European Reference Network for Rare Vascular Diseases (VASCERN) position statement on cerebral screening in adults and children with hereditary haemorrhagic telangiectasia (HHT).

Hereditary haemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia inherited as an autosomal dominant trait. Approximately 10 % of patients have cerebral vascular malformations, a proportion being cerebral arteriovenous malformations (AVMs) and fistulae that may lead to potentially devastating consequences in case of rupture. On the other hand, detection and treatment related-risks are not negligible, and immediate. While successful treatment can be undertaken in individual cases, current data do not support the treatment of unruptured AVMs, which also present a low risk of bleeding in HHT patients. Screening for these AVMs is therefore controversial.Structured discussions, distinctions of different cerebrovascular abnormalities commonly grouped into an "AVM" bracket, and clear guidance by neurosurgical and neurointerventional radiology colleagues enabled the European Reference Network for Rare Vascular Disorders (VASCERN-HHT) to develop the following agreed Position Statement on cerebral screening:1) First, we emphasise that neurological symptoms suggestive of cerebral AVMs in HHT patients should be investigated as in general neurological and emergency care practice. Similarly, if an AVM is found accidentally, management approaches should rely on expert discussions on a case-by-case basis and individual risk-benefit evaluation of all therapeutic possibilities for a specific lesion.2) The current evidence base does not favour the treatment of unruptured cerebral AVMs, and therefore cannot be used to support widespread screening of asymptomatic HHT patients.3) Individual situations encompass a wide range of personal, cultural and clinical states. In order to enable informed patient choice, and avoid conflicting advice, particularly arising from non-neurovascular interpretations of the evidence base, we suggest that all HHT patients should have the opportunity to discuss knowingly brain screening issues with their healthcare provider.4) Any screening discussions in asymptomatic individuals should be preceded by informed pre-test review of the latest evidence regarding preventative and therapeutic efficacies of any interventions. The possibility of harm due to detection of, or intervention on, a vascular malformation that would not have necessarily caused any consequence in later life should be stated explicitly.We consider this nuanced Position Statement provides a helpful, evidence-based framework for informed discussions between healthcare providers and patients in an emotionally charged area.

Non-randomised comparison of acute and long-term outcomes of robotic versus manual ventricular tachycardia ablation in a single centre ischemic cohort.

INTRODUCTION: Robotically guided radiofrequency (RF) ablation offers greater catheter stability that may improve lesion depth. We performed a non-randomised comparison of patients undergoing ventricular tachycardia (VT) ablation either manually or robotically using the Hansen Sensei system for recurrent implantable defibrillator (ICD) therapy. METHODS: Patients with infarct-related scar underwent VT ablation using the Hansen system to assess feasibility compared with patients undergoing manual VT ablation during a similar time period. Power delivery during robotic ablation was restricted to 30 W at 60 s. VT inducibility was checked at the end of the procedure. Pre-ablation ICD therapy burdens over 6 months were compared with post-ablation therapy averaged to a 6-month period. RESULTS: Twelve consecutive patients who underwent robotic VT ablation were compared to 12 consecutive patients undergoing a manual ablation. Patient demographics and comorbidities were similar in the two groups. A higher proportion of robotic cases were urgent (9/12 (75%)) vs. manual (4/12 (33%)) (p = 0.1). Post-ablation VT stimulation did not induce clinical VT in 11/12 (92%) in each group. There were no peri-procedural complications related to ablation delivery. Patients were followed up for approximately 2 years. Averaged over 6 months, robotic ICD therapy burdens fell from 32 (5-400) events to 2.5 (0-11) (p = 0.015). Therapy burden fell from 14 (10-25) to 1 (0-5) (p = 0.023) in the manual group. There was no difference in long-term outcome (p = 0.60) and mortality (4/12 (33%), p = 1.0). CONCLUSION: Robotically guided VT ablation is both feasible and safe when compared to manual ablation with good acute and long-term outcomes.

Chest pain and palpitations: taking a closer look.

This case highlights the importance of considering a wide differential diagnosis in a young patient with chest pain and an abnormal ECG. Rarer causes of myocarditis such as GCM should be sought in patients who develop ventricular arrhythmias or high-grade heart block because the treatment is different and dramatically influences outcome. Our patient is the first reported case of GCM and a concurrent diagnosis of tuberculosis. It is most likely that the histological appearance of GCM was due to the presence of mycobacterial infection within the myocardium, and we believe that effective antituberculous therapy has led to resolution of the GCM without the need for continued long-term immunosuppression.

Hybrid iFR-FFR decision-making strategy: implications for enhancing universal adoption of physiology-guided coronary revascularisation.

AIMS: Adoption of fractional flow reserve (FFR) remains low (6-8%), partly because of the time, cost and potential inconvenience associated with vasodilator administration. The instantaneous wave-Free Ratio (iFR) is a pressure-only index of stenosis severity calculated without vasodilator drugs. Before outcome trials test iFR as a sole guide to revascularisation, we evaluate the merits of a hybrid iFR-FFR decision-making strategy for universal physiological assessment. METHODS AND RESULTS: Coronary pressure traces from 577 stenoses were analysed. iFR was calculated as the ratio between Pd and Pa in the resting diastolic wave-free window. A hybrid iFR-FFR strategy was evaluated, by allowing iFR to defer some stenoses (where negative predictive value is high) and treat others (where positive predictive value is high), with adenosine being given only to patients with iFR in between those values. For the most recent fixed FFR cut-off (0.8), an iFR of <0.86 could be used to confirm treatment (PPV of 92%), whilst an iFR value of >0.93 could be used to defer revascularisation (NPV of 91%). Limiting vasodilator drugs to cases with iFR values between 0.86 to 0.93 would obviate the need for vasodilator drugs in 57% of patients, whilst maintaining 95% agreement with an FFR-only strategy. If the 0.75-0.8 FFR grey zone is accounted for, vasodilator drug requirement would decrease by 76%. CONCLUSION: A hybrid iFR-FFR decision-making strategy for revascularisation could increase adoption of physiology-guided PCI, by more than halving the need for vasodilator administration, whilst maintaining high classification agreement with an FFR-only strategy.

Fast pathway ablation using cryotherapy for a patient with a long PR interval in sinus rhythm and AVNRT.

A 53-year-old man with previous aortic valve surgery presented with paroxysmal narrow complex tachycardia, induced by exercise. His PR interval was greater than 400 ms when in sinus rhythm and atrioventricular nodal reentry tachycardia (AVNRT) was diagnosed with invasive electrophysiological studies. Single echoes were repeatedly inducible with single-paced extrastimuli. Cryotherapy was then used to ablate the fast pathway using single echoes to monitor anterograde slow pathway and retrograde fast pathway function during ablation.

Development and validation of a new adenosine-independent index of stenosis severity from coronary wave-intensity analysis: results of the ADVISE (ADenosine Vasodilator Independent Stenosis Evaluation) study.

OBJECTIVES: The purpose of this study was to develop an adenosine-independent, pressure-derived index of coronary stenosis severity. BACKGROUND: Assessment of stenosis severity with fractional flow reserve (FFR) requires that coronary resistance is stable and minimized. This is usually achieved by administration of pharmacological agents such as adenosine. In this 2-part study, we determine whether there is a time when resistance is naturally minimized at rest and assess the diagnostic efficiency, compared with FFR, of a new pressure-derived adenosine-free index of stenosis severity over that time. METHODS: A total of 157 stenoses were assessed. In part 1 (39 stenoses), intracoronary pressure and flow velocity were measured distal to the stenosis; in part 2 (118 stenoses), intracoronary pressure alone was measured. Measurements were made at baseline and under pharmacologic vasodilation with adenosine. RESULTS: Wave-intensity analysis identified a wave-free period in which intracoronary resistance at rest is similar in variability and magnitude (coefficient of variation: 0.08 ± 0.06 and 284 ± 147 mm Hg s/m) to those during FFR (coefficient of variation: 0.08 ± 0.06 and 302 ± 315 mm Hg s/m; p = NS for both). The resting distal-to-proximal pressure ratio during this period, the instantaneous wave-free ratio (iFR), correlated closely with FFR (r = 0.9, p < 0.001) with excellent diagnostic efficiency (receiver-operating characteristic area under the curve of 93%, at FFR <0.8), specificity, sensitivity, negative and positive predictive values of 91%, 85%, 85%, and 91%, respectively. CONCLUSIONS: Intracoronary resistance is naturally constant and minimized during the wave-free period. The instantaneous wave-free ratio calculated over this period produces a drug-free index of stenosis severity comparable to FFR. (Vasodilator Free Measure of Fractional Flow Reserve [ADVISE]; NCT01118481).

Pulmonary venous isolation by antral ablation with a large cryoballoon for treatment of paroxysmal and persistent atrial fibrillation: medium-term outcomes and non-randomised comparison with pulmonary venous isolation by radiofrequency ablation.

BACKGROUND: To prevent atrial fibrillation (AF) recurrence after catheter ablation, pulmonary venous isolation (PVI) at an antral level is more effective than segmental ostial ablation. Cryoablation around the pulmonary venous (PV) ostia for AF therapy is potentially safer compared to radiofrequency ablation (RFA). The aim of this study was to investigate the efficacy of a strategy using a large cryoablation balloon to perform antral cryoablation with 'touch-up' ostial cryoablation for PVI in patients with paroxysmal and persistent AF. METHODS: Paroxysmal and persistent AF patients undergoing their first left atrial ablation were recruited. After cryoballoon therapy, each PV was assessed for isolation and if necessary, treated with focal ostial cryoablation until PVI was achieved. Follow-up with Holter monitoring was performed. Clinical outcomes of the cryoablation protocol were compared, with consecutive patients undergoing PVI by RFA. RESULTS: 124 consecutive patients underwent cryoablation. 77% of paroxysmal and 48% of persistent AF subjects were free from AF at 12 months after a single procedure. Over the same time period, 53 consecutive paroxysmal AF subjects underwent PVI with RFA and at 12 months, 72% were free from AF at 12 months (p=NS). There were too few persistent AF subjects (n=8) undergoing solely PVI by RFA as a comparison group. Procedural and fluoroscopic times during cryoablation were significantly shorter than RFA. CONCLUSIONS: PV isolation can be achieved in less than 2 h by a simple cryoablation protocol with excellent results after a single intervention, particularly for paroxysmal AF.