scholarly journals POEMS syndrome causing left ventricular hypertrophy, myocardial dysfunction and pericardial effusion: a case report

2021 ◽  
Author(s):  
Shanshan Wang ◽  
Xuejie Wu ◽  
Hui Li
Keyword(s):  
Pulmonary Hypertension ◽  
Pericardial Effusion ◽  
Left Ventricular Hypertrophy ◽  
Cardiovascular System ◽  
Ventricular Hypertrophy ◽  
Myocardial Dysfunction ◽  
Left Ventricular ◽  
Poems Syndrome ◽  
Study Patient ◽  
Initial Symptoms

Abstract Background POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a paraneoplastic syndrome caused by a plasma cell proliferative disorder. Characteristics of POEMS syndrome include elevated pro-inflammatory and angiogenic cytokine levels that lead to multi-organ dysfunction. Patients have a variety of initial symptoms, but cardiac involvement is not common. Case summary We report a case of a 31-year-old Chinese woman with chief complaints of chest pain and dyspnoea who was diagnosed with POEMS syndrome. The cardiovascular system in the case study patient was characterized by pericardial effusion, enlarged left atria, abnormal myocardial segmental deformation, left ventricular hypertrophy, pulmonary hypertension, and increased glucose metabolism in the left and right ventricular myocardium. The pericardial effusion diminished, while cardiac function, left ventricular wall thickness, and pulmonary hypertension gradually returned to normal with dexamethasone and bortezomib treatment. Discussion This case suggests that cardiovascular system damage may be related to systemic diseases. Cardiovascular system damage caused by POEMS syndrome is recoverable after chemotherapy treatment. Echocardiography readily visualizes the changes in the heart of a patient with POEMS syndrome, clearly reflecting the changes in the structure and function of the heart before and after treatment.

Abstract 3435: Left Ventricular Hypertrophy is Resistant to Inhibition of Expression of the R403Q Alpha-Myosin Heavy Chain Cardiac Hypertrophy-Inducing Mutant Protein

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Leah Cannon ◽  
Tadeusz Marciniec ◽  
Bryony Mearns ◽  
Robert M Graham ◽  
Diane Fatkin
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Ventricular Hypertrophy ◽  
Interstitial Fibrosis ◽  
Myocardial Dysfunction ◽  
Left Ventricular ◽  
Familial Hypertrophic Cardiomyopathy ◽  
Lv Function ◽  
Dependent Manner ◽  
Cardiovascular Morbidity And Mortality ◽  
Lv Mass

Left ventricular hypertrophy (LVH) develops as a compensatory response to myocardial dysfunction due to diverse causes, but is nonetheless a major risk factor for premature cardiovascular morbidity and mortality. It is thus unclear if regressing LVH is beneficial or may worsen patient outcome. To evaluate the effects of LVH regression, we developed a transgenic mouse model in which the expression of a familial hypertrophic cardiomyopathy (FHC)-inducing mutation (R403Q alpha-MHC) can be regulated in a temporal and dose-dependent manner. In this model, transgene expression can be shut off by feeding with a tetracycline analogue (doxycycline). Serial echocardiography and histology studies were performed in a cohort of mice expressing the FHC mutant (“gene-on”) and in wildtype (WT) littermates. A second cohort of WT and 403/+ mice was randomised to placebo or doxycycline (“gene off”) from 6 (Dox6) or 20 weeks (Dox20) and evaluated at 40 weeks of age. Compared to WT littermates, “gene on” 403/+ mice showed increased LV mass, LV end-diastolic diameter (LVDD) and left atrial diameter (LAD), and reduced fractional shortening (LVFS), with changes evident from 12 weeks of age. LV sections from 403/+ mice showed typical features of FHC: myofibre disarray and interstitial fibrosis. LV mass, LV function and myocardial histology were unchanged in both male and female placebo- vs Dox6 or Dox20 mice at 40 weeks (Table 1 ). Thus, consistent with the major LV thickening in FHC humans occurring in adolescence, overexpression of R403Q for only 6 weeks is sufficient to trigger the complete LVH phenotypic response. Moreover, switching off the genetic trigger for LVH in 403/+ mice at 6 weeks (prior to overt disease manifestation) or 20 weeks (established disease) does not induce regression of LVH or exacerbate contractile dysfunction. Interventions to induce LVH regression may, therefore, need to be directed at downstream factors in hypertrophic pathways. Table 1. Echo data for male WT and 403/+ mice aged 40 weeks

MO746CARDIAC REMODELING AND PULMONARY HYPERTENSION IN HEMODIALYSIS PATIENTS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Ekaterina Borodulina ◽  
Alexander M Shutov
Keyword(s):  
Pulmonary Hypertension ◽  
Left Ventricular Hypertrophy ◽  
Arterial Pressure ◽  
Ventricular Hypertrophy ◽  
Pulmonary Arterial Pressure ◽  
Hemodialysis Patients ◽  
Left Ventricular ◽  
Hemodialysis Treatment ◽  
Systolic Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

Abstract Background and Aims An important predictor of cardiovascular mortality and morbidity in hemodialysis patients is left ventricular hypertrophy. Also, pulmonary hypertension is a risk factor for mortality and cardiovascular events in hemodialysis patients. The aim of this study was to investigate cardiac remodeling and the dynamics of pulmonary arterial pressure during a year-long hemodialysis treatment and to evaluate relationship between pulmonary arterial pressure and blood flow in arteriovenous fistula. Method Hemodialysis patients (n=88; 42 males, 46 females, mean age was 51.7±13.0 years) were studied. Echocardiography and Doppler echocardiography were performed in the beginning of hemodialysis treatment and after a year. Echocardiographic evaluation was carried out on the day after dialysis. Left ventricular mass index (LVMI) was calculated. Left ventricular ejection fraction (LVEF) was measured by the echocardiographic Simpson method. Arteriovenous fistula flow was determined by Doppler echocardiography. Pulmonary hypertension was diagnosed according to criteria of Guidelines for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology. Results Pulmonary hypertension was diagnosed in 47 (53.4%) patients. Left ventricular hypertrophy was revealed in 71 (80.7%) patients. Only 2 (2.3%) patients had LVEF<50%. At the beginning of hemodialysis correlation was detected between systolic pulmonary arterial pressure and LVMI (r=0.52; P<0.001). Systolic pulmonary arterial pressure negatively correlated with left ventricular ejection fraction (r=-0.20; P=0.04). After a year of hemodialysis treatment LVMI decreased from 140.49±42.95 to 123.25±39.27 g/m2 (р=0.006) mainly due to a decrease in left ventricular end-diastolic dimension (from 50.23±6.48 to 45.13±5.24 mm, p=0.04) and systolic pulmonary arterial pressure decreased from 44.83±14.53 to 39.14±10.29 mmHg (р=0.002). Correlation wasn’t found between systolic pulmonary arterial pressure and arteriovenous fistula flow (r=0.17; p=0.4). Conclusion Pulmonary hypertension was diagnosed in half of patients at the beginning of hemodialysis treatment. Pulmonary hypertension in hemodialysis patients was associated with left ventricular hypertrophy, systolic left ventricular dysfunction. After a year-long hemodialysis treatment, a regress in left ventricular hypertrophy and a partial decrease in pulmonary arterial pressure were observed. There wasn’t correlation between arteriovenous fistula flow and systolic pulmonary arterial pressure.

Gαi-biased apelin analog protects against isoproterenol-induced myocardial dysfunction in rats

2021 ◽  
Vol 320 (4) ◽  
pp. H1646-H1656
Author(s):  
David Coquerel ◽  
Eugénie Delile ◽  
Lauralyne Dumont ◽  
Frédéric Chagnon ◽  
Alexandre Murza ◽  
...  
Keyword(s):  
Cardiovascular Diseases ◽  
Left Ventricular Hypertrophy ◽  
Ventricular Hypertrophy ◽  
Myocardial Dysfunction ◽  
Negative Inotropic Effect ◽  
Left Ventricular ◽  
Inotropic Effect ◽  
Camp Production ◽  
Data Point

By using more potent Gαi-biased APJ agonists that strongly inhibit cAMP production, these data point to the negative inotropic effect of APJ-mediated Gαi signaling in the heart and highlight the potential protective impact of APJ-dependent Gαi signaling in cardiovascular diseases associated with left ventricular hypertrophy.

scholarly journals The cardiovascular system in patients with symptomatic and mild primary hyperparathyroidism

2009 ◽  
Vol 55 (3) ◽  
pp. 25-29 ◽  
Author(s):  
I V Voronenko ◽  
N G Mokrysheva ◽  
L Ya Rozhinskaya ◽  
A L Syrkin
Keyword(s):  
Parathyroid Hormone ◽  
Left Ventricular Hypertrophy ◽  
Primary Hyperparathyroidism ◽  
Cardiovascular System ◽  
Qt Interval ◽  
Ventricular Hypertrophy ◽  
Left Ventricular Diastolic Dysfunction ◽  
Left Ventricular ◽  
Ionized Calcium ◽  
Mild Primary Hyperparathyroidism

The cardiovascular system was analyzed in patients with symptomatic (n = 31) and mild primary hyperparathyroidism (n = 34) whose mean age was 54.6 years; 95% females). In the patients with symptomatic primary hyperparathyroidism, the PQ interval was longer and the QT interval was significantly shorter than those in patients with mild hyperparathyroidism. Left ventricular hypertrophy was noted in 45.2% of patients with symptomatic and in 15.2% of those with mild hyperparathyroidism (p = 0.013). Left ventricular diastolic dysfunction was also more common in the group of symptomatic hyperparathyroidism. There was a statistically significant correlation between the levels of parathyroid hormone, total and ionized calcium and the duration of QT interval and the determinants of diastolic function and left ventricular hypertrophy. The revealed cardiovascular disorders in patients with primary hyperparathyroidism are presumed to depend on the increase rate of parathyroid hormone and total and ionized calcium.

Abstract 13499: A 34-year-old Man With Debilitating Polyneuropathy, Pericardial Effusion and Monoclonal Gammopathy Presents With Right Heart Failure

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Kenta Nakamura ◽  
Mary H Zhang ◽  
Jordan T Shin
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Pericardial Effusion ◽  
Monoclonal Gammopathy ◽  
Right Heart Failure ◽  
Left Ventricular ◽  
Poems Syndrome ◽  
Right Heart ◽  
Peripheral Edema ◽  
Vegf Level

A 34-year-old man with debilitating polyneuropathy and monoclonal gammopathy presents with progressive lower extremity edema, pericardial effusion and dyspnea. Pulmonary edema, elevated jugular venous pressure and peripheral edema suggested right heart failure, and dyspnea responded initially to diuresis. Echocardiography revealed occult pericardal effusion, right heart dysfunction and estimated right ventricular systolic pressure of 54 mmHg. The differential diagnosis included pulmonary hypertension secondary to chronic pulmonary or left heart processes, pericardial constriction, restrictive myopathy or a syndromic condition capturing his neurologic and hematologic findings. Chest CT and VQ-scan were negative for acute and chronic pulmonary thromboembolic disease, respectively. Cardiac magnetic resonance imaging was most consistent with amyloidosis. Pericardial enhancement was normal, arguing against constriction. Amyloidosis was thought to be unlikely given preserved left ventricular size and function, increase in both serum free κ and λ light chains, and was ultimately excluded by fat pad biopsy. The paraneoplastic disease Crow-Fukase or POEMS syndrome, classically described as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes explained the patient’s constellation of signs and symptoms. Pulmonary hypertension subsequent to cor pulmonale is common and reversible with treatment of the underlying gammopathy. Diagnosis of the POEMS syndrome was confirmed by the presence of sclerotic bone lesions and markedly elevated vascular endothelial growth factor (VEGF) level of 1028 pg/mL (ref. range 31-86 pg/mL). VEGF is highly induced in the POEMS syndrome and correlates closely with disease activity. VEGF-mediated microangiopathy, neovascularization, and vasopermeability likely underlie multiple clinical sequelae of extravascular volume overload such as pericardial effusion and peripheral edema. The patient responded to sildenafil for pulmonary arterial hypertension and immunomodulatory therapy for the monoclonal gammopathy. At six months of treatment, the patient has recovered substantial strength, heart failure has not reoccurred and repeat VEGF level reduced to 383 pg/mL.

scholarly journals P722 Unrepaired complex severe aortic coarctation determining restrictive cardiomyopathy with pulmonary hypertension in adulthood

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
F Graziani ◽  
E Panaioli ◽  
R Lillo ◽  
A B Delogu ◽  
G Perri ◽  
...  
Keyword(s):  
Emergency Department ◽  
Pulmonary Hypertension ◽  
Left Ventricular Hypertrophy ◽  
Arterial Hypertension ◽  
Aortic Coarctation ◽  
Left Atrial ◽  
Ventricular Hypertrophy ◽  
Wall Motion ◽  
Restrictive Cardiomyopathy ◽  
Left Ventricular

Abstract A 54-year-old female presented to our Emergency Department with acute pulmonary edema. On physical examination she was tachypneic with arterial oxygen saturation of 91% and a grade 3/6 systolic murmur in the apex and left second intercostal space that was irradiated to the intrascapular area. Bilateral femoral and pedal pulses were quite faint on palpation. The blood pressure was 260/120 mmHg. Results of routine blood chemistry were normal with the exception of increased NT-proBNP (2746 pg/mL). Twelve-lead electrocardiogram revealed left ventricular hypertrophy with repolarization abnormalities and left atrial enlargement. The two dimensional (2D) echocardiography showed significant left ventricular hypertrophy (RWT 0.6; LVmass index 150 g/m2), no wall-motion abnormalities and normal left ventricular ejection fraction (EF 60%) but with reduced longitudinal components at TDI evaluation (6 cm/s). Severe ventricular diastolic dysfunction was detected with E/A 2,11 and E/e" 26 with a significant left atrial dilatation (LAVi 73 ml/m2) and severe pulmonary arterial hypertension ( PASP of 85 mmHg) . We were unable to visualize the aortic arch but reduced/absent pulsatile wall motion of abdominal aorta was identified. The patient reported to be affected by unrepaired aortic coarctation diagnosed at the age of 32 during pregnancy. She refused the surgical treatment twice and the she did not undergo any specific follow up but she had several admissions in emergency department for uncontrolled arterial hypertension. Our further evaluation with CT angiography showed a severe narrowing of the post isthmic aortic lumen with significant development collateral vessels (Figure 1). The patient was discharged after resolution of pulmonary edema and titration of hypertension treatment with a planning of hybrid approach to treat the aortic coarctation. The case shows a restrictive cardiomyopathy with post-capillary pulmonary hypertension due to severe unrepaired aortic coarctation. Abstract P722 Figure. CT angiography,echo, right heart cath

scholarly journals P1300CARDIOVASCULAR COMPLICATIONS IN HEMODIALYSIS PATIENTS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Helaoui Fadwa
Keyword(s):  
Pulmonary Hypertension ◽  
Left Ventricular Hypertrophy ◽  
Ventricular Hypertrophy ◽  
Clinical Status ◽  
Vena Cava ◽  
Cardiovascular Complications ◽  
Hemodialysis Patients ◽  
Left Ventricular ◽  
Chronic Hemodialysis ◽  
Cardiac Ultrasound

Abstract Background and Aims Cardiovascular complications are the leading cause of morbidity and mortality among dialysis patients. We aim to determine the cardiac damage showed by trans-thoracic ultrasound and to identify the various factors associated with it. Method Fourty chronic hemodialysis patients have benefited of transthoracic ultrasound during this year (2019). Clinical findings were collected from patients’ medical files. Data collected included demographics, Clinical Status and echocardiographic parameters. All the patients included in this study gave their consent. Data were collected and analysed using SPSS software. Chi-squared test with a level of significance of 0.05 was used for the qualitative variables. Results The mean age of our population is 53.1 (range: 26-78) with a sex ratio equal to 0.9. 72.5% of patients are hypertensive; 27.5% of patients are diabetic. Only 2 patients to be coronary. In this study, 11 patients remained asymptomatic while 29 patients (75.5%) presented with cardiac symptomatology: 39.7% complained of chest pain, 27.5% presented with acute dyspnea and 24,1% a pericardial rub. Rhythm disorders were objectified in electrocardiography in 3 patients (1 ventricular extrasystole and 2 cardiac arrhythmia by atrial fibrillation). Twenty percent of the cardiac ultrasounds performed are without abnormalities. Cardiac ultrasound revealed the presence of valvulopathies in 40% with 20% valve calcifications. Left ventricular hypertrophy is evident in 55% of patients, 45% of whom are hypertensive. Segmental hypokinesia was observed in 27.5% and pulmonary hypertension was observed in 37.5%. Cardiac ultrasound also showed dilated right cavities in 22.5% of cases and dilated left cavities in 32.5%. Dilatation of the inferior vena cava is observed only in 3 patients. Pericarditis is highlighted in 37.5% of which 36.3% are of medium abundance and 18.1% are of great abundance without signs of compression. Clinical data were correlated with echocardiographic findings. A correlation was found between left venticular hypertrophy and hypervolemia (p=0.001) and between perdialytic hemodynamic instability and ischemic cardiopathy (p=0.04) as well as pulmonary hypertension and vascular access dysfunction (p=0.02). Conclusion Cardiovascular complications are very common in chronic hemodialysis population. The left ventricular hypertrophy is the main cause of heart disease with predominance of high blood pressure as an associated comorbidity.

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