Hemochromatosis as Junctional Tachycardia, a Rare Presentation.
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ABSTRACT:
SUBMITTER: Ghani A
PROVIDER: S-EPMC5543826 | biostudies-other | 2017 Jun
REPOSITORIES: biostudies-other
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Project description:Differential diagnosis is challenging in poor conditioned neonates referred to the emergency room. Infectious disease is common, yet tachycardia should alert the clinician to look for cardiac arrhythmia and comprise. Tachycardia can lead to cardiomyopathy and should warrant further diagnostics for myocarditis, especially in rare or unusual combination of arrhythmias.
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Project description:Cryoablation of slow pathway doesn't usually cause junctional beats. If this occurs, the nearness to AV compact node is supposed. 3d electroanatomical mapping during this unusual finding may help to clarify the relationship between junctional beats (JBs) during cryomapping/cryoablation and Koch's triangle.
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Project description:Junctional ectopic tachycardia (JET) is a potentially life-threatening postoperative arrhythmia in children with specific congenital heart defects and can contribute significantly to postoperative morbidity for at-risk populations. In adults, β1-adrenergic receptor (ADRB1) and β2-adrenergic receptor (ADRB2) genotypes have been associated with increased risk for arrhythmias. However, their association with arrhythmia risk in children is unknown. We aimed to test associations between ADRB1 and ADRB2 genotypes and postoperative JET in patients with congenital heart defects. Children who underwent cardiac surgery were genotyped for the ADRB1 p.Ser49Gly (rs1801252; c.145A>G), p.Arg389Gly (rs1801253; c.1165C>G), ADRB2 p.Arg16Gly (rs1042713; c.46A>G), and p.Glu27Gln (rs1042714; c.79G>C) polymorphisms. The occurrence of postoperative JET was assessed via cardiologist-interpreted electrocardiograms. Genotype associations with JET were analyzed via logistic regression, adjusted for clinical variables associated with JET, with separate analysis in patients not on a β-blocker. Of the 343 children included (median age 8 months, 53% boys, 69% European ancestry), 45 (13%) developed JET. The Arg389Arg genotype was not significantly associated with JET in the overall population (odds ratio [OR] = 1.96, 95% confidence interval [CI] = 0.96-4.03, p = 0.064), but was nominally associated in patients not taking a β-blocker (n = 324, OR = 2.25, 95% CI = 1.05-4.80. p = 0.034). None of the other variants were associated with JET. These data suggest that the ADRB1 Arg389Arg genotype may predict risk for JET following cardiac surgery in pediatric patients in the absence of β-blockade. Whether treatment with a β-blocker ameliorates this association requires further research.
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Project description:BackgroundFabry disease is an inherited rare metabolic disease caused by mutation in the GLA gene, encoding lysosomal enzyme alpha-galactosidase A. The disorder is a systemic disease that manifests as cerebrovascular and cardiac disease, chronic renal failure, skin lesion, peripheral neuropathy, and other abnormalities. Ventricular tachycardia as a Fabry disease presentation is very rare.Case summaryA 36-year-old man self-presented to a general practitioner complaining of episodes of shortness of breath together with a 6-month history of malaise. The 12-lead electrocardiogram (ECG) prompted a decision to transfer him immediately to a percutaneous coronary intervention (PCI) capable hospital under the suspicion of acute coronary syndrome. Whilst awaiting transport, he experienced acute onset of dyspnoea together with non-specific chest heaviness. A repeat ECG monitor strip showed ventricular tachycardia transforming to ventricular fibrillation. The patient was successfully defibrillated. Coronary angiography was performed upon arrival at hospital and demonstrated unobstructed coronary arteries. Transthoracic echocardiography revealed concentric left ventricular hypertrophy (LVH) and normal systolic function, with severe diastolic dysfunction. Magnetic resonance imaging (MRI) confirmed the LVH, and did not demonstrate any late gadolinium enhancement.DiscussionOur case illustrates the pivotal role of critical clinical thinking in the diagnosis of rare but treatable hereditary cardiomyopathy. The uncommon cardiac presentation of Fabry disease promotes further research linking different phenotypes of Fabry disease with different pathogenic mutations.
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Project description:Postural orthostatic tachycardia syndrome (POTS) presents heterogeneously and is diagnosed when appropriate symptoms are present in conjunction with a heart rate increase of at least 30 beats-per-minute upon standing without orthostatic hypotension. Much of the current understanding of POTS is based on clinical expertise, particularly regarding POTS phenotypes and their potential role in targeting pharmacologic treatment. This study describes the symptom presentation of POTS by phenotypes at a subspecialty POTS clinic. Data was collected prospectively during clinical visits between April 17, 2014 and February 8, 2021. This data was abstracted retrospectively by chart review. Most of the 378 study participants were female (89.9%) with a mean age 23.0 ± 4.9 years. Lightheadedness was the most common (97.6%) symptom and the most disruptive of quality of life (29.9%). Patients reported substantial functional impairment across multiple life domains, with 3.0 ± 2.8 days lost and 4.7 ± 2.3 unproductive days per week. There were no differences in symptom presentation among POTS phenotypes. POTS phenotypes are not distinguishable based on symptoms alone; if phenotyping is sought, testing is necessary. Further research is needed in better classifying POTS phenotypes with the potential goal of tailoring treatment.
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Project description:Background Junctional ectopic tachycardia (JET) is a prevalent life-threatening arrhythmia in children with congenital heart disease (CHD), with marked resemblance to normal sinus rhythm (NSR) often leading to delay in diagnosis. Objective To develop a novel automated arrhythmia detection tool to identify JET. Methods A single-center retrospective cohort study of children with CHD was performed. Electrocardiographic (ECG) data produced by bedside monitors is captured automatically by the Sickbay platform. Based on the detection of R and P wave peaks, 2 interpretable ECG features are calculated: P prominence median and PR interval interquartile range (IQR). These features are used as input to a simple logistic regression classification model built to distinguish JET from NSR. Results This study analyzed a total of 64.5 physician-labeled hours consisting of 509,833 cardiac cycles (R-R intervals), from 40 patients with CHD. The extracted P prominence median feature is much smaller in JET compared to NSR, whereas the PR interval IQR feature is larger in JET compared to NSR. The area under the receiver operating characteristic curve for the unseen patient test cohort was 93%. Selecting a threshold of 0.73 results in a true-positive rate of 90% and a false-positive rate of 17%. Conclusion This novel arrhythmia detection tool identifies JET, using 2 distinctive features of JET in ECG—the loss of a normal P wave and PR relationship—allowing for early detection and timely intervention.
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Project description:Background "Palpitations" are one of the most common complaints prompting medical attention. Textbooks of medicine and cardiology as well as guideline documents and position papers describe palpitations as a common symptom of ventricular tachycardia (VT). However, data to support this description are lacking. The aim of our study was to evaluate the symptomatology of sustained monomorphic VT with emphasis on the prevalence of palpitations. Methods and Results Consecutive patients presenting to our center with a first event of a regular sustained monomorphic VT (n=59) or a regular supraventricular tachycardia (SVT; n=109) between January 2012 and September 2019 were interviewed regarding their symptoms during the arrhythmic event. We included only patients with a first arrhythmic event to avoid the influence of previous medical encounters on our patients' terminology. As expected, patients with VT were older (age 68.8±13.6 versus 52.6±16.8 years; P<0.001), more often of male sex (94.9% versus 37.6%; P<0.001), had lower left ventricular ejection fraction (37±11% versus 59±2%, P<0.001) and more comorbidities (87.6% versus 40.5%; P<0.001) compared with patients with SVT. Importantly, even though the heart rate upon presentation did not differ between the 2 groups (165±26 beats/min during VT versus 171±32 beats/min during SVT; P=0.16), symptomatology differed significantly; specifically, palpitations were reported in only 8.8% of VT patients, compared with 90.7% of SVT patients (P<0.001). Common symptoms in the VT group included chest pain (64%), dyspnea (21%), and dizziness (26%). Conclusions Despite similar heart rate, patients with VT rarely report having palpitations, whereas patients with SVT do so commonly. This finding may assist with decision making in patients reporting palpitations in whom an ECG tracing is not available.
| S-EPMC7763738 | biostudies-literature
Project description:BackgroundInfective endocarditis with paravalvular abscess can be complicated by atrioventricular block (AVB), but junctional ectopic tachycardia (JET) has as yet never been described.Case summaryA 68-year-old male recently admitted with Staphylococcal aureus endocarditis of his aortic valve bioprosthesis, presented with a regular tachycardia at 240 b.p.m. with a pre-existent right bundle branch block pattern. Haemodynamic collapse necessitated electrical cardioversion, following which high-grade AVB was observed. Multiple recurrences of the same tachycardia required repeated electrical cardioversions and emergent electrophysiological study, which indicated JET. The tachycardia was unresponsive to overdrive pacing, adenosine and intravenous amiodarone, and external cardioversions. Radiofrequency catheter ablation of the atrioventricular node was performed emergently with interruption of the tachycardia. A temporary external pacemaker was implanted via a jugular route. The tachycardia recurred after 48 h at a slower rate, and the patient underwent redo ablation. Transoesophageal echocardiography revealed a pseudoaneurysm of the right sinus of Valsalva probably corresponding to an evacuated abscess. A permanent pacemaker was implanted after active infection had been ruled out. At 3 months of follow-up, the patient had complete AVB, without arrhythmia recurrence.DiscussionThis is the first case report of JET complicating a paravalvular abscess of the aortic valve with concomitant AVB. Junctional ectopic tachycardia is very rare arrhythmia which is usually seen in children as a congenital arrhythmia or following surgical correction of paediatric heart disease. The differential diagnosis is discussed in detail in the article.
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Project description:Postoperative junctional ectopic tachycardia is one of the most serious arrhythmias that occur after pediatric cardiac surgery, difficult to treat and better to be prevented. Our aim was to assess the efficacy of prophylactic dexmedetomidine in preventing junctional ectopic tachycardia after pediatric cardiac surgery.A prospective controlled study was carried out on 90 children who underwent elective cardiac surgery for congenital heart diseases. Patients were randomized into 2 groups. Group I (dexmedetomidine group): 60 patients received dexmedetomidine; Group II (Placebo group): 30 patients received the same amount of normal saline intravenously. The primary outcome was the incidence of postoperative junctional ectopic tachycardia. Secondary outcomes included bradycardia, hypotension, vasoactive inotropic score, ventilation time, pediatric cardiac care unit stay, length of hospital stay, and perioperative mortality. The incidence of junctional ectopic tachycardia was significantly reduced in the dexmedetomidine group (3.3%) compared with the placebo group (16.7%) with P<0.005. Heart rate while coming off cardiopulmonary bypass was significantly lower in the dexmedetomidine group (130.6±9) than the placebo group (144±7.1) with P<0.001. Mean ventilation time, and mean duration of intensive care unit and hospital stay (days) were significantly shorter in the dexmedetomidine group than the placebo group (P<0.001). However, there was no significant difference between the 2 groups as regards mortality, bradycardia, or hypotension (P>0.005).Prophylactic use of dexmedetomidine is associated with significantly decreased incidence of postoperative junctional ectopic tachycardia in children after congenital heart surgery without significant side effects.
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