators during or just after medical school with the potential to contribute to developing meaningful skills and noteworthy careers. Collaborations between international universities, such as the MD-bridge, are feasible and should be embraced by other institutions.

The value of the 12-lead electrocardiogram in localizing the scar in non-ischaemic cardiomyopathy.

AIMS: Patients with non-ischaemic cardiomyopathy (NICM) and ventricular tachycardia can be categorized as anteroseptal (AS) or inferolateral (IL) scar sub-types based on imaging and voltage mapping studies. The aim of this study was to correlate the baseline electrocardiogram (ECG) with endo-epicardial voltage maps created during ablation procedures and identify the ECG characteristics that may help to distinguish the scar as AS or IL. METHODS AND RESULTS: We assessed 108 baseline ECGs; 72 patients fulfilled criteria for dilated cardiomyopathy whereas 36 showed minimal structural abnormalities. Based on the unipolar low-voltage distribution, the scar pattern was classified as predominantly AS (n = 59) or IL (n = 49). Three ECG criteria (PR interval < 170 ms or QRS voltage in inferior leads <0.6 mV or a lateral q wave) resulted in 92% sensitivity and 90% specificity for predicting an IL pattern in patients with preserved ejection fraction (EF). The four-step algorithm for dilated cardiomyopathy included a paced ventricular rhythm or PR > 230 ms or QRS > 170 ms or an r ≤ 0.3 mV in V3 having 92 and 81% of sensitivity and specificity, respectively, in predicting AS scar pattern. A significant negative correlation was found between the extension of the endocardial unipolar low voltage area and left ventricular EF (rs = -0.719, P < 0.001). The extent of endocardial AS unipolar low voltage was correlated with PR interval and QRS duration (rs = 0.583 and rs = 0.680, P < 0.001, respectively) and the IL epicardial unipolar low voltage with the mean voltage of the limb leads (rs = -0.639, P < 0.001). CONCLUSION: Baseline ECG features are well correlated with the distribution of unipolar voltage abnormalities in NICM and may help to predict the location of scar in this population.

Risk stratification for sudden cardiac death: current status and challenges for the future.

Sudden cardiac death (SCD) remains a daunting problem. It is a major public health issue for several reasons: from its prevalence (20% of total mortality in the industrialized world) to the devastating psycho-social impact on society and on the families of victims often still in their prime, and it represents a challenge for medicine, and especially for cardiology. This text summarizes the discussions and opinions of a group of investigators with a long-standing interest in this field. We addressed the occurrence of SCD in individuals apparently healthy, in patients with heart disease and mild or severe cardiac dysfunction, and in those with genetically based arrhythmic diseases. Recognizing the need for more accurate registries of the global and regional distribution of SCD in these different categories, we focused on the assessment of risk for SCD in these four groups, looking at the significance of alterations in cardiac function, of signs of electrical instability identified by ECG abnormalities or by autonomic tests, and of the progressive impact of genetic screening. Special attention was given to the identification of areas of research more or less likely to provide useful information, and thereby more or less suitable for the investment of time and of research funds.