scholarly journals Intracardiac hemodynamic abnormalities in patients with sarcoidosis

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. 

2021 ◽  
pp. 204589402110136
Author(s):  
Tailong Zhang ◽  
Weitao Liang ◽  
Longrong Bian ◽  
Zhong Wu

Right heart thrombus (RHT) accompanied by chronic thromboembolic pulmonary hypertension (CTEPH) is a rare entity. RHT may develop in the peripheral veins or in situ within the right heart chambers. The diagnosis of RHT is challenging, since its symptoms are typically non-specific and its imaging features resemble those of cardiac masses. Here, we report two cases of RHT with CTEPH that presented as right ventricular masses initially. Both patients underwent simultaneous pulmonary endarterectomy (PEA) and resection of the ventricular thrombi. Thus, when mass-like features are confirmed by imaging, RHT should be suspected in patients with CTEPH, and simultaneous RHT resection is required along with PEA.


2021 ◽  
Vol 8 ◽  
Author(s):  
Hong Meng ◽  
Wu Song ◽  
Sheng Liu ◽  
David Hsi ◽  
Lin-Yuan Wan ◽  
...  

Background: There have been no systemic studies about right heart filling pressure and right ventricular (RV) distensibility in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Therefore, we aimed to explore combinations of echocardiographic indices to assess the stages of RV diastolic dysfunction.Methods and Results: We recruited 32 healthy volunteers and 71 patients with CTEPH. All participants underwent echocardiography, cardiac catheterization (in patients with CTEPH), and a 6-min walk test (6MWT). The right atrial (RA) end-systolic area was adjusted for body surface area (BSA) (indexed RA area). RV global longitudinal diastolic strain rates (SRs) and RV ejection fraction (EF) were measured by speckle tracking and three-dimensional echocardiography (3D echo), respectively. All 71 patients with CTEPH underwent pulmonary endarterectomy. Of the 71 patients, 52 (73%) had decreased RV systolic function; 12 (16.9%), 26 (36.6%), and 33 (46.5%) patients had normal RV diastolic pattern, abnormal relaxation (stage 1), and pseudo-normal patterns (stage 2), respectively. The receiver operating characteristic curve analysis showed that the optimal cut-off values of early diastolic SR <0.8 s−1 and indexed RA area > 8.8 cm2/BSA had the best accuracy in identifying patients with RV diastolic dysfunction, with 87% sensitivity and 82% specificity. During a mean follow-up of 25.2 months after pulmonary endarterectomy, the preoperative indexed RA area was shown as an independent risk factor of the decreased 6MWT distance.Conclusions: Measuring early diastolic SR and indexed RA area would be useful in stratifying RV diastolic function.


2021 ◽  
Author(s):  
James P. Pirruccello ◽  
Paolo Di Achille ◽  
Victor Nauffal ◽  
Mahan Nekoui ◽  
Samuel N. Friedman ◽  
...  

The heart evolved hundreds of millions of years ago. During mammalian evolution, the cardiovascular system developed with complete separation between pulmonary and systemic circulations incorporated into a single pump with chambers dedicated to each circulation. A lower pressure right heart chamber supplies deoxygenated blood to the lungs, while a high pressure left heart chamber supplies oxygenated blood to the rest of the body. Due to the complexity of morphogenic cardiac looping and septation required to form these two chambers, congenital heart diseases often involve maldevelopment of the evolutionarily recent right heart chamber. Additionally, some diseases predominantly affect structures of the right heart, including arrhythmogenic right ventricular cardiomyopathy (ARVC) and pulmonary hypertension. To gain insight into right heart structure and function, we fine-tuned deep learning models to recognize the right atrium, the right ventricle, and the pulmonary artery, and then used those models to measure right heart structures in over 40,000 individuals from the UK Biobank with magnetic resonance imaging. We found associations between these measurements and clinical disease including pulmonary hypertension and dilated cardiomyopathy. We then conducted genome-wide association studies, identifying 104 distinct loci associated with at least one right heart measurement. Several of these loci were found near genes previously linked with congenital heart disease, such as NKX2-5, TBX3, WNT9B, and GATA4. We also observed interesting commonalities and differences in association patterns at genetic loci linked with both right and left ventricular measurements. Finally, we found that a polygenic predictor of right ventricular end systolic volume was associated with incident dilated cardiomyopathy (HR 1.28 per standard deviation; P = 2.4E-10), and remained a significant predictor of disease even after accounting for a left ventricular polygenic score. Harnessing deep learning to perform large-scale cardiac phenotyping, our results yield insights into the genetic and clinical determinants of right heart structure and function.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Kanda ◽  
T Nagai ◽  
N Kondou ◽  
K Tateno ◽  
M Hirose ◽  
...  

Abstract Introduction and purpose The number of patients with right heart failure due to pulmonary hypertension has been increasing. Although several drugs have reportedly improved pulmonary hypertension, no treatments have been established for decompensated right heart failure. The heart has an innate ability to regenerate, and cardiac stem or progenitor cells (e.g., side population [SP] cells) have been reported to contribute to the regeneration process. However, their contribution to right ventricular pressure overload has not been clarified. Here, this regeneration process was evaluated using a genetic fate-mapping model. Methods and results We used Cre-LacZ mice, in which more than 99.9% of the cardiomyocytes in the left ventricular field were positive for 5-bromo-4-chloro-3-indolyl-β-D-galactoside (X-gal) staining immediately after tamoxifen injection. Then, we performed either a pulmonary binding (PAB) or sham operation on the main pulmonary tract. In the PAB-treated mice, the right ventricular cavity was significantly enlarged (right-to-left ventricular [RV/LV] ratio, 0.24±0.04 in the sham group and 0.68±0.04 in the PAB group). Increased peak flow velocity in the PAB group (1021±80 vs 1351±62 mm/sec) was confirmed by echocardiography. One month after the PAB, the PAB-treated mice had more X-gal-negative (newly generated) cells than the sham mice (94.8±34.2 cells/mm2 vs 23.1±10.5 cells/mm2; p<0.01). The regeneration was biased in the RV free wall (RV free wall, 225.5±198.7 cells/mm2; septal area, 88.9±56.5/mm2; LV lateral area, 46.8±22.0/mm2; p<0.05). To examine the direct effects of PAB on the cardiac progenitor cells, bromodeoxyuridine was administered to the mice daily until 1 week after the PAB operation. Then, the hearts were isolated and SP cells were harvested. The SP cell population increased from 0.65±0.23% in the sham mice to 1.87% ± 1.18% in the PAB-treated mice. Immunostaining analysis revealed a significant increase in the number of BrdU-positive SP cells, from 11.6±2.0% to 44.0±18%, therefore showing SP cell proliferation. Conclusions Pulmonary pressure overload stimulated cardiac stem or progenitor cell-derived regeneration with a RV bias, and SP cell proliferation may partially contribute to this process. Acknowledgement/Funding JSPS KAKENHI Grant Number JP 17K17636, GSK Japan Research Grant 2016


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Angel Lopez-Candales ◽  
Ananth Eleswarapu ◽  
Kathy Edelman

Background : Left ventricular diastolic dysfunction is associated with worse outcomes in patients with severe chronic elevation in pulmonary artery systolic pressures (PASP); however, little is known if right ventricular diastolic dysfunction (RVDD) is also present in these patients. Methods : Color M-mode to measure RV propagation velocity (Vp) and tissue Doppler imaging (TDI) of the tricuspid valve annulus (TVa) were done in 21 patients (Group I: mean age 54 ± 15, mean PASP 80 ± 31 mmHg) and in 19 healthy individuals (Group II: mean age 51 ± 14 years, PASP 30 ± 7 mmHg). All subjects were in normal sinus rhythm and normal left ventricular systolic function. Results : Despite elevation in PASP, Group I RV systolic function measured by both TVa excursion (2.01 ± 0.7 cm) and TVa systolic velocity (0.10 ± 0.04 cm/s) were no different than Group II (2.5 ± 0.5 cm and 0.12 ± 0.03 cm/s, respectively). Even though there was no difference in either early (0.11 ± 0.04 versus 0.13 ± 0.04 cm/s) versus late (0.15 ± 0.05 versus 0.13 ± 0.04 cm/s) diastolic velocities by TDI; a lower Vp was seen in Group I (28 ± 12 cm/s) than Group II (41 ± 15 cm/s, p < 0.01) with a significantly shorter diastolic interval (259 ± 90 versus 390 ± 80 ms, p < 0.0001). Representative color M-mode images are shown in the Figure . Conclusions : Routine use of TVA TDI appears to be less useful than color M-mode to appreciate RV diastole as it shows a slower Vp with flows shifted later in diastole. In addition, these patients also demonstrate a shorter diastolic interval. Further studies are now required to prospectively assess the effects of pulmonary hypertension in clinical outcomes in patients manifesting RVDD. Figure. (A) Color M-mode image of a patient artery systolic pressures demonstrating predominantly early diastolic flow. (B) Color M-mode image from a patient with pulmonary hypertension showing predominantly late diastolic flow.


2022 ◽  
Author(s):  
Alexey N. Sumin ◽  
Nina S. Gomozova ◽  
Anna V. Shcheglova ◽  
Oleg G. Arkhipov

Abstract Objective of this study was to compare right ventricular echocardiography parameters in urbanized hypertensive patients of the Shor and non-indigenous ethnic groups in the Mountain Shoria region. Methods The study included patients with arterial hypertension: 58 Shors and 50 non-indigenous urbanized residents, comparable in age, and divided by ethnicity and gender into 4 groups: Shors men (n = 20), Shors women (n = 38), non-indigenous men (n = 15) and non-indigenous women (n = 35). All underwent echocardiographic examination, and the right heart parameters were studied. Results Shor men with arterial hypertension had the lowest values ​​of the pulmonary artery index, the right atrium dimensions, and the highest values ​​of the blood flow velocity in the right ventricle, et' and st' in comparison with non-indigenous men. Shor women have the lowest values Et and Et/At ratios. RV diastolic dysfunction was detected mainly in women, somewhat more often in Shors. Ethnicity was one of the factors associated with the right ventricular diastolic dysfunction presence. Among the factors associated with the RV diastolic dysfunction were risk factors (smoking, obesity), blood pressure, gender, ethnicity, and left ventricular parameters (diastolic dysfunction and the myocardial mass increase). Conclusion Our study established the influence of ethnic differences on the right heart echocardiographic parameters in Shors and Caucasians with arterial hypertension. The revealed differences should improve the assessment of the right heart structure and function in patients with arterial hypertension from small ethnic groups, which will help to improve the diagnosis and treatment of such patients.


Circulation ◽  
2005 ◽  
Vol 112 (9_supplement) ◽  
Author(s):  
Sydney L. Gaynor ◽  
Hersh S. Maniar ◽  
Jeffrey B. Bloch ◽  
Paul Steendijk ◽  
Marc R. Moon

Background— Increased mortality in patients with chronic pulmonary hypertension has been associated with elevated right atrial (RA) pressure. However, little is known about the effects of chronic right ventricular (RV) pressure overload on RA and RV dynamics or the adaptive response of the right atrium to maintain RV filling. Methods and Results— In 7 dogs, RA and RV pressure and volume (conductance catheter) were recorded at baseline and after 3 months of progressive pulmonary artery banding. RA and RV elastance (contractility) and diastolic stiffness were calculated, and RA reservoir and conduit function were quantified as RA inflow with the tricuspid valve closed versus open, respectively. With chronic pulmonary artery banding, systolic RV pressure increased from 34±7 to 70±17 mm Hg ( P <0.001), but cardiac output did not change ( P >0.78). RV elastance and stiffness both increased ( P <0.05), suggesting preserved systolic function but impaired diastolic function. In response, RA contractility improved (elastance increased from 0.28±0.12 to 0.44±0.13 mm Hg/mL; P <0.04), and the atrium became more distensible, as evidenced by increased reservoir function (49±14% versus 72±8%) and decreased conduit function (51±14% versus 28±8%; P <0.002). Conclusions— With chronic RV pressure overload, RV systolic function was preserved, but diastolic function was impaired. To compensate, RA contractility increased, and the atrium became more distensible to maintain filling of the stiffened ventricle. This compensatory response of the right atrium likely plays an important role in preventing clinical failure in chronic pulmonary hypertension.


2018 ◽  
Vol 32 (5) ◽  
pp. 1541-1548 ◽  
Author(s):  
Tommaso Vezzosi ◽  
Oriol Domenech ◽  
Giulia Costa ◽  
Federica Marchesotti ◽  
Luigi Venco ◽  
...  

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