Thalassemia Associated Pulmonary Hypertension
Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise. The prevalence of PH is known to be higher in TI than in TM. Moreover, the pathophysiology of PH in thalassemia appears to be sophisticated and complex. Risk factors for occurrence of PH consists of non-transfusion dependent thalassemia (NTDT), sub-optimally transfused transfusion dependent thalassemia (TDT), splenectomy, thrombocytosis, anemia, NRBC ≥ 300 × 106, iron accumulation, history of thrombosis and older age. Other parameters which aggravate the risk of PH include hemolysis, oxidative stress, hypoxemia, alteration of erythrocyte membrane, decline of nitric oxide biological availability, arginine abnormal regulation and arginase excess. The screening method for PH is Doppler echocardiography but the gold standard for detection of PH is right heart catheterization (RHC). Current medical therapeutic options in PH comprise hydroxyurea, L- Carnitine, sildenafil, calcium channel antagonists, endothelin 1-receptor blockers and prostacyclin agonists. The only curative surgical method for the refractory and severe cases of PH is pulmonary endarterectomy. In this article, the etiology, pathophysiology, diagnostic methods and novel therapies of thalassemia associated PH are discussed.
Prevalence and Risk Factors of Pulmonary Arterial Hypertension in Thalassemia Major Patients of Ilam, 2014
Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality rates. Research has shown that PAH has a prevalence rate of 10-79% in thalassemia major patients. This cross-sectional study was carried out in 2014 to determine the prevalence and risk factors of PAH in all thalassemia major patients of over 18 years of age in Ilam, Iran. A cardiologist measured sy...متن کامل
OBJECTIVE To compare pulmonary artery systolic pressure (PASP) between thalassemic patients with pulmonary arterial hypertension (PAH) for whom acetylsalicylic acid (ASA) was and was not prescribed after 1 year. METHODS A retrospective cohort study was conducted at the hematological outpatient clinic at Chiang Rai Hospital, Chiang Rai, Thailand. All new cases of thalassemia with PAH from Janu...متن کامل
The occurrence of pulmonary arteriosclerosis and arteriolosclerosis has long been recognized. Such structural changes in the pulmonary vasculature have usually been considered to represent an adaptation to alterations in the haemodynamics of the lesser circulation. In particular, their presence has been related to pulmonary hypertension, and they have been compared to the changes which occur in...متن کامل
Necrotizing arteritis limited to the pulmonary arteries has been described occasionally as a complication of mitral stenosis (Parker and Weiss, 1936), of Eisenmenger's complex (Old and Russell, 1947), and in patients dying with right heart failure of uncertain causation (McKeown, 1952). One such case in this last category, in which cardiac catheterization gave clear evidence of pulmonary hypert...متن کامل
Pulmonary hypertension is a common complication of chronic obstructive pulmonary disease (COPD). The increase in pulmonary artery pressures is often mild to moderate, but some patients may suffer from severe pulmonary hypertension, and present with a progressively downhill clinical course because of right-sided heart failure added to ventilatory handicap. The cause of pulmonary hypertension in ...متن کامل
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دوره 11 شماره 1
صفحات 51- 63
تاریخ انتشار 2020-12
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