ReviewAnnals of the New York Academy of Sciences2016
Pulmonary hypertension associated with thalassemia syndromes.
Review in Annals of the New York Academy of Sciences, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 63 citations in OpenAlex.
- The Association between β-Thalassemia Major (β-TM) and Cardiac Complications: Recent Insights.Current cardiology reviews · 2026Review
- Is splenectomy one of the contributory factors to pulmonary hypertension? An analysis of splenectomized hemolytic anemia and immune thrombocytopenia patients.Annals of hematology · 2025Article
- Circulating Red Cell and Platelet Microparticles in Pediatric Non-transfusion Dependent Beta-thalassemia: A Case Control Study.Oman medical journal · 2025Article
- Pulmonary hypertension aetiologies in different parts of the world.International journal of cardiology. Congenital heart disease · 2025Review
- Luspatercept Treatment in a β-Thalassemia Patient with Pulmonary Arterial Hypertension: A Case Report.Mediterranean journal of hematology and infectious diseases · 2025Article
- Review
- The Discovery of Selective Protein Arginine Methyltransferase 5 Inhibitors in the Management of β-Thalassemia through Computational Methods.Molecules (Basel, Switzerland) · 2024Article
- Macitentan Administration for Pulmonary Hypertension Due to β-thalassemia with Multiple Organ Failure.Internal medicine (Tokyo, Japan) · 2024Article
- The Prevalence of Pulmonary Arterial Hypertension in Patients with Beta Thalassemia Major.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2024Article
- The predicting formula and scoring system for cardiac iron overload for thalassaemia children: Study from a middle-income country.PloS one · 2024Article
- Extramedullary hematopoiesis in ribs and severe pulmonary hypertension disease following intermediate beta-thalassemia: a case report.Journal of medical case reports · 2023Article
- In Silico Molecular Docking and Dynamics Simulation Analysis of Potential Histone Lysine Methyl Transferase Inhibitors for Managing β-Thalassemia.Molecules (Basel, Switzerland) · 2023Article
- Pulmonary function in patients with transfusion-dependent thalassemia and its associations with iron overload.Scientific reports · 2023Observational
- Non-Transfusion-Dependent Thalassemia: A Panoramic Review.Medicina (Kaunas, Lithuania) · 2022Review
- Article
- How does iron deposition modify the myocardium? A feature-tracking cardiac magnetic resonance study.The international journal of cardiovascular imaging · 2021Article
- Luspatercept: A Gigantic Step in the Treatment of Transfusion-Dependent β-Thalassemia Patients-a Quick Review.Advances in therapy · 2021Review
- Pulmonary thromboendarterectomy for pulmonary hypertension linked to thalassemia.Clinical case reports · 2021Article
- Prevalence and risk factors of high echocardiographic probability of pulmonary hypertension in myeloproliferative neoplasms patients.International journal of hematology · 2020Article
- Pulmonary hypertension: From an orphan disease to a global epidemic.Global cardiology science & practice · 2020Review
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Chronic hemolytic anemia has increasingly been identified as an important risk factor for the development of pulmonary hypertension (PH). Within the thalassemia syndromes, there are multiple mechanisms, both distinct and overlapping, by which PH develops and that differ among β-thalassemia major or intermedia patients. PH in β-thalassemia major correlates with the severity of hemolysis, yet in patients whose disease is well treated with chronic transfusion therapy, the development of PH can be related to cardiac dysfunction and the subsequent toxic effects of iron overload rather than hemolysis. β-Thalassemia intermedia, on the other hand, has a higher incidence of PH owing to the low level of hemolysis that exists over years without the requirement for frequent transfusions, while splenectomy is shown to play an important role in both types. Standard therapies such as chronic transfusion have been shown to mitigate PH, and appropriate chelation therapy can avoid the toxic effects of iron overload, yet is not indicated in many patients. Limited evidence exists for the use of pulmonary vasodilators or other therapies, such as l-carnitine, to treat PH associated with thalassemia. Here, we review the most recent findings regarding the pathogenic mechanisms, epidemiology, presentation, diagnosis, and treatment of PH in thalassemia syndromes.
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