Interatrial Septal Mass Secondary to Rosai-Dorfman Disease: A Rare Cause of Complete Heart Block.

Varying degrees of atrioventricular block can be associated with old age or a manifestation of an ischemic, metabolic, or infective pathology. In patients with no clear explanation, it is important to investigate secondary causes. Our case describes the first case of an adult with Rosai-Dorfman histiocytosis presenting with complete heart block. (Level of Difficulty: Advanced.).

Is heart failure with mid range ejection fraction (HFmrEF) a distinct clinical entity or an overlap group?

BACKGROUND: The new category of heart failure (HF), Heart Failure with mid range Ejection Fraction (HFmrEF) has recently been proposed with recent publications reporting that HFmrEF represents a transitional phase. The aim of this study was to determine the prevalence and clinical characteristics of patients with HFmrEF and to establish what proportion of patients transitioned to other types of HF, and how this affected clinical outcomes. METHODS AND RESULTS: Patients were diagnosed with HF according to the 2016 ESC guidelines. Clinical outcomes and variables were recorded for all consecutive in-patients referred to the heart failure service. In total, 677 patients with new HF were identified; 25.6% with HFpEF, 21% with HFmrEF and 53.5% with HFrEF. While clinical characteristics and prognostic factors of HFmrEF were intermediate between HFrEF and HFpEF, HFmrEF patients had the best outcome, with higher mortality in the HFrEF population (p 0.02) and higher HF rehospitalisation rates in the HFpEF population (p < 0.01).38.7% of the HFmrEF patients transitioned (56.4% to HFpEF and 43.6% to HFrEF) with fewest deaths in the patients that transitioned to HFpEF (p 0.04), and fewest HF readmissions in the patients that remained as HFmrEF (<0.01). CONCLUSION: HFmrEF patients had the best outcomes, compared to high rates of mortality seen in patients with HFrEF and high rates of HF readmissions seen in patients with HFpEF. Only 1/3 of HFmrEF patients transitioned during follow up, with the lowest mortality seen in patients transitioning to HFpEF.

A cost effectiveness study establishing the impact and accuracy of implementing the NICE guidelines lowering plasma NTproBNP threshold in patients with clinically suspected heart failure at our institution.

AIMS: The 2014 National Institute of Clinical Excellence (NICE) guidelines on the management of acute heart failure recommended using a plasma NT-proBNP threshold of 300pg/ml to assist in ruling out the diagnosis of heart failure (HF), updating previous guidelines recommending using a threshold of 400pg/ml. NICE based their recommendations on 6 studies performed in other countries. This study sought to determine the diagnostic and economic implications of using these thresholds in a large unselected UK population. METHODS: Patient and clinical demographics were recorded for all consecutive suspected HF patients over 12months, as well as clinical outcomes including time to HF hospitalisation and time to death (follow up 15.8months). RESULTS: Of 1995 unselected patients admitted with clinically suspected HF, 1683 (84%) had a NTproBNP over the current NICE recommended threshold, of which 35% received a final diagnosis of HF. Lowering the threshold from 400 to 300pg/ml would have involved screening an additional 61 patients and only would have identified one new patient with HF (sensitivity 0.985, NPV 0.976, area under the curve (AUC) at 300pg/ml 0.67; sensitivity 0.983, NPV 0.977, AUC 0.65 at 400pg/ml). The economic implications of lowering the threshold would have involved additional costs of £42,842.04 (£702.33 per patient screened, or £ 42,824.04 per new HF patient). CONCLUSION: Applying the recent updated NICE guidelines to an unselected real world population increases the AUC but would have a significant economic impact and only identified one new patient with heart failure.

The role of multi modality imaging in selecting patients and guiding lead placement for the delivery of cardiac resynchronization therapy.

INTRODUCTION: Cardiac resynchronization therapy (CRT) is an effective pacemaker delivered treatment for selected patients with heart failure with the target of restoring electro-mechanical synchrony. Imaging techniques using echocardiography have as yet failed to find a metric of dyssynchrony to predict CRT response. Current guidelines are thus unchanged in recommending prolonged QRS duration, severe systolic function and refractory heart failure symptoms as criteria for CRT implantation. Evolving strain imaging techniques in 3D echocardiography, cardiac MRI and CT may however, overcome limitations of older methods and yield more powerful CRT response predictors. Areas covered: In this review, we firstly discuss the use of multi modality cardiac imaging in the selection of patients for CRT implantation and predicting the response to CRT. Secondly we examine the clinical evidence on avoiding areas of myocardial scar, targeting areas of dyssynchrony and in doing so, achieving the optimal positioning of the left ventricular lead to deliver CRT. Finally, we present the latest clinical studies which are integrating both clinical and imaging data with X-rays during the implantation in order to improve the accuracy of LV lead placement. Expert commentary: Image integration and fusion of datasets with live X-Ray angiography to guide procedures in real time is now a reality for some implanting centers. Such hybrid facilities will enable users to interact with images, allowing measurement, annotation and manipulation with instantaneous visualization on the catheter laboratory monitor. Such advances will serve as an invaluable adjunct for implanting physicians to accurately deliver pacemaker leads into the optimal position to deliver CRT.

Early experience with the Occlutech PLD occluder for mitral paravalvar leak closure through a hybrid transapical approach.

AIMS: We sought to evaluate the feasibility and efficacy of hybrid transapical closure of paravalvar mitral leaks using a new Occlutech PLD occluder in patients with heart failure and/or haemolytic anaemia. METHODS AND RESULTS: Retrospective analysis of clinical and procedural data was undertaken for patients who had attempted closure of paravalvar mitral leaks via a hybrid transapical approach with the Occlutech PLD occluder. Eight patients (four males, median age 69 years) underwent closure of 10 mitral paravalvar leaks using eight Occlutech PLD occluders and two AMPLATZER Vascular Plugs (AVP II). Successful deployment, with significant reduction of the paravalvar leak was achieved in seven patients with short procedure (median 131 min) and fluoroscopy times (median 22 min). One patient had mechanical interference with prosthetic valve function, requiring surgery. Another patient with a high EuroSCORE (48.8%) died of multi-organ failure two days after the procedure. Clinical improvement in either heart failure or haemolysis was seen in all discharged patients. CONCLUSIONS: In our series of patients with challenging anatomy, the Occlutech PLD occluders performed well when implanted via a hybrid transapical approach. Further work is needed to assess this methodology fully for a wider population and to assess other deployment approaches for this promising new occluder.

Novel System for Real-Time Integration of 3-D Echocardiography and Fluoroscopy for Image-Guided Cardiac Interventions: Preclinical Validation and Clinical Feasibility Evaluation.

Real-time imaging is required to guide minimally invasive catheter-based cardiac interventions. While transesophageal echocardiography allows for high-quality visualization of cardiac anatomy, X-ray fluoroscopy provides excellent visualization of devices. We have developed a novel image fusion system that allows real-time integration of 3-D echocardiography and the X-ray fluoroscopy. The system was validated in the following two stages: 1) preclinical to determine function and validate accuracy; and 2) in the clinical setting to assess clinical workflow feasibility and determine overall system accuracy. In the preclinical phase, the system was assessed using both phantom and porcine experimental studies. Median 2-D projection errors of 4.5 and 3.3 mm were found for the phantom and porcine studies, respectively. The clinical phase focused on extending the use of the system to interventions in patients undergoing either atrial fibrillation catheter ablation (CA) or transcatheter aortic valve implantation (TAVI). Eleven patients were studied with nine in the CA group and two in the TAVI group. Successful real-time view synchronization was achieved in all cases with a calculated median distance error of 2.2 mm in the CA group and 3.4 mm in the TAVI group. A standard clinical workflow was established using the image fusion system. These pilot data confirm the technical feasibility of accurate real-time echo-fluoroscopic image overlay in clinical practice, which may be a useful adjunct for real-time guidance during interventional cardiac procedures.

Multi-site left ventricular pacing as a potential treatment for patients with postero-lateral scar: insights from cardiac magnetic resonance imaging and invasive haemodynamic assessment.

AIMS: Multi-site left ventricular (LV) pacing may be superior to single-site stimulation in correcting dyssynchrony and avoiding areas of myocardial scar. We sought to characterize myocardial scar using cardiac magnetic resonance imaging (CMR). We aimed to quantify the acute haemodynamic response to single-site and multi-site LV stimulation and to relate this to the position of the LV leads in relation to myocardial scar. METHODS: Twenty patients undergoing cardiac resynchronization therapy had implantation of two LV leads. One lead (LV1) was positioned in a postero-lateral vein, the second (LV2) in a separate coronary vein. LV dP/dtmax was recorded using a pressure wire during stimulation at LV1, LV2, and both sites simultaneously (LV1 + 2). Patients were deemed acute responders if ΔLV dP/dtmax was ≥ 10%. Cardiac magnetic resonance imaging was performed to assess dyssynchrony as well as location and burden of scar. Scar anatomy was registered with fluoroscopy to assess LV lead position in relation to scar. RESULTS: LV dP/dtmax increased from 726 ± 161 mmHg/s in intrinsic rhythm to 912 ± 234 mmHg/s with LV1, 837 ± 188 mmHg/s with LV2, and 932 ± 201 mmHg/s with LV1 and LV2. Nine of 19 (47%) were acute responders with LV1 vs. 6/19 (32%) with LV2. Twelve of 19 (63%) were acute responders with simultaneous LV1 + 2. Two of three patients benefitting with multi-site pacing had the LV1 lead positioned in postero-lateral scar. CONCLUSION: Multi-site LV pacing increased acute response by 16% vs. single-site pacing. This was particularly beneficial in patients with postero-lateral scar identified on CMR.

The acute hemodynamic response to LV pacing within individual branches of the coronary sinus using a quadripolar lead.

BACKGROUND: It is not clear whether there is a large difference in acute hemodynamic response (AHR) to left ventricle (LV) pacing in different regions of the same coronary sinus (CS) vein. Using the four electrodes available on a Quartet LV lead, we evaluated the AHR to pacing within individual branches of the CS. METHODS: An acute hemodynamic study was attempted in 20 patients. In each patient, we assessed AHR in a number of CS veins and along a significant proportion of each CS branch using three different bipolar configurations. We compared the AHR achieved when pacing using each different vector and also the highest AHR achieved in any position within the same patient with the lowest achieved in that patient. RESULTS: Sixty-four different CS positions in 19 patients were successfully assessed. No significant difference in AHR was found overall between the three vectors tested. The mean percentage difference in AHR between the CS branch vectors with the lowest and highest dP/dt(max) was +6.5 ± 5.4% (P < 0.001). A much larger difference of +16.9 ± 6.1% (P < 0.001) was seen when comparing the highest and lowest AHR achieved using any vector in any position within the same patient. CONCLUSION: A small difference in AHR is seen when pacing within the same branch of the CS compared to pacing in different branches in the same patient. This suggests that although the site of LV lead placement is important, the position within a CS branch is less important than choosing the right vein.

Real-time 3D echo in patient selection for cardiac resynchronization therapy.

OBJECTIVES: this study investigated the use of 3-dimensional (3D) echo in quantifying left ventricular mechanical dyssynchrony (LVMD), its interhospital agreement, and potential impact on patient selection. BACKGROUND: assessment of LVMD has been proposed as an improvement on conventional criteria in selecting patients for cardiac resynchronization therapy (CRT). Three-dimensional echo offers a reproducible assessment of left ventricular (LV) structure, function, and LVMD and may be useful in selecting patients for this intervention. METHODS: we studied 187 patients at 2 institutions. Three-dimensional data from baseline and longest follow-up were quantified for volume, left ventricular ejection fraction (LVEF), and systolic dyssynchrony index (SDI). New York Heart Association (NYHA) functional class was assessed independently. Several outcomes from CRT were considered: 1) reduction in NYHA functional class; 2) 20% relative increase in LVEF; and 3) 15% reduction in LV end-systolic volume. Sixty-two cases were shared between institutions to analyze interhospital agreement. RESULTS: there was excellent interhospital agreement for 3D-derived LV end-diastolic and end- systolic volumes, EF, and SDI (variability: 2.9%, 1%, 7.1%, and 7.6%, respectively). Reduction in NYHA functional class was found in 78.9% of patients. Relative improvement in LVEF of 20% was found in 68% of patients, but significant reduction in LV end-systolic volume was found in only 41.5%. The QRS duration was not predictive of any of the measures of outcome (area under the curve [AUC]: 0.52, 0.58, and 0.57 for NYHA functional class, LVEF, and LV end-systolic volume), whereas SDI was highly predictive of improvement in these parameters (AUC: 0.79, 0.86, and 0.66, respectively). For patients not fulfilling traditional selection criteria (atrial fibrillation, QRS duration <120 ms, or undergoing device upgrade), SDI had similar predictive value. A cutoff of 10.4% for SDI was found to have the highest accuracy for predicting improvement following CRT. CONCLUSIONS: the LVMD quantification by 3D echo is reproducible between centers. SDI was an excellent predictor of response to CRT in this selected patient cohort and may be valuable in identifying a target population for CRT irrespective of QRS morphology and duration.

Percutaneous closure of an atrial septal defect guided by live three-dimensional transesophageal echocardiography.

A 55-year-old man with a history of cryptogenic stroke presented to the authors' department for investigation. On transthoracic echocardiography, he was found to have a small secundum atrial septal defect, and transesophageal echocardiography was performed for a more detailed assessment. Following this, the defect was deemed suitable for percutaneous closure. The case demonstrates the utility and benefits of live three-dimensional transesophageal echocardiography for the assessment of this type of defect and guidance of transcatheter closure.

Echo determinants of dyssynchrony (atrioventricular and inter- and intraventricular) and predictors of response to cardiac resynchronization therapy.

Cardiac resynchronization therapy (CRT) has revolutionized not only the treatment of chronic heart failure but also how we assess left ventricular (LV) dysfunction on echo. Increasingly, it has become clear that identifying and quantifying delays in events during the cardiac cycle is an important assessment in LV dysfunction as it has prognostic implications for patients undergoing CRT. The delays in atrioventricular, right-to-left ventricular, and LV segmental contraction have been shown to be important components in cardiac performance, and this review provides an overview of the commonest methods used for these assessments and their implications for selecting patients for biventricular pacing.

Optimization of cardiac resynchronization therapy.

Cardiac resynchronization is now an accepted and widespread therapy for patients with left ventricular (LV) systolic dysfunction. However, there are still a significant number of patients that do not appear to gain benefit, and this is currently the focus of a great deal of research. Contemporary resynchronization devices allow manipulation of both atrioventricular (AV) and ventricular-to-ventricular (VV) delays and there is evidence that optimization of these delays has a positive effect on hemodynamics. However, there are many ways that optimization can be performed and there is little consensus on how, if at all, it should be incorporated into clinical practice.