Annals of Thoracic Medicine Official publication of the Saudi Thoracic Society, affiliated to King Saud University
 
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GUIDELINES
Year : 2014  |  Volume : 9  |  Issue : 5  |  Page : 67-73

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia


Department of Medicine, Division of Pulmonary Medicine, King Specialist Hospital and Research Center, Riyadh 11211, Saudi Arabia

Correspondence Address:
Sarfraz Saleemi
MD, FCCP, Division of Pulmonary Medicine, Department of Medicine, King Faisal Specialist Hospital and Research Center, PO Box 3354, Riyadh 11211
Saudi Arabia
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DOI: 10.4103/1817-1737.134039

PMID: 25077000

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Hereditary hemoglobin disorders affecting the globin chain synthesis namely thalassemia syndromes and sickle cell disease (SCD) are the most common genetic disorders in human. Around 7% of the world population carries genes for these disorders, mainly the Mediterranean Basin, Middle and Far East, and Sub-Saharan Africa. An estimated 30 million people worldwide are living with sickle cell disease, while 60-80 million carry beta thalassemia trait. About 400,000 children are born with severe hemoglobinopathies each year. Cardiovascular complications of hemoglobinopathies include left and right ventricular (RV) dysfunction, arrhythmias, pericarditis, myocarditis, valvular heart disease, myocardial ischemia, and notably pulmonary hypertension (PH). Because of a unique pathophysiology, pulmonary hypertension associated with hemolytic disorders was moved from WHO group I to group V PH diseases. Treatment strategies are also unique and include blood transfusion, iron chelation, hydroxyurea, and oxygen therapy. The role of PH-specific agents has not been established.


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