Simone Romano

The European Lead Extraction ConTRolled (ELECTRa) study: a European Heart Rhythm Association (EHRA) Registry of Transvenous Lead Extraction Outcomes

Aims The European Lead Extraction ConTRolled Registry (ELECTRa), is a prospective registry of consecutive transvenous lead extraction (TLE) procedures conducted by the European Heart Rhythm Association (EHRA) in order to identify the safety and efficacy of the current practice of TLE. Methods and results European centres performing TLE, invited by the organizing committee on behalf of EHRA, prospectively recruited all consecutive patients undergoing TLE at their institution. The primary endpoint was TLE safety defined by pre-discharge major procedure-related complications including death. Secondary endpoints included clinical and radiological success and overall complication rates. Outcomes were compared between Low Volume (LoV) vs. High Volume (HiV) centers (LoV < 30 and HiV ≥ 30 procedures/year). A total of 3555 consecutive patients (pts) of whom 3510 underwent TLE at 73 centres in 19 European countries were enrolled between November 2012 and May 2014. The primary endpoint of in-hospital procedure-related major complication rate was 1.7% [95% CI 1.3-2.1%] (58/3510 pts) including a mortality of 0.5% [95% CI 0.3-0.8%] (17/3510 pts). Approximately two-thirds (37/58) of these complications occurred during the procedure and one-third (21/58) in the post-operative period. The most common procedure related complications were those requiring pericardiocentesis or chest tube and/or surgical repair (1.4% [95% CI 1.0-1.8%]). Complete clinical and radiological success rates were 96.7% [95% CI 96.1-97.3%] and 95.7% [95% CI 95.2-96.2%], respectively. The all cause in-hospital major complications and deaths were significantly lower in HiV centres vs. LoV centres (2.4% [95% CI 1.9-3.0%] vs. 4.1% [95% CI 2.7-6.0%], P = 0.0146; and 1.2% [95% CI 0.8-1.6%] vs. 2.5% [95% CI 1.5-4.1%] P = 0.0088), although those related to the procedure did not reach statistical significance. Radiological and clinical successes were more frequent in HiV vs. LoV centres. Conclusion The ELECTRa study is the largest prospective registry on TLE and confirmed the safety and efficacy of the current practice of TLE. Lead extraction was associated with a higher success rate with lower all cause complication and mortality rates in high volume compared with low volume centres.

Abdominal Pain-Associated Functional Gastrointestinal Disorder Prevalence in Children and Adolescents with Celiac Disease on Gluten-Free Diet: A Multinational Study

Objective To test the hypothesis that children with celiac disease (CD) on gluten‐free diet are at increased risk of abdominal pain (AP) associated‐functional gastrointestinal disorders (FGIDs). Study design This was a multinational cross‐sectional study performed from 2014 to 2015. Patients 4‐18 years of age with CD on gluten‐free diet for longer than 6 months were recruited from pediatric CD clinics in US and Italy. Control groups included siblings of children with CD (with normal tissue transglutaminase levels) and unrelated controls. Subjects or parents completed the Questionnaire on Pediatric Gastrointestinal Symptoms‐Rome III. Results Children (n = 289) were recruited (55% US, 45% Italy): 96 children with CD, 96 sibling controls, and 97 unrelated controls. Chronic AP was present in 30 (30.9%) subjects with CD, 22 (22.7%) sibling controls, and 21 (21.6%) unrelated controls (P = .26 patients with CD vs siblings; P = .18 patients with CD vs unrelated; P = .96 siblings vs unrelated). AP‐FGIDs were present in 8 (8.2%) subjects with CD, 8 (8.2%) sibling controls, and 2 (2.1%) unrelated controls (P = 1.00 subjects with CD vs sibling controls; P = .06 subjects with CD vs unrelated controls; P = .06 sibling controls vs unrelated controls). Conclusion This multinational study evaluated the prevalence of chronic abdominal pain and AP‐FGIDs in the pediatric population with CD. We found that subjects with CD and controls have a similar prevalence of chronic AP and AP‐FGIDs. This suggests that not all types of gastrointestinal inflammation result in AP‐FGIDs in children.

Association of Feature-Tracking Cardiac Magnetic Resonance Imaging Left Ventricular Global Longitudinal Strain with All-Cause Mortality in Patients with Reduced Left Ventricular Ejection Fraction

Direct assessment of myocardial fiber deformation with echocardiographic global longitudinal strain (GLS) has shown promise in providing prognostic information that is incremental to ejection fraction (EF) in patients with left ventricular dysfunction.1 Cardiac magnetic resonance (CMR) imaging has now evolved into a major tool for evaluation of patients with left ventricular dysfunction, providing precise measurements of EF and viability assessment with late gadolinium enhancement (LGE). Recent development of CMR feature-tracking techniques now allow assessment of GLS from standard cine CMR images without the need for specialized pulse sequences or additional scanning time.2 We therefore hypothesized that CMR feature-tracking derived GLS may provide incremental prognostic information in these patients. Consecutive patients (n=470) undergoing CMR with both cine and LGE imaging for evaluation of ischemic or nonischemic dilated cardiomyopathy with EF <50% were included in this retrospective study. Comprehensive phenotyping, including clinical history, imaging, and cardiac catheterization, classified 330 individuals with ischemic and 140 with nonischemic cardiomyopathy. Institutional review board approval was obtained. Steady-state free-precession cine images were acquired in multiple short-axis and 3 long-axis views. LGE imaging was performed 10 to 15 minutes after Gadolinium contrast (0.15 mmol/kg) administration using a 2D-segmented gradient echo inversion recovery sequence in …

Succinate dehydrogenase gene mutation with cardiac paraganglioma: multimodality imaging and pathological correlation

Simone Romano, Cristiano Fava, Pietro Minuz, and Afshin Farzaneh-Far* Division of Cardiology, Department of Medicine, University of Illinois at Chicago, Chicago, IL; Department of Internal Medicine, University of Verona, Verona, Italy; and Division of Cardiology, Department of Medicine, Duke University, Durham, NC, USA * Corresponding author. Tel: 312-996-6730, Fax: 312-413-2948, Email: afshin@uic.edu

AJM onlineImages in radiologyResistant Hypertension

DIAGNOSIS Three-dimensional contrast-enhanced magnetic resonance angiography showed significant focal aortic coarctation, just beyond the origin of the left subclavian artery (Figure 1 and Online Video 1). Coarctation is a vascular disease with narrowing of the aorta and is a common congenital malformation, accounting for up to approximately 1 in 2500 live births. It can be classified anatomically on the basis of its location relative to the fetal ductus arteriousus as preductal, juxtaductal, or postductal. The postductal and juxtaductal types are the most common forms seen in adults. The majority of patients (50%-85%) have associated bicuspid aortic valve disease, as in this case (Figure 2 and Online Video 2) with an associated aortic ejection click on physical examination.

Reversal of right-ventricular dysfunction in pulmonary arterial hypertension following sildenafil therapy.

A 42-year-old-woman with known chronic pulmonary-sarcoidosis on long-term prednisone and home-oxygen therapy was referred to cardiology with symptoms of exertional chest tightness. Physical examination was notable for a palpable right-ventricular (RV) heave and a loud second heart sound (P2). Plasma B-natriuretic peptide (BNP) levels were elevated at 183 pg/mL. Transthoracic-echocardiography was suggestive of severe RV enlargement and systolic dysfunction with an estimated pulmonary systolic pressure of 60 mmHg. Cardiac magnetic resonance imaging confirmed severe RV enlargement (RVEDV = 218 mL) …

Left ventricular long-axis function assessed with cardiac cine mr imaging is an independent predictor of all-cause mortality in patients with reduced ejection fraction: A multicenter study

Purpose To evaluate the prognostic value of a simple index of left ventricular (LV) long-axis function-lateral mitral annular plane systolic excursion (MAPSE)-in a large multicenter population of patients with reduced ejection fraction (EF) who were undergoing cardiac magnetic resonance (MR) imaging. Materials and Methods This retrospective study included 1040 consecutive patients (mean age, 59.5 years ± 15.8) at four U.S. medical centers who were undergoing cardiac MR imaging for assessment of LV dysfunction with EF less than 50%. Lateral MAPSE was measured in the four-chamber cine view. The primary end point was all-cause death. Cox proportional hazards regression modeling was used to examine the independent association between lateral MAPSE and death. The incremental prognostic value of lateral MAPSE was assessed in nested models. Results During a median follow-up of 4.4 years, 132 patients died. With Kaplan-Meier analysis, the risk of death increased significantly with decreasing tertiles of lateral MAPSE (log-rank P = .0001). Patients with relatively preserved lateral MAPSE (>9 mm) had very few deaths, regardless of whether their EF was above or below 35%. Patients with late gadolinium enhancement (LGE) and low lateral MAPSE had significantly reduced survival compared to those with LGE and high lateral MAPSE (log-rank P < .0001). Lateral MAPSE was independently associated with risk of death after adjustment for clinical and imaging risk factors, which were univariate predictors (age, body mass index, diabetes, LV end-diastolic volume index, LGE, EF) (hazard ratio = 2.051 per mm decrease; 95% confidence interval [CI]: 1.520, 2.768; P < .001). Inclusion of lateral MAPSE in this model resulted in significant improvement in model fit (likelihood ratio test P < .0001) and C statistic (increasing from 0.675 to 0.844; P < .0001). Continuous net reclassification improvement was 1.036 (95% CI: 0.878, 1.194). Conclusion Lateral MAPSE measured during routine cine cardiac MR imaging is a significant independent predictor of mortality in patients with LV dysfunction, incremental to common clinical and cardiac MR risk factors-including EF and LGE.

Proarrhythmic effect of pacing mode reprogramation in a patient with a congenital long-QT syndrome.

A 34-year-old woman with a congenital long-QT syndrome (LQTS) type 2, implanted 10 years before with a single chamber implantable defibrillator (ICD) for secondary prevention, was admitted to our hospital to remove the malfunctioning ventricular ICD lead (Endotak Reliance S 0128, Boston Scientific, St. Paul, MN, USA). She had been previously treated with a sympatechtomy in 1989 and with a single chamber pacemaker (PM) implantation in 1991. In 2004, the PM had been transvenously removed and replaced with a single chamber ICD. Medical therapy included betablockers and mexiletine. No further arrhythmic episodes had been documented under medical therapy and perma-

Cardiac MRI for Detection of Cardiac Sarcoidosis

Online supplemental material is available for this article.

Life-saving maneuvers during a tilt test

The vasovagal syncope is a frequent clinical problem, representing 21 % of all types of syncope; this percentage is probably underestimated since 36.6 % of vasovagal syncope is of unknown origin [1]. The cardio-inhibitory syncope is classified as type 2 vasovagal syncope (VASIS classification), and can be triggered by the tilt test. It is defined as asystolic (type 2B; 4–33 %) when asystole occurs for more than 3 s. Occasionally, asystole can last more than 5 s (9.1 %); however, only very few cases are reported in the literature of asystole duration[30 s [2] and notably, none of those reported required life-saving maneuvers. In the present report, we describe the cases of a 32-yearold woman and a 31-year-old man with a history of several syncope episodes of uncertain origin, for which there was an indication to obtain a tilt test. The syncopal episodes in both cases looked similar to vasovagal episodes, as they occurred in the standing position with few presyncopal symptoms. No one noted an absence of heart beat. In addition, no elements of fear were reported or predictive signs of an asystolic event. The tilt test was performed according to the so-called ‘‘Italian protocol,’’ i.e., both patients, free from drugs, were administered sublingual nitroglycerin after 20 min of passive orthostatic standing at 60 . A few minutes later, the patients had an asystolic syncopal episode (Fig. 1), which lasted approximately 40 and 42 s (during which we recorded a few ventricular escape beats). Placing the patients in the Trendelenburg position did not restore the sinus rhythm, therefore, external cardiac massage was provided. The sinus rhythm resumed after a few seconds, likely because of the cardiac massage. Although syncopal recurrences are common, and may clearly decrease the quality of life, tilt-induced asystole does not necessarily imply increased risk of death, or the need to undergo major therapeutic procedures. In addition, syncope recurrence in patients with tilt-induced asystole is comparable to that in patients who did not have an asystolic response during tilt testing [2]. The treatment for patients with vasovagal syncope with asystole is the same as for patients with other forms of vasovagal syncope, including all preventive measures related to life style. It has been reported that pharmacological therapy does not significantly influence the clinical outcome [2]. Pacemaker implantation could be relatively beneficial only in the vasovagal asystolic syncope although its real-life effectiveness has not been demonstrated [3]. Thus, it should be considered as the last option in a selected number of patients with recurrent syncopal episodes, who do not respond to behavioral therapies, and who have their quality of life compromised [3]. Here, we report that in a cohort of 100 individuals subjected to the tilt test, we observed two very long-lasting, non-spontaneously resolving asystole during vasovagal syncope, both lasted more than 30 s, and both required cardiopulmonary resuscitation. Such prolonged events, although reported in the literature, are very unusual. In addition, in all reported cases, the restoration of sinus rhythm occurred spontaneously, just placing the patient in Trendelenburg position without the need for life-saving & Simone Romano simone.romano83@hotmail.com