A Case of Empty Sella Syndrome with the First Clinical Manifestation of Sick Sinus Syndrome.

Empty sella syndrome (ESS) is characterized by the herniation of cerebrospinal fluid into the sella, which results in the enlargement of the sella and compression of the pituitary gland. ESS commonly accompanies pituitary dysfunction and abnormal secretion of one or more hormones, which manifests as symptoms like cold intolerance, fatigue, and memory impairment. However, the occurrence of sick sinus syndrome (SSS) in ESS has not been reported. A 66-year-old female patient was admitted to the hospital with complaints of dizziness and fatigue. Electrocardiogram (ECG) revealed sinus arrest, junctional escape rhythm, and a heart rate of 40 bpm. Then, the patient was diagnosed with SSS. Thyroid function test indicated decreased thyroxine levels and slightly elevated thyroid-stimulating hormone levels. Additionally, hyposecretion of cortisol and insulin-like growth factors was observed. Magnetic resonance imaging of the pituitary gland confirmed the diagnosis of ESS. The patient was treated with hydrocortisone and euthyrox, relieving the symptoms of dizziness and fatigue. Thyroid function tests during the follow-up period showed normal hormone levels, and ECG examination revealed no abnormalities.

Transient Sinus Node Dysfunction Associated with Herpes Simplex Encephalitis.

Sinus arrest and inappropriate sinus bradycardia are sinus node dysfunction (SND), which cause loss of consciousness. Cardiac pacing is recommended in patients with symptoms, such as syncope or dizziness. Several conditions can induce these arrhythmias; however, whether or not intracranial infectious diseases, such as herpes simplex encephalitis (HSE), can cause secondary SND is unclear. We encountered a patient with sinus arrest and transient sinus bradycardia associated with HSE. Since cardiac pause was never monitored and the bradycardia improved after HSE treatment, HSE was suspected to be the cause of SND. Although the underlying mechanisms have not yet been completely elucidated, HSE may cause secondary SND.

Pacing mode survival in patients with single chamber atrial pacemaker for sinus node dysfunction.

BACKGROUND: Single chamber atrial pacemaker should be sufficient for patients with sinus node dysfunction (SND) with normal atrioventricular (AV) conduction. However, most patients undergo dual chamber pacemaker implantation because of concern of new onset AV block. The annual incidence of new AV block has been reported from 0.6 to 4.4 % in various studies. OBJECTIVES: Our aim is to assess mode survival in sinus node dysfunction with normal AV conduction patients implanted with AAIR. METHODS: Patients who underwent single chamber atrial pacemaker implantation for SND with normal AV conduction between January 2014 and December 2021 were followed up for pacemaker device change, new onset AV block, bundle branch block, atrial fibrillation (AF), lead complications, reoperation and mortality rate. RESULTS: A total of 113 patients underwent single chamber atrial pacemaker implantation for SND during the study period. Mean age was 55.6 ± 12.7 years. During a mean follow up of 48.7 ± 24.9 months, none of the patients required pacemaker device change to VVIR/DDDR. Nine patients underwent reoperation, 5 for lead dislodgment, 1 for high threshold, 1 for pocket site erosion and 3 for pulse generator change. None developed AV block or AF with slow ventricular rate. Only 4 patients developed AF (3 paroxysmal,1 permanent). There were 3 deaths during follow up and none were sudden deaths. CONCLUSION: Single chamber atrial pacing is an acceptable mode of pacing in patients with SND in developing countries. Development of AV conduction abnormalities is rare in this relatively younger population.

Oral Albuterol Treatment in Three Pediatric Patients with Bradycardia: A Novel Therapy.

Clinically significant bradycardia is an uncommon problem in children, but one that can cause significant morbidity and sometimes necessitates implantation of a pacemaker. The most common causes of bradycardia are complete heart block (CHB), which can be congenital or acquired, and sinus node dysfunction, which is rare in children with structurally normal hearts. Pacemaker is indicated as therapy for the majority of children with CHB, and while early mortality is lower in postnatally diagnosed CHB than in fetal CHB, it is still up to 16%. In young children, less invasive transvenous pacemaker systems can be technically challenging to place and carry a high risk of complications, often necessitating surgical epicardial pacemaker placement, which usually entails a median sternotomy. We report three cases of pediatric patients referred for pacemaker implantation for different types of bradycardia, treated at our institution with oral albuterol with therapeutic results that avoided the need for surgical pacemaker implantation at that time.

An unmet need: arrhythmia detection by implantable loop recorder in the systemic right ventricle.

AIMS: Patients with systemic right ventricles are at high risk of sudden cardiac death. Arrhythmia is a significant risk factor. Routine Holter monitoring is opportunistic with poor adherence. The aim of this study was to determine if continuous rhythm monitoring with an implantable loop recorder (ILR) could allow early detection of clinically important arrhythmias. METHODS AND RESULTS: Implantable loop recorder implantation was offered to patients with atrial switch repair for transposition of the great arteries. Recordings were made with symptoms or, automatically for pauses, significant bradycardia or tachycardia and reviewed by the multi-disciplinary team. Twenty-four out of 36 eligible patients underwent ILR implantation with no complication. Forty-two per cent had preserved ventricular function, 75% were NYHA functional class I, 88% had low sudden cardiac death risk, 33% had previous intra-atrial re-entrant tachycardia (IART), and none had known conduction disease. Eighteen out of 24 (75%) patients made 52 recordings (52% automated) over 39.5 months (1.6-72.5). Thirty-two out of 52 (62%) recordings in 15/24 (63%) of the cohort were clinically significant and included sinus node disease (two patients), atrioventricular block (two patients), IART (seven patients), and IART with sinus node disease or atrioventricular block (four patients). Implantable loop recorder recordings prompted medication change in 11 patients [beta-blockers (n = 9), anti-coagulation (n = 5), and stopping anti-coagulation (n = 1)] and device therapy recommendation in seven patients [five pacemakers (three: atrioventricular block) and two defibrillators]. Two patients declined intervention; one suffered an arrhythmic death. Intra-atrial re-entrant tachycardia and clinically relevant conduction disease were detected in patients irrespective of sudden cardiac death risk. CONCLUSION: Continuous monitoring with an ILR in patients with systemic right ventricle following atrial switch detects clinically relevant arrhythmias that impact decision-making. In this cohort, clinically relevant arrhythmias did not correlate with sudden cardiac death risk.

Sinus Node Dysfunction.

Sinus node dysfunction (SND) is a multifaceted disorder most prevalent in older individuals, but may also occur at an earlier age. In most cases, the SND diagnosis is ultimately established by documenting its ECG manifestations. EPS has limited utility. The treatment strategy is largely dictated by symptoms and ECG manifestations. Not infrequently, both bradycardia and tachycardia coexist in the same patients, along with other diseases common in the elderly (e.g., hypertension, coronary artery disease), thereby complicating treatment strategy. Prevention of the adverse consequences of both bradyarrhythmia and tachyarrhythmia is important to reduce susceptibility to syncope, falls, and thromboembolic complications.

The PhysioVP-AF study, a randomized controlled trial to assess the clinical benefit of physiological ventricular pacing vs. managed ventricular pacing for persistent atrial fibrillation prevention in patients with prolonged atrioventricular conduction: design and rationale.

AIMS: In patients with prolonged atrioventricular (AV) conduction and pacemaker (PM) indication due to sinus node disease (SND) or intermittent AV-block who do not need continuous ventricular pacing (VP), it may be difficult to determine which strategy to adopt. Currently, the standard of care is to minimize unnecessary VP by specific VP avoidance (VPA) algorithms. The superiority of this strategy over standard DDD or DDD rate-responsive (DDD/DDDR) in improving clinical outcomes is controversial, probably owing to the prolongation of the atrialventricular conduction (PR interval) caused by the algorithms. Conduction system pacing (CSP) may offer the most physiological-VP approach, providing appropriate AV conduction and preventing pacing-induced dyssynchrony. METHODS AND RESULTS: PhysioVP-AF is a prospective, controlled, randomized, single-blind trial designed to determine whether atrial-synchronized conduction system pacing (DDD-CSP) is superior to standard DDD-VPA pacing in terms of 3-year reduction of persistent-AF occurrence. Cardiovascular hospitalization, quality-of-life, and safety will be evaluated. Patients with indication for permanent DDD pacing for SND or intermittent AV-block and prolonged AV conduction (PR interval > 180 ms) will be randomized (1:1 ratio) to DDD-VPA (VPA-algorithms ON, septal/apex position) or to DDD-CSP (His bundle or left bundle branch area pacing, AV-delay setting to control PR interval, VPA-algorithms OFF). Approximately 400 patients will be randomized in 24 months in 13 Italian centres. CONCLUSION: The PhysioVP-AF study will provide an essential contribution to patient management with prolonged AV conduction and PM indication for sinus nodal disease or paroxysmal 2nd-degree AV-block by determining whether CSP combined with a controlled PR interval is superior to standard management that minimizes unnecessary VP in terms of reducing clinical outcomes.

Sinus node dysfunction and atrial fibrillation-Relationships, clinical phenotypes, new mechanisms, and treatment approaches.

Although the anatomical basis of the pathogenesis of sinus node dysfunction (SND) and atrial fibrillation (AF) is located primarily in the left and right atria, increasing evidence suggests a strong correlation between SND and AF, in terms of both clinical presentation and formation mechanisms. However, the exact mechanisms underlying this association are unclear. The relationship between SND and AF may not be causal, but is likely to involve common factors and mechanisms, including ion channel remodeling, gap junction abnormalities, structural remodeling, genetic mutations, neuromodulation abnormalities, the effects of adenosine on cardiomyocytes, oxidative stress, and viral infections. Ion channel remodeling manifests primarily as alterations in the "funny" current (If) and Ca2+ clock associated with cardiomyocyte autoregulation, and gap junction abnormalities are manifested primarily as decreased expression of connexins (Cxs) mediating electrical impulse propagation in cardiomyocytes. Structural remodeling refers primarily to fibrosis and cardiac amyloidosis (CA). Some genetic mutations can also cause arrhythmias, such as SCN5A, HCN4, EMD, and PITX2. The intrinsic cardiac autonomic nervous system (ICANS), a regulator of the heart's physiological functions, triggers arrhythmias.In addition, we discuss arrhythmias caused by viral infections, notably Coronavirus Disease 2019 (COVID-19). Similarly to upstream treatments for atrial cardiomyopathy such as alleviating CA, ganglionated plexus (GP) ablation acts on the common mechanisms between SND and AF, thus achieving a dual therapeutic effect.

Supraventricular tachyarrhythmia and sinus node dysfunction as a first manifestation of short QT syndrome in a pediatric patient. Case Report.

Short QT syndrome (SQTS) represents a diagnosis challenge where the symptoms may vary from palpitations in an otherwise asymptomatic patient to sudden death. Is a recently discovered rare channelopathy, identified by Gussak in 2000, characterized by short QT intervals on the electrocardiogram and a tendency to develop atrial and ventricular arrhythmias in the absence of structural heart disease, hyperkalemia, hypercalcemia, hyperthermia, acidosis and endocrine disorders. We present the case of a 16-year-old patient with short QT-type channelopathy, who presented with sinus arrest and junctional rhythm, who later developed atrial tachycardia and atrial flutter.

Catheter ablation for treatment of bradycardia-tachycardia syndrome: is it time to consider it the therapy of choice? A systematic review and meta-analysis.

BACKGROUND: Atrial fibrillation catheter ablation (AFCA) should be considered as a strategy to avoid pacemaker (PM) implantation for patients with bradycardia-tachycardia syndrome (BTS), but lack of evidence is remarkable. METHODS: Our aim was to conduct a random-effects model meta-analysis on safety and efficacy data from controlled trials and observational studies. We compared atrial fibrillation (AF) recurrence, AF progression, procedural complication, additional procedure, cardiovascular death, cardiovascular hospitalization, heart failure and stroke in patients undergoing AFCA vs. PM implantation. RESULTS: PubMed/MEDLINE, Cochrane Database and Google Scholar were screened, and four retrospective studies were selected. A total of 776 patients (371 in the AFCA group, 405 in the PM group) were included. After a median follow-up of 67.5 months, lower AF recurrence [odds ratio (OR) 0.06, confidence interval (CI) 0.02-0.18, I2 = 82.42%, P < 0.001], AF progression (OR 0.12, CI 0.06-0.26, I2 = 0%, P < 0.001), heart failure (OR 0.12, CI 0.04-0.34, I2 = 0%, P < 0.001), and stroke (OR 0.30, CI 0.15-0.61, I2 = 0%, P = 0.001) were observed in the AFCA group. No differences were observed in cardiovascular death and hospitalization (OR 0.48, CI 0.10-2.28, I2 = 0%, P = 0.358 and OR 0.43, CI 0.14-1.29, I2 = 87.52%, P = 0.134, respectively). Higher need for additional procedures in the AFCA group was highlighted (OR 3.65, CI 1.51-8.84, I2 = 53.75%, P < 0.001). PM implantation was avoided in 91% of BTS patients undergoing AFCA. CONCLUSIONS: AFCA in BTS patients seems to be more effective than PM implantation in reducing AF recurrence and PM implantation may be waived in most BTS patients treated by AFCA. Need for additional procedures in AFCA patients is balanced by long-term benefit in clinical end points.

An Adolescent Patient with Sick Sinus Syndrome Complicated by Hypothyroidism Carrying an SCN5A Variant.

Previous studies have reported that hypothyroidism can lead to sick sinus syndrome (SSS) or other rhythm disturbances. Variants in the alpha subunit of the cardiac sodium channel (SCN5A) are known to be among the genetic causes of SSS. We encountered an adolescent patient with SSS and hypothyroidism who also harbored an SCN5A variant. The patient was a 13-year-old girl who was referred to our hospital because of bradycardia identified during a school electrocardiography screening. Clinical examination revealed severe hypothyroidism due to Hashimoto thyroiditis and SSS. After levothyroxine supplementation, her symptoms of hypothyroidism improved; however, the SSS did not. Genetic testing revealed a heterozygous variant (c.1066 G>A, p.Asp356Asn) in SCN5A. This is the first report of the coexistence of SSS due to an SCN5A variant and severe hypothyroidism in an adolescent patient. While patients with SCN5A variants exhibit phenotypic heterogeneity due to the presence of various modifiers, the presence of severe hypothyroidism may affect the development of SSS. This case highlights the importance of genetic analysis, including testing for SCN5A variants, in patients with hypothyroidism complicated by SSS or cardiac conduction disorders.

Systemic sclerosis and tachycardia-bradycardia syndrome: a case report.

BACKGROUND: Systemic sclerosis is a multisystemic character autoimmune disease. It is characterized by vascular dysfunction and progressive fibrosis affecting mainly the skin but also different internal organs. All heart structures are commonly affected, including the pericardium, myocardium, and conduction system. However, tachycardia-bradycardia syndrome is not common in the literature as a cardiac complication of systemic sclerosis. Case presentation We report a case of tachycardia-bradycardia syndrome in a 46-year-old Moroccan woman followed for systemic sclerosis with cutaneous, vascular, and articular manifestations. The diagnosis was based mainly on patient-reported symptoms and electrocardiogram data. A permanent pacemaker was implanted, allowing the introduction of beta-blockers with good outcomes. CONCLUSIONS: This case aims to show that even minor electrocardiogram abnormalities should be monitored in this group of patients, preferably by 24-hour ambulatory electrocardiogram because they could be a good indicator of the activity and progression of cardiac fibrosis.

Early and Late Outcomes of the Warden and Modified Warden Procedure.

BACKGROUND: Operative repair of partial anomalous pulmonary venous connection (PAPVC) remains challenging due to risks of sinus node dysfunction, baffle obstruction, and superior vena cava (SVC) obstruction. METHODS: Traditional or modified Warden procedures were performed in 75 of 318 consecutive patients (24%) with PAPVC repaired surgically at our institution during 1993 to 2021. Clinical characteristics, echocardiography data, operative details, and early and late outcomes were collected. Cumulative incidence of reintervention and Kaplan-Meier survival analysis are reported. RESULTS: Median age was 39 years (interquartile range, 21-57 years). Fifty-nine (79%) had normal sinus rhythm preoperatively. Seventeen (23%) had intact atrial septa. Traditional and modified Warden procedures were performed in 15 (20%) and 60 (80%), respectively. Frequent concomitant procedures included 15 (20%) tricuspid valve repairs and 12 (16%) atrial fibrillation procedures. There were no early deaths. Postoperative complications included atrial fibrillation in 17 (23%), sinus node dysfunction in 15 (20%), pneumothorax in 3 (4%), pleural effusion in 2 (3%), and pacemaker implantation in 1 (1%). At hospital discharge, sinus node dysfunction persisted in 8 (11%). Over a median follow-up of 6 years (interquartile range, 4-10 years), baffle obstruction developed in 1 patient and SVC obstruction developed in 7. None required reoperation and 6 were treated with SVC stents. At 1, 5, and 10 years, the cumulative incidence of reintervention was 5%, 7%, and 14%, and survival was 99%, 94%, and 94%, respectively. CONCLUSIONS: Traditional and modified Warden procedures can be performed with satisfactory early and late survival. Persistent sinus node dysfunction and need for permanent pacing are low. Late SVC obstruction is uncommon and can often be managed nonoperatively.

Predictors of permanent pacemaker insertion after mitral valve replacement: A systematic review.

As the established surgical mitral valve replacement (MVR) expands toward various contemporary techniques and access routes, the predictors and burden of procedure-related complications including the need for permanent pacemaker (PPM) implantation need to be identified. Digital databases were searched systematically to identify studies reporting the incidence of PPM implantation after MVR. Detailed study and patient-level baseline characteristics including the type of study, sample size, follow-up, number of post-MVR PPM implantations, age, gender, and baseline ECG abnormalities were abstracted. A total of 12 studies, recruiting 37,124 patients were included in the final analysis. Overall, 2820 (7.6%) patients required a PPM with the net rate ranging from 1.7% to 10.96%. Post-MVR atrioventricular (AV) block was the most commonly observed indication for PPM, followed by sinoatrial (SA) node dysfunction, and bradycardia. Age, male gender, pre-existing comorbid conditions, prior CABG, history of arrhythmias or using antiarrhythmic drugs, atrial fibrillation ablation, and double valve replacement were predictors of PPM implantation post-MVR. Age, male gender, comorbid conditions like diabetes and renal impairment, prior CABG, double valve replacement, and antiarrhythmic drugs served as positive predictors of PPM implantation in patients undergoing MVR.

Sinus Node Dysfunction Co-occurring with Immune Checkpoint Inhibitor-associated Myocarditis.

Immune checkpoint inhibitor (ICI)-induced myocarditis is a potentially life-threatening adverse event. We herein report a rare case of sick sinus syndrome (SSS) co-occurring with ICI-associated myocarditis. A 71-year-old woman with lung cancer undergoing pembrolizumab monotherapy was admitted owing to a fever, worsening kidney function, and sinus bradycardia. She was diagnosed with multi-organ immune-related adverse events, including myocarditis. Pulse steroid therapy was initiated immediately under the support of a temporary pacemaker, which resulted in the resolution of SSS in a few days. Biopsy specimens of the endomyocardium showed active myocarditis. Thus, we should be aware that SSS can co-occur with ICI-induced myocarditis.

Implantation of a dual-chamber pacemaker in a patient with dextrocardia and sick sinus syndrome: a case report.

Dextrocardia is a congenital abnormal position of the heart in which the main part of the heart is in the right chest, and the long axis of the heart points to the lower right. Cases of a combination of dextrocardia and sick sinus syndrome are rare. A 65-year-old female patient was admitted to hospital with palpitations and dizziness for 1 week. Mirror-image dextrocardia and sick sinus syndrome were diagnosed by an electrocardiogram, echocardiography, Holter monitoring, and X-rays. Finally, we successfully implanted a dual-chamber pacemaker into the patient. The patient had an uneventful recovery and was discharged when her symptoms had greatly improved 1 week later. When dextrocardia is present, using active fixation leads in the atrial and ventricular leads is easier for finding the pacing position with optimal sensing and pacing thresholds, and they reduce the incidence of falling off.

Sick sinus syndrome as the initial manifestation of neuromyelitis optica spectrum disorder: a case report.

BACKGROUND: Sick sinus syndrome (SSS) is known to occur due to lesions in the medulla oblongata. Although medullary lesions have occurred in patients with neuromyelitis optica spectrum disorder (NMOSD), there are few reports of SSS associated with NMOSD. We report a patient with NMOSD who developed refractory nausea, vomiting and SSS as the initial manifestation. CASE PRESENTATION: A 77-year-old female developed refractory nausea and frequent episodes of syncope. The patient was diagnosed with SSS because sinus pauses lasting five to six seconds were observed, and pacemaker implantation was performed. Two months later, she was referred to our hospital because of limb weakness and sensory impairment that progressed over a month. The patient was confirmed to have muscle weakness; manual muscle testing revealed grade 4 in the upper extremities and grade 3 in the lower extremities. Tendon reflexes were diminished, while no pathological reflexes were present. Thermal and pain sensations were impaired in the upper and lower extremities, and vibration sensation was impaired in both lower extremities. Bladder and rectal disturbances were also noted. Optic neuritis was not detected. T2-weighted magnetic resonance imaging (MRI) showed high-intensity lesions in the dorsal part of the medulla oblongata and C3-6 cervical cord. Her serum was positive for antibodies against aquaporin 4, and a diagnosis of NMOSD was made. She was treated with two courses of an intravenous methylprednisolone pulse and one course of plasma exchange. Then, she was transferred to another hospital for rehabilitation. CONCLUSIONS: Because SSS is a life-threatening complication, clinicians should be aware of the possibility that medullary lesions in NMOSD can cause SSS as the initial manifestation.

Beta-Blocker-Related Atrioventricular Conduction Disorders-A Single Tertiary Referral Center Experience.

Background and Objectives: Drug-related bradyarrhythmia is a well-documented major adverse event among beta-blocker users and a potential cause for hospitalization or additional interventions. Whether beta-blocker use is associated with specific bradyarrhythmia presentations, and how this relates to other predisposing factors, is not well known. We aim to evaluate the association between beta-blocker use and the type of atrioventricular (AV) conduction disorder in patients with symptomatic bradycardia. Materials and Methods: We conducted a retrospective cohort study on 596 patients with a primary diagnosis of symptomatic bradyarrhythmia admitted to a single tertiary referral center. Of the cases analyzed, 253 patients were on beta-blocker treatment at presentation and 343 had no bradycardic treatment. We analyzed demographics, clinical and paraclinical parameters in relation to the identified AV conduction disorder. A multivariate regression analysis was performed to explore factors associated with beta-blocker use. Results: Of the 596 patients (mean age 73.9 ± 8.8 years, 49.2% male), 261 (43.8%) had a third-degree AV block, 92 (15.4%) had a second-degree AV block, 128 (21.5%) had slow atrial fibrillation, 93 (15.6%) had sick sinus syndrome and 21 (3.5%) had sinus bradycardia/sinus pauses. Beta-blocker use was associated with the female gender (p < 0.001), emergency admission (p < 0.001), dilated cardiomyopathy (p = 0.003), the lower left ventricular ejection fraction (p = 0.02), mitral stenosis (p = 0.009), chronic kidney disease (p = 0.02), higher potassium levels (p = 0.04) and QRS duration > 120 ms (p = 0.02). Slow atrial fibrillation (OR = 4.2, p < 0.001), sick sinus syndrome (OR = 2.8, p = 0.001) and sinus bradycardia/pauses (OR = 32.9, p < 0.001) were more likely to be associated with beta-blocker use compared to the most common presentation (third-degree AV block), after adjusting for other patient characteristics. Conclusions: Beta-blocker use is more likely to be associated with slow atrial fibrillation, sick sinus syndrome and sinus bradycardia/pauses, compared to a second- or third-degree AV block, after adjusting for other patient factors such as gender, admission type, ECG, comorbidities, cardiac function and lab testing.