Congenital Long QT Syndrome Type 2 with Symptomatic 2:1 Atrioventricular Block and Ventricular Arrhythmia in a Preterm Baby Who Presented with Fetal Ventricular Tachycardia and Hydrops.
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SUBMITTER: Shin JA
PROVIDER: S-EPMC8424453 | biostudies-literature | 2021 Sep
REPOSITORIES: biostudies-literature
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Congenital Long QT Syndrome Type 2 with Symptomatic 2:1 Atrioventricular Block and Ventricular Arrhythmia in a Preterm Baby Who Presented with Fetal Ventricular Tachycardia and Hydrops.
Shin Ju Ae JA Choi Yeon U YU Kim Kyung Min KM Yoon Ji Hong JH Lee Jae Young JY
Korean circulation journal 20210901 9
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Project description:Background Sarcoidosis is a granulomatous disease usually affecting the lungs, although cardiac morbidity may be common. The risk of these outcomes and the characteristics that predict them remain largely unknown. This study investigates the epidemiology of heart failure, atrioventricular block, and ventricular tachycardia among patients with and without sarcoidosis. Methods and Results We identified California residents aged ≥21 years using the Office of Statewide Health Planning and Development ambulatory surgery, emergency, or inpatient databases from 2005 to 2015. The risk of sarcoidosis on incident heart failure, atrioventricular block, and ventricular tachycardia were each determined. Linkage to the Social Security Death Index was used to ascertain overall mortality. Among 22 527 964 California residents, 19 762 patients with sarcoidosis (0.09%) were identified. Sarcoidosis was the strongest predictor of heart failure (hazard ratio [HR], 11.2; 95% CI, 10.7-11.7), atrioventricular block (HR, 117.7; 95% CI, 103.3-134.0), and ventricular tachycardia (HR, 26.1; 95% CI, 24.2-28.1) identified among all risk factors. The presence of any cardiac involvement best predicted each outcome. Approximately 22% (95% CI, 18%-26%) of the relationship between sarcoidosis and increased mortality was explained by the presence of at least 1 of these cardiovascular outcomes. Conclusions The magnitude of risk associated with sarcoidosis as a predictor of heart failure, atrioventricular block, and ventricular tachycardia, exceeds all established risk factors. Surveillance for and anticipation of these outcomes among patients with sarcoidosis is indicated, and consideration of a sarcoidosis diagnosis may be prudent among patients with heart failure, atrioventricular block, or ventricular tachycardia.
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Project description:BackgroundAcute myocardial infarction (AMI) complicated by tachyarrhythmias or high-grade atrioventricular block (HAVB) may lead to increased mortality.PurposeTo evaluate the sex differences in patients with AMI complicated by tachyarrhythmias and HAVB and their associated outcomes.Materials and methodsWe analyzed the incidence rates of arrhythmias following AMI from the Acute Coronary Syndrome Israeli Survey database from 2000 to 2018. We assessed the differences in arrhythmias incidence and the associated mortality risk between men and women.ResultsThis cohort of 14,280 consecutive patients included 3,159 (22.1%) women and 11,121 (77.9%) men. Women were less likely to experience early ventricular tachyarrhythmia (VTA), (1.6% vs. 2.3%, p = 0.034), but had similar rates of late VTA (2.3% vs. 2.2%, p = 0.62). Women were more likely to experience atrial fibrillation (AF) (8.6% vs. 5.0%, p < 0.001) and HAVB (3.7% vs. 2.3%, p < 0.001). The risk of early VTAs was similar in men and women [adjusted Odds Ratio (aOR) = 0.76, p = 0.09], but women had a higher risk of AF (aOR = 1.27, p = 0.004) and HAVB (aOR = 1.30, p = 0.03). Early [adjusted hazard ratio (aHR) = 2.84, p < 0.001] and late VTA (aHR =- 4.59, p < 0.001), AF (aHR = 1.52, p < 0.001) and HAVB (aHR = 2.83, p < 0.001) were associated with increased 30-day mortality. Only late VTA (aHR = 2.14, p < 0.001) and AF (aHR = 1.44, p = 0.002) remained significant in the post 30 days period.ConclusionsDuring AMI women experienced more AF and HAVB but fewer early VTAs than men. Early and late VTAs, AF, and HAVB were associated with increased 30-day mortality. Only late VTA and AF were associated with increased post-30-day mortality.
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Project description:BackgroundInappropriate sinus tachycardia (IST) is characterized by a continuum of symptoms, and the aetiology of IST is imprecise. IST-induced autonomic dysfunction is well known, but IST-induced atrio-ventricular block is not reported to our knowledge.Case summaryA 67-year-old female presented with a 4-day history of random intermittent difficulty in breathing, chest tightness, palpitations, and dizziness, with a recorded heart rate of 30 beats per minute (BPM) on home monitoring equipment. The initial electrocardiogram (ECG) demonstrated sinus rhythm with intermittent Mobitz type I second degree atrio-ventricular (AV) block, with continuous cardiac monitoring demonstrating frequent episodes of Wenckebach phenomenon throughout the day, with a sinus rate of 100-120 BPM. Echocardiogram showed no significant structural abnormalities. The patient was on bisoprolol, and hence, it was suspected Wenckebach may be due to that and so stopped. However, there was no tangible effect on rhythm 48 hours after stopping bisoprolol, leading to a suspicion of IST-induced Mobitz type I second degree AV block; and so decided to introduce ivabradine 2.5 mg twice daily. After 24 hours of Ivabradine, the patient remained in sinus rhythm with no documented episodes of Wenckebach phenomenon on cardiac monitor, a finding subsequently confirmed by 24-hour Holter monitoring. During a recent follow-up visit in clinic, the patient remained symptom-free, with an ECG demonstrating sinus rhythm at a physiological rate.DiscussionMobitz type I second degree AV block is usually due to reversible conduction block at the level of the AV node whereby malfunctioning AV nodal cells tend to progressively fatigue until they fail to conduct an impulse. Under conditions of increased vagal tone and autonomic dysfunction, Wenckebach occurrence will be increased. Thus, selective impulse conduction within the sinoatrial (SA) node by ivabradine to reduce beat conduction to the AV node in patients with IST/dysautonomia-induced Mobitz type I AV will reduce the occurrence of Wenckebach.
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Project description:AimsLong QT syndrome (LQTS) carries a risk of life-threatening polymorphic ventricular tachycardia (Torsades de Pointes, TdP) and is a major cause of premature sudden cardiac death. TdP is induced by R-on-T premature ventricular complexes (PVCs), thought to be generated by cellular early-afterdepolarisations (EADs). However, EADs in tissue require cellular synchronisation, and their role in TdP induction remains unclear. We aimed to determine the mechanism of TdP induction in rabbit hearts with acquired LQTS (aLQTS).Methods and resultsOptical mapping of action potentials (APs) and intracellular Ca2+ was performed in Langendorff-perfused rabbit hearts (n = 17). TdP induced by R-on-T PVCs was observed during aLQTS (50% K+/Mg++ & E4031) conditions in all hearts (P < 0.0001 vs. control). Islands of AP prolongation bounded by steep voltage gradients (VGs) were consistently observed before arrhythmia and peak VGs were more closely related to the PVC upstroke than EADs, both temporally (7 ± 5 ms vs. 44 ± 27 ms, P < 0.0001) and spatially (1.0 ± 0.7 vs. 3.6 ± 0.9 mm, P < 0.0001). PVCs were initiated at estimated voltages of ∼ -40 mV and had upstroke dF/dtmax and Vm-Ca2+ dynamics compatible with ICaL activation. Computational simulations demonstrated that PVCs could arise directly from VGs, through electrotonic triggering of ICaL. In experiments and the model, sub-maximal L-type Ca2+ channel (LTCC) block (200 nM nifedipine and 90% gCaL, respectively) abolished both PVCs and TdP in the continued presence of aLQTS.ConclusionThese data demonstrate that ICaL activation at sites displaying steep VGs generates the PVCs which induce TdP, providing a mechanism and rationale for LTCC blockers as a novel therapeutic approach in LQTS.
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