scholarly journals Right Heart Changes Impact on Clinical Phenotype of Amyloid Cardiac Involvement: A Single Centre Study

Author(s):  
Sebastiano Cicco ◽  
Antonio Giovanni Solimando ◽  
Roberta Buono ◽  
Nicola Susca ◽  
Gianfranco Inglese ◽  
...  

Amyloidosis is due to deposition of an excessive amount of protein in many parenchymal tissues, including myocardium. The onset of cardiac Amyloidosis (CA) is an inauspicious prognostic factor, that can lead to sudden death. We retrospectively analyzed 135 patients with systemic amyloidosis, admitted to our ward between 1981 and 2019. Among them, 54 patients (46.30% F / 53.70% M, age 63.95±12.82 years) presented CA at baseline. In 53 patients, there was associated with multiorgan involvement, while in one there was primary myocardial deposition. As control group, we enrolled 81 patients (49.30% F / 50.70% M, aged 58.33±15.65) who did not meet the criteria for CA. In 44/54 of patients CA was associated with AL, 5/54 with AA, 3/54 of patients with ATTR, in 1/54 AL was related to hemodialysis and 1/54 to Gel-Amyloidosis. The most common AL type was IgG (28/44); less frequent forms were either IgA (7/40) or IgD (2/40), while seven patients had a λ free light chain form. The 32 AL with complete Ig were 31 λ-chain and just one k-chain. CA patients presented normal BP (SBP 118.0±8.4 mmHg; DBP 73.8±4.9 mmHg), while those with nCA had increased proteinuria (p=0.02). TnI and NT-proBNP were significantly increased compared to nCA (p= 0.031 and p=0.047, respectively). In CA patients we found an increased LDH compared to nCA (p=0.0011). CA patients were also found to have an increased interventricular septum thickness compared to nCA (p=0.002), a decreased Ejection Fraction % (p=0.0018) and Doppler velocity E/e’ ratio (p=0.0095). Moreover, CA patients were seen to have an enhanced right atrium area (p=0.0179), right ventricle basal diameter (p= 0.0112) and wall thickness (p=0.0471) as compared to nCA, as well as an increased inferior cava vein diameter (p=0.0495). TAPSE was the method chosen to evaluate systolic function of the right heart. In CA subjects very poor TAPSE levels were found compared to nCA patients (p=0.0495). Additionally, we found a significant positive correlation between TAPSE and lymphocyte count (r=0.47; p=0.031) as well as Gamma globulins (r=0.43, p=0.033), Monoclonal component (r=0.72; p=0.047) and IgG values (r=0.62, p=0.018). On the contrary, a significant negative correlation with LDH (r=-0.57, p=0.005), IVS (r=-0.51, p=0.008) and diastolic function evaluated as E/e’ (r=-0.60, p=0.003) were verified. CA patients had very poor survival compared to controls (30 vs. 66 months in CA vs nCA, respectively, P=0.15). Mean survival of CA individuals was worse also when stratified according to NT-proBNP levels, using 2500 pg/mL as class boundary (174 vs. 5.5 months, for patients with lower vs higher values than the median, respectively p=0.013). In much the same way, decreased right heart systolic function was correlated with a worse prognosis (18.0 months median survival, not reached in subjects with lower values than 18mm, p=0.0186). Finally, our data highlight the potential prognostic and predictive value of right heart alterations characterizing amyloidosis, as a novel clinical parameter correlated to increased LDH and immunoglobulins levels. Overall, we confirm the clinical relevance of cardiac involvement and propose that right heart evaluation may be considered as a new marker for clinical risk stratification in patients with amyloidosis.

Life ◽  
2020 ◽  
Vol 10 (10) ◽  
pp. 247 ◽  
Author(s):  
Sebastiano Cicco ◽  
Antonio Giovanni Solimando ◽  
Roberta Buono ◽  
Nicola Susca ◽  
Gianfranco Inglese ◽  
...  

Amyloidosis is due to deposition of an excessive amount of protein in many parenchymal tissues, including myocardium. The onset of cardiac Amyloidosis (CA) is an inauspicious prognostic factor, which can lead to sudden death. We retrospectively analyzed 135 patients with systemic amyloidosis, admitted to our ward between 1981 and 2019. Among them, 54 patients (46.30% F/53.70% M, aged 63.95 ± 12.82) presented CA at baseline. In 53 patients, it was associated with a multiorgan involvement, while in one there was a primary myocardial deposition. As a control group, we enrolled 81 patients (49.30% F/50.70% M, aged 58.33 ± 15.65) who did not meet the criteria for CA. In 44/54 of patients CA was associated with AL, 5/54 with AA and 3/54 of patients with ATTR, and in 1/54 AL was related to hemodialysis and in 1/54 to Gel-Amyloidosis. The most common AL type was IgG (28/44); less frequent forms were either IgA (7/44) or IgD (2/44), while seven patients had a λ free light chain form. The 32 AL with complete Ig were 31 λ-chain and just one k-chain. CA patients presented normal BP (SBP 118.0 ± 8.4 mmHg; DBP 73.8 ± 4.9 mmHg), while those with nCA had an increased proteinuria (p = 0.02). TnI and NT-proBNP were significantly increased compared to nCA (p = 0.031 and p = 0.047, respectively). In CA patients we found an increased LDH compared to nCA (p = 0.0011). CA patients were also found to have an increased interventricular septum thickness compared to nCA (p = 0.002), a decreased Ejection Fraction % (p = 0.0018) and Doppler velocity E/e’ ratio (p = 0.0095). Moreover, CA patients had an enhanced right atrium area (p = 0.0179), right ventricle basal diameter (p = 0.0112) and wall thickness (p = 0.0471) compared to nCA, and an increased inferior cava vein diameter (p = 0.0495) as well. TAPSE was the method chosen to evaluate systolic function of the right heart. In CA subjects very poor TAPSE levels were found compared to nCA patients (p = 0.0495). Additionally, we found a significant positive correlation between TAPSE and lymphocyte count (r = 0.47; p = 0.031) as well as Gamma globulins (r = 0.43, p = 0.033), Monoclonal components (r = 0.72; p = 0.047) and IgG values (r = 0.62, p = 0.018). Conversely, a significant negative correlation with LDH (r = −0.57, p = 0.005), IVS (r = −0.51, p = 0.008) and diastolic function evaluated as E/e’ (r = −0.60, p = 0.003) were verified. CA patients had very poor survival rates compared to controls (30 vs. 66 months in CA vs. nCA, respectively, p = 0.15). Mean survival of CA individuals was worse also when stratified according to NT-proBNP levels, using 2500 pg/mL as class boundary (174 vs. 5.5 months, for patients with lower vs. higher values than the median, respectively p = 0.013). In much the same way, a decreased right heart systolic function was correlated with a worse prognosis (18.0 months median survival, not reached in subjects with lower values than 18 mm, p = 0.0186). Finally, our data highlight the potential prognostic and predictive value of right heart alterations characterizing amyloidosis, as a novel clinical parameter correlated to increased LDH and immunoglobulins levels. Overall, we confirm the clinical relevance of cardiac involvement suggests that right heart evaluation may be considered as a new marker for clinical risk stratification in patients with amyloidosis.


2019 ◽  
Vol 70 (1) ◽  
pp. 63-68 ◽  
Author(s):  
Venera Cristina Dinescu ◽  
Ileana Puiu ◽  
Sorin Nicolae Dinescu ◽  
Diana Rodica Tudorascu ◽  
Elena Catalina Bica ◽  
...  

The aim of this study was to identify correlations between electrocardiographic and echocardiographic changes in patients with silicosis prior to the occurrence of chronic pulmonary heart disease. We conducted a prospective, descriptive, analytical study, in which we included a group of 67 patients consecutively admitted to the Health Promotion and Occupational Medicine Clinic between December 2016 and January 2018, aged 47 to 78 years.There was a biochemical and electrocardiographic evaluation for each patient as well as a right ventricle echocardiographic evaluation (diameters, volumes, function). A control group, including 25 patients with benign minor diseases that required a cardiologist consultation, was also used. From the electrocardiographic point of view, slight changes were observed regarding the waves of electrical activity of the right ventricle. Taking into account the degree of ventilatory dysfunction (depending on FEV1), changes in right heart echocardiographic parameters were identified. Thus, in what the most important right ventricular parameters, including the tricuspid annular plane systolic excursion (TAPSE) or the RV index of myocardial performance (RVMPI) were concerned, values at the upper limit of normality were recorded in most patients with moderate and severe ventilatory dysfunction. Values of echocardiographic parameters of the right heart at the upper limit of normality, correlated with the degree of ventilatory dysfunction, are early markers for cardiovascular damage in patients with pulmonary silicosis prior to the occurrence of chronic pulmonary heart disease also known ascor pulmonale.


2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Agata Popielarz-Grygalewicz ◽  
Jakub S. Gąsior ◽  
Aleksandra Konwicka ◽  
Paweł Grygalewicz ◽  
Maria Stelmachowska-Banaś ◽  
...  

To determine whether the echocardiographic presentation allows for diagnosis of acromegalic cardiomyopathy. 140 patients with acromegaly underwent echocardiography as part of routine diagnostics. The results were compared with the control group comprising of 52 age- and sex-matched healthy volunteers. Patients with acromegaly presented with higher BMI, prevalence of arterial hypertension, and glucose metabolism disorders (i.e., diabetes and/or prediabetes). In patients with acromegaly, the following findings were detected: increased left atrial volume index, increased interventricular septum thickness, increased posterior wall thickness, and increased left ventricular mass index, accompanied by reduced diastolic function measured by the following parameters: E’med., E/E’, and E/A. Additionally, they presented with abnormal right ventricular systolic pressure. All patients had normal systolic function measured by ejection fraction. However, the values of global longitudinal strain were slightly lower in patients than in the control group; the difference was statistically significant. There were no statistically significant differences in the size of the right and left ventricle, thickness of the right ventricular free wall, and indexed diameter of the ascending aorta between patients with acromegaly and healthy volunteers. None of 140 patients presented systolic dysfunction, which is the last phase of the so-called acromegalic cardiomyopathy. Some abnormal echocardiographic parameters found in acromegalic patients may be caused by concomitant diseases and not elevated levels of GH or IGF-1 alone. The potential role of demographic parameters like age, sex, and/or BMI requires further research.


2020 ◽  
Vol 10 (5) ◽  
pp. 27-34
Author(s):  
Eugene V. Timofeev ◽  
Eduard G. Malev ◽  
Nina N. Parfenova ◽  
Eduard V. Zemtsovsky

For many hereditary connective tissue disorders (HCTD), especially Marfan syndrome, remodeling of the heart and main vessels is described, which is manifested by a decrease in the systolic function of the left ventricle and expansion of the thoracic aorta. Evaluation of morphometric characteristics of the heart and main vessels in patients with other HCTD, in particular marfanoid habitus (MH) has not been previously carried out. Materials and methods. Weexamined 90 young men and 74 young women between the ages of 18 to 25 years, 111 patients older age groups with stable over coronary heart disease (mean age 64.66.2 years) and 9 patients with verified Marfan syndrome (mean age 27.99.3years). All survey phenotypic and performed anthropometric survey identifying bone signs of dysembryogenesis as well as Echocardiography study on standard protocol. The results.Patients with MH as compared with control group revealed a relatively larger diameter of aortic root (30.44.7 vs 28.03.6 mm,p= 0.03) and the ascending aorta (26.64.9 vs 24.63.2 mm,p= 0.05). Also young with MH turned out to be significantly thicker myocardium of left ventricular posterior wall (8.30.8 vs 7.71.1 mm,p= 0.02) and interventricular septum (8.81.2vs 8.21.1mm,p= 0.04). When performing correlation analysis identified reliable positive correlation between such highly specialized bone signs as high palate (r= 0.31), infundibular deformation of the chest (r= 0.43), arachnodactyly (r= 0.45) andZ-test (p 0.05 for all). Expansion of the aorta (Z-criterion 2.0) have found 24% of older patients with MH. Conclusion.Inpatients with MH revealed significant structural changes of heart and main vessels which are progredient character thickening of the left ventricular myocardium and expansion of the aortic root.


2016 ◽  
Vol 47 (3) ◽  
pp. 869-875 ◽  
Author(s):  
Marcin Koć ◽  
Maciej Kostrubiec ◽  
Waldemar Elikowski ◽  
Nicolas Meneveau ◽  
Mareike Lankeit ◽  
...  

Our aim was the assessment of the prognostic significance of right heart thrombi (RiHT) and their characteristics in pulmonary embolism in relation to established prognostic factors.138 patients (69 females) aged (mean±sd) 62±19 years with RiHT were included into a multicenter registry. A control group of 276 patients without RiHT was created by propensity scoring from a cohort of 963 contemporary patients. The primary end-point was 30-day pulmonary embolism-related mortality; the secondary end-point included 30-day all-cause mortality. In RiHT patients, pulmonary embolism mortality was higher in 31 patients with systolic blood pressure <90 mmHg than in 107 normotensives (42% versus 12%, p=0.0002) and was higher in the 83 normotensives with right ventricular dysfunction (RVD) than in the 24 normotensives without RVD (16% versus 0%, p=0.038). In multivariable analysis the simplified Pulmonary Embolism Severity Index predicted mortality (hazard ratio 2.43, 95% CI 1.58–3.73; p<0.0001), while RiHT characteristics did not. Patients with RiHT had higher pulmonary embolism mortality than controls (19% versus 8%, p=0.003), especially normotensive patients with RVD (16% versus 7%, p=0.02).30-day mortality in patients with RiHT is related to haemodynamic consequences of pulmonary embolism and not to RiHT characteristics. However, patients with RiHT and pulmonary embolism resulting in RVD seem to have worse prognosis than propensity score-matched controls.


2020 ◽  
pp. 127-286

This chapter deals with the anatomy and pathology of the chambers and vessels in transthoracic echocardiography. It covers the left ventricle, global systolic function, regional systolic function, diastolic function and dysfunction, heart failure, the right ventricle, pulmonary hypertension, the left and right atria, the interatrial septum and interventricular septum, pericardium, pericardial effusion, cardiac tamponade, pericarditis, constrictive pericarditis, the aorta, primary cardiac tumours and other masses, extra-cardiac tumours, cardiomyopathies, and athlete’s heart.


2014 ◽  
Vol 23 (134) ◽  
pp. 476-487 ◽  
Author(s):  
Robert Naeije ◽  
Alessandra Manes

Pulmonary arterial hypertension (PAH) is a right heart failure syndrome. In early-stage PAH, the right ventricle tends to remain adapted to afterload with increased contractility and little or no increase in right heart chamber dimensions. However, less than optimal right ventricular (RV)–arterial coupling may already cause a decreased aerobic exercise capacity by limiting maximum cardiac output. In more advanced stages, RV systolic function cannot remain matched to afterload and dilatation of the right heart chamber progressively develops. In addition, diastolic dysfunction occurs due to myocardial fibrosis and sarcomeric stiffening. All these changes lead to limitation of RV flow output, increased right-sided filling pressures and under-filling of the left ventricle, with eventual decrease in systemic blood pressure and altered systolic ventricular interaction. These pathophysiological changes account for exertional dyspnoea and systemic venous congestion typical of PAH. Complete evaluation of RV failure requires echocardiographic or magnetic resonance imaging, and right heart catheterisation measurements. Treatment of RV failure in PAH relies on: decreasing afterload with drugs targeting pulmonary circulation; fluid management to optimise ventricular diastolic interactions; and inotropic interventions to reverse cardiogenic shock. To date, there has been no report of the efficacy of drug treatments that specifically target the right ventricle.


2018 ◽  
Vol 28 (5) ◽  
pp. 567-575
Author(s):  
E. I. Leonova ◽  
E. I. Shmelev ◽  
R. B. Amansakhedov

The aim of this study was to evaluate intracardiac hemodynamic abnormalities in patients with pulmonary sarcoidosis and to investigate relevant factors. Methods. The study involved 42 patients with chronic pulmonary sarcoidosis. Chest computed tomography (CT), echocardiography, body plethysmography, spirometry, 6-minute walking test, and measurements of lung diffusing capacity (DLCO), blood gases and blood C-reactive protein (CRP) were performed in all patients. Results. Chronic cor pulmonale was detected in 26.2% of patients. The right heart diastolic dysfunction was found in 14.3% of patients, the left heart diastolic dysfunction was found in 23.8% of patients, the mean pulmonary artery pressure (mPAP) was increased in 19% of patients. The right ventricular diameter was found to be related to the inspiratory bronchial resistance (Rin) (R = 0.480; p = 0.02), the intrathoracic gas volume (ITGV) (R = –0.670; p = 0.001), DLCO (R = 0.438; p = 0.013), and pulmonary fibrosis. The right heart systolic function (TAPSE) was related to DLCO (R = 0.518; p = 0.006), the total lung capacity (TLC) (R = 0.639; p = 0.001) and pulmonary fibrosis. The increased mPAP was related to the extension of disseminated lung lesions (R = 0.716; p = 0.018), blood oxygen partial pressure (R = 0.486; p = 0.017) and CRP level. The 6-min distance was related to Rin, the right ventricular diameter, parameters of the right heart diastolic function (tricuspid E / A ratio) (R = 0.486; p = 0.01), and ITGV (R = 0.494; p = 0.006). Conclusion. The development of cor pulmonale in patients with pulmonary sarcoidosis was related to DLCO, Rin and pulmonary fibrosis. The extension of disseminated lung lesions, hypoxemia and increased CRP were related to pulmonary hypertension. Different factors associated with cor pulmonale and pulmonary hypertension in sarcoidosis could prompt further investigation of different phenotypes of this disease with the intrinsic central hemodynamic abnormalities. 


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I Cabac-Pogorevici ◽  
V Revenco

Abstract Purpose In arterial hypertension (HTN), vascular structural changes develop as a consequence of haemodynamic as well as neurohumoral factors. Indeed, in the peripheral vessels a decrease in arterial compliance and, in the heart remodelling occurs respectively. The significant impact of HTN on left heart remodelling and its interconnection with intrarenal hemodynamics (IRH) has been widely studied. In the same time, there is lack of data concerning the affinity of HTN, IRH and right heart remodelling, as another important cluster of factors for prediction of mortality and morbidity in this group of patients. Methods The study included 104 patients (50 females and 54 males, mean age 48,26±11,2 years) with grade I-III arterial hypertension. All subjects underwent careful clinical history and physical examination to reveal risk factors, cardiovascular history and treatments. Blood test, echocardiography focused on right heart evaluation, intrarenal Doppler measurements were repeated in three parts of both kidneys (superior, median, and lower) untill three reproducible waveforms were obtained. The following IRH parameters were obtained: renal resistive index (RRI), renal pulsatile index (RPI), acceleration time (AT), renal volume (RV) and RV/RRI ratio. Results The mean RRI was 0,6672±0,0452, mean RPI 1,2533±0,178, mean AT 66,68±2,324 ms, mean RV 129,67±23,79 ml and mean RV/IRR ratio 195,52±41,587 ml. Mean 24 hours SBP was 146,12±13,96 mmHg, mean 24 hours DBP 86,59±6,78 mmHg. The mean pulse pressure (PP) was 59,10±22,90 mmHg. The mean 24 hours heart rate (HR) was 75,14±26,86 beats/minute. Mean value for the right ventricular (RV) basal diameter (4Ch) was 29,5±3,71 mm, right atrium (RA) diameter 39,71±3,38 mm, RA area 16,98±2,89 mm2, Sm RV 0,11±0,05 mm/sec, TAPSE 19,2±1,66 mm, SPAP 28,3±5,321 mmHg. We found an important positive correlation between RA diameter and RA area with RRI (r=0,364, p<0,01), (r=0,371, p<0,01), RPI (r=0,296, p<0,01), (r=0,320, p<0,01) and AT (r=0,155, p<0,05), (r=0,148, p<0,05), and negative correlation of RV Sm with RRI (r=−0,259, p<0,01), RPI (r=−0,232, p<0,01), AT (r=−0,162, p<0,05), meanwhile PASP showed an important positive connection with RRI (r=0,354, p<0,01), RPI (r=0,330, p<0,01) and AT (r=0,218, p<0,01). Conclusions The assumption that cardiac structural changes are progressing in parallel with extracardiac target damage could have an important scientific substrate, emphasizing the potential connection of IRH and the heart remodelling, including the right heart in HTN, and the eventual strengthening the IRH positions as a predictor of cardiovascular outcomes in this subset of population.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yaoshi Hu ◽  
Zhe Wei ◽  
Chaoyong Zhang ◽  
Chuanghong Lu ◽  
Zhiyu Zeng

AbstractLevosimendan exerts positive inotropic and vasodilatory effects. Currently, its effects on right heart function remain uncertain. This systematic review and meta-analysis is intended to illustrate the impacts of levosimendan on systolic function of the right heart in patients with heart dysfunction. We systematically searched electronic databases (PubMed, the Cochrane Library, Embase and Web of Science) up to November 30, 2020, and filtered eligible studies that reported the impacts of levosimendan on right heart function. Of these, only studies whose patients suffered from heart dysfunction or pulmonary hypertension were included. Additionally, patients were divided into two groups (given levosimendan or not) in the initial research. Then, RevMan5.3 was used to conduct further analysis. A total of 8 studies comprising 390 patients were included. The results showed that after 24 h of levosimendan, patients’ right ventricular fractional area change [3.17, 95% CI (2.03, 4.32), P < 0.00001], tricuspid annular plane systolic excursion [1.26, 95% CI (0.35, 2.16), P = 0.007] and tricuspid annular peak systolic velocity [0.86, 95% CI (0.41, 1.32), P = 0.0002] were significantly increased compared to the control group. And there is an increasing trend of cardiac output in levosimendan group [1.06, 95% CI (− 0.16, 2.29), P = 0.09 ] .Furthermore, patients’ systolic pulmonary arterial pressure [− 5.57, 95% CI (− 7.60, − 3.54), P < 0.00001] and mean pulmonary arterial pressure [− 1.01, 95% CI (− 1.64, − 0.37), P = 0.002] were both significantly decreased, whereas changes in pulmonary vascular resistance [− 55.88, 95% CI (− 206.57, 94.82), P = 0.47] were not significant. Our study shows that in patients with heart dysfunction, levosimendan improves systolic function of the right heart and decreases the pressure of the pulmonary artery.


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