This paper presents the case of a young girl with idiopathic pulmonary hypertension, who developed signs of severe heart failure within a short period of time. Pharmacotherapy with sildenafil and bosentan (among other drugs) was ineffective. Heart catheterization revealed suprasystemic pressure in the pulmonary artery. At the age of 7.5 years, the patient underwent a surgical Potts shunt (namely, a direct side-by-side anastomosis from the left pulmonary artery to the descending aorta). The procedure resulted in a significant improvement of the clinical, echocardiographic, and biochemical parameters, which persists after one and a half years of follow-up. After the surgery, pharmacotherapy with bosentan was gradually discontinued.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4550015 | PMC |
| http://dx.doi.org/10.5114/kitp.2015.52864 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Secondary organizing pneumonia after infection.
World J Clin Cases
December 2024
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand.
This editorial explores the clinical implications of organizing pneumonia (OP) secondary to pulmonary tuberculosis, as presented in a recent case report. OP is a rare condition characterized by inflammation in the alveoli, which spreads to alveolar ducts and terminal bronchioles, usually after lung injuries caused by infections or other factors. OP is classified into cryptogenic (idiopathic) and secondary forms, the latter arising after infections, connective tissue diseases, tumors, or treatments like drugs and radiotherapy.
View Article and Find Full Text PDFEndothelial CD38-induced endothelial-to-mesenchymal transition is a pivotal driver in pulmonary fibrosis.
Cell Mol Life Sci
December 2024
National Engineering Research Center for Bioengineering Drugs and the Technologies, Institute of Translational Medicine, Jiangxi Medical College, Nanchang University, Nanchang, 330031, China.
Idiopathic pulmonary fibrosis (IPF) is a prevalent interstitial lung disease with high mortality. CD38 is a main enzyme for intracellular nicotinamide adenine dinucleotide (NAD) degradation in mammals. It has been reported that CD38 participated in pulmonary fibrosis through promoting alveolar epithelial cells senescence.
View Article and Find Full Text PDFHigh Shear Stress Reduces ERG Causing Endothelial-Mesenchymal Transition and Pulmonary Arterial Hypertension.
Arterioscler Thromb Vasc Biol
December 2024
Department of Pediatrics (T.S., J.-R.M., Y.H.C., J.M.S., J. Kaplan, A.C., L.W., D.G., S.T., S.I., M.D., W.Y., A.L.M., M.R.).
Background: Computational modeling indicated that pathological high shear stress (HSS; 100 dyn/cm) is generated in pulmonary arteries (PAs; 100-500 µm) in congenital heart defects causing PA hypertension (PAH) and in idiopathic PAH with occlusive vascular remodeling. Endothelial-to-mesenchymal transition (EndMT) is a feature of PAH. We hypothesize that HSS induces EndMT, contributing to the initiation and progression of PAH.
View Article and Find Full Text PDFPulmonary and Medullary Tuberculosis: An Uncommon Cause of Evans Syndrome in Adults.
Cureus
November 2024
Hematology, Cheikh Khalifa International University Hospital, Mohammed VI University of Sciences and Health, Casablanca, MAR.
Evans syndrome (ES) is a rare syndrome characterised by the association of autoimmune idiopathic hemolytic anemia (AIHA) with immune thrombocytopenia (ITP) and, less commonly, autoimmune neutropenia (AIN). ES may be primary or secondary to some aetiology, including, exceptionally, tuberculosis. We describe a case of association between pulmonary and medullary tuberculosis and Evans syndrome with an effective response to antitubercular treatment and corticosteroids.
View Article and Find Full Text PDFCorticosteroid therapy for treating acute exacerbation of interstitial lung diseases: a systematic review.
Thorax
December 2024
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Introduction: Acute exacerbation of interstitial lung disease (AE-ILD) often results in death and poses significant challenges in clinical management. While corticosteroids are frequently employed, the optimal regimen and their clinical efficacy remain uncertain. To address this knowledge gap, we undertook a systematic review to evaluate the impact of steroid therapy on clinical outcomes in patients experiencing AE-ILD.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!