scholarly journals Author Correction: Left atrial dysfunction in sickle cell anemia is associated with diffuse myocardial fibrosis, increased right ventricular pressure and reduced exercise capacity

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Tarek Alsaied ◽  
Omar Niss ◽  
Justin T. Tretter ◽  
Adam W. Powell ◽  
Clifford Chin ◽  
...  
Blood ◽  
2017 ◽  
Vol 130 (2) ◽  
pp. 205-213 ◽  
Author(s):  
Omar Niss ◽  
Robert Fleck ◽  
Fowe Makue ◽  
Tarek Alsaied ◽  
Payal Desai ◽  
...  

Key Points Diffuse myocardial fibrosis is a common and novel mechanism of heart disease in SCA that can be detected noninvasively. Diffuse myocardial fibrosis is strongly associated with diastolic dysfunction in individuals with SCA.


Author(s):  
Onur Sinan Deveci ◽  
Caglar Ozmen ◽  
Muhammet Bugra Karaaslan ◽  
Aziz Inan Celik ◽  
Hatice Rahimova ◽  
...  

Objective Vaso-occlusive crisis (VOC) is a common clinical manifestation of sickle cell anemia (SCA) and is associated with increased proinflammatory mediators. Copeptin is the C-terminal part of the prohormone for pro-vasopressin and seems clinically relevant in various clinical conditions. Right ventricular (RV) dysfunction significantly appears in SCA patients due to pulmonary hypertension. This study aimed to investigate the association of copeptin levels in VOC patients and evaluate RV dysfunction. Materials and Methods A total of 108 patients were enrolled in the study. Twenty-eight SCA patients in steady state (30.2±10.9 years), 25 SCA patients in VOC (36.8±11.8 years), and 55 healthy individuals (31.9±9.4 years) with HbAA genotype were included. Clinical, echocardiographic, and laboratory data were recorded. ELISA was used for the determination of serum levels of copeptin. Results VOC patients had significantly higher copeptin level compared both with controls and SCA subjects in steady-state (22.6±13.0 vs. 11.3±5.7 pmol/l, 22.6±13.0 vs. 12.4±5.8 pmol/l, p=0.009 for both). Additionally, the copeptin level was significantly higher in SCA patients with RV dysfunction than those without RV dysfunction (23.2±12.2 vs. 15.3±9.5 pmol/l, p=0.024). Multiple logistic regression analysis revealed that hs-CRP and copeptin levels were found to be associated with VOC. Conclusion The study showed that copeptin and hs-CRP levels were increased in patients with VOC, and a significant relationship was found between RV dysfunction in VOC patients. As a conclusion copeptin can be used as a potential biomarker in predicting VOC crisis in SCA patients and in early detection of patients with SCA who have the potential to develop RV dysfunction.


Author(s):  
Heiner Latus ◽  
Danik Born ◽  
Nerejda Shehu ◽  
Heiko Stern ◽  
Alfred Hager ◽  
...  

Background Right atrial (RA) dilatation and impaired right ventricular (RV) filling are common in patients with RV outflow tract dysfunction. We aimed to study potential correlations between atrial function with clinically relevant hemodynamic parameters and to assess the predictive impact of atrial performance on the recovery of exercise capacity and RV pump function after percutaneous pulmonary valve implantation (PPVI). Methods and Results Altogether, 105 patients with right ventricular outflow tract dysfunction (median age at PPVI, 19.2 years; range, 6.2–53.4 years) who underwent cardiac magnetic resonance imaging before and 6 months after PPVI were included. RA and left atrial maximal and minimal volumes as well as atrial passive and active emptying function were assessed from axial cine slices. RA emptying function was inversely related to invasive RV end‐diastolic pressure, and RA passive emptying correlated significantly with peak oxygen uptake. After PPVI, a significant decrease in RA minimum volume was observed, whereas RA passive emptying function improved, and RA active emptying function decreased significantly. Patients with predominant right ventricular outflow tract stenosis showed more favorable changes in RA active and left atrial passive emptying than those with primary volume overload. None of the RA and left atrial emptying parameters was predictive for recovery of peak oxygen uptake or RV ejection fraction. Conclusions In patients with right ventricular outflow tract dysfunction, impaired RA emptying assessed by cardiac magnetic resonance imaging was associated with increased RV filling pressures and lower exercise capacity. PPVI leads to a reduction in RA size and improved passive RA emptying function. However, RA function was not associated with improved exercise performance and RV pump function.


1983 ◽  
Vol 74 (5) ◽  
pp. 757-764 ◽  
Author(s):  
Samuel Charache ◽  
Eugene R. Bleecker ◽  
Dean S. Bross

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