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An 80-year-old woman received a dual chamber pacemaker (Boston Scientific Accolade MRI DR) for pre-syncopal episodes associated with transient type 1 and 2:1 II degree atrioventricular block, recorded in 24-hour Holter monitoring. Due to residual AV conduction, pacemaker was set with RYTHMIQ algorithm, in order to reduce inappropriate ventricular pacing. A month later, the patient began to complain of severe asthenia and bradycardia (46-48 bpm). Telemetry-supported pacemaker control revealed III degree AV block with junctional escape rhythm, unmasking failing to switch of RYTHMIQ algorithm. Why did it happen? The RythmIQ algorithm has been designed to promote intrinsic conduction in Boston Scientific devices. With RythmIQ the device provides AAI(R)[i] at the lower rate limit and/or sensor indicated rate. It provides backup VVI pacing at a rate 15 bpm slower than the programmed lower rate limit, with backup VVI pacing rate limited to no slower than 30 bpm and no faster than 60 bpm. If 3 slow ventricular beats are detected in a window of 11 beats, the device automatically switches to DDD(R) mode. A slow beat is defined as a ventricular paced or ventricular sensed event that is at least 150 ms slower than the AAI(R) pacing rate. Slow ventricular beats are any of the following: - V paced event- V sensed event > AAI lower rate limit + 150 ms- V sensed event > AAI(R) sensor indicated rate + 150 ms In our case LRL was set at 50 bpm (1200 ms). During III degree AV block, patient's ventricular rate was always about 46-48 bpm, due to stable junctional escape rhythm. Thus no ventricular sensed event occurred with a V-V cycle greater than AAI lower rate limit + 150 ms, namely 1350 ms (around 44 bpm). Moreover the device didn't provide backup VVI pacing at a rate 15 bpm slower, namely 35 bpm. This two conditions impaired Rhythm IQ switching from AAI with VVI backup to DDD. The device was set manually in DDD with restoring of AV synchrony and adequate ventricular rate followed by complete resolution of symptoms. Rhythm-IQ algorithm doesn't check for AV synchrony, providing switch from AAI with VVI backup to DDD just in case of sudden and marked bradycardia. Loss of AV synchrony doesn't represent a switching criteria and moreover it is not recognized by the device. This condition could be dangerous failing to correct AV conduction disturbances in pacemaker recipient, especially if LRL was set ≤ 50 bpm. For this reason, adequate knowledge of the algorithm would indicate greater caution in using RythmIQ in certain categories of patients, such as the one in the case just described.

Background The COMPASS trial showed that rivaroxaban plus aspirin was associated with fewer adverse cardiovascular events, but more major bleeding events, as compared with aspirin alone in patients with chronic vascular disease. The clinical benefit was particularly favorable in high-risk subgroups, who are frequently undertreated because of the fear of severe bleeding events. Purpose Our aim is to evaluate objective effects of anticoagulation strategies with rivaroxaban among patients with stable cardiovascular disease. Methods We considered a population of patients with stable cardiovascular disease (coronary and/or peripheral artery disease) which has had dual antiplatelet therapy (DAPT) for 1 year. Between them we selected 65 patients (52 males and 13 females, mean age 59±7 years) with high risk of ischemic events and low bleeding risk. We used DAPT Score and HAS BLED Score to enroll patients. We stopped them DAPT and starting a dual therapy combining rivaroxaban 2,5 mg twice daily plus aspirin 100 mg. We excluded patients with high bleeding risk and heart failure with less than 35% ejection fraction. At baseline they underwent blood tests, transthoracic echocardiography, six minutes walking test (6MWT), Kansas City Cardiomyopathy Questionnaire (KCCQ) Score, evaluation of carotid– femoral pulse wave velocity (cf-PWV) and ankle brachial index (ABI). We established 3, 6 and 12 months follow-up. Results At 3 months follow-up we evaluated 41 patients (the study is going on) repeating those exams and comparing them with the baseline ones. We observed that 35% of population had reduction of cf-PWV values and in 40% of population ABI increased. 15% of patients had also improvement of more than 100 meters in 6MWT. Particularly, reduction of cf-PWV and improvement of ABI values suggest that rivaroxaban 2,5 mg twice daily may have effects on vascular protection and arterial stiffness through different mechanisms such as improvement of endothelial functionality and fibrinolytic activity at endothelium, anti-inflammatory properties and platelet-dependent thrombin generation. Nevertheless none of the patients experienced subjective clinical improvement and the KCCQ Score was unmodified. This element indicates that patients at 3 months follow-up have imperceptible changes that can be documented only by diagnostic imaging evaluation and not by anamnestic data. Finally, an important evidence was that none of the patients at 3 months follow-up reported major bleeding events. Conclusion Preliminary data suggest that the addiction of rivaroxaban 2,5 mg to aspirin exerts vascular protection and its effects can be primarly documented by evaluation of cf-PWV and ABI. The 6MWT seems to play a minor role at 3 months assessment. The KCCQ may not be useful to fill in at 3 months follow-up because patients seem not to perceive subjective clinical improvement in this phase. If confirmed on a large cohort these results may give rivaroxaban a higher relevance not only for the power but also for the immediacy of its effects. Anyway, we are continuing our check to give our data more statistical significance and to test any role of the other parameters at 6 and 12 months follow-up.

A male patient, 33 years old, smoker, with a 20-year history of SLE, consulted the ER for right hand fatigue. He didn't assume his medications constantly. Computed Tomography showed a fronto-parieto-occipital cerebral infarction, and the angio-CT showed a plaque in left internal carotid artery. The patient was admitted to the Stroke Unit for minor ischemic stroke. The ECG showed pathological Q waves in the anterior leads from V1 to V4 revealing a likely previous silent myocardial infarction. The echocardiogram demonstrated moderate left ventricular dysfunction with 40% EF and segmental wall motion abnormalities, namely ipoakinesia of anterior wall and apex. This findings were confirmed by cardiac MRI highliting subendocardial LGE in these areas suggesting an ischemic genesis. Due to the hemorrhagic risk related to recent cerebral ischemic event, coronary angiography was postponed a month later. The coronary angiography revealed a significant stenosis of the proximal left anterior descending artery (iFR < 0.65). Qualitative assessment of the lesion with IVUS showed a “mixed” plaque. The lesion was treated with PCI and the implant of two overlapped DES and the patient underwent dual antiplatelet therapy. Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with heterogeneous presentation, characterised by alternating periods of flares and remission, and irreversible organ damage. The skin, joints, heart, kidneys, central nervous system and haematologic system are some of the most affected organs. Although recent data suggest that mortality decreased in patients with SLE over the last 30 years, mortality due to cardiovascular disease (CVD), usually defined as a combination of coronary, cerebrovascular and/or peripheral arterial disease, has remained high. Stroke and myocardial infarction (MI) are major CVD events that are potentially life-threatening. Lupus specific cardiovascular risk factors are believed to contribute to the high risk for MI, such as renal involvement, that has been associated with increased subclinical atherosclerosis and ischaemic heart disease, and antiphospholipid syndrome (APS), defined by venous, arterial or small vessel thrombosis and/or obstetric complications together with persistent positivity for antiphospholipid antibodies (aPL). Though several risk factors have been suggested, the exact mechanisms behind the high MI incidence in SLE remain essentially unknown. MIs in SLE are in most cases associated with coronary atherosclerosis. Subclinical atherosclerosis has been reported in many case–control studies, but a direct causal relationship between coronary artery disease (CAD) and MI in SLE has not yet been well documented. The pathogenesis of cardiovascular diseases in SLE is not fully understood. The inflammatory nature of SLE is believed to be an important factor in accelerating atherosclerosis. Systemic inflammation may lead to an abnormal lipid profile with elevated triglycerides, total cholesterol, and low-density lipoprotein cholesterol and dysfunctional high-density lipoprotein cholesterol. Additionally, promotes endothelial dysfunction and vascular injury. Our case showed the key role of an integrated imaging approach with echocardiography and cardiac MRI to confirm diagnosis of past myocardial infarction in a very young man affected by LES. IVUS played a great role to characterize the nature of the lesion, confirming the association between coronary atherosclerosis and MIs in SLE.

A 73 year-old man was admitted for syncope and sustained ventricular arrhythmia complicated by cardiogenic shock treated with electrical cardioversion and restoration of sinus rhythm. Former smoker, he suffered by arterial hypertension, hypercholesterolemia and known heart failure with reduced ejection fraction. In the 1995 the patient underwent aortic valve replacement surgery with a mechanical prosthetic valve. The patient underwent coronary angiography that showed multivessel coronary artery disease with functional occlusion of posterior interventricular artery (rehabilitated by hetero-coronary circles) and critical stenosis of the middle left anterior descending artery. In this angiographic framework, the indication was collegial revaluation considering other patient's comorbidities (chronic renal dysfunction, mitral moderate-severe regurgitation). The patient underwent ICD implantation for secondary prevention. Despite maximal medical therapy, the patient experienced new episodes of sustained VT complicated by hemodynamic instability. Hypokalemia, hypomagnesemia and hyperthyroidism were excluded as triggering factors for arrhythmias on laboratory investigations. In the following days due to persistent and symptomatic arrhythmias, configuring electrical storm, we decided to proceed with anatomical stellate ganglion block, guarantying a free interval from ventricular arrhythmia about six hours. The anesthetic has been injected at the C6 or C7 vertebral level with the Chassignac's tubercle, the cricoid cartilage, and the carotid artery serving as the anatomic landmarks to the procedure. An aspiration test must be done to avoid the suction of blood or cerebrospinal fluid, then a local anesthetic is injected, and the diffusion of the injectate is seen in real-time. Local anesthetic (lidocaine mixed with bupivacaine) is injected until the fluid spread along the paravertebral fascia to the stellate ganglion. The period free from VA allowed us to transfer the patient in another center in order to receive myocardial revascularization supported by ECMO. Left ganglion stellate block has a central role in the treatment of the refractory ventricular arrhythmias and may offer effective arrhythmia control giving time to rescue and/or other bridge therapy. In our case, it had a key role to perform an inter-hospital transfer and subsequent “rescue PCI therapy”. Thanks to Stellate ganglion block, the sinus rhythm was retained immediately, there were no ventricular tachycardia episodes for at least six hours allowing to perform myocardial revascularization supported by ECMO. No further ventricular arrhythmias occurred after revascularization, corroborating the ischemic trigger of electrical storm.

The proband was patient A2, suffering by severe left ventricular dysfunction (EF 20%) and II degree Mobitz type 1 atrioventricular block, requiring dual chamber ICD implantation, and retinitis pigmentosa. He had three brothers A1, A4, A5 suffering also by dilated cardiomyopathy and ICD/CRTD recipients. His sister A3, his daughter B1 and the firstborn brother A1 were also affected by retinitis pigmentosa. Genetic analysis was performed in A2 by NGS of a panel of genes related to heart disease and retinitis pigmentosa. It documented the presence of a missense pathogenetic variant (class 4) of the LMNA gene in heterozygosis (c.949G>A). This variant has been described in several scientific papers as associated with cardiac impairment in patients suffering from atrioventricular block and dilated cardiomyopathy. Laminin A/C is a fundamental protein of the nuclear envelope of the cell. Germline mutations in the LMNA gene, present on chromosome 22 (1q22), encoding the A/C lamina, have been causally linked to four different diseases with 42 reported mutations: Dilated cardiomyopathy (DCM) with disease of the conduction system; Limb girdle muscular dystrophy (LGMD); Autosomal dominant variant of Emery-Dreifuss muscular dystrophy (EDMD); Autosomal dominant partial lipodystrophy. LMNA-related dilated cardiomyopathy (DCM) is characterized by left ventricular enlargement and/or reduced systolic function frequently preceded or accompanied by significant conduction system disease. Family studies suggest that conduction system disease commonly precedes the development of DCM by a few years to a decade or more. Conduction system involvement usually starts with disease of the sinus node and/or atrioventricular node that can manifest as sinus bradycardia, sinus node arrest with junctional rhythms, or heart block (commonly first-degree heart block that progresses to second- and third-degree block). The following are also common: symptomatic bradyarrhythmias requiring cardiac pacemakers, supraventricular arrhythmias including atrial flutter, atrial fibrillation, supraventricular tachycardia, and the sick sinus syndrome (i.e., tachycardia-bradycardia syndrome), ventricular arrhythmias including frequent premature ventricular contractions and ventricular tachycardia Sudden cardiac death may occur with progressive disease. Although more malignant, life-threatening arrhythmias may occur with longstanding and usually previously symptomatic DCM, sudden cardiac death can also be the presenting manifestation of LMNA-related DCM, with minimal or no left ventricular dysfunction. In the cardiological setting, the AVB associated with DCM is a reliable marker for LMNA gene molecular screening. Regarding retinitis pigmentosa, the variant in heterozygosis c538C>G was found in the RHO gene in the proband, classifiable as pathogenetic. This gene encodes a protein necessary for the function of retinal photoreceptors and pathogenic mutations have been found in 30-40% of hereditary forms of retinitis pigmentosa. In conclusion, we believe that the tests carried out confirmed the hypothesis of a hereditary form of cardiomyopathy and retinitis pigmentosa, which seem to segregate independently in the family. Both mutations can be transmitted in 50% of cases, regardless of sex. For this reason we recommended to extend genetic analysis to all the first-degree relatives (siblings and children).

Background QT interval varies with the heart rate (HR), so a correction in QT calculation is needed (QTc). Atrial fibrillation (AF) is associated with elevated heart rate (HR) and beat-to-beat variation in the length of the QT and RR interval making correction of the QT interval challenging. Aim to evaluate which correction formula better correlates with QTc in sinus rhythm after electrical cardioversion (ECV). Methods During a 3-month period, we considered patients who underwent 12-lead ECG recording for standard clinical indications and received an AF diagnosis. Exclusion criteria were the following: a QRS duration >120 ms, chronical therapy with QT prolonging drugs, a rate control strategy, a non-electrical cardioversion. The final population was composed by 60 patients. The QT interval was measured in V2 lead as the 10-beat average and corrected using Bazzett's, Framingham, Fridericia and Hodges formulas during the last ECG taken during AF and the first one immediately after ECV. Moreover, QTc mean was calculated as mQTc (average of 10 QTc calculated beat per beat) and as QTcM (QTc calculated from the average of 10 raw QT and RR for each beat). Results Population was composed by 26 women (43%) with a mean age was 79 ± 11 years. Good correlations between mQTc and QTcM are present for each formula, even in atrial fibrillation or sinus rhythm. Comparing atrial fibrillation and sinus rhythm a significant change in HR was seen (63,9 ± 14,9 vs 99,5 ± 27,9, p Conclusion In ECG during atrial fibrillation, due its high heart rate, Bazzett's formula, although the most used, seems to be the most imprecise in QTc estimation.

Introduction Dipyridamole stress echocardiography (Dipy-stress) compared to exercise ECG, has an higher specificity in non-invasive detection of coronary artery disease (CAD). Two-dimensional strain echocardiography (2D-SE) is able to detect even minimal abnormalities of systolic function. The aim of the study was to observe changes in 2D-SE parameters during dipy-stress and to assess prognosis in a group of patients who had previously a non-diagnostic dipy-stress test result. Methods In the first phase 71 patients were enrolled and a dipy-stress test was performed. Each patient was then studied by off-line measurement of 2D-SE and coronary computed tomography angiography (CCTA), to check the presence of CAD. In the follow-up phase, an echocardiographic re-evaluation and outcome analysis during a mean follow-up of 78 months was carried out. Results In the first phase, Global Longitudinal Strain (GLS) was reduced (p < .0001) in the CCTA positive group compared to the CCTA negative group (23±3 vs 26±2 at rest; 20±3 vs 26±2 after stress). CCTA negative group and the CCTA positive group did not differ in terms of clinical features, cardiovascular risk factors, or treatments. Despite a trend in reduction for positive CCTA group (23±3 vs 20±3 at rest; 20±3 vs 19±3 after stress), no statistically significant changes were seen in the analysis of GLS rest and stress values, between baseline and follow-up in the two groups. None of the enrolled patients experimented cardiac events during follow-up. Conclusions Left ventricular GLS analysis improves the accuracy of dipy-stress echocardiography in the detection of mild CAD compared with the assessment of Wall Motion changes, particularly in those with preserved left ventricular ejection fraction. Patients may have a better mid-term prognosis thanks to close follow-up and early treatment of all cardiovascular risk factors.

Background Gliflozins proved effectiveness in reducing hospitalization and mortality in patients with heart failure and reduced ejection fraction (HFrEF). Recent studies showed how SGLT2-i improve loading condition and afterload by increasing extracellular Na excretion, downregulating RAAS and reducing vascular resistances. This latter aspect seems the result of a combined action on endothelial cells and vascular tone: experimental studies hypnotized that SGLT2-i may inhibit Na-H exchanger on endothelial cells and directly interact with PKG pattern and K+ channels determining vasodilation. On the other hand, 40 to 72% of patients with HFrEF are estimated to have postcapillary pulmonary hypertension. Of note, increased Pulmonary Artery Systolic Pressure (PASP) and Pulmonary vascular resistances (PVR) are associated with a worse outcome among HFrEF patients. However, effect of gliflozins on pulmonary artery pressures have never been investigated. Aim This pilot study aims to assess whether Gliflozins may have positive effects on pulmonary artery by a non-invasive evaluation. Materials and methods We retrospectively analysed transthoracic echocardiograms (TTE) of 24 patients with HFrEF who added gliflozins on top of optimal medical therapy. ETT before and after three months of Dapagliflozin therapy were compared, with particular attention to PASP and right chambers dimension and function. Results At three months, no significant differences in Left Ventricle (LV) dimensions were found (116 ml/mq vs 101.61, p=0.096), while there was an improvement in LV Ejection Fraction (27.9% vs 30,04 p=0.040). E wave velocity and mean E/e’ significantly decreased (respectively 52.9 vs 37,11 p=0.03 and 11.2 vs 8.01 p Discussion Gliflozins positive action on cardiac remodeling, preload and afterload are well known. However, as long as we know, there are no studies investigating Gliflozins effects on pulmonary vascular resistances and pressures. We observed a significative improvement on pulmonary pressures when gliflozins are added on top of OMT. One could speculate that reduction in mean E/e’ and, particularly, in E wave velocity, may be the expression of decrease LA pressure and LA volume due to LV function improvement with decrease in LA overload. In this context, a drop in postcapillary pressures may explain the increase in PV AT, which is an indirect sign of pulmonary pressures. In respect to PASP, which was also reduced, but significantly, PV AT may be more sensitive, explaining the difference in significance of these two results. No significative relation between PV AT increase and E wave velocity, mean E/e’ ratio and LAVi decrease was found, nevertheless a larger population may be required. Further studies, with a larger numerosity, are need to confirm our hypothesis. Conclusion Gliflozins, on top of OMT, appear to improve pulmonary pressures by reducing left atrial pressures after three months of therapy at a non-invasive evaluation.

We reported the cases of two young men with II degree AV block. The first one was a 17 yo asymptomatic boy, soccer player, undergoing 24 hours Holter-ECG for sporadic RVOT ventricular extrasystoles at resting ECG. During the night, an apparent Mobitz type 2 II degree AV block was observed. Detailed analysis showed: Sinusal bradycardia at 50 bpm;Absence of prolongation of P-R intervals before the pause;The pause is longer than double the basic P-P interval;Prolongation of P-P interval in the beats before the pause. The second case was also a young man suffering by atypical episodes of palpitations. The 24h Holter-ECG showed, during the night, an apparent Mobitz type 2 II degree AV block. Also in this case analysis of the tracing highlighted a) progressive slowing of sinus cycle length before the pause b) pause longer than double of sinus cycle and c) absence of prolongation of P-R intervals before the pause. Vagally mediated atrioventricular block and pseudo-Mobitz atrioventricular block. Vagally mediated AV block can have heterogeneous presentation: Wenckebach type, pseudo-Mobitz type II, 2:1, advanced-degree, complete AV block or a combination of different types of AV block and ventricular asystole. The mechanism is mediated by a vagal input, which depresses contemporarily sinus node and AV junction. The site of vagally mediated AV block is usually within the AV node. Generally, it results in a Wenckebach II degree AV block associated with sinus bradycardia, which occurs more often during the night in young/trained people. So the blocked P wave is generally preceded by Wenckebach phenomenon. However, in some patients the prolongation of the P-R interval is not present and the AV block appears abruptly. In these cases, Mobitz type II AV block may be erroneously diagnosed if sinus slowing is ignored. A differential diagnosis between true Mobitz II AV block and pseudo-Mobitz II block is essential for clinical purposes. Simultaneous slowing of the sinus rate and a pause longer than the double of the sinus cycle length indicate clearly a vagal mechanism, ruling out true Mobitz type 2 AV block, especially in young asymptomatic healthy men. In case of doubt, an electrophysiological study may be indicated.

A young 47 years old female patient was hospitalized in our department for recurrent traumatic syncopal episodes in order to undergo Head Up Tilt Test and Electrophysiology Study (EPS). Her ECG was normal and also transthoracic echocardiography didn't show pathological findings. 24 hour Holter ECG recording showed a short run of polymorphic ventricular tachycardia. Regarding familiar history her mother had died at a young age of sudden death. Head-up tilt test resulted negative for induced-syncope. During hospitalization the patient complained onset of malaise rapidly exiting in syncope: telemetry showed two pauses of 3.8 and 5.1 sec respectively, due to paroxysmal III degree atrio-ventricular block. The day after, the patient underwent electrophysiological study. During programmed ventricular stimulation from RV apex with train and V extrastimolous S1 300 S2 220 induction of syncopal ventricular fibrillation was observed, promptly treated with external DC shock. Cardiac MRI was performed showing diagnostic signs of Left Ventricular non-Compaction Cardiomyopathy. Considering the history of traumatic syncope the patient underwent dual chamber ICD implantation and was discharged. No clinical events in a 6 month follow-up. Genetical analysis was performed and we are waiting for the results. The term left ventricular non-compaction (LVNC) identifies a cardiomyopathy, characterized by intrauterine arrest of the compaction process of the ventricular myocardium during the end of the fourth week, thus leading to the development of prominent trabeculae of the left ventricle, deep intertrabecular recesses and a ventricular wall divider into two different layers of myocardium, the first compact, the other not. This nosological entity is difficult to classify (the European Society of Cardiology considers it as not classifiable among other cardiomyopathies, unlike the American Heart Association) mostly from the point of view of clinical implications, often unpredictable. In fact, we may have completely asymptomatic forms, which, according to some authors, should be considered as normal and completely benign variants, and various manifestations, in which the lack of compaction represents a morphological trait shared by phenotypically distinct forms of cardiomyopathy, such as hypertrophic, dilated and restrictive. LVNC carries an increased risk of ventricular dysfunction resulting in chronic heart failure, thromboembolic events and especially arrhythmic, often ventricular and life-threatening manifestations. A large spectrum of arrhythmias has been observed in patients with non-compact ventricles: in particular, supraventricular and ventricular tachycardias, Wolff-Parkinson-White (WPW) syndrome, but also bradyarrhythmias, which include sinus bradycardia, Sick Sinus Syndrome and various degrees of atrioventricular block, up to complete block. Ventricular tachycardias, including those that progress to ventricular fibrillation, occur in 38–47% of adult patients with LVNC, hence sudden death is often the cause of clinical presentation. Seen the features of the pathology, early diagnosis and family clinical evaluation are necessary aspects in patient management.

Introduction The Implantable Cardioverter Defibrillator (ICD) has been demonstrated to successfully prevent sudden cardiac death in children and young adults. A wide range of device-related complications/malfunctions have been described, which depend on the intrinsic design of the defibrillation system [Transvenous (TV-ICD) vs Subcutaneous (S-ICD)]. Objective To compare the device-related complications and inappropriate shocks with TV-ICD vs S-ICD. Methods Electronic databases were queried for studies focusing on the prevention of SCD in children and young adults with TV-ICD or S-ICD. The effect size was estimated using a random-effect model as Odds Ratio (OR) and relative 95% Confidence Interval (CI). The primary endpoint was a composite of any device-related complications and inappropriate shocks. Results We identified a total of 5 studied including 236 patients (Group S-ICD: 76 patients; Group TV-ICD: 160 patients) with a mean follow-up time of 54.2 ± 24.9 months. S-ICD implantation contributed to a significant reduction in the risk of the primary endpoint of any device-related complications and inappropriate shock (OR:0.18; 95% CI: 0.05 - 0.73; p=0.02)(Figure 1). S-ICD was also associated with a significantly lower incidence of inappropriate shocks (OR:0.28; 95% CI: 0.11 - 0.74; p=0.01) and lead-related complications (OR:0.18; 95% IC: 0.05 - 0.66; p=0.01). Otherwise, a trend towards a higher risk of pocket complications (OR:5.91; 95% CI: 0.98 - 35.63; p=0.05) was recorded in patients with S-ICD. Conclusion Children and young adults undergoing S-ICD implantation may have a lower risk of a composite of device-related complications and inappropriate shocks, compared to TV-ICD patients.

Introduction Transvenous lead implantation for pacemaker implantation is commonly performed by the cephalic vein cutdown (CVC), subclavian (SVP), or axillary vein puncture (AVP)(1). However, the CVC or AVP should be considered as first choice, according to the last guidelines, due to high rate of lead complications and pneumothorax of SVP. Objective To compare efficacy and safety of AVP compared with CVC for CIED implantation by a meta-analysis. Methods We systematically searched Medline, Embase and Cochrane electronic databases up to September 5th, 2022, for studies that evaluated the efficacy and safety of AVP and CVC reporting at least one clinical outcome of interest. The primary outcome was acute procedural success. The secondary outcomes were pneumothorax, lead failure, pocket hematoma/bleeding, device infection, total procedure time, venous access time and fluoroscopy time. The effect size was estimated using a random-effect model as Risk Ratio (RR) and relative 95% Confidence Interval (CI). Results A total of 8 studies were included enrolling 1926 patients with 3532 leads and average age of 72.3±14.8 years. AVP compared to CVC showed a significant increase in the procedural success (95.7% vs 76.1%; RR: 1.24; 95% CI: 1.09-1.40; p=0.001), (Figure 1). Total procedural time (Mean Difference [MD]: -8.25 min; 95%CI: -10.23- -6.27; p Conclusion Our meta-analysis proved that AVP improves procedural success and reduces total procedural time and venous access time compared to CVC. Figure 1 – Forest plots comparing Acute Procedural Success between Axillary Vein Puncture Versus Cephalic Vein Cutdown.

A 38-year-old male patient (A2) was referred to our ambulatory for paroxysmal atrial fibrillation in young age and ventricular preexcitation. Echocardiographic evaluation showed left ventricle hypertrophy (localized particularly at inter-ventricular septum (SIV) and normal LV ejection fraction. He also had a positive family history of hypertrophic cardiomyopathy since the latter was already diagnosed with the maternal grandmother (M0), the mother (M1) and the two brothers (A1 and A3). His father was apparently healthy (P1- consanguineous third degree of the mother M1). Specifically, the mother (M1) was suffering from non-obstructive hypertrophic cardiomyopathy, and tachy-brady (paroxysmal atrial flutter, sinus arrest) syndrome requiring pacemaker implantation. The firstborn (A1) was affected by paroxysmal atrial fibrillation, ventricular prexcitation and mild hypertrophy of the interventricular septum (SIV). The third child (A3) had a diagnosis of hypertrophic cardiomyopathy and a history of successfully ablation of a left postero-septal accessory pathway. A3 suffered also by paroxysmal typical atrial common flutter. Genetic analysis of the proband A2 confirmed a heterozygous pathological mutation of the PRKAG2 gene. Thus, genetic analysis was extended to the other two brothers A1 and A3 and the mother. We proposed to the all three brothers electrophysiological study in order to assess arrhythmic substrate and appropriate therapy (strict FUP, PM or ICD implantation). Mutation of the PRKAG2 gene is a rare disease, classified as non-lysosomal cardiac glycogenosis with clinical onset in late adolescence or in the third decade of life and presents an autosomal dominant inheritance with complete penetrance. It is associated with cardiac arrhythmias, such as ventricular preexcitation (Wolff-Parkinson-White syndrome), sinus node disease, atrioventricular block, atrial fibrillation. Genetic analysis has a crucial role especially in the evaluation of inheritance with autosomal dominant transmission and regarding the prognostic impact, since PRKAG2 gene mutation involved a greater arrhythmic risk comparing with other subtypes of hypertrophic cardiomyopathy. Affected patients should be closely monitored to facilitate early detection of arrhythmia and conduction problems. PRKAG2 mutation should be considered in patients with LVH who develop AF or require permanent pacemakers at a young age. Early recognition is important to allow prompt identification and appropriate management of genetic carriers.

Background The diagnostic value of the β-angle in the diagnosis of patients with Brugada Syndrome (BrS) is still unclear. Objective to evaluate the diagnostic accuracy of the β-angle and establish its best cut-off value. Methods We searched databases for studies evaluating sensitivity and specificity of the β-angle in patients with suspected BrS undergoing Sodium Channel Blocker Provocation Test (SCBPT). The pooled sensitivity and specificity were calculated, and the Summary Receiver Operating Characteristic curve was constructed. The effect size was estimated using a random-effect model as Odds Ratio. Results we included 4 studies enrolling 1471 patients (Positive SCBPT: 382 patients; Negative SCBPT: 1089 patients). Patients with positive SCBPT had a higher mean β-angle value than those with negative SCBPT (39.25° vs 22.52°; p Conclusion A β-angle ≥58° represents the best diagnostic predictor for patients with suspected BrS.

Introduction The anomalous connection of the left circumflex artery (LCx) to the right coronary artery (RCA) or sinus is the most frequent coronary artery (CA) anomaly. Among them, only those with an interarterial course are regarded as hidden conditions at risk of myocardial ischemia (MI) and sudden cardiac death (SCD). We report an uncommon of anomalous origin of LCx from the right sinus of Valsalva and a retroaortic path causing MI. Case presentation A 61-year-old man presented to the emergency department complaining palpitations and chest discomfort for an hour. He only had history of hypertension. Physical examination was unremarkable. The ECG demonstrated atrial flutter with a 2:1 conduction ratio and a ventricular rate of 157 bpm and ST segment depression in leads V4-6. Transthoracic echocardiography did not reveal segmental kinetic anomalies but a five-chamber apical view showed a “RAC sign”, typical of anomalous retroaortic course of the left coronary artery. The patient was treated with intravenous infusion of amiodarone. He restored sinus rhythm and symptoms regressed completely, but the ECG taken after conversion showed flattened T waves in leads V5-6 and negative T waves in I and aVL. Cardiac enzymes had transient increase. After the acute episode ended the patient underwent cardiac computed tomography angiography (CTA) with evidence of anomalous origin of LCx from the right sinus with a retroaortic course. A coronary angiography excluded obstructive atherosclerotic coronary lesions. Nuclear myocardial perfusion imaging revealed reversible small subsegmental perfusion defects in mid inferolateral wall and apical lateral wall. We established a medical treatment with beta-blocker. Discussion Our patient had anomalous connection of the LCx branch to the right sinus of Valsalva with a retroaortic course. Although this anomaly is usually considered benign, cases of association with SCD and MI have been reported. The factor responsible for this pathogenicity could be high orifice, ostial stenosis, slit-like/fish-mouth-shaped orifice and acute-angle take-off. As cardiac CTA did not reveal any of these characteristics, we hypothesized that the increased cardiac output and expansion of the great vessels during tachycardia could cause compression of the retroaortic segment or angling at its origin and generate ischemia. Repolarization abnormalities at ECG are well documented during supraventricular tachycardia as a response to pacing-induced stress. These changes are usually diffused and disappear after conversion to sinus rhythm. In this case they appeared hours later, accompanied by cardiac enzyme buildup. As the epicardial coronary arteries did not show any pathology, we suggest that the patient had transient ischemia due to LCx anomaly. We confirmed it by myocardial perfusion imaging. As for the management of this anomaly in adults, surgery is recommended as class IC in patients with typical angina symptoms who present with evidence of stress-induced myocardial ischemia in a matching territory or high-risk anatomy. Our patient has never had clear manifestations of angina. All these elements together with the age of our patient motivated us to use a conservative approach. Conclusions We report a case of anomalous origin of LCx from right sinus of Valsalva causing transient myocardial ischemia in a patient that has always been asymptomatic. This anomaly has been and continues to be considered benign, nevertheless we suggest to judge the clinical significance of this kind of CA anomaly on a case-by-case integrated approach.

Background Anderson-Fabry Disease (AFD, OMIM 301500) is a rare X-linked lysosomal storage disorder caused by GLA gene mutation resulting in a deficit or absent activity of the α-galactosidase A enzyme (α-Gal A). This deficiency involves the impossibility of cleavage of glycophospholipids, resulting in an intralisosomal accumulation of them in different tissues. Due to an incidence of 1 in 80000, AFD is considered the second most common glycosphingolipid storage disorder after Gaucher disease. Cardiac manifestations include left ventricular hypertrophy (LVH) and arrhythmias. The rate of pacemaker implantation (PMI) in AFD has been described to be 25 times higher than in the general population, and the requirement of PMI has been reported to be as high as 8% in some AFD series. In this context, the presence of conduction abnormalities in young patients may suggest an undiagnosed AFD.1 Therefore, early diagnosis is important in AFD because appropriate therapies seem to be more effective when initiated promptly. Purpose Our aim is to detect AFD among relatively patients with unexplained conduction disturbances requiring PMI, not submitted to newborn screening. Methods Among 650 patients afferent to our ambulatory for routinary pacemaker follow-up, we considered a selected population with diagnosis of sinus node dysfunction or atrioventricular block (confirmed by atrial pacing rate ≥ 60% or ventricular pacing percentage ≥ 80%) and an age, at the time of PMI, ranging between ≥40 and ≤70 years old. The exclusion criteria were: patients with previous myocardial infarction; patient whit known cardiac disease (such as hypertrophic cardiomyopathy); patients who underwent cardiac surgery and patients with extracardiac disease with cardiac involvement such as autoimmune disorders. For this cohort of 26 adult patients (13 males; 13 females; mean age 63 ± 7 years) a prospective screening study for AFD was performed. After clinical evaluation, transthoracic echocardiography (analyzing signs of left ventricular hypertrophy) and pacemaker check, a dried blood spot sampled in filter paper was analyzed. This filter paper assay was performed in male patients in order to evaluate the α-Galactosidase A enzyme activity through the detection of Fabry disease biomarkers; only in the case of abnormal values, genetic investigation was performed. In female patients, the analysis was exclusively genetic. Results The analyses revealed 58% (15/26) of patients affected by mild LVH (IVS diameter ranging from 11 to 15 mm). No patient had severe LVH (IVS diameter ≥15 mm) or moderate-severe renal dysfunction (more than stage 3B, GFR below than 30-44 mL/min). In the restrict cohort considered, we found one 69 yo female patient with heterozygosis GLA pathogenic mutation, NM_000169.2:c.638A>C p.(Lys213Thr). She had normal value of liso-Gb3 1,1 ng/ml (n.v. ≤ 1,8 ng/ml). She had mild LVH (IVS diameter 12 mm) and no renal dysfunction. Familiar screening was programmed. Conclusion In a highly selected sample of relatively young patients with conduction disturbances requiring pacemaker implantation, a female patient with genetic mutation causing AFD has been identified. Therefore, it seems that screening efforts should be increased in this patient population. 1 Hemelsoet D, De Keyser J, Van Heuverswyn F et al. Screening for Fabry Disease in Male Patients With Arrhythmia Requiring a Pacemaker or an Implantable Cardioverter-Defibrillator. Circulation. 2021 Feb 23;143(8):872-874. doi: 10.1161/CIRCULATIONAHA.120.051400.

Aims Trans-venous lead-related thrombosis is an undervalued issue. Since the lack of guidelines or evidences regarding the best therapeutic option, treatment of endocavitary clots on pacemaker wire is left to individual decision between surgical catheter extraction, thrombolysis, or anticoagulation therapy. In some patients, the labile equilibrium between hemorrhagic and thrombotic events may further complicate management. Methods and results We report the case of a 86-years-old man with heart failure (HF) severely reduced ejection fraction due to chronic ischaemic cardiomyopathy and several co-morbidities. He had a reacution of HF requiring hospitalization secondary to new-onset atrial flutter. During hospital stay, in light of episodes of severe bradycardia, he underwent single-lead PM implantation. Few days after discharge he developed deep vein thrombosis in situ of PM electro catheter insertion (poor compliance to anticoagulation therapy was reported) which improved after few days of regular therapy assumption. After few months anticoagulation therapy was dismitted due to etiology unknown-anemization requiring transfusion, but 20 days later he was once again admitted, this time because of pulmonary embolism. Trans thoracic echocardiography (TTE) enlightened a severely dysfunctioning right ventricle and a mass in right atrium hanging the wire. Trans oesophageal echocardiography was then performed showing hyper-reflective and highly mobile material with numerous ‘arborizations’. Intravenous heparin was started as pulmonary embolism therapy. Once infective etiology was excluded, total body computer tomography was performed to investigate a possible paraneoplastic origin. Presence of a meningioma was enlightened. Heart-team evaluation excluded catheter extraction in light of patient’s frailty, while thrombolysis was controindicated. Vitamin K antagonists was then started. At 1 month follow up no further embolic neither haemorrhagic events were reported. TTE showed a reduction in mass dimension and an improvement in right ventricle function. Conclusions In a guidelines-lacking field, VKA can be an effective option in cases of lead-related thrombosis when surgery or thrombolysis is not suitable. Further studies are needed to establish their real effectiveness in management of E-C-related endocavitary thrombi.

Aims Along with relevant progress in technology, pacemaker implantation is continuously improving its safety and efficacy in treating patients with bradyarrhythmias. Despite this, this procedure has several complications, including haematoma, pneumothorax, lead dislodgement, infection, lead perforation, and tamponade. Methods and results A 64-year-old woman underwent loop recorder implantation, after recurrent loss of consciousness, in order to assess arrhythmic causes of syncope. Two weeks later, an episode of paroxysmal complete AV block, conditioning a pause of 3 s, was recorded. Thus, the patient was scheduled for urgent dual-chamber pacemaker implantation. No complication apparently occurred during the procedure. An active fixation ventricular lead was positioned in right ventricular septal apex while passive fixation atrium lead in the right appendage. Soon after implantation the patient started to suffer by non-productive cough, clearly related to ventricular stimulation, either in DDD or in VVI pacing modality. During spontaneous ventricular activation (RBBB) no symptoms occurred. Transthoracic echocardiography, performed the day after implantation, revealed a small pericardial effusion (diastolic diameter < 10 mm) along the apical segments, near the tip of the right ventricular lead. Suspicion of right ventricular lead perforation arised. The patient underwent urgent contrast chest CT confirming pericardial effusion, and showing an intramyocardium placement of the right ventricular apical lead. No active bleeding in pericardium was observed. Due to persistence of symptoms, we decided to perform right ventricular lead repositioning in right middle septum, with pericardiocentesis back-up promptly available. Post-procedure, palpitation, and cough abruptly disappeared. After 3 months follow-up, no significant symptoms were reported and pericardial effusion gradually disappeared. Conclusions We describe a singular case of cough, as atypical symptom immediately after pacemaker implantation. Pericardial effusion and contrast-CT showing intra-myocardial position of the tip guided our suspicion to a possible right ventricular lead microperforation. Although right ventricular lead parameters were completely normal this findings didn’t exclude RV perforation. The lead perforation is known as a rare complication of device implantation. Typical symptoms of RV lead perforation are chest pain and hypotension. The patient described in our case showed a haemodynamically stable pericardial effusion accompanied by non-productive cough, clearly time-related to RV stimulation. In literature, there is only another similar case report. The cough is a rare and not well recognized symptom of lead perforation. Early diagnosis of RV perforation allows to perform urgently and safely (pericardiocentesis back-up) lead replacement/repositioning. Echocardiography and contrast-CT could be useful in order to assess a possible pericardial effusion or intramyocardial/pericardial position of RV lead tip.

Aims Acute coronary syndrome (ACS) has a huge potential impact on patients’ life, and little is known on the long-term effect on mental health in young patients. Methods and results We included consecutive young patients (≤45 years at presentation) with ACS referred from October 2013 until March 2021 to our clinic. Physical and mental status was evaluated through the standardized SF12 scale for physical (PCS) and mental status (MCS). Depression and perceived stress were evaluated with the patient health questionnaire 9 (PHQ9) and perceived stress scale (PSS). A total of 91 patients have been included and followed for a median of 40 months. History of anxiety or depression at baseline was reported in 1.1% and 2.2%, respectively. Physical and mental health status was impaired in 65.8% and 42.1% of patients, respectively (Figure). Predictors of poorer physical health were lower LVEF, WMSI, and multivessel disease. Predictors of poorer mental health were age and nuisance bleeding during follow-up. Mild/moderate depression was reported in 47.3%. Moderate/high stress was reported in 78.1%. 37.1% patients affirmed that their career trajectory worsened after the event and 37.8% declared a reduction of income. 53.8% declared mild to significant changes in their sexual activity, while 83.8% declared to have received no advises on sexual activity from their Doctor. Conclusions In conclusion, ACS is a dramatic event with a significant impact on young patients’ life at a physical, mental, and social level. Measures of social/psychological support in the aftermath of the event merit consideration in future research.

Aims Takotsubo syndrome (TTS) is an acute stress-induced cardiomyopathy showing left ventricular (LV) dysfunction without obstructive coronary arteries disease. A sudden massive surge of circulatory catecholamines from an intense physical or emotional stress may play a central role in the pathogenesis of TTS. We report the case of an 87 years-old woman who developed TTS with uncommon presentation after permanent pacemaker (PM) implantation. Methods and results The patient was referred to our hospital for PM implantation because of advanced atrio-ventricular block (3:1). She suffered by rheumatoid arthritis (RA), arterial hypertension, and chronic kidney disease. Echocardiogram, performed before PM implantation, showed normal LV kinesis and normal ejection fraction (EF 60%). She was initially administered with infusion of Isoprenaline 2 mcg/min. The subsequent day, she underwent permanent dual-chamber pacemaker implantation without any complications. After 3 days, the patient complained severe asthenia and fever, together with increase of white blood cells and C reactive protein. Blood cultures were negative. We started antibiotic therapy and, suspecting a reactivation of RA, steroid therapy with infusion of methylprednisolone 40 mg/die. Electrocardiogram showed normal sinus rhythm and paced ventricular rhythm. PM interrogation showed normal function. Surprisingly, echocardiogram showed LV dysfunction with apical and medium segments akinesia, and severe EF reduction (35%). Coronary angiography documented absence of coronary obstructive lesions, assessing diagnosis of TTS. The patient was discharged 1 week after admission in good clinical condition. One week later, an echocardiogram showed apical akinesia, partial recovery of medium segments motility, and slight increase of EF (40%). The excess of catecholamines could lead to decreased cardiac muscular function and to spasm of coronary arteries: these events can lead to acute heart failure and decrease of LVEF. Furthermore, about 90% of patients with TTS are women, especially in postmenopausal period. Peculiarities of this case were the atypical symptoms of TTS and the combination of different predisposing stressors factors: female sex in postmenopausal period, anamnesis of chronic inflammatory disease, use of stress-inducing drugs (methylprednisolone and isoprenaline, the last associated with TTS after PM-implantation), atrio-ventricular block itself, and PM implantation procedure. Our findings remark that even a low-risk procedure could be a possible cause of TTS in patient with such risk factors. In our opinion, in this subset of patients, conscious sedation could be useful to reduce the stress load, together with an early procedure and consequently the minimal use of exogenous stress drugs like Isoprenaline, even if the patient is in a good clinical condition. Conclusions This case highlights TTS as a potential complication after PM implantation, especially in post-menopausal women with high pre-existing stress load.

Introduction The Impella Heart Pump device (Abiomed, Danvers, MA, USA) is commonly used to provide mechanical circulatory support during high-risk percutaneous coronary intervention (PCI) and has demonstrated both efficacy and safety in patients with cardiogenic shock. Left ventricular assist devices (LVADs) alter the pathophysiological impact of ventricular arrhythmias in advanced heart failure; for example, life-threatening arrhythmias such as ventricular fibrillation (VF). We present a case of sustained VF tolerance in a patient with IMPELLA CP® support. Methods and results A 64-year-old man was admitted with typical chest pain that began 3 days earlier and an anterior myocardial infarction with ST-segment elevation. Urgent coronary angiogram showed a left anterior descending artery treated with angioplasty and stent implantation (TIMI 3). An initial echocardiogram, performed after PCI, showed a reduced left ventricular ejection fraction (LVEF) of approximately 35% with good right ventricular function. Two days later, the ECG tracing showed persistence of the ST elevation, and the patient developed recurrent ventricular tachycardia and an episode of acute pulmonary oedema; the echocardiogram showed a significant worsening of LVEF (15%). A percutaneous mechanical circulatory support device (Impella CP; Abiomed) was inserted through the right common femoral artery in order to preserve adequate systemic perfusion (Figure 1A). Twelve hours later, the patient developed rapid VT degenerating into VF without loss of consciousness (Figure 1C). During the arrhythmia, the patient was alert and his mental status was normal, Impella flow was 2.4–3.0 l/min, and invasive blood pressure (IBP) was 80/65 mmHg (Figure 1D). Intravenous lidocaine was administered without effect. After approximately 10 min of incessant VF, the patient received sedation with propofol from the anesthesiologist. A single unsynchronized DC shock of 200 J converted the patient to sinus rhythm. A bedside transthoracic echocardiogram was performed to check the optimal position of the Impella device (Figure 1B). In the following days, the patient had two new episodes of asymptomatic VF treated with DC-shock after sedation and was transferred to the cardiac surgery department to undergo urgent LVAD implantation. Conclusions Impella CP was helpful in the management of this patient with severe heart failure and malignant ventricular tachyarrhythmias, reducing the hemodynamic and neurological impact of this latter catastrophic arrhythmic event.

Background Recent reports evidenced gender differences in the knowledge, perception and awareness of cardiovascular risk factors and cardiovascular diseases. Purposes To evaluate trends in awareness of cardiovascular risk, as well as knowledge of symptoms and preventive behaviors related to cardiovascular disease (CVD). Methods As part of Vivi con il Cuore (campaign with the goal of raising awareness about women's heart disease) a nationwide survey was conducted. Standardized questions on awareness of CVD risk, as well as prevention behaviors and barriers, were provided through a computer-assisted web interviewing platform to a large sample of Italians citizens ranging from 40 to 70 years old. The sample was representative of the population by age, sex, and area of geographical residence. Results A total of 1,000 subjects were included in this survey, of which 511 (51%) female. About 60% of women indicated cancer as the leading cause of death among female sex, while only 22% indicated cardiovascular disease. Similarly, when the same question was asked to men, 44% indicated cancer and 21% cardiovascular disease, observations that were consistent across age categories. Although a well sizable part of the population (90% of the interviewees) have consulted the general practitioner in the last year, only 45% of women and 56% of men declared to receive information about CVD risk. Almost 84% among men and women, recognized the importance of knowing how to understand the symptoms of a heart attack due to the possibility of survival. An additional survey was conducted among young cardiologists under 40 years old, including a total of 200 young cardiologists, similarly distributed among men and women (47% and 53% respectively). About 24% of young cardiologists indicated breast cancer as the leading cause of death in women, while only 70% reported cardiovascular diseases. Furthermore, 35% of participants reported that they did not have adequate training on the risks of heart disease in women. Conclusions In this survey, only 22% of women were aware that cardiovascular disease is the number one killer of women. Likewise, a well sizable part of young cardiologists (30%) does not recognize cardiovascular disease as the leading cause of death in women. These data should inform public health campaigns to focus on evidence-based strategies to prevent CVD and to help target messages that resonate and motivate women to take action. Funding Acknowledgement Type of funding source: Private company. Main funding source(s): Abbott