Objectives: Systemic sclerosis (SSc) leads to pulmonary circulation dysfunctionand there are some indications of systemic circulation impairment. We evaluated the influence of SSc on the elastic properties of large systemic arterial walls and potential correlations between systemic and pulmonary circulation involvement.
Method: We examined 75 consecutive women (mean age 53.13±10.1 years) with confirmed SSc [mean disease duration (DD) 7.1±9.1 years] and 21 age-matched female volunteers (mean age 52.6±8.3 years, ns). Pulse wave velocity (PWV) and transthoracic echocardiography were performed. SSc patients were divided into two groups according to the median of DD: ≤3 years (39 patients) and >3 years (36 patients).
Results: Patients with DD>3 years had higher PWV than those with DD≤3 years and controls (log PWV: 2.23±0.23 vs. 2.13±0.16 and vs. 2.11±0.16 m/s; p=0.028 and 0.029, respectively). In addition, echocardiographic indices showed impaired right ventricular (RV) function in the patients with DD>3 years. Also in these SSc patients, PWV correlated with clinical and echocardiographic parameters of pulmonary circulation: age (r=0.64, p<0.0001), acceleration time of pulmonary ejection (AcT; r=-0.38, p=0.021), and tricuspid regurgitation peak gradient (TRPG; r=0.34, p=0.04). Multiple linear regression analysis showed that PWV was independently associated with DD (β=0.22, p==0.02), AcT (β=-0.215, p=0.03), and age (β=0.44, p<0.001).
Conclusions: In patients with SSc lasting more than 3 years, the disease is characterized by increased stiffness of the large systemic arteries. Longer duration of SSc leads simultaneously to the increased stiffness of the large systemic arteries and to the progressive impairment of RV function and its coupling to the pulmonary arterial bed.
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http://dx.doi.org/10.3109/03009742.2015.1021710 | DOI Listing |
J Appl Physiol (1985)
January 2025
Medical Physics Graduate Program, Duke University, Durham, North Carolina, United States.
Hyperpolarized Xe MRI/MRS enables quantitative mapping of function in lung airspaces, membrane tissue, and red blood cells (RBCs) within the pulmonary capillaries. The RBC signal also exhibits cardiogenic oscillations that are reduced in pre-capillary pulmonary hypertension (PH). This effect is obscured in patients with concomitant defects in transfer from airspaces to RBCs, which increase RBC oscillation amplitudes.
View Article and Find Full Text PDFInterstitial lung disease (ILD) can lead to pulmonary hypertension (ILD-PH), worsening prognosis and increasing mortality. Diagnosing ILD-PH is challenging due to the limitations of imaging methods. Right heart catheterization (RHC) is the gold standard for diagnosing PH but is limited to ILD patients considered for lung transplantation.
View Article and Find Full Text PDFEur Cardiol
December 2024
Department of Respiratory Medicine, King George's Medical University Lucknow, Uttar Pradesh, India.
Pulmonary arterial hypertension (PAH) is a long-term condition characterised by increased resistance to blood flow in the pulmonary circulation. The disease has a progressive course and is associated with a poor prognosis. Without treatment, PAH is associated with mortality in <3 years.
View Article and Find Full Text PDFCardiol Young
January 2025
Children's Heart Centre, Motol University Hospital, Prague, Czech Republic.
Aims: To evaluate the prevalence, long-term mortality, and clinical characteristics in total cavopulmonary connection patients with excellent functional outcomes.
Methods And Results: A retrospective study of cardiopulmonary exercise test results in 288 patients after total cavopulmonary connection from a single-centre nationwide database. A subgroup of 88 (30.
Bioconjug Chem
January 2025
Department of Pharmacology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104-5127, United States.
Red blood cells (RBCs) serve as natural transporters and can be modified to enhance the pharmacokinetics and pharmacodynamics of a protein cargo. Affinity targeting of Factor IX (FIX) to the RBC membrane is a promising approach to improve the (pro)enzyme's pharmacokinetics. For RBC targeting, purified human FIX was conjugated to the anti-mouse glycophorin A monoclonal antibody Ter119.
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