scholarly journals Cardiac and non-cardiac characteristics of Jervell-Lange-Nielsen syndrome

2021 ◽  
Vol 28 (3) ◽  
pp. 37-44
Author(s):  
R. A. Ildarova ◽  
N. V. Sherbakova ◽  
V. V. Bereznitskaya ◽  
M. A. Shkolnikova
Keyword(s):  
Beta Blockers ◽  
Stress Test ◽  
Ventricular Tachyarrhythmia ◽  
Hypochromic Anemia ◽  
Cardiac Events ◽  
Genetic Characteristics ◽  
Cardiac Symptoms ◽  
Delayed Initiation ◽  
The Mean ◽  
Multiple Recurrences

We aimed to analyze the management of children with Jervell-Lange-Nielsen syndrome based on their clinical and genetic characteristics and to assess the effectiveness of their therapy.Methods. We analyzed medical data from 12 Jervell and Lange-Nielsen syndrome patients. Mean follow-up was 8.3±5.1 years. Comprehensive cardiological examination include the standard 12-lead electrocardiography (ECG), echocardiography, stress-test, 24-hour Holter recordings, blood tests, thyroid ultrasound, and assessment of thyroid hormone levels, and if necessary, consultation with a neurologist.Results. At the first visit, the mean age was 3.0±2 years old, girls prevailed (67%). All patients are alive now. The mean QTc was 578.9±57.3 ms. Cardiac events had 10 patients (83%), mean age at the first event was 1.9±1.6 years. In 67% of children, syncope was initially regarded as epileptic. All patients received beta-blockers, which were prescribed at mean age 3.0±2.3 years (from 1 month to 7 years). In 8 patients cardiac events recurred despite regular and adequate beta-blocker therapy, all of them were implanted with implanted cardioverter-defibrillator. It was found that patients with the first syncope under the age of 1.5 and delayed initiation of therapy (at the mean age 4.3±1.7 years) had multiple recurrences of syncope despite therapy. Non-cardiac symptoms include sensorineural hearing loss in 100% of cases and hypochromic anemia in 75% of cases.Conclusion. In Jervell-Lange-Nielsen patients who are characterized by resistant ventricular tachyarrhythmia aggressive antiarrhythmic therapy at a very early age is essential for increasing the risk of life-threatening cardiac events. Consequently, diagnosis of the disease in the first days of life is extremely relevant. ECG screening is crucial in solving the problem of early diagnosis of the disease. Extracardiac pathology in patients with Jervell-Lange-Nielsen syndrome necessitates the interaction of doctors of different specialties together when managing a patient.

scholarly journals ASSOCIATION BETWEEN BODY MASS INDEX AND CARDIORESPIRATORY FITNESS AMONG PATIENTS UNDERGOING CARDIAC STRESS TEST IN DR HASAN SADIKIN GENERAL HOSPITAL BANDUNG

2021 ◽  
Vol 4 (1) ◽  
pp. 14
Author(s):  
Grace Octania ◽  
Mohammad Rizki Akbar ◽  
Sofiati Dian
Keyword(s):  
Body Mass Index ◽  
Body Mass ◽  
Cardiorespiratory Fitness ◽  
Beta Blockers ◽  
Stress Test ◽  
Pearson Correlation ◽  
Vascular Medicine ◽  
Cross Sectional ◽  
The Mean ◽  
The Relationship

Body mass index (BMI) and cardiorespiratory fitness are considered risk factors for cardiovascular disease. Chronic inflammation associated with adiposity as well as the hemodynamic changes that occur when there is an increase in BMI suggests a possible association between BMI and cardiorespiratory fitness. This study aims to analyze the relationship between BMI and cardiorespiratory fitness. A cross-sectional study was conducted using weighted cardiac training test results for the period January 1st, 2014 to December 31, 2019, from the Non-Invasive Diagnostic Division, the Department of Cardiology and Vascular Medicine, Dr. Hasan Sadikin Bandung. Subjects included in the study were those who achieved the maximum estimated heart rate based on age or less than 10 beats per minute, and/or the exercise test was stopped due to fatigue with a Borg 17 scale. Patients with multiple conditions were excluded from the study (taking beta-blockers, having a history of heart failure and diabetes mellitus, currently undergoing cardiac rehabilitation), along with patients with incomplete data. The maximum oxygen consumption in the form of the metabolic equivalent of tasks (METs) was estimated based on the speed and inclination of the stage reached during the treadmill training test using the Bruce protocol. The percentage of fitness is obtained by comparing the METs achieved with the estimated maximum METs based on age and gender. The relationship between BMI and percentage of fitness was analyzed using the Pearson correlation test. The total number of subjects included in the study was 51 subjects. The mean BMI of the subjects was 25.65 ± 3.22 kg / m2. The mean fitness percentage was 107.29 ± 23.89. Analysis of the relationship between BMI and fitness showed a negative but insignificant relationship (r = -0.135, p = 0.345). An increase in body mass index has a tendency to be associated with a decrease in cardiorespiratory fitness.

scholarly journals The Efficacy of Anti-Arrhythmic Drugs in Children With Idiopathic Frequent Symptomatic or Asymptomatic Premature Ventricular Complexes With or Without Asymptomatic Ventricular Tachycardia: a Retrospective Multi-Center Study

2021 ◽  
Author(s):  
Robin A. Bertels ◽  
Janneke A. E. Kammeraad ◽  
Anna M. Zeelenberg ◽  
Luc H. Filippini ◽  
Ingmar Knobbe ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Beta Blockers ◽  
Control Group ◽  
Premature Ventricular Complexes ◽  
Percentage Points ◽  
Asymptomatic Children ◽  
Group A ◽  
The Mean ◽  
Multi Center Study ◽  
Center Study

AbstractThe aim of the study is to compare the efficacy of flecainide, beta-blockers, sotalol, and verapamil in children with frequent PVCs, with or without asymptomatic VT. Frequent premature ventricular complexes (PVCs) and asymptomatic ventricular tachycardia (VT) in children with structurally normal hearts require anti-arrhythmic drug (AAD) therapy depending on the severity of symptoms or ventricular dysfunction; however, data on efficacy in children are scarce. Both symptomatic and asymptomatic children (≥ 1 year and < 18 years of age) with a PVC burden of 5% or more, with or without asymptomatic runs of VT, who had consecutive Holter recordings, were included in this retrospective multi-center study. The groups of patients receiving AAD therapy were compared to an untreated control group. A medication episode was defined as a timeframe in which the highest dosage at a fixed level of a single drug was used in a patient. A total of 35 children and 46 medication episodes were included, with an overall change in PVC burden on Holter of -4.4 percentage points, compared to -4.2 in the control group of 14 patients. The mean reduction in PVC burden was only significant in patients receiving flecainide (− 13.8 percentage points; N = 10; p = 0.032), compared to the control group and other groups receiving beta-blockers (− 1.7 percentage points; N = 18), sotalol (+ 1.0 percentage points; N = 7), or verapamil (− 3.9 percentage points; N = 11). The efficacy of anti-arrhythmic drug therapy on frequent PVCs or asymptomatic VTs in children is very limited. Only flecainide appears to be effective in lowering the PVC burden.

scholarly journals P1398 Echocardiographic continuous monitoring of exercise stress test using probe fixation device

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
K Wdowiak-Okrojek ◽  
P Wejner-Mik ◽  
Z Bednarkiewicz ◽  
P Lipiec ◽  
J D Kasprzak
Keyword(s):  
Image Quality ◽  
Physical Exercise ◽  
Stress Test ◽  
Exercise Stress Test ◽  
Bicycle Ergometer ◽  
Exercise Stress ◽  
Fixation Device ◽  
The Mean ◽  
First Time

Abstract Background Stress echocardiography (SE) plays an important role among methods of noninvasive diagnosis of ischemic disease. Despite the advantages of physical exercise as the most physiologic stressor, it is difficult (bicycle ergometer) or impossible (treadmill) to obtain and maintain the acoustic window during the exercise. Recently, an innovative probe fixation device was introduced and a research plan was developed to assess the feasibility of external probe fixation during exercise echocardiography on a supine bicycle and upright treadmill exercise for the first time. Methods 37 subjects (36 men, mean age 39 ± 16 years, 21 healthy volunteers, 16 patients with suspected coronary artery disease) were included in this study. This preliminary testing stage included mostly men due to more problematic probe fixation in women. All subjects underwent a submaximal exercise stress test on a treadmill (17/37) or bicycle ergometer (11/37). Both sector and matrix probes were used. We assessed semi-quantitatively the quality of acquired apical views at each stage – the four-point grading system was used (0-no view, 1-suboptimal quality, 2-optimal quality, 3-very good quality), 2-3 sufficient for diagnosis. Results The mean time required for careful positioning of the probe and image optimization was 12 ± 3 min and shortened from 13,7 to 11,1 minutes (mean) in first vs second half of the cohort documenting learning curve. At baseline, 9 patients had at least one apical view of quality precluding reliable analysis. Those patients were excluded from further assessment. During stress, 17 patients maintained the optimal or very good quality of all apical views, whereas in 11 patients the quality significantly decreased during the stress test and required probe repositioning. The mean image quality score at baseline was 2,61 ± 0,48 and 2,25 ± 0,6 after exercise. Expectedly, good image quality was easier to obtain and maintain in the supine position (score 2,74 ± 0,44) points as compared with upright position (score 2,25 ± 0,57). Conclusion This preliminary, unique experience with external probe fixation device indicates that continuous acquisition and monitoring of echocardiographic images is feasible during physical exercise, and for the first time ever - also on the treadmill. This feasibility data stem from almost exclusively male patients and the estimated rate of sufficient image quality throughout the entire test is currently around 60%. We are hoping, that gaining more experience with the product could increase the success rate on exercise tests. Abstract P1398 Figure. Treadmill and ergometer stress test

P3517Failure to achieve adequate heart rate control in women during therapy optimization

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Kutyifa ◽  
J W Erath ◽  
A Burch ◽  
B Assmus ◽  
D Bondermann ◽  
...  
Keyword(s):  
Heart Failure ◽  
Heart Rate ◽  
Beta Blocker ◽  
Rate Control ◽  
Beta Blockers ◽  
Heart Rate Control ◽  
Blocker Therapy ◽  
Cardioverter Defibrillator ◽  
Beta Blocker Therapy ◽  
The Mean

Abstract Background Previous studies highlighted the importance of adequate heart rate control in heart failure patients, and suggested under-treatment with beta-blockers especially in women. However, data on women achieving effective heart rate control during beta-blocker therapy optimization are lacking. Methods The wearable cardioverter defibrillator (WCD) allows continuous monitoring of heart rate (HR) trends during WCD use. In the current study, we assessed resting HR trends (nighttime: midnight-7am) in women, both at the beginning of WCD use and at the end of WCD use to assess the adequacy of beta-blockade following a typical 3 months of therapy optimization with beta-blockers. An adequate heart rate control was defined as having a nighttime HR <70 bpm at the end of the 3 months. Results There were a total of 21,453 women with at least 30 days of WCD use (>140 hours WCD use on the first and last week). The mean age was 67 years (IQR 58–75). The mean nighttime heart rate was 72 bpm (IQR 65–81) at the beginning of WCD use, that decreased to 68 bpm (IQR 61–76) at the end of WCD use with therapy optimization. Women had an insufficient heart rate control with resting heart rate ≥70 bpm in 59% at the beginning of WCD use that decreased to 44% at the end of WCD use, but still remained surprisingly high. Interestingly, there were 21% of the women starting with HR ≥70 bpm at the beginning of use (BOU) who achieved adequate heart rate control by the end of use (EOU). Interestingly, 6% of women with adequate heart rate control at the start of therapy optimization ended up having higher heart rates >70 bpm at the end of the therapy optimization time period (Figure). Figure 1 Conclusions A significant proportion of women with heart failure and low ejection fraction do not reach an adequate heart rate control during the time of beta blocker initiation/titration. The wearble cardioverter defibrillator is a monitoring device that has been demonstrated in this study to appropriately identify patients with inadequate heart rate control at the end of the therapy optimization period. The WCD could be utilized to improve management of beta-blocker therapy in women and improve the achievement of adequate heart rate control in women.

Abstract 13785: Left Ventricular Contractile Entropy in 99mTc-Sestamibi SPECT is a Novel Prognostic Predictor in Patients with Non-ischemic Dilated Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Naoaki Kano ◽  
Takahiro Okumura ◽  
Akinori Sawamura ◽  
Naoki Watanabe ◽  
Hiroaki Mori ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Sampling Point ◽  
Cardiac Events ◽  
Ventricular Ejection Fraction ◽  
Qrs Duration ◽  
Non Ischemic Dilated Cardiomyopathy ◽  
The Mean ◽  
99Mtc Sestamibi

Background: It has been reported that mechanical dispersion of myocardial contraction is increased in failing myocardium. However little is known about the association between contractile entropy evaluated by myocardial scintigraphy and prognosis in patients with non-ischemic dilated cardiomyopathy (NIDCM). Purpose: We aimed to investigate the prognostic value of contractile entropy in patients with NIDCM. Methods: Forty-seven patients (38 male, 55.1 years) with NIDCM were performed gated 99mTc-sestamibi myocardial perfusion SPECT (GMPS) and endomyocardial biopsy. Entropy was automatically calculated as a result of contractile phase analysis for each myocardial sampling point from GMPS, and it reflects a dispersion of global mechanical contraction. All patients were allocated into two groups based on the median of entropy; HE-group: entropy≥0.61 and LE-group: entropy<0.61. All patients were followed up at the mean of 2.8 years. Results: The mean QRS duration, left ventricular ejection fraction (LVEF) and plasma brain natriuretic peptide (BNP) levels were 114 msec, 35% and 225 pg/mL, respectively. Although there were no significant differences in QRS duration and plasma BNP levels between the two groups, LVEF was lower in the HE-group than in the LE-group (31.1% vs 39.8%, p=0.002). In Kaplan-Meier survival analysis, cardiac event rate was significantly higher in the HE-group (Figure). Cox proportional hazard analysis revealed that the HE-group was a significant determinant of cardiac events (Hazard Ratio: 7.66; 95%CI: 0.070-2.532; p=0.033). The mRNA expression level of sarcoplasmic endoplasmic reticulum Ca2+ ATPase (SERCA2a) in biopsy specimens was significantly lower in the LE-group (p=0.015). Conclusion: Contractile entropy, reflecting an impairment of global left ventricular contraction, might be useful to predict a poor prognosis in patients with NIDCM.

scholarly journals Capabilities of Cardioprotective Therapy in the Treatment and Prevention of Heart Failure in the Postinfarction Period

2020 ◽  
pp. 7-26
Author(s):  
Valerii Batushkin
Keyword(s):  
Heart Failure ◽  
Antihypertensive Drugs ◽  
Diastolic Pressure ◽  
Beta Blockers ◽  
Stress Test ◽  
Group Versus ◽  
Stage I ◽  
Lipid Lowering ◽  
Treatment And Prevention ◽  
Hawthorn Extract

Recently, numerous and quite convincing data has been accumulated on the effectiveness and safety of lipid-lowering drugs, beta-blockers, antiplatelet and antihypertensive drugs in patients with chronic heart failure (CHF), depending on the origin of the latter. The practitioner is suggested to use several drugs of different classes at the same time in order to reduce cardiovascular mortality, as well as the risk of recurrent myocardial infarction and ischemic stroke. In CHF, metabolism in cardiomyocytes varies depending on the stage of the disease. The changes that occur in the postinfarction period are compensatory in nature, which contributes to the partial improvement of impaired metabolism, while others, on the contrary, further inhibit the processes of energy production in the myocardium. In our research paper we will discuss some capabilities of metabolic therapy of CHF and prospects in the treatment and prevention using hawthorn extract; analyze the features of interaction of some well-known cardioprotective drugs with long-term antiplatelet therapy in the postinfarction period. Initiation of therapy with a new drug in addition to clopidogrel, such as trimetazidine, may adversely affect antiplatelet activity of clopidogrel (TRACER study, 2019). As a compromise, some herbal cardioprotective drugs may be used. Hawthorn preparations containing vaso- and cardioactive substances have significant potential in the treatment of cardiovascular diseases. Diversified mechanism of action of hawthorn has a significant impact on various parts of the cardiovascular system. Clinical trials of more than 4,000 patients confirm that standardized hawthorn extracts are effective as adjunctive therapy in the treatment of NYHA stage I–III CHF. The main two-year results of the WISO cohort study showed that the three pivotal symptoms of heart failure — fatigue (p = 0.036), stress dyspnea (p = 0.020) and palpitations (p = 0.048) — were significantly less marked after treatment in the hawthorn group versus comparative group. Cochrane analysis (2009) of studies investigating hawthorn extract included 14 studies where hawthorn was used primarily as an adjunct to conventional treatment. Exercise tolerance increased significantly during the treatment with hawthorn extract. Thus, the weighted difference between the average double multiplication rates during cardiac stress test (CST) was 122.76 W/min, whereas end-diastolic pressure in the right ventricle and myocardial oxygen consumption decreased with hawthorn treatment (a weighted mean difference was 19.22 mmHg per 1 min). The reported side effects were infrequent, mild and transient. A special hawthorn extract is indicated for the treatment of patients with NYHA stage II heart failure as an alternative and supplement to the standard evidence-based drug therapy. The beneficial effect on clinical symptoms allowed patients in the Crataegus group to reduce the use of angiotensin-converting enzyme (ACE) inhibitors from 54 to 36% (p = 0.004), cardiac glycosides from 37 to 18% (p = 0.001), diuretics from 61 to 49% (p = 0.061), beta-blockers from 33 to 22% (p = 0.052). At the same time, SPICE and HERB CHF studies show greater efficacy of Crataegus preparations in the treatment of mild to moderate heart failure (NYHA stage I–II). Higher doses (1800 mg) may be required for critically ill patients in order to achieve sustained improvement. Analysis of the data available to date is promising but suggests the need for a more focused approach to dosing based on the disease severity.

scholarly journals .BETA.-blockers Reduce the Incidence of Cardiac Events in Post-myocardial Infarction Patients.

2000 ◽  
Vol 41 (3) ◽  
pp. 279-294 ◽  
Author(s):  
Ishikawa Kinji ◽  
Miyataka Masaru ◽  
Kanamasa Ken ◽  
Hayashi Takahiro ◽  
Takenaka Toshihiko ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Beta Blockers ◽  
Post Myocardial Infarction ◽  
Cardiac Events

Abstract P297: Comparing Blood Pressure Control in Diabetic verses Non-Diabetic Veterans following Percutaneous Coronary Intervention (PCI)

2011 ◽  
Vol 4 (suppl_1) ◽  
Author(s):  
Sloane A McGraw ◽  
Michael Scholfield ◽  
Ragu Murthy ◽  
Anupama Shivaraju ◽  
Burhan Mohamedali ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Beta Blockers ◽  
Coronary Intervention ◽  
Pressure Control ◽  
Joint National Committee ◽  
National Committee ◽  
Diabetic Patients ◽  
Channel Blockers ◽  
The Mean ◽  
Artery Disease

Background: Blood pressure (BP) control in patients with coronary artery disease (CAD) is beneficial on morbidity and mortality, however the US Joint National Committee VII (JNC-7) also recommends systolic BP (SBP) <130 and diastolic BP (DBP) <80 for diabetic patients because diabetes itself is an additional risk for a cardiac event. This can be attained using beta-blockers (BB), angiotensin agonists (ACE-I/ARB), calcium channel blockers, diuretics and nitrates. Methods: We conducted a retrospective cohort study focusing on attaining JNC-7 guidelines, comparing outcomes between 302 diabetic to the 469 non-diabetic patients; all underwent PCI between September 2004 and September 2008 at the Jesse Brown Veterans Hospital in Chicago, IL. We collected data of BP values and antihypertensive regimens on admission and at six month follow up, and correlated these into percentages of which have attained goals. Results: Among diabetics, mean SBP decreased from 134 to 130mmHg (p = 0.002) and mean DBP decreased from 72 to 70mmHg (p= 0.004); in the non-diabetics, the mean SBP decreased from 133 to 127mmHg (p<0.0001) and the mean DBP decreased from 73 to 71mmHg (p<0.0012). With regards to guidelines, the percent of diabetics at SBP goal increased from 41% to 51% (124 to 154 of 302) (p= 0.006), however the percent at DBP goal was not significant. In non-diabetics, percent at goal for SBP increased 46% to 57% (216 to 267 of 469) (p=0.0002) and for DBP increased 69% to 76% (324 to 356 of 469) (p=0.0131). At 6 months, among diabetics the medication usage increased with BB, 80% to 92% (241 to 278 of 302) (p<0.0001) and nitrates 30% to 36% (91 to 109 of 302) (p=0.035). Similarly, among non-diabetics, use of BB, 68% to 87% (319 to 408 of 469) (p<0.0001) and nitrates 19% to 24% (89 to 113 of 469) (p=0.006) increased, as well as ACE-I/ARB 52% to 71% (244 to 333 if 469) (p<0.0001). Conclusions: There were improvements in BP among both populations at six months post-PCI; both attained JNC-7 SBP goal, but only non-diabetics achieved DBP goal. Medication use increased for both groups with BB and nitrates, but also with ACE-I/ARB for non-diabetics only. This analysis suggests that tighter control needs to be obtained among diabetics, especially because they are a higher risk population than those solely with CAD.

Abstract 274: Primary Care Physician Status is an Important Factor in Addressing Healthcare Disparities in Patients With Cardiovascular Disease

2020 ◽  
Vol 13 (Suppl_1) ◽  
Author(s):  
Ryan King ◽  
Dalia Giedrimiene
Keyword(s):  
Myocardial Infarction ◽  
Cardiovascular Disease ◽  
Primary Care ◽  
Logistic Regression ◽  
Primary Care Physician ◽  
Beta Blocker ◽  
Care Physician ◽  
Cardiac Events ◽  
Cvd Risk ◽  
The Mean

Recent improvements in survival and management of patients with cardiovascular disease (CVD) have resulted from timely use of medications such as beta blockers according to established guidelines. Without medical care provided by a primary care physician (PCP), patients may experience a significant healthcare disparity leading to CVD risk factors not being addressed or not receiving effective preventative therapies. If patients do not have a PCP, then their risk of experiencing CVD complications including an acute myocardial infarction (AMI) may be increased without access to appropriate treatment. We hypothesized that the utilization of preventative therapy depends on patient’s ability to have a PCP. Patients who do not have a PCP are less likely to receive a timely prescription of a beta blocker. Data for this study was collected through a retrospective chart review for 250 patients who presented to the Hartford Hospital Emergency Department for an AMI and were subsequently admitted between August 1, 2016 and April 30, 2018. A Chi square, independent t-test, and logistic regression were used for statistical analysis. A total of 17 patients were excluded due to incomplete documentation. The mean age of 233 patients was 64.64 ± 14.03 years old (range 26-89, males-144, females-89). There were 179 (76.8%) of these patients who had a documented PCP. Out of those with a PCP there were 104 (72.2%, of 144) males as compared to 75 (84.3%, of 89) females, p<0.034. Of the 223 with confirmed information about a beta blocker prescription there were 116 (52.0%) using a beta blocker before this admission for AMI and 99 (85.3%, of 116) of them had a PCP. There were 69 (59.5%, of 116) men and 47 (40.5%, of 116) women using a beta blocker. The mean age of patients using a beta blocker was 69.38 ± 12.9 years vs. 58.99 ± 13.08 years for those without a prescription (p < 0.001). A significant association was also found using logistic regression between PCP status and age groups (> 55 y vs < 55 y), p=0.032, gender, p=0.047, and beta blocker use, p=0.018. Our study shows that being prescribed a beta blocker significantly depends on the patient’s ability to have a PCP. Our study shows that among subjects with AMI, having access to a PCP is an important factor in being prescribed a beta blocker. Identifying barriers to PCP access may improve prevention measures and help bridge disparities resulting in major cardiac events such as myocardial infarction.

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