Prevalence of pulmonary arterial hypertension (PAH) was studied by Echocardiography and Doppler in 43 splenectomised patients with various disorders 1-20 years after splenectomy. PAH was detected only in thalassemia major, intermedia, hereditary sphereocytosis and myelofibrosis groups comprising a total of 21 patients. Six patients out of 21 was found to have PAH with mean pulmonary arterial pressure of 46.28 ± 28.17 mmHg. Twenty one controls having similar duration and type of disease also were assessed for PAH in this case control study 3/21 had PAH in this control group. The difference in number of patients showing pulmonary hypertension between case and control was not statistically significant (chi-square test p = 0.29-though the difference in pulmonary arterial pressure between case and control were significantly different (t-test p<0.0029) with control group showing a mean pulmonary arterial pressure of 25 ± 19 mmHg.Platelet count in the splenectomised group was significantly higher (p = 0.0029) than the controls. Pulmonary thromboembolism was equally high in the PAH patients with and without splenectomy. Patients undergoing splenectomy due to trauma, immune thrombocytopenia, sideroblastic anemia, extra hepatic portal hypertension, autoimmune hemolytic anemia did not show PAH after splenectomy even years after the procedure PAH following splenectomy is common after certain disorders and control patients with these diseases have tendency to develop PAH even without splenectomy. Pulmonary thromboembolism may be an important pathophysiological mechanism leading to this condition. Patients having hemolytic anemia and myelofibrosis should have regular evaluation of pulmonary arterial pressure whether he/she has been splenectomised or not. This is particularly important as availability of phosphodiesterase inhibitors like sildenafil allows one to manage these cases.
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http://dx.doi.org/10.1007/s12288-010-0002-x | DOI Listing |
J Cardiovasc Magn Reson
January 2025
Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA; Department of Biomedical Engineering, McCormick School of Engineering, Northwestern University, Evanston, Illinois, USA. Electronic address:
Delivery of health care, including medical imaging, generates substantial global greenhouse gas emissions. The cardiovascular magnetic resonance (CMR) community has an opportunity to decrease our carbon footprint, mitigate the effects of the climate crisis, and develop resiliency to current and future impacts of climate change. The goal of this document is to review and recommend actions and strategies to allow for CMR operation with improved sustainability, including efficient CMR protocols and CMR imaging workflow strategies for reducing greenhouse gas emissions, energy, and waste, and to decrease reliance on finite resources, including helium and waterbody contamination by gadolinium-based contrast agents.
View Article and Find Full Text PDFEur Respir J
January 2025
INSERM UMR_S 999 « Pulmonary Hypertension: Pathophysiology and Novel Therapies », Hôpital Marie Lannelongue, Le Plessis-Robinson, France
Background: European guidelines recommend initial monotherapy in PAH patients with cardiovascular (CV) comorbidities based on the limited of evidence for combination therapy in this growing population.
Methods: A retrospective analysis was conducted on incident PAH patients enrolled in the French Pulmonary Hypertension Registry between 2009 and 2020. Propensity score matching was used to investigate initial dual oral combination therapy oral monotherapy in patients with at least one CV comorbidity (, hypertension, obesity, diabetes and coronary artery disease).
Eur Respir J
January 2025
Université Paris-Saclay, INSERM Unité Mixte de Recherche en Santé 999 (HPPIT), Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital Bicêtre (Assistance Publique-Hôpitaux de Paris), Le Kremlin-Bicêtre, France.
Introduction: Pulmonary arterial hypertension (PAH) is a progressive disease associated with significant morbidity and mortality. Sotatercept is a first-in-class activin signalling inhibitor that acts to restore the balance between the growth-promoting and growth-inhibiting signalling pathways.
Methods: This post-hoc, exploratory, pooled analysis combines data from the double-blind placebo periods of the phase 2 PULSAR (NCT03496207) and phase 3 STELLAR (NCT04576988) studies.
BMJ Case Rep
January 2025
Department of Allergy, Immunology and Respiratory Medicine, Alfred Hospital, Melbourne, Victoria, Australia.
We describe a woman in her late 20s with newly diagnosed systemic lupus erythematosus (SLE), who presented with fulminant pulmonary arterial hypertension (PAH) requiring inotropic and extracorporeal support. She was established on triple pulmonary vasodilator therapy with concurrent aggressive immunosuppression; however, treatment was complicated by infection and diffuse alveolar haemorrhage, necessitating delays in immunosuppression and withdrawal of epoprostenol. Despite this, with ongoing suppression of her SLE, her pulmonary haemodynamics improved, with normal pressures on right heart catheterisation several months later allowing stepdown to sildenafil monotherapy.
View Article and Find Full Text PDFJ Thromb Haemost
January 2025
Department of Cardiology, Inselspital, Bern University Hospital, University of Bern, Switzerland.
Background: Ultrasound-assisted catheter-directed thrombolysis (USAT) is nowadays available as an alternative reperfusion approach for acute pulmonary embolism (PE). The lytic agent recombinant tissue-type plasminogen activator (rt-PA) activates the effector protease plasmin to induce fibrinolysis. The aim of this study was to identify predictive markers for the efficacy of USAT in patients with acute PE.
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