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Balloon pulmonary angioplasty is a promising option in thalassemic patients with inoperable chronic thromboembolic pulmonary hypertension
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نویسنده
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Panagiotis Karyofyllis ,Dimitris Tsiapras ,Varvara Papadopoulou ,Michael D. Diamantidis ,Paraskevi Fotiou ,Eftychia Demerouti ,Vassilis Voudris
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منبع
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journal of thrombosis and thrombolysis - 2018 - دوره : 46 - شماره : 4 - صفحه:516 -520
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چکیده
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pulmonary hypertension (ph), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. β-thalassaemia, among other haematological disorders, develop ph which is not an infrequent finding and worsens the prognosis. haemolysis, iron overload and hypercoagulable state are among the main pathogenetic mechanisms. haemoglobinopathies and congenital haemolytic anaemia constitute a unique patients population more predisposed to developing chronic thromboembolic pulmonary hypertension (cteph). although pulmonary endarterectomy is accepted as the best treatment for cteph, surgery in these patients poses significant practical challenges given the distinct nature of the disease. as drug therapy is not expected to offer relief from the mechanical obstructions of pulmonary arteries and the use of specific pulmonary arterial hypertension drugs is not established in thalassaemia patients, the novel technique of balloon pulmonary angioplasty (bpa) may emerge as a new therapeutic option for patients with inoperable cteph and thalassaemia. we are reporting the case of a thalassaemic patient with a history of splenectomy suffering from progressive severe pulmonary hypertension related to chronic thromboembolic disease, who was successfully treated by bpa with substantial improvement.
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کلیدواژه
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Thalassemia ,Hemoglobinopathies ,Chronic thromboembolic pulmonary hypertension (CTEPH) ,Balloon pulmonary angioplasty (BPA)
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آدرس
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Onassis Cardiac Surgery Centre, Division of Interventional Cardiology, Greece, Onassis Cardiac Surgery Centre, Noninvasive Cardiology Diagnostic Unit, Greece, Onassis Cardiac Surgery Centre, Division of Interventional Cardiology, Greece, General Hospital of Larissa, Thalassemia Unit, Department of Hematology, Greece, General Hospital of Larissa, Thalassemia Unit, Department of Hematology, Greece, Onassis Cardiac Surgery Centre, Noninvasive Cardiology Diagnostic Unit, Greece, Onassis Cardiac Surgery Centre, Division of Interventional Cardiology, Greece
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Authors
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