Refractory coronary vasospasm complicated by complete atrio-ventricular block during neck surgery: is there an indication for pacing?

Vasospastic angina is a clinical condition characterized by coronary artery spasm in angiographically normal coronary arteries. Vasospastic angina can often lead to ventricular arrhythmias, sudden cardiac death, or life-threatening bradyarrhythmias, such as high-degree atrioventricular block or asystole. We present the unusual case of a woman with depressive syndrome who underwent emergency surgery for hemostasis of a neck lesion that caused hemorrhagic shock after a suicide attempt. During surgery, the electrocardiogram revealed inferior and posterior ST-segment elevation, total atrioventricular block and torsades de pointes; the patient also suffered 4 minutes of cardiac arrest. A temporary pacemaker was placed. Coronary angiography showed right coronary artery vasospasm. Following a second similar episode after tracheostomy, a permanent pacemaker was implanted. The indication for definitive electrostimulation in such a context and the stimulation mechanisms of the carotid sinus underlying vasospasm constitute the interesting points of this clinical case. LEARNING OBJECTIVE: The indication for definitive electrostimulation in a context of recurrent episodes of high-degree atrioventricular block during vasospastic angina and the stimulation mechanisms of the carotid sinus underlying vasospasm constitute the interesting points of this clinical case.

Calmodulin mutation in long QT syndrome 15 associated with congenital heart defects further complicated by a functional 2:1 atrioventricular block: Management from foetal life to postpartum.

We report a long QT syndrome 15 whose diagnosis was suspected during foetal life and confirmed at birth and was associated with congenital heart disease. Genetic testing revealed a rare mutation associated with the CALM2 gene. At 23 weeks of gestation, severe foetal sinus bradycardia (∼100 bpm) was detected. In the third trimester, the foetus developed severe right ventricular hypertrophy. At birth, the electrocardiogram showed a long QT interval of 640 ms, and after 1 hour, the newborn showed functional 2:1 atrioventricular block at ventricular rate of 50 bpm. After further pharmacological therapies, epicardial wires were surgically implanted for transient pacing in VVI mode at 90 bpm. Echocardiogram showed aneurysmatic left atrial appendage, dilated right segments, hypertrophied right ventricle, ostium secundum type atrial septal defect, and muscular ventricular septal defect. At two weeks of postpartum, a permanent dual-chamber pacemaker was implanted in the DDD mode and the patient was discharged with a prescription of beta-blockers and calcium therapy.

The implantation of AV leadless pacemaker - a case report.

In early 2020 Food and Drug Administration (FDA) approved Micra AV - a new type of leadless pacemaker with atrioventricular synchrony, to treat patients with atrio-ventricular (AV) blocks. We describe one of the first in Poland case of a patient who was implanted with Micra AV pacemaker. CASE REPORT: A 38-year-old female patient was admitted to the clinic due to the 29-seconds event of a complete AV block without an escape rhythm and was implanted with a dual chamber pacemaker without any complications. After several months she was admitted again with suspicion of ventricular perforation by the pacemaker electrode and underwent a replacement procedure of both pacemaker's leads. Nevertheless, one week later the patient developed a fever with significantly elevated inflammatory markers. The blood cultures were negative but in the transesophageal echocardiography features of cardiac device-related infective endocarditis were observed. Empirical antibiotic therapy was administered, and the device was removed. The Heart Team qualified the patient for the implantation of a MicraTM AV leadless pacemaker. The procedure was performed without any complications and the device was implanted to the right ventricle. All parameters were correct, and the patient was discharged. CONCLUSIONS: Micra AV may be a feasible and safe option for young patients with paroxysmal AV block after device-related complications.

Bradyarrhythmias in patients with SARS-CoV-2 infection: A narrative review and a clinical report.

Several cardiovascular diseases and arrhythmic disorders have been described in COVID-19 era as likely related to SARS-CoV-2 infection. The prognostic relevance of bradyarrhythmias during the infection has not been yet described and no data are available about long-term heart conduction disorders. A review of literature concerning the association between hypokinetic arrhythmias and COVID-19 from January 2020 to February 2021 was performed. The key-words used for the research were: "sinus node disfunction," "sick sinus syndrome (SSS)," "sino-atrial block," "atrio-ventricular block (AVB)," "bradyarrhythmias," and "COVID-19″ or "SARS-CoV-2.″ Excluding "relative bradycardia," a total of 38 cases of bradyarrhythmia related to SARS-CoV-2 infection have been described, even in very young people, requiring in many cases a definitive pacemaker implantation. Furthermore, we report a case of non-hospitalized 47-years old man with a SSS developed as a consequence of mild SARS-CoV-2 infection. While in all described cases heart conduction disorders were found at presentation of the infection or during hospitalization for COVID-19, in our case the diagnosis of SSS was made after the resolution of the infection. Although rarely, heart conduction disorders may occur during COVID-19 and the present case highlights that a cardiological follow up may be desirable even after the resolution of infection, especially in the presence of symptoms suggesting a possible heart involvement.

Functional modulation of atrio-ventricular conduction by enhanced late sodium current and calcium-dependent mechanisms in Scn5a1798insD/+ mice.

AIMS: SCN5A mutations are associated with arrhythmia syndromes, including Brugada syndrome, long QT syndrome type 3 (LQT3), and cardiac conduction disease. Long QT syndrome type 3 patients display atrio-ventricular (AV) conduction slowing which may contribute to arrhythmogenesis. We here investigated the as yet unknown underlying mechanisms. METHODS AND RESULTS: We assessed electrophysiological and molecular alterations underlying AV-conduction abnormalities in mice carrying the Scn5a1798insD/+ mutation. Langendorff-perfused Scn5a1798insD/+ hearts showed prolonged AV-conduction compared to wild type (WT) without changes in atrial and His-ventricular (HV) conduction. The late sodium current (INa,L) inhibitor ranolazine (RAN) normalized AV-conduction in Scn5a1798insD/+ mice, likely by preventing the mutation-induced increase in intracellular sodium ([Na+]i) and calcium ([Ca2+]i) concentrations. Indeed, further enhancement of [Na+]i and [Ca2+]i by the Na+/K+-ATPase inhibitor ouabain caused excessive increase in AV-conduction time in Scn5a1798insD/+ hearts. Scn5a1798insD/+ mice from the 129P2 strain displayed more severe AV-conduction abnormalities than FVB/N-Scn5a1798insD/+ mice, in line with their larger mutation-induced INa,L. Transverse aortic constriction (TAC) caused excessive prolongation of AV-conduction in FVB/N-Scn5a1798insD/+ mice (while HV-intervals remained unchanged), which was prevented by chronic RAN treatment. Scn5a1798insD/+-TAC hearts showed decreased mRNA levels of conduction genes in the AV-nodal region, but no structural changes in the AV-node or His bundle. In Scn5a1798insD/+-TAC mice deficient for the transcription factor Nfatc2 (effector of the calcium-calcineurin pathway), AV-conduction and conduction gene expression were restored to WT levels. CONCLUSIONS: Our findings indicate a detrimental role for enhanced INa,L and consequent calcium dysregulation on AV-conduction in Scn5a1798insD/+ mice, providing evidence for a functional mechanism underlying AV-conduction disturbances secondary to gain-of-function SCN5A mutations.

Cardiac manifestations of sarcoidosis: diagnosis and management.

Approximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Cardiac symptoms are usually dominant over extra-cardiac as most patients with clinically manifest disease have minimal extra-cardiac disease and up to two-thirds have isolated cardiac sarcoidosis (CS). It is estimated that another 20-25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In addition, the extent of myocardial late gadolinium enhancement is emerging as an important prognostic factor. The literature shows some controversy regarding outcomes for patients with clinically silent CS and larger studies are needed. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest CS despite minimal data supporting it. Fluorodeoxyglucose Positron Emission Tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators.

Swallow-induced syncope and carotid sinus hypersensitivity: Coincident or associated conditions?

Swallow induced syncope is a rare clinical condition which is thought to result from an abnormal vagal reflex leading to bradycardia and cerebral hypoperfusion. It mostly occurs in patients with organic or functional disorders of the esophagus, and often requires permanent pacemaker implantation, along with treatment of the underlying esophageal pathology. In the following case, we report of a 71-year-old male with achalasia post per oral endoscopic myectomy, who presented with syncope and documented AV-block while eating solid food. In addition, long sinus pauses were noted during carotid sinus massage, suggesting that the two distinct entities may be associated, and further supporting the mechanism of neurally mediated syncope in the pathophysiology of swallow-induced syncope.

Hypoplastic Left Heart Syndrome with Congenital Complete Heart Block.

Congenital complete atrioventricular block (CCAVB) is a rare condition with an incidence of 1 of 20,000 live births. Hypoplastic left heart syndrome (HLHS) occurs more frequently than CCAVB and occurs in 1 of 5000 live births. HLHS in association with CCAVB is exceedingly rare. In this report, we describe a rare case of HLHS and CCAVB diagnosed in utero. Postnatal diagnosis, management and outcome are presented as well as review of the medical literature.

[The PRKAG2 cardiac syndrome with familial hypertrophic cardiomyopathy: an inherited burden. A case report. Review of the literature]. / La cardiomyopathie hypertrophique familiale dans le contexte de la mutation PRKAG2 : un lourd héritage. A propos d'un cas. Revue de la littérature.

We report the case of a young man without prior medical history who presented with a left ventricular heart failure because of a new onset atrial fibrillation. The evolution is characterized by a sinus rythm dysfunction, a complete atrioventricular block, a non-sustained ventricular tachycardia (which required a dual-chamber defibrillator implantation) and an ischemic testicular necrosis treated by orchidectomy. After ruling out differential diagnosis we evoked an hypertrophic cardiomyopathy. Medical family anamnesis revealed an hereditary component, and we concluded to a PRKAG2 cardiac syndrome presenting as a familial hypertrophic cardiomyopathy. Two years later, he presented with a type B aortic dissection. We review the literature and differentials diagnosis.

Le cas que nous présentons est celui d'un jeune patient sans antécédent médical qui présente une décompensation cardiaque gauche sur fibrillation auriculaire de novo. L'évolution est caractérisée par une dysfonction sinusale, un bloc atrioventriculaire syncopal, de la tachycardie ventriculaire non soutenue avec nécessité d'implanter un défibrillateur double chambre et par une nécrose testiculaire d'origine ischémique indiquant une orchidectomie. Plusieurs diagnostics différentiels sont évoqués. Les investigations plus poussées mettent en évidence une cardiomyopathie hypertrophique jusqu'alors asymptomatique. L'anamnèse familiale révèle une composante héréditaire qui nous amènera à évoquer le diagnostic de cardiomyopathie hypertrophique familiale liée à la mutation PRKAG2. Deux ans plus tard, il se présente avec une dissection aortique de type B. La littérature concernant cette pathologie et les diagnostics différentiels sont revus à cette occasion.

Pulmonary Thromboembolism and Myocarditis Resulting from a Large Pacing-Lead-Associated Right Ventricular Thrombus in a Dog with Chronic Cough as Presenting Sign.

In the present case report, we describe the clinical course and postmortem findings of a 12-year-old Labrador retriever dog with a third-degree atrio-ventricular block that developed a chronic cough, and later dyspnea and weakness as a result of massive pulmonary thromboembolism 3 years after implantation of a transvenous permanent pacemaker. A large soft tissue mass was seen in the right ventricular chamber around the pacing lead with echocardiography. Initially, this was thought to be caused by mural bacterial endocarditis based on hyperthermia, severe leukocytosis and the appearance of runs of ventricular tachycardia, the latter suggesting myocardial damage. While blood culture results were pending, antibiotics were administered without a positive effect. Due to clinical deterioration, the owner elected for euthanasia and a post-mortem examination confirmed a right ventricular thrombus and surrounding myocarditis, without signs of bacterial infection, and a massive pulmonary thromboembolism. We conclude that pulmonary thromboembolism should be considered in dogs with a cough that have an endocardial pacing lead implanted. Serial screening for proteinuria before and after implantation of an endocardial pacing lead would allow timely initiation of prophylactic antiplatelet therapy. Local myocarditis can develop secondary to an intracavitary thrombus, which can subsequently lead to runs of ventricular tachycardia.

Dual challenge inside the womb: a case report of concomitant fetal atrio-ventricular block associated with maternal anti-SSA antibodies and fetal tachyarrhythmia diagnosed as Wolff-Parkinson-White syndrome after birth.

Fetal autoimmune atrioventricular block (AVB) is a rare but potentially life-threatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first-degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described.

Unveiling a Rare Side Effect: A Report of a Unique Case of Second-Degree Type 2 Sinoatrial Node Exit Block Induced by Adenosine Infusion.

Adenosine is a widely used pharmacologic agent in the field of cardiology, predominantly for the termination of supraventricular tachycardias and diagnostic purposes. Most of the side effects are short-lasting due to its very short half-life. Fatal complications of adenosine are rare but can include ventricular fibrillation, ventricular tachycardia, and asystole. Proper medical supervision and monitoring are crucial to minimize risks. We report a unique case of a second-degree type 2 sinoatrial node exit block following intravenous adenosine administration in a 25-year-old male presenting with palpitations.

Involvement of CD73, equilibrative nucleoside transporters and inosine in rhythm and conduction disturbances mediated by adenosine A1 and A2A receptors in the developing heart.

We previously established that exogenous adenosine (ADO) induces transient arrhythmias in the developing heart via the adenosine A1 receptor (A1AR) and downstream activation of NADPH oxidase/ERK and PLC/PKC pathways. Here, we investigated the mechanisms by which accumulation of endogenous ADO and its derived compound inosine (INO) in the interstitial compartment induce rhythm and conduction troubles. The validated model of the spontaneously beating heart obtained from 4-day-old chick embryos was used. Quantitative RT-PCR showed that enzymes involved in ADO and INO metabolism (CD39, CD73 and eADA) as well as equilibrative (ENT1, -3, -4) and concentrative (CNT3) nucleoside transporters were differentially expressed in atria, ventricle and outflow tract. Inactivation of ENTs by dipyridamole, 1) increased myocardial ADO level, 2) provoked atrial arrhythmias and atrio-ventricular blocks (AVB) in 70% of the hearts, 3) prolonged P wave and QT interval without altering contractility, and 4) increased ERK2 phosphorylation. Blockade of CD73-mediated phosphohydrolysis of AMP to ADO, MEK/ERK pathway inhibition or A1AR inhibition prevented these arrhythmias. Exposure to exogenous INO also caused atrial ectopy associated with AVB and ERK2 phosphorylation which were prevented by A1AR or A2AAR antagonists exclusively or by MEK/ERK inhibitor. Inhibition of ADA-mediated conversion of ADO to INO increased myocardial ADO and decreased INO as expected, but slightly augmented heart rate variability without provoking AVB. Thus, during cardiogenesis, disturbances of nucleosides metabolism and transport, can lead to interstitial accumulation of ADO and INO and provoke arrhythmias in an autocrine/paracrine manner through A1AR and A2AAR stimulation and ERK2 activation.

Cardiac Sarcoidosis: A Literature Review of Current Recommendations on Diagnosis and Management.

Cardiac sarcoidosis (CS) is a rare multisystem disorder characterized by granulomatous infiltration of the myocardium, which can lead to significant morbidity and mortality. Its clinical manifestations range from asymptomatic conduction abnormalities to severe heart failure (HF) and sudden cardiac death. This comprehensive review aims to provide an overview of the diagnosis, clinical features, and current medical management strategies for CS. Additionally, the role of implantable cardioverter-defibrillators (ICDs) and the potential use of positron emission tomography in guiding management decisions are explored. A comprehensive understanding of the medical management of CS is essential for improving patient outcomes and guiding future research endeavors.

Successful Clindamycin Therapy of an Infected Subcutaneous Permanent Pacing Lead in a Dog after a Failed Course with Potentiated Amoxicillin and Enrofloxacin.

Though permanent pacemaker implantation is the only effective therapy for certain bradyarrhythmias in dogs, it is not without risks. Bacterial infection of the device is one of the most common complications. Human guidelines recommend besides systemic antibiotics, surgical explantation of the pacing lead and pulse generator in case of device-infection. This report describes a 13.5-year-old dog that received a transvenous endocardial permanent pacemaker because of syncopal episodes resulting from paroxysmal third-degree atrio-ventricular block. Five days after an uneventful surgery, a painful swelling appeared around the subcutaneous part of the lead where this was inserted into the jugular vein. A 4-week course of amoxicillin and clavulanic acid combined with enrofloxacin failed to clear the infection on long-term. Ultrasound-guided puncture of the abscess was performed to gain a sample for bacterial culture and antibiogram. Oral clindamycin of 4 weeks' duration successfully resolved the infection with Staphylococcus aureus without having to explant the device. Repeated ultrasonographic examinations and fine-needle aspiration biopsies were used to evaluate for persistent local inflammation, guiding the length of the antibiotic therapy. Though the described approach has traditionally been ill-advised because of the risk of introducing bacteria and damaging the pacemaker lead, it was successful in our case.

Teetering on the Edge: Second-Degree Atrioventricular Block Following Long-Acting Second-Generation Antipsychotic.

Our case report highlights the importance of understanding various mechanisms of an atrioventricular block (AVB) and recognizing potential iatrogenic culprits. Despite the prevalent use of second-generation antipsychotics and the growing popularity of long-acting formulations, it is not routinely recognized as a cause for AVB. Second-generation antipsychotics such as risperidone have a dose-dependent pro-arrhythmic effect and are known to cause first-degree AVB. Our case presents an opportunity to recognize an unappreciated cause for AVB and switch to safer alternatives. In the era of long-acting injectables, it is important to monitor for these effects prior to escalating doses and risking high-degree AVB.

Retrograde penetration pacing into the conduction system as an alternative approach of his-bundle pacing: Retrograde penetration pacing into the conduction system.

BACKGROUND: The optimal right ventricular (RV) pacing site during pacemaker implantation is still unclear due to left ventricular (LV) dyssynchrony by traditional RV pacing. His-bundle (HIS) pacing has achieved narrow QRS and maintained LV synchrony but high failure rate. RV septal pacing occasionally has QRS waveform with wide and narrow component in the early and late phase, respectively, and maintains LV synchrony, reflecting the normal conduction system. We aimed to define this QRS waveform as retrograde penetration pacing into the conduction system (RPP-CS) and compared its effect on LV synchrony as an alternative approach of HIS pacing. METHODS AND RESULTS: We enrolled 42 patients with atrio ventricular block (AVB) or bradycardia atrial fibrillation (AF) requiring pacemaker implantation (RPP-CS, n = 27; no RPP-CS, n = 15). Baseline characteristics were similar between the groups. RPP-CS was observed in 96% and 26% of the RV septum and apex area, respectively. RPP-CS had a significantly shorter QRS width (p < 0.001). The frequency of maintaining LV synchrony was significantly higher in RPP-CS (67% vs. 20%, p = 0.003). The QRS interval's optimal cut-off value during RPP-CS was 132 ms for prediction of LV synchrony (sensitivity 83%, specificity 89%, positive predictive value 94%, and negative predictive value 73%). During RPP-CS, shorter QRS intervals (QRS ≤ 132 ms) had better postoperative LV ejection fraction than longer intervals (p < 0.001). CONCLUSIONS: RPP-CS, especially with short QRS intervals (≤132 ms), had a high frequency of LV synchrony, maintained postoperative cardiac function, and may be an adequate first-line RV pacing site strategy for AVB or bradycardia AF as an alternative approach of HIS pacing.

Tricuspid leaflet flail after Micra™ leadless pacemaker implantation: a case report.

Background: Currently, the leadless pacemaker indications are limited to few and highly selected cases. Case summary: We describe the first reported case of an atrioventricular Micra™ leadless pacemaker implantation complicated by tricuspid posterior leaflet flail with severe regurgitation in a 29-year-old man affected by asymptomatic and progressive high degree atrio-ventricular block. The patient was then treated with endoscopic tricuspid valve repair, leadless pacemaker removal and implantation of an epicardial pacemaker. Discussion: Leadless pacemaker complications are multiple, hence it is essential to ensure a safe procedure, especially in the younger patients. We thought that the application of a transesophageal echocardiography guidance might mitigate the risk of major complications.

The Role of TRPM4 Gene Mutations in Causing Familial Progressive Cardiac Conduction Disease: A Further Contribution.

Progressive cardiac conduction disease (PCCD) is a relatively common condition in young and elderly populations, related to rare mutations in several genes, including SCN5A, SCN1B, LMNA and GJA5, TRPM4. Familial cases have also been reported. We describe a family with a large number of individuals necessitating pacemaker implantation, likely due to varying degrees of PCCD. The proband is a 47-year-old-patient, whose younger brother died at 25 years of unexplained sudden cardiac death. Three paternal uncles needed a pacemaker (PM) implantation between 40 and 65 years for unspecified causes. At the age of 42, he was implanted with a PM for two episodes of syncope and the presence of complete atrioventricular block (AVB). NGS analysis revealed the missense variation c. 2351G>A, p.Gly844Asp in the exon 17 of the TRPM4 gene. This gene encodes the TRPM4 channel, a calcium-activated nonselective cation channel of the transient receptor potential melastatin (TRPM) ion channel family. Variations in TRPM4 have been shown to cause an increase in cell surface current density, which results in a gain of gene function. Our report broadens and supports the causative role of TRPM4 gene mutations in PCCD. Genetic screening and identification of the causal mutation are critical for risk stratification and family counselling.

3-Dimensional computed tomographic assessment predicts conduction block and paravalvular leakage after rapid-deployment aortic valve replacement.

OBJECTIVES: Complications like complete atrioventricular block (CAVB) and paravalvular leakage (PVL) following rapid deployment aortic valve (AV) replacement (RDAVR) remain unresolved. Selecting an optimal size of the valve might be important to minimize the incidence of these complications. We sought to determine the impact of prosthesis size relative to the anatomic profile of the AV on the occurrence of CAVB or PVL after RDAVR. METHODS: Preoperative computed tomographic (CT) images were evaluated in patients receiving RDAVR (INTUITY ELITE) between February 2016 and December 2019. The occurrence of CAVB requiring permanent pacemaker implantation and PVL (≥ mild) was evaluated. The relative size of implants against the cross-sectional dimensions of recipients' AV annulus and left ventricular outflow tract (LVOT) were calculated. RESULTS: Among 187 eligible patients, CAVB and PVL (≥ mild) occurred in 12 (6.4%) and 11 patients (5.9%), respectively. CAVB was associated with oversized RDAVR (RDAVR frame width minus average diameter of LVOT calculated from the cross-sectional area [ΔLVOTarea]: odds ratio, 2.05; 95% confidence interval, 1.28-3.30): this was with an area under the curve of 0.78 (P = 0.005). The projected probability of CAVB was <3% when the ΔLVOTarea was <1.3. In contrast, PVL was associated with under-sized RDAVR (RDAVR size divided by the longest diameter of AV annulus [index Annlong]: odds ratio, 0.64; 95% confidence interval, 0.51-0.79): This was with an area under curve of 0.94 (P < 0.001). CONCLUSIONS: CT parameters of the AV annulus and LVOT are highly reliable in the prediction of CAVB or PVL after RDAVR. Our data might justify CT-based sizing of prosthesis for RDAVR.