Premature Ventricular Contractions Research Articles

Protection by L-arginine against Epinephrine-induced Arrhythmia and Cardiotoxicity

Nitric oxide is important in control of coronary blood flow, cardiomyocyte contractility, cardiac rhythm, and thrombogenicity. We aimed to investigate the effect of the nitric oxide precursor L-arginine on cardiac arrhythmia and myocardial injury induced by epinephrine in male Sprague-Dawley rats. The possible modulation of L-arginine effects by methylene blue (MethyB), a nitric oxide synthase inhibitor was also examined. Cardiac arrhythmia was induced with 10μg/kg epinephrine given intravenously (i.v.). L-arginine (200mg/kg) or L-arginine and MethyB (100mg/kg) were intraperitoneally (i.p.) administered prior to i.v. epinephrine. Other groups received i.p. saline, only L-arginine or only MethyB (100mg/kg). Results showed that compared with the saline control, epinephrine caused a significant bradycardia (188.7±24.4 vs. 405.6±1.19beats/min), increased QRS duration (0.039±0.006 vs. 0.0185±0.0001s), decreased R wave amplitude (0.18±0.01 vs. 0.23±0.011mv), and ventricular extrasystoles. L-arginine alone showed no significant effect on heart rate (380.8±10.0 vs. 405.6±1.19beats/min) but increased QRS duration (0.029±0.0002 vs. 0.0185±0.0001s), and R wave amplitude (0.47±0.01 vs. 0.23±0.011mv). L-arginine given prior to epinephrine prevented bradycardia, shortened the duration of arrhythmia and decreased the number of extrasystoles. The administration of L-arginine and MethyB almost completely suppressed epinephrine-induced ventricular arrhythmias. Epinephrine caused severe degeneration of muscle fibres, widening of intercellular spaces, cellular infiltrations and interstitial haemorrhage. L-arginine markedly attenuated, while L-arginine/MethyB completely prevented these changes. It is concluded that L-arginine protects against cardiac arrhythmias and cardiac tissue damage caused by epinephrine.

Application of high-power ablation mode in patients with idiopathic ventricular extrasystole

Background. A new method of high-power short-duration ablation (HPSDA) with high impact energy, ranging from 60 to 90 W, is becoming widespread in the world. However, the number of studies on the use of HPSDA in patients with idiopathic ventricular arrhythmias (IVA) is limited. It is relevant to study the feasibility of using HPSDA to improve the outcomes of radiofrequency ablation (RFA) in patients with IVA. The aim of the study: to evaluate the immediate results of HPSDA in RFA for IVA and to compare it with the classic ablation mode. Materials and methods. We have retrospectively analyzed the results of RFA in 54 patients with symptomatic IVA. Their average age was 45.0 ± 11.7 years. The majority were men (72.3 %). Given the possible effect of intracardiac mapping on the results of ablation, the patients were divided into two groups. Group I include 27 (50 %) participants in whom mapping was performed using 3D navigation. Group II include 27 (50 %) patients in whom 3D navigation was not used. The decision to use HPSDA or classic ablation was made empirically, without considering any factors or characteristics of a patient. The following ablation parameters were used during HPSDA: temperature 45 °C, exposure time 7 seconds, energy 70 W. Results. In group I, where 3D navigation was used to localize the substrate, HPSDA was performed in 12 patients (44.4 %), and the classic mode was used in 15 cases (55.6 %). With conventional mapping, HPSDA was applied in 10 (37.0 %) patients, and the classic mode in 17 (63.0 %). Сlassic ablation allowed to achieve complete arrhythmia suppression in all patients, regardless of the mapping method. When using only HPSDA, the disappearance of arrhythmia was observed only in 45.5 % of cases. With the use of the classic mode, recurrence of IVA during the control time was observed in 16.6 % of patients, while with the use HPSDA in 33.3 %. Conclusions. The high-power regimen demonstrated significantly lower efficacy for permanent suppression of IVA compared to classic ablation methods (p = 0.007). The use of HPSDA to suppress IVA is associated with a high risk of arrhythmia recurrence during control time (p = 0.0010). It is advisable to convert the ablation mode for complete suppression of arrhythmia when the HPSDA is ineffective.

CARDIOPULMONARY SUPPORT WITH EXTRACORPOREAL MEMBRANE OXYGENATION (ECMO) DURING CATHETER ABLATION OF VENTRICULAR FIBRILLATION: A CASE REPORT

Abstract The clinical case deals with a 46–year–old man, severely obese and suffering from type II diabetes mellitus and arterial hypertension, a smoker. He was admitted to our department for iterative ventricular fibrillation (VF) following a recent acute myocardial infarction with ST–segment elevation treated with percutaneous coronary angioplasty (PTCA) and implantation of drug–eluting stents on the anterior interventricular. Control coronary angiography showed good results of previous PTCA. On the echocardiogram, left ventricular pump function moderately reduced (EF 45%) with district alterations of the kinetics and aneurysmal dilatation of the ascending aorta. During hospitalization, iterative episodes of VF despite maximal antiarrhythmic therapy, promptly treated with DC shock. Continuous telemetry monitoring showed frequent early premature ventricular complex (PVC) triggering VF episodes. A 12–lead Holter ECG was performed to better identify the morphology of these PVC, documenting PVC of morphology compatible with origin from Purkinjie fibres. An electrophysiological study and mapping of the left ventricle were performed with extracorporeal membrane oxygenation (ECMO) support. During mapping of the middle left interventricular septum, VF occurred several times and was promptly treated with DC shock. In this area have been documented Purkinje fibers and low voltage and multifragmented areas; ablation was then performed by delivering radiofrequency in this area until the all potentials were completely eliminated. After ablation, no more ventricular arrhythmias were induced. Cardiac magnetic resonance imaging was performed which showed large areas of late gadolinium enhancement. The patient underwent automatic defibrillator implantation. Ventricular arrhythmias were no longer documented during telemetric monitoring. In this complex case of significant arrhythmic instability in ischemic heart disease with moderate left ventricular pump dysfunction, ECMO assistance allowed us to effectively and safely perform the ablation of the arrhythmic substrate triggering the iterative episodes of VF.

A LABILE EQUILIBRIUM: THE CONTROVERSIAL ROLE OF BETA–BLOCKERS IN A CASE OF MID–VENTRICULAR TAKOTSUBO SYNDROME

Abstract Case Report Takotsubo syndrome (TTS) is a non–ischemic heart disease mimicking acute coronary syndrome. The mid–ventricular variant is almost rare (15% of TTS cases). A 62 years old woman was referred to our hospital for oppressive chest pain radiating to the jaw lasting at least 30 minutes after an attempted strangulation by a family member. She denied any conventional cardiovascular risk factor; she was taking beta–blockers (BB) to reduce symptomatic and idiopathic premature ventricular complexes (PVCs) burden. On arrival, the patient was hemodynamically stable, reporting only swallowing difficulties. Physical examination revealed neck bruising and irregular pulse. The initial ECG showed sinus rhythm with a heart rate of 62 bpm, PVCs and a biphasic T wave in lead aVL. Hs–cTnI first dosage was 145 pg/mL. Transthoracic echocardiography (TTE) found moderate left ventricular (LV) dysfunction and severe hypokinesis of mid septal and mid inferior segments. Coronary angiography revealed normal coronary arteries. Left ventriculogram showed hypokinesis of the midventricular section with a hyperdynamic base and apex suggestive of mid–ventricular variant of TTS. After a few hours, she experienced a lipothymic episode and telemetry showed marked sinus bradycardia followed by multiple sinus pauses. A TTE was immediately performed, demonstrating a worsening LV ejection fraction with new development of severe symmetrical akinesia of the LV apex. Successively, psychotherapy was started. Wall motion abnormalities and LV systolic function completely recovered over a few days. At present the patient is in good health status. Discussion domestic violence against women is prevalent, but it is often omitted as a potential stress trigger that affects their hearts. In Italy, it’s reported that 32% of women had experienced physical violence. Strangulation is very uncommon as cause of TTS and psychotherapy may prevent recurrences and recover in a shorter time. Bradycardia due to atrioventricular block and asystole has been described in the acute phase of TTS and it is often attributed to BB therapy. Although BB have been proposed to add further benefit of blunting the effect of catecholamine excess, their use should be used cautiously in the acute phase of TTS. Moreover, beta–adrenoceptor hypersensitivity may generate a parasympathetic drive when on BB infusion, provoking a worsening LV dysfunction and a transient sick sinus syndrome as we described.

THE CONUNDRUM OF DRUG INTERACTIONS AND HOLTER MONITORING: LAMIN GENE MUTATION DISCOVERED BY A TORSADE DE POINTES EPISODE FOLLOWED BY OUT–OF–THE–HOSPITAL RESUSCITATION

Abstract The prolongation of the QTc interval on the electrocardiogram is known to be associated with an increased risk of developing life–threatening ventricular arrhythmias such as torsades de pointes (TdP). QTc values exceeding 500 ms can double or even triple the risk of TdP. Many drugs, including amiodarone and some SSRI antidepressants like paroxetine, can prolong the QTc interval. A 57–year–old patient experienced cardiac arrest during an overnight Holter recording prescribed to exclude brady–tachy syndrome in the presence of persistent atrial fibrillation. ECG ambulatory monitoring revealed significant bradycardia with the onset of idioventricular rhythm, followed by TdP triggered by premature ventricular contractions, subsequently degenerating into ventricular fibrillation. The patient was resuscitated by family members and later by emergency medical personnel using 3 DC shocks, leading to the restoration of spontaneous circulation. The patient was transferred to the cardiology intensive care unit where a coronary angiography showed normal results. Blood tests indicated low potassium levels (3.3 mEq/L), with a moderately reduced left ventricular ejection fraction at 45%. The patient was discharged after ICD implantation and during the diagnostic follow–up the genetic testings for long QT syndrome were negative, but showed a mutation of the LMNA lamin A/C gene. During the two months previous to hospital admission, the patient had complained of fatigue in the presence of atrial fibrillation with a low ventricular response. Nonetheless, she had been prescribed oral amiodarone to optimize the chances of successful electrical cardioversion, in combination with paroxetine that the patient was already taking at home. The combination of these two drugs and the patient‘s idiopathic bradycardia contributed to a marked prolongation of the QT interval, leading to TdP degenerated into ventricular fibrillation. This case, notable for the prolonged recording of ventricular fibrillation and subsequent resuscitation chest compressions during a Holter monitoring, suggests caution in the use of antiarrhythmic drugs unless strongly indicated in the presence of potentially ominous drug interactions. It is encouraging to note how the widespread adoption of cardiopulmonary resuscitation culture has enabled individuals who might not have otherwise received medical attention to be effectively resuscitated.

A COMPLEX CASE OF EARLY ONSET ATRIAL FIBRILLATION IN AN ENDURANCE ATHLETE: THE IMPORTANCE OF FAMILY HISTORY

Abstract Introduction Early onset atrial fibrillation (AF) in an endurance athlete represents a challenge for the cardiologist, especially in apparent absence of an overt structural heart disease. Different genetic variants have been associated with early onset AF but indications to genetic testing are still under definition. Case A 54–year–old man (BMI 19 kg/sqm), previous competitive athlete (Nordic running and walking) presented to the Emergency Department with sudden onset palpitations and dyspnea due to high penetrance AF (145 bpm). Family history (FH) was positive for cerebrovascular accidents in both parents and for early onset (<60 years) AF in the father. The man had a recent history of borderline hypertension, of effort related syncope of and of infundibular, isolated, premature ventricular complexes during a maximal exercise stress test (otherwise negative for ischemia) which ultimately led to the denial of competitive sport eligibility. Sinus rhythm (SR) was acutely restored with flecainide and the patient was discharged with low dose nadolol. Cardiac US showed normal left ventricular (LV) dimensions and thicknesses (10 mm), with regular LVEF (60%) and global longitudinal strain (–18%), mildly dilated left atrium (LAVI 36 ml/sqm) and bi–leaflet mitral valve prolapse without significant mitral regurgitation, mitro–anular disjunction nor Pickelhaube sign; 24h Holter ECG showed SR with rare supraventricular (SV) ectopic beats and a 4 beats SV run. Due to the recurrence of short episodes of palpitations, rhythm control with flecainide and antithrombotic prophylaxis were started and AF ablation was planned. Genetic testing for early onset AF identified a nonsense variant (c.84665delG; p.Gly28222fs*2) in TTN gene, classified as likely pathogenic (C4), so far not reported in scientific literature. Also, despite a moderate–low cardiovascular risk as assessed by SCORE2, mostly due to the FH and the desire to engage in high intensity exercise, coronary CT was prescribed, which showed a critical coronary disease leading to stenting on left anterior descending artery and intermediate branch. Cardiac MRI was planned. Conclusions Early onset AF in endurance athletes should not be underrated and a thorough examination including a detailed family history should be performed. TTN is the single gene most frequently associated with early onset AF in absence of an overt structural heart disease, but the subsequent clinical evolution is still largely unknown.

DON’T FORGET ABOUT CARDIAC AMYLOIDOSIS: WHEN RED FLAGS MATTER

Abstract Case Report A 78–year–old man came to our attention for worsening dyspnea, asthenia and frequent lipothymic episodes in the last few weeks. Nothing remarkable was reported in anamnesis except for periodic cardiological check–ups ascribed to hypertensive heart disease and moderate aortic stenosis. Other cardiovascular risk factors were obesity, previous smoking habits and dyslipidemia. A recent 24–hours ECG monitoring revealed slow permanent atrial fibrillation (AF) with 3–seconds pauses, frequent premature ventricular complexes (PVCs) and some PVCs couplets. On arrival, NT–proBNP was elevated (2952 pg/mL), while ECG showed AF with a heart rate of 50/m and low voltages in peripheral leads. Transthoracic echocardiogram revealed a biatrial dilatation, a severe left ventricular (LV) concentric hypertrophy with a preserved LV ejection fraction (EF) and a moderate aortic stenosis. In addition, a restrictive LV filling pattern, a decreased longitudinal myocardial contraction and an apical sparing of longitudinal strain were reported. Invasive coronary angiography was performed with evidence of normal coronary arteries. Given the echocardiographic suspicion of amyloidosis, a 99mTc–Hydroxymethylene diphosphonate (HMDP) scintigraphy was performed, showing a moderate cardiac uptake and an attenuated bone uptake (Perugini Score 2). Serum and urine immunofixation resulted negative. Genetic testing did not find any mutations in Transthyretin (TTR) gene. Therefore, the diagnosis of TTR wild–type amyloidosis was made. Before discharge, putting it all together, we opted to implant a leadless pacemaker in order to protect the patient from life–threatening bradyarrhythmias. Discussion Although it is currently considered rare, there is growing evidence that cardiac amyloidosis may be more common than once thought. First of all, it should be investigated in HFpEF–patients (heart failure with preserved EF) with LV hypertrophy. Despite several red flags (low QRS voltages, conduction disorders, elevated NT–proBNP, severe LV hypertrophy and aortic stenosis) and various medical consultations, our patient had to wait 4 years or more to be diagnosed with cardiac amyloidosis. Inevitably, a diagnostic delay means loss of time in treatment initiation. Earlier diagnosis would enable us to improve our patients’ survival and heart failure hospitalization. Awareness alone is not enough, we must be right on target too.

REAL WORLD USAGE OF RANOLAZINE FOR ANTIARRHYTHMIC PURPOSES: SINGLE CENTER EXPERIENCE

Abstract Introduction Ranolazine is a late sodium current blocking drug developed as an antianginal agent, with good preliminary efficacy data even for antiarrhythmic purposes (both at atrial and at ventricular level). Objectives To evaluate patients’ clinical characteristics and antiarrhythmic efficacy of ranolazine in subjects with a history of atrial and/or ventricular arrhythmias, isolated or repetitive. Patients and methods we included patients who received a first prescription of ranolazine for antiarrhythmic purposes in our center from 1/2022 to 11/2023 and evaluated the survival free from major arrhythmic relapses/ablation. Results 93 patients were enrolled (66±13 years, 15% women): 59% with arterial hypertension, 26% diabetics, 46% with post–ischemic dilated heart disease, 32% with primary cardiomyopathy (including 11% with hypertrophic cardiomyopathy, 10% with LMNA, 14% with TTN), 46% with implantable cardioverter defibrillator, 9% with pacemaker. The mean LV ejection fraction was 42 ± 15%, the mean serum creatinine 1 ± 0.3 mg/dL. Half (50%) had a history of atrial arrhythmias in the 6 months before starting the drug, mainly paroxysmal atrial fibrillation (AF), 67% of ventricular arrhythmias (VAs), mainly isolated and repetitive premature ventricular beats (PVB, 47% of the total), but also sustained or treated VAs (n=18, 19%). Overall, 18% of patients had already undergone AF ablation, 13% ventricular tachycardia ablation, 3% cardiac sympathetic denervation. Finally, 95% of patients were on beta–blocker therapy and 28% were on amiodarone. Ranolazine was prescribed at an average initial dose of 535± 150 mg. The majority (54%) of patients (n=50) had a follow–up longer than 3 months (median 9 months, IQR 6–14); in 6% the drug was stopped due to intolerance and the average dose at the last FU was 641 ± 150 mg. Twenty–eight percent of patients with paroxysmal/persistent atrial arrhythmias had relapses, requiring ablation in 6%. Among patients with major VAs, 57% suffered recurrences, but only 19% required ablation (invasive in 2 cases, non–invasive in 1 case). Finally, among the patients with PVBs, none suffered major VAs or required PVB/VA ablation. Conclusions This is the largest available case–series reporting about the real–world usage of ranolazine for antiarrhythmic purposes. Our single center experience supports ranolazine’s good safety and tolerability and shows encouraging efficacy results in terms of clinical stabilization of complex patients.

ACUTE MYOCARDIAL INJURY IN A PATIENT WITH TYPE–1 NEUROFIBROMATOSIS: A CASE OF SUSPECTED PHEOCROMOCYTOMA–ASSOCIATED MYOCARDITIS

Abstract A 54–year–old patient with an history of type–1 neurofibromatosis presented to the emergency department (ED) due to recurrent abdominal pain with diarrhea. Clinical examination didn’t show anything remarkable. She was mildly hypertensive, apyretic. During her staying in the ED, she complained about chest pain and an electrocardiogram (ECG) was performed. ECG showed sinus rhythm, normal heart rate, left ventricular (LV) hypertrophy with marked horizontal ST segment depression in the precordial leads and bimorphic ventricular ectopic beats with prevalent right bundle branch block superior axis morphology, likely from the medio–basal inferior LV. Troponin T (peak 958 ng/L, normal <15 ng/L) and NTproBNP (peak 2357 ng/L, normal <180 ng/L) were elevated, hemoglobin, creatinine and liver function were normal, C–reactive protein peaked at 120 mg/dL. Echocardiogram showed mildly reduced left ventricular ejection fraction (LVEF) with inferior and inferolateral hypokinesia and moderate mitral regurgitation (MR). She was admitted to cardiac intensive care unit with the suspected diagnosis of an acute coronary syndrome and was started on acetylsalicylic acid, metoprolol, furosemide and potassium canrenoate. Amiodarone was added due to frequent ventricular tachycardias. Due to the symptoms and the history of neurofibromatosis a whole body computed tomography (CT) was done. CT showed a 53 mm mass on the left adrenal gland with intense enhancement, suspected for a neoplastic origin (Figure 2). Plasmatic and urinary cathecolamines and metanephrines were increased. Positron–emission tomography (PET)/CT with 68Ga showed left adrenal uptake, compatible with a neuroendocrine tumor. Coronary arteriography showed normal coronary arteries. The patient underwent a cardiac magnetic resonance (CMR) study in the suspect of acute myocarditis. CMR showed normal biventricular function, an inferior basal hypokinesia with focal intramyocardial late gadolinium enhancement (LGE) suspected for a post–inflammatory origin and mild pericardial effusion. After pharmacological preparation a laparoscopic left adrenalectomy was performed, and histology confirmed the diagnosis of a composite pheocromocytoma. Three months after, the patient was asymptomatic, biochemistry showed normalization of both troponin T and endocrinological biomarkers, the echocardiogram showed preserved LVEF without areas of hypokinesia and mild MR. A follow–up CMR at six months from the acute injury has been scheduled.

A RARE CASE OF INTERVENTRICULAR SEPTUM DUPLICATION

Abstract Case Report A 62–year–old man, history of hypertension. In 2015, there was suspicion of non–compaction cardiomyopathy, and he underwent cardiac MRI at another facility (report not available). The patient reported palpitations and dyspnea with exertion, leading to a follow–up visit. EKG showed sinus rhythm 90/min, nonspecific intraventricular conduction delay and isolated monomorphic ventricular ectopic beats. Echo revealed a mildly dilated left ventricle (end–diastolic volume 88 ml/m), systolic function at the lower limits of normal (EF 53%), and prominent muscular structures near the posterior interventricular septum (SIV), likely related to anomalies of the papillary muscles (prominent and possibly duplicated posterior–medial papillary muscle). There was apical trabecular protrusion, which did not meet the criteria for non–compaction (NC/C ratio in systole approximately 1), with no other significant findings. The cardiac MRI, performed to assess dilated cardiomyopathy, revealed a conspicuous structural abnormality in the septo–papillary region, located medio–apically and paraseptal–endocardial in the left ventricle. This anomaly lacked evident chordae tendineae with the mitral valve leaflets and exhibited synchronous systo–diastolic motion with the left ventricular wall, suggesting septal duplication. The septal thickness was slightly reduced, particularly in the medio–apical region. Additionally, there was hypertrabeculation of the posterior and lateral walls of the left ventricle, exceeding the threshold for non–compaction (NC/C ratio in end–diastole: 2.6 – normal range: <2.3). Left ventricular mass was normal (mass: 173.52 g; mass/body surface area (BSA): 86.58 g/m). Basal septum, medio–apical septum and medio–apical lateral wallshowed hypokinetic contractility. The left ventricle was dilated (end–diastolic diameter: 55.0 mm; end–systolic diameter: 45.3 mm – end–diastolic volume: 230 ml; stroke volume/BSA: 57.6 ml/m² – EF: 51%). Areas of late gadolinium enhancement (LGE) were observed in the anterior medio–basal septum and at the site of the described structural muscular anomaly, both displaying an intramural non–ischemic pattern. Conclusions The described findings suggest a picture of dilated cardiomyopathy and an associated structural septo–papillary anomaly of uncertain diagnostic interpretation (septum duplication with associated non–compacted myocardium? duplicated posterior–medial papillary muscle with associated non–compacted myocardium?).

PERCUTANEOUS LEFT STELLATE GANGLION BLOCK TO INCREASE VENTRICULAR FIBRILLATION THRESHOLD AND DEFIBRILLATION SUCCESS RATE IN ACUTE FULMINANT MYOCARDITIS WITH ELECTRICAL STORM

Abstract Introduction Acute fulminant myocarditis represents a predisposing substrate for life–threatening ventricular arrhythmias (VAs). Percutaneous left stellate ganglion block (PLSGB) usage has been reported for the prevention of recurrent VAs, but clinical data on its acute effect on ongoing ventricular fibrillation (VF) are missing. Case A 57–years–old man suffering gastroenteritis and fever in the past week presented in the ED with typical chest pain. ECG depicted infero–lateral ST–segment elevation, cardiac ultrasound showed a mildly reduced LVEF and coronary angiography ruled out coronary lesions. In the following hours the patient developed frequent premature ventricular complexes with R on T phenomenon triggering short coupled torsade de pointes with rapid degeneration into VF. VAs episodes, initially responsive to single DC shock, kept recurring despite intravenous (iv) amiodarone, iv lidocaine and iv correction of potassium and magnesium. The patient was therefore treated with deep sedation, intubation and ECMO VA placement; still, he reached a status of persistently ongoing VF, refractory even to DC shocks (n=20). Therefore, ultrasound–guided PLSGB using a local bolus of lidocaine 150 mg + ropivacaine 50 mg was performed. After 5 minutes, a single DC shock was delivered, effective in achieving a stable sinus rhythm at 35 bpm with no more recurrences. Despite electrical stabilization, the patient developed a severe cardiogenic shock due to bradycardia and severe biventricular disfunction, treated with vasopressin and dobutamine. In the next 48 hours we observed a significant improvement, resumption of biventricular contractility and absence of VAs recurrences, so the patient was slowly weaned off ECMO and vasopressors. Immunological tests showed Enterovirus IgM positivity, while endomyocardial biopsy was negative for inflammatory infiltrates in the analyzed sections. Cardiac MRI (performed 10 days after onset) showed edema and non–ischemic LGE in the antero–lateral and infero–lateral basal subepicardial regions of the left ventricle, LVEF was 55%. After 15 days from admission, despite ongoing amiodarone and bisoprolol, the patient developed a run of monomorphic VA of 30 beats at 170 bpm, therefore he underwent sICD implantation. Conclusion PLSGB played a key role for the acute management of refractory VF unresponsive to DC shocks, allowing for acute stabilization. More clinical data are needed on acute neuromodulation for electrical storm.

LMNA–RELATED DILATED CARDIOMYOPATHY PRESENTING WITH REGIONAL WALL AKINESIS AND TRANSMURAL LATE GADOLINIUM ENHANCEMENT

Abstract A 26–year–old girl was admitted at hospital in December 2022 for a syncopal episode. She referred fatigue and dyspnea in the last 2 months. EKG was markedly abnormal showing peripheral low voltages, poor R wave progression in prechordial leads, negative T waves V4–V6, DII, DIII and aVF, fragmented QRS (Fig.1). Echo showed moderately dilated left ventricle (LV) with apical akinesia and apical aneurysm (no thrombi) and hypo–akinesia and hyperechoic appearance of the middle segments of the infero–posterior and lateral wall (Fig.2a). EF was 38%. There was no family history for sudden cardiac death and cardiomyopathy. Creatine kinase and neurological examination were normal. The diagnostic work–up included: myocardial scintigraphy which showed fixed tracer uptake deficit at apical and posterolateral levels (Fig.2b); cardiac magnetic resonance (CMR): apical aneurysm with extended late gadolinium enhancement (LGE) due to fibrosis, focally transmural, on the left ventricle’s midapical and infero–lateral thinned–hypokinetic myocardial walls (Fig.3a, 3b); Coronary angiography showed no stenosis. Due to myocardial aneurysm in absence of CAD, Chagas disease was excluded. We performed LV endomyocardial biopsy which showed cardiomyopathic changes and replacement fibrosis. Due to fibrosis extension, complex ventricular extrasystoles detected on monitoring and syncope, ICD was implanted. Finally genetic testing showed c.1621C>Tp.Arg541Cys LMNA pathogenetic mutation leading to the diagnosis of LMNA–related LV cardiomyopathy, with extensive fibrosis in multiple areas of left ventricle including the apical segments at a very young age. Patients with LMNA mutation-related heart disease are characterized by conduction abnormalities, ventricular tachyarrhythmias (VA) and high risk of sudden cardiac death with mildly impaired systolic function, often without chamber dilation. About 88% LMNA– cardiomyopathy have typical myocardial fibrosis, predominantly in the mid-myocardium of the basal septum. However, our patient and previous reported cases with the same p.R541 LMNA mutation presents with a specific phenotype including regional LV akinesis, segmental transmural LGE, significant LV dilatation and systolic dysfunction and VA without conduction abnormality. Of note, EKG shows normal A–V conduction but low voltage and negative T waves in precordial and inferior leads. These features are not typical for LMNA–disease and underline the phenotypic variability of cardiomyopathies.

Prognostic relevance of ventricular arrhythmias in surgical patients with gastrointestinal tumors.

Individuals diagnosed with gastrointestinal tumors are at an increased risk of developing cardiovascular diseases. Among which, ventricular arrhythmia is a prevalent clinical concern. This suggests that ventricular arrhythmias may have predictive value in the prognosis of patients with gastrointestinal tumors. To explore the prognostic value of ventricular arrhythmias in patients with gastrointestinal tumors receiving surgery. We retrospectively analyzed data from 130 patients undergoing gastrointestinal tumor resection. These patients were evaluated by a 24-h ambulatory electrocardiogram (ECG) at the Sixth Affiliated Hospital of Sun Yat-sen University from January 2018 to June 2020. Additionally, 41 general healthy age-matched and sex-matched controls were included. Patients were categorized into survival and non-survival groups. The primary endpoint was all-cause mortality, and secondary endpoints included major adverse cardiovascular events (MACEs). Colorectal tumors comprised 90% of cases. Preoperative ambulatory ECG monitoring revealed that among the 130 patients with gastrointestinal tumors, 100 (76.92%) exhibited varying degrees of premature ventricular contractions (PVCs). Ten patients (7.69%) manifested non-sustained ventricular tachycardia (NSVT). The patients with gastrointestinal tumors exhibited higher PVCs compared to the healthy controls on both conventional ECG [27 (21.3) vs 1 (2.5), P = 0.012] and 24-h ambulatory ECG [14 (1.0, 405) vs 1 (0, 6.5), P < 0.001]. Non-survivors had a higher PVC count than survivors [150.50 (7.25, 1690.50) vs 9 (0, 229.25), P = 0.020]. During the follow-up period, 24 patients died and 11 patients experienced MACEs. Univariate analysis linked PVC > 35/24 h to all-cause mortality, and NSVT was associated with MACE. However, neither PVC burden nor NSVT independently predicted outcomes according to multivariate analysis. Patients with gastrointestinal tumors exhibited elevated PVCs. PVCs > 35/24 h and NSVT detected by 24-h ambulatory ECG were prognostically significant but were not found to be independent predictors.

Assessment of cardiac adverse events following COVID-19 vaccination by speckle tracking echocardiography

Cardiac discomfort has been reported periodically in COVID-19-vaccinated individuals. Thus, this study aimed to evaluate the role of myocardial strains in the early assessment of the clinical presentations after COVID-19 vaccination. Totally, 121 subjects who received at least one dose of vaccine within 6 weeks underwent laboratory tests, electrocardiogram (ECG), and echocardiogram. Two-dimensional speckle tracking echocardiography (2D-STE) was implemented to analyze changes in the left ventricular myocardium. After vaccination, 66 individuals (55.4 ± 17.4 years) developed cardiac discomforts, such as chest tightness, palpitations, dyspnea, and chest pain. The ECG readings exhibited both premature ventricular contractions and premature atrial contractions (n = 24, 36.4%), while none of the individuals in the control group manifested signs of cardiac arrhythmia. All had normal serum levels of creatine phosphokinase, creatine kinase myocardial band, troponin, N-terminal pro b-type natriuretic peptide, platelets, and D-dimer. Left ventricular ejection fraction in the symptomatic group (71.41% ± 7.12%) and the control group (72.18% ± 5.11%) (p = 0.492) were normal. Use of 2D-STE presented global longitudinal strain (GLS) and global circumferential strain (GCS) was reduced in the symptomatic group (17.86% ± 3.22% and 18.37% ± 5.22%) compared to the control group (19.54% ± 2.18% and 20.73% ± 4.09%) (p = 0.001 and p = 0.028). COVID-19 vaccine-related cardiac adverse effects can be assessed early by 2D-STE. The prognostic implications of GLS and GCS enable the evaluation of subtle changes in myocardial function after vaccination.

Electrocardiographic Patterns and Ejection Fraction in Patients with Nonischemic Dilated Cardiomyopathy: A Cross-sectional Retrospective Study

Background:: The relationship between ECG changes in dilated nonischemic cardiomyopathy and ejection fraction (EF) is complex and poorly understood. Thus, this study aimed to identify the most common patterns of ECG associated with dilated nonischemic cardiomyopathy and their relationship to EF. Methods:: A retrospective cross-sectional study was conducted between 2019 and 2022, including 100 consecutive patients with nonischemic dilated cardiomyopathy. ECG data were rate, axis, left (LBBB) and right bundle branch block (RBBB), first-degree heart block, premature ventricular contractions, depth of S wave in leads V1 and V2, length of R wave in leads V2 and V6, the ratio of R/S in the lead V4, left ventricular hypertrophy (LVH), maximum R wave length in leads I, II, and III, poor R wave progression, ST-segment elevation, and T wave inversion. Results:: The most common ECG pattern associated with cardiomyopathy was T-wave inversion (47%). The EF was 23.9± 8.87% in patients with LBBB and 25.5± 8.07% in patients without LBBB (P= 0.361). In patients with T-wave inversion, the EF was 23.4± 8.21%, and it was 26.28± 8.35% in patients without T-wave inversion (P= 0.086). In patients with LVH, the EF was 23.89± 7.84%, and it was 25.5± 8.66% in patients without LVH (P= 0.354). A negative nonsignificant correlation existed between SV1, SV2, RV2, RV6, R/S V4, and maximum R in I, II, III, and EF. Conclusions:: The study showed that T-wave inversion was the most common pattern associated with dilated nonischemic cardiomyopathy. The study provided insight into the negative correlation between EF and SV1, SV2, RV2, RV6, R/S V4, and maximum R in I, II, and III, although it did not reach a significant level.

Cognitive Behavioral Therapy for Symptom Preoccupation Among Patients With Premature Ventricular Contractions: Nonrandomized Pretest-Posttest Study.

Premature ventricular contractions (PVCs) are a common cardiac condition often associated with disabling symptoms and impaired quality of life (QoL). Current treatment strategies have limited effectiveness in reducing symptoms and restoring QoL for patients with PVCs. Symptom preoccupation, involving cardiac-related fear, hypervigilance, and avoidance behavior, is associated with disability in other cardiac conditions and can be effectively targeted by cognitive behavioral therapy (CBT). The aim of this study was to evaluate the effect of a PVC-specific CBT protocol targeting symptom preoccupation in patients with symptomatic idiopathic PVCs. Nineteen patients diagnosed with symptomatic idiopathic PVCs and symptom preoccupation underwent PVC-specific CBT over 10 weeks. The treatment was delivered by a licensed psychologist via videoconference in conjunction with online text-based information and homework assignments. The main components of the treatment were exposure to cardiac-related symptoms and reducing cardiac-related avoidance and control behavior. Self-rated measures were collected at baseline, post treatment, and at 3- and 6-month follow-ups. The primary outcome was PVC-specific QoL at posttreatment assessment measured with a PVC-adapted version of the Atrial Fibrillation Effects on Quality of Life questionnaire. Secondary measures included symptom preoccupation measured with the Cardiac Anxiety Questionnaire. PVC burden was evaluated with 5-day continuous electrocardiogram recordings at baseline, post treatment, and 6-month follow-up. We observed large improvements in PVC-specific QoL (Cohen d=1.62, P<.001) and symptom preoccupation (Cohen d=1.73, P<.001) post treatment. These results were sustained at the 3- and 6-month follow-ups. PVC burden, as measured with 5-day continuous electrocardiogram, remained unchanged throughout follow-up. However, self-reported PVC symptoms were significantly lower at posttreatment assessment and at both the 3- and 6-month follow-ups. Reduction in symptom preoccupation had a statistically significant mediating effect of the intervention on PVC-specific QoL in an explorative mediation analysis. This uncontrolled pilot study shows preliminary promising results for PVC-specific CBT as a potentially effective treatment approach for patients with symptomatic idiopathic PVCs and symptom preoccupation. The substantial improvements in PVC-specific QoL and symptom preoccupation, along with the decreased self-reported PVC-related symptoms warrant further investigation in a larger randomized controlled trial. ClinicalTrials.gov NCT05087238; https://clinicaltrials.gov/study/NCT05087238.

A Case Report of Arrhythmia Caused by Skin Expander Implantation

A 51-year-old female patient suffered facial burns during her youth. Following the healing of the wound, a skin expander was implanted on 7 December 2021 to address scar contracture. The preoperative electrocardiogram (ECG) indicated sinus rhythm within the normal range. Color Doppler ultrasound revealed no abnormalities. The patient had no history of hypertension, heart disease, diabetes, arrhythmia, or other cardiovascular and cerebrovascular conditions. On 16 June 2022, the skin expander was removed, and facial scar resection was performed. The routine ECG showed ventricular premature beats. During the operation, when the surgeon pulled and compressed the neck dilator, significant arrhythmia was observed, with the heart rate dropping below 50 beats per minute and frequent ventricular premature beats occurring. The surgeon promptly halted the procedure, resulting in immediate relief of the patient's pain. Subsequently, the ventricular premature beats ceased, and normal sinus rhythm was restored. Once the heart rate exceeded 60 beats per minute, the surgeon resumed the operation, but ventricular premature beats persisted. After removing the dilator, the heart rate gradually returned to normal, and no further arrhythmia occurred. The patient recovered smoothly post-operation, with stable vital signs and no reported discomfort. She safely returned to the ward. It was determined that the patient's arrhythmia was caused by the compression of the carotid sinus due to the stretching of the neck dilator.

Diabetes insipidus with dilated cardiomyopathy and premature ventricular contractions: case report

Diabetes insipidus with dilated cardiomyopathy and premature ventricular contractions: case report

Dynamic Regularized Adaptive Cluster Optimization (DRACO) for Quantitative Cardiac Cine MRI in Complex Arrhythmias.

Irregular cardiac motion can render conventional segmented cine MRI nondiagnostic. Clustering has been proposed for cardiac motion binning and may be optimized for complex arrhythmias. To develop an adaptive cluster optimization method for irregular cardiac motion, and to generate the corresponding time-resolved cine images. Prospective. Thirteen with atrial fibrillation, four with premature ventricular contractions, and one patient in sinus rhythm. Free-running balanced steady state free precession (bSSFP) with sorted golden-step, reference real-time sequence. Each subject underwent both the sorted golden-step bSSFP and the reference Cartesian real-time imaging. Golden-step bSSFP images were reconstructed using the dynamic regularized adaptive cluster optimization (DRACO) method and k-means clustering. Image quality (4-point Likert scale), signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), edge sharpness, and ventricular function were assessed. Paired t-tests, Friedman test, regression analysis, Fleiss' Kappa, Bland-Altman analysis. Significance level P < 0.05. The DRACO method had the highest percent of images with scores ≥3 (96% for diastolic frame, 93% for systolic frame, and 93% for multiphase cine) and the percentages were significantly higher compared with both the k-means and real-time methods. Image quality scores, SNR, and CNR were significantly different between DRACO vs. k-means and between DRACO vs. real-time. Cardiac function analysis showed no significant differences between DRACO vs. the reference real-time. DRACO with time-resolved reconstruction generated high quality images and has early promise for quantitative cine cardiac MRI in patients with complex arrhythmias including atrial fibrillation. Stage 2.

Role of Exercise Stress Test in Evaluating Athletes with Premature Ventricular Beats: A Retrospective Study

Role of Exercise Stress Test in Evaluating Athletes with Premature Ventricular Beats: A Retrospective Study