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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cardiac valve disease resulting from elastic tissue defects has been demonstrated in patients with beta-thalassemia; however, valve disorders of patients with alpha-thalassemia have been rarely discussed. We present the case of a patient with alpha-thalassemia and severe aortic regurgitation with left ventricular dysfunction. The patient underwent successful aortic valve replacement with mechanical prosthesis. Histopathology of the excised valve showed elastic tissue disruption and chronic thrombus on the ventricular side. Hypercoagulative states have been observed in patients with alpha-thalassemia as beta-thalassemia; therefore special attention should be taken in perioperative anticoagulation therapy.
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PMID:Aortic valve replacement in a patient with alpha-thalassemia. 2103 Sep 27

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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PMID:Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia. 2507

Outcome after heart valve surgery in patients affected by thalassemia is an unreported issue and to the best of our knowledge only 7 cases have been described in the literature. Heart valve disease is commonly encountered in thalassemia patients and heart valve replacement carries high risk of prosthesis complications including thrombosis and embolization despite optimal anticoagulation management. We report a successful long-term outcome after a case of aortic valve repair after mycotic valve endocarditis.
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PMID:Thalassemia and heart surgery: aortic valve repair after endocarditis. 2555 80