Dynamic Left Ventricular Arterial Pressure Gradient and Sick Sinus Syndrome with Heterozygous Fabry's Disease Improved Following Implantation of a Dual Chamber Pacemaker

1999 ◽  
Vol 22 (7) ◽  
pp. 1114-1115 ◽  
Author(s):  
YASUNORI NAKAYAMA ◽  
KEI TSUMURA ◽  
NAOTOSHI YAMASHITA ◽  
KIYOMICHI YOSHIMARU
Keyword(s):  
Arterial Pressure ◽  
Pressure Gradient ◽  
Sick Sinus Syndrome ◽  
Left Ventricular ◽  
Dual Chamber ◽  
Fabry’S Disease ◽  
Dual Chamber Pacemaker ◽  
Fabry's Disease

scholarly journals Evaluation of left ventricular longitudinal function and synchrony after dual chamber pacemaker implantation

Medicina ◽  
2014 ◽  
Vol 50 (6) ◽  
pp. 340-344
Author(s):  
Kristina Baronaitė-Dūdonienė ◽  
Jolanta Vaškelytė ◽  
Aras Puodžiukynas ◽  
Vytautas Zabiela ◽  
Tomas Kazakevičius ◽  
...  
Keyword(s):  
Pacemaker Implantation ◽  
Left Ventricular ◽  
Dual Chamber ◽  
Dual Chamber Pacemaker ◽  
Longitudinal Function

Cardiac Fabry's disease: an unusual cause of left ventricular hypertrophy

2007 ◽  
Vol 4 (11) ◽  
pp. 630-633 ◽  
Author(s):  
José Pedro L Nunes ◽  
Ovídio Costa ◽  
Maria do Sameiro Faria ◽  
Pedro Bernardo Almeida ◽  
Lúcia Lacerda
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Fabry’S Disease ◽  
Fabry's Disease

scholarly journals Chronic Apical and Nonapical Right Ventricular Pacing in Patients with High-Grade Atrioventricular Block: Results of the Right Pace Study

2018 ◽  
Vol 2018 ◽  
pp. 1-7
Author(s):  
Carmine Muto ◽  
Valeria Calvi ◽  
Giovanni Luca Botto ◽  
Domenico Pecora ◽  
Daniele Porcelli ◽  
...  
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Left Ventricular ◽  
Secondary Outcome ◽  
X Rays ◽  
Ventricular Pacing ◽  
Dual Chamber ◽  
Dual Chamber Pacemaker ◽  
Right Ventricular Pacing ◽  
The Right

Objective. The aim of the study was to compare the two approaches to chronic right ventricular pacing currently adopted in clinical practice: right ventricular apical (RVA) and non-RVA pacing. Background. Chronic RVA pacing is associated with an increased risk of atrial fibrillation, morbidity, and even mortality. Non-RVA pacing may yield more physiologic ventricular activation and provide potential long-term benefits and has recently been adopted as standard procedure at many implanting centers. Methods. The Right Pace study was a multicenter, prospective, single-blind, nonrandomized trial involving 437 patients indicated for dual-chamber pacemaker implantation with a high percentage of RV pacing. Results. RV lead-tip target location was the apex or the interventricular septum. RVA (274) and non-RVA patients (163) did not differ in baseline characteristics. During a median follow-up of 19 months (25th–75th percentiles, 13–25), 17 patients died. The rates of the primary outcome of death due to any cause or hospitalization for heart failure were comparable between the groups (log-rank test, p=0.609), as were the rates of the composite of death due to any cause, hospitalization for heart failure, or an increase in left ventricular end-systolic volume ≥ 15% as compared with the baseline evaluation (secondary outcome, p=0.703). After central adjudication of X-rays, comparison between adjudicated RVA (239 patients) and non-RVA (170 patients) confirmed the absence of difference in the rates of primary (p=0.402) and secondary (p=0.941) outcome. Conclusions. In patients with indications for dual-chamber pacemaker who require a high percentage of ventricular stimulation, RVA or non-RVA pacing resulted in comparable outcomes. This study is registered with ClinicalTrials.gov (identifier: NCT01647490).

scholarly journals PERSISTANT LEFT SUPERIOR VENA CAVA IN A PATIENT WITH AN IMPLANTED DUAL CHAMBERPASING SYSTEM

2015 ◽  
Vol 6 (2) ◽  
pp. 24-26
Author(s):  
A A Kocharyan ◽  
R S Ovchinnikov ◽  
V N Lesniak ◽  
A V Ardashev
Keyword(s):  
Superior Vena Cava ◽  
Clinical Case ◽  
Sick Sinus Syndrome ◽  
Superior Vena ◽  
Vena Cava ◽  
Dual Chamber ◽  
Left Superior Vena Cava ◽  
Dual Chamber Pacemaker

We described a clinical case of a 65-years patient with sick sinus syndrome with persistant left superior vena cava, whom was implanted dual chamber pacemaker.

scholarly journals Fabry’s disease in cardiological aspect

2020 ◽  
pp. 22-27
Author(s):  
L. D. Khidirova ◽  
A. Kh. Magomedova ◽  
A. A. Vasilenko ◽  
V. S. Dudchenko
Keyword(s):  
Subsequent Development ◽  
Heart Rhythm ◽  
Cardiovascular Complications ◽  
Left Ventricular ◽  
Fabry’S Disease ◽  
Fabry's Disease ◽  
Enzyme Replacement ◽  
Heart Rhythm Disorders ◽  
Lysosomal Accumulation ◽  
Gla Gene

Hereditary genetic X-linked disease Fabry’s disease belongs to the group of lysosomal accumulation diseases and is caused by mutations in the GLA gene and is characterized by a decrease in functional activity or complete absence of the enzyme α-galactosidase A. This pathology belongs to the group of orphan diseases. Mutation of the GLA gene leads to the formation of defective forms of the enzyme α-galactosidase A, which contributes to the violation of the catabolism of glycosphingolipids, their further accumulation in the lysosomes of various cell cultures, and the development of lysosomal cell dysfunction. The prevalence of Fabry disease is about 1 in 117,000 live-born boys. According to screening studies in newborns, this figure can be about 1 in 3,100 and affects to the same extent representatives of all ethnic groups. Fabri’s disease has become actively studied in Russia, but more than 5,000 people (according to estimates) remain undiagnosed. In the first place among the causes of death in Fabry’s disease is heart disease, in particular left ventricular hypertrophy with the subsequent development of diastolic dysfunction and heart failure. Heart rhythm disorders are often observed. Early diagnosis of Fabri disease will lead to the appointment of genotype-specific enzyme replacement therapy and reduce the risk of cardiovascular complications.

An Atypical Variant of Fabry's Disease in Men with Left Ventricular Hypertrophy

1995 ◽  
Vol 333 (5) ◽  
pp. 288-293 ◽  
Author(s):  
Shoichiro Nakao ◽  
Toshihiro Takenaka ◽  
Masato Maeda ◽  
Chihaya Kodama ◽  
Akihiro Tanaka ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Fabry’S Disease ◽  
Fabry's Disease ◽  
Atypical Variant

scholarly journals Relationship between right ventricular pacing and non-sustained ventricular arrhythmias in patients with dual-chamber pacemaker and normal range left ventricular ejection fraction

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Rayan S. El-Zein ◽  
Anish K. Amin ◽  
Sreedhar R. Billakanty ◽  
Eugene Y. Fu ◽  
Allan J. Nichols ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Pacing ◽  
Normal Range ◽  
Dual Chamber ◽  
Dual Chamber Pacemaker ◽  
Right Ventricular Pacing ◽  
The Impact

Abstract Background Right ventricular pacing (RVP) increases heart failure, AF, and death rates in pacemaker patients and ventricular arrhythmias (VAs) in defibrillator patients. However, the impact of RVP on VAs burden and its clinical significance in pacemaker patients with normal range LVEF of > 50–55% remains unknown. We sought to evaluate the relationship of RVP and VAs and its clinical impact in a pacemaker patient population. Methods Records of 105 patients who underwent denovo dual-chamber pacemaker implant or a generator change (Medtronic™ or Boston Scientific™) for AV block and sinus node disease at a tertiary care center between September 1, 2015, and September 1, 2016, were retrospectively reviewed. Results Data from 105 patients (51% females, mean age 76 ± 1 years, mean LVEF 61 ± 0.7%) without history of VAs (98.2%) were reviewed over 1044 ± 23 days. Dependent patients (100% RVP) exhibited the lowest VAs burden when compared to < 100% RVP (isolated PVCs, PVC runs of < 4 beats, and NSVT; p ≤ 0.001). Patients with < 1% RVP also exhibited low VA burden with intermediate RVP (1–99.9%) being most arrhythmogenic for PVC runs (p = 0.04) and for isolated PVCs (p = 0.006). Antiarrhythmics/beta and calcium channel blockers use and stress tests performed to evaluate VAs which were positive requiring intervention did not differ significantly. Burden of > 1/h of PVC runs and increasing PVC runs/h were significantly associated with hospitalization (p = 0.04) and all-cause mortality (p = 0.03), respectively. Conclusions In pacemaker patients with normal range LVEF (> 50–55%), 100% RVP is associated with the lowest burden of NSVT. Furthermore, patients with < 1% RVP also exhibit low VA burden; however, intermittent RVP seems to significantly correlate with non-sustained VAs.

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