scholarly journals Left ventricular systolic dysfunction with concomitant bradyarrhythmia in a patient with POEMS syndrome: a case report

Author(s):  
Joo Hor Tan ◽  
Min Sen Yew ◽  
Wenjie Huang ◽  
Kenny Tan

Abstract Background POEMS syndrome (PS) is a paraneoplastic disorder from plasma cell dyscrasia, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Vascular endothelial growth factors (VEGFs)-driven fluid extracellular matrix expansion plays a key role in this condition. Associated cardiac involvement has been sparsely reported thus far. Case summary A 55-year-old woman with PS presented with a pleural effusion and respiratory failure requiring mechanical ventilation. Transthoracic echocardiogram revealed left ventricular (LV) systolic dysfunction with a moderate pericardial effusion. She developed intermittent complete heart block and ventricular standstill, requiring temporary transcutaneous pacing. Further evaluation revealed no significant coronary stenosis on coronary angiogram and cardiac magnetic resonance (CMR) showed elevated T1 and extracellular volume suggestive of myocardial oedema with possible early cardiac infiltration. She had a dual-chamber permanent pacemaker implanted in view of recurrent high-grade heart block. She was initiated on a daratumumab-based chemotherapy regimen prior to discharge. She recovered well subsequently with a promising clinical response to chemotherapy. Discussion We describe the first case of LV systolic dysfunction with concomitant significant bradyarrhythmia in a patient with PS. CMR revealed evidence suggestive of LV myocardial oedema and/or possible early infiltration. VEGF overexpression could explain oedema-related LV dysfunction which reversed with adequate diuresis, as well as damage to the conduction system. Early cardiac amyloidosis, which can be associated with PS, is an important differential diagnosis. Pacemaker implantation, adequate diuresis, and definitive chemotherapy are key to the management of concomitant ventricular myocardial and electrical dysfunction in such rare case.

2019 ◽  
Vol 12 (3) ◽  
pp. e227143
Author(s):  
Muhammad Hamza Saad Shaukat ◽  
Fadi Fahad ◽  
David Weinreb ◽  
Mikhail Torosoff

A previously healthy 44-year-old Caucasian man presented with recurrent syncope and was found to have a complete heart block with a ventricular rate of 24 bpm. No biochemical abnormalities were identified. Tick borne illnesses were ruled out. Paced echocardiogram revealed left ventricular systolic dysfunction with septal hypokinesis. Chest radiography and subsequent CT scan did not reveal adenopathy. However, a positron emission tomography scan demonstrated increased fluorodeoxyglucose uptake in the spleen, a right retro-clavicular lymph node, right ventricle and the interventricular septum of the heart. Excision biopsy of the retro-clavicular lymph node revealed non-caseating granulomas consistent with sarcoidosis. Complete heart block persisted despite steroid treatment. A pacemaker/biventricular implantable cardioverter defibrillator was placed for complete heart block and primary prevention of ventricular tachycardia and sudden cardiac death.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Ken Matsuoka ◽  
Masami Nishino ◽  
Masahiko Hara ◽  
Shinpei Nakatani ◽  
Akihito Hashimoto ◽  
...  

Backgrounds: Although right ventricular apical (RVA) pacing has been reported to result in asynchronous patterns of left ventricular (LV) contraction reducing the LV ejection fraction (EF), LV torsion in RVA pacing has been unknown. We hypothesized that RVA pacing impairs rotation of LV apex and base and synchrony between LV apical and basal rotation, leading to the reduction of LV torsion. Methods: We studied 30 adult patients with sick sinus syndrome who had undergone DDD pacemaker implantation. Right ventricular pacing leads were positioned at the right ventricular apex. Changing from the intrinsic AV conduction to RVA pacing, we assessed the acute effect on echocardiac parameters; LV EF, tissue Doppler imaging (TDI) derived intraventricular delay, LV rotation and torsion, and LV apical-basal rotation dyssynchrony. Results: Results were shown in a table . Changing from the intrinsic AV conduction to RVA pacing, LV EF reduced (P=0.0003) and TDI derived intraventricular delay prolonged (P<0.0001). During RVA pacing, values of peak rotation in LV apex and LV base were significantly lower than those during intrinsic AV conduction (P=0.007 and 0.003, respectively). Correspondingly, LV torsion decreased significantly (P<0.0001). During the intrinsic AV conduction, LV apex and base rotated at the almost same time, while, during RVA pacing, LV basal rotation was delayed as compared to LV apical rotation. Consequently, apical-basal rotation dyssynchrony during RVA pacing was significantly longer than that during the intrinsic AV conduction (P=0.02). Conclusion: RVA pacing decreases LV rotation and torsion and induces LV apical-basal rotation dyssynchrony, resulting in partially LV systolic dysfunction. Comparison between Intrinsic AV Conduction and RVA Pacing


2020 ◽  
Author(s):  
Junqiao Niu ◽  
Zeng Mu ◽  
Yan Wang ◽  
Jun Liu ◽  
Jia Wang ◽  
...  

Abstract Background : Evaluation of tissue fibrosis and myocardial hypertrophy in left ventricular (LV) remodeling is the basis of post-treatment evaluation of hypertensive heart disease(HHD). Extracellular volume (ECV) and myocardial strain parameters can indirectly reflect the changes of both. Our objective was to analyze the characteristics of ECV and strain parameters in LV myocardium of HHD with varying degrees of systolic dysfunction, and to explore the changes of both after treatment for hypertension.Methods: A total of 62 HHD patients were divided into 3 groups according to ejection fraction (EF<30%, 30%≦EF<50%, EF≧50%). Twenty-one of these patients underwent cardiac magnetic resonance(CMR)reexamination more than six months after receiving antihypertensive medication. The initial T1 time and post-enhancement T1 time of each segment were measured, and the ECV was calculated. Radial strain (RS), circumferential strain (CS) and longitudinal strain (LS) of LV were measured by cvi42 software, and the differences in CMR parameters between different groups and before and after treatment were compared.Results: ①The mean, basal and middle ECV value of HHD groups with different EF were all higher than that of the control group ( p <0.05), but the difference between HHD groups was not statistically significant. ② With the decrease of EF, the absolute value of both the global or local strain decreased. Strain is related to LVMI and ECV. ③In general, ECV, global RS (GRS) and global CS(GCS) improved after treatment, but the improvement of LS impairment in HHD patients is difficult.Conclusions: ECV and myocardial strain parameters are more sensitive to myocardial abnormalities, and ECV, GRS and GCS are more sensitive to treatment. Although it is difficult to improve longitudinal strain impairment in HHD patients, it is more important for prognosis evaluation. ECV and myocardial strain parameters can be used as good makers for long-term monitoring of the efficacy of HHD patients.


Heart ◽  
2020 ◽  
Vol 106 (16) ◽  
pp. 1236-1243 ◽  
Author(s):  
Rong Bing ◽  
Haotian Gu ◽  
Calvin Chin ◽  
Lingyun Fang ◽  
Audrey White ◽  
...  

ObjectiveFirst-phase ejection fraction (EF1) is a novel measure of early left ventricular systolic dysfunction. We investigated determinants of EF1 and its prognostic value in aortic stenosis.MethodsEF1 was measured retrospectively in participants of an echocardiography/cardiovascular magnetic resonance cohort study which recruited patients with aortic stenosis (peak aortic velocity of ≥2 m/s) between 2012 and 2014. Linear regression models were constructed to examine variables associated with EF1. Cox proportional hazards were used to determine the prognostic power of EF1 for aortic valve replacement (AVR, performed as part of clinical care in accordance with international guidelines) or death.ResultsTotal follow-up of the 149 participants (69.8% male, 70 (65–76) years, mean gradient 33 (21–42) mm Hg) was 238 029 person-days. Sixty-seven participants (45%) had a low baseline EF1 (<25%) despite normal ejection fraction (67% (62%–71%)). Patients with low EF1 had more severe aortic stenosis (mean gradient 39 (34–45) mm Hg vs 24 (16–35) mm Hg, p<0.001) and more myocardial fibrosis (indexed extracellular volume (iECV) (24.2 (19.6–28.7) mL/m2 vs 20.6 (16.8–24.3) mL/m2, p=0.002; late gadolinium enhancement (LGE) prevalence 52% vs 20%, p<0.001). Zva, iECV and infarct LGE were independent predictors of EF1. EF1 improved post-AVR (n=57 with post-AVR EF1 available, baseline 16 (12–24) vs follow-up 27% (22%–31%); p<0.001). Low baseline EF1 was an independent predictor of AVR/death (HR 5.6, 95% CI 3.4 to 9.4), driven by AVR.ConclusionEF1 quantifies early, potentially reversible systolic dysfunction in aortic stenosis, is associated with global afterload and myocardial fibrosis, and is an independent predictor of AVR.


2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Carlos A. Carmona ◽  
Fatma Levent ◽  
Kelvin Lee ◽  
Bhavya Trivedi

Cardiac manifestations in multisystem inflammatory syndrome in children (MIS-C) can include coronary artery aneurysms, left ventricular systolic dysfunction, and electrocardiographic disturbances. We report the clinical course of three children with MIS-C while focusing on the unique considerations for managing atrioventricular conduction abnormalities. All initially had normal electrocardiograms but developed bradycardia followed by either PR prolongation or QTc elongation. Two had mild left ventricular ejection fraction dysfunction prior to developing third-degree heart block and/or a junctional escape rhythm; one had moderate left ventricular systolic dysfunction that normalized before developing a prolonged QTc. On average, our patients presented to the hospital 4 days after onset of illness. Common presenting symptoms included fevers, abdominal pain, nausea, and vomiting. Inflammatory and coagulation factors were their highest early on, and troponin peaked the highest within the first two days; meanwhile, peak brain-natriuretic peptide occurred at hospital days 3-4. The patient’s lowest left ventricular ejection fraction occurred at days 5-6 of illness. Initial electrocardiograms were benign with PR intervals below 200 milliseconds (ms); however, collectively the length of time from initial symptom presentation till when electrocardiographic abnormalities began was approximately days 8-9. When comparing the timing of electrocardiogram changes with trends in c-reactive protein and brain-natriuretic peptide, it appeared that the PR and QTc elongation patterns occurred after the initial hyperinflammatory response. This goes in line with the proposed mechanism that such conduction abnormalities occur secondary to inflammation and edema of the conduction tissue as part of a widespread global myocardial injury process. Based on this syndrome being a hyperinflammatory response likely affecting conduction tissue, our group was treated with different regimens of intravenous immunoglobulin, steroids, anakinra, and/or tocilizumab. These medications were successful in treating third-degree heart block, prolonged QTc, and a junctional ectopic rhythm.


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