Balloon pulmonary angioplasty is a promising option in thalassemic patients with inoperable chronic thromboembolic pulmonary hypertension

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 over...

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Bibliographic Details
Published in:Journal of thrombosis and thrombolysis 2018-11, Vol.46 (4), p.516-520
Main Authors: Karyofyllis, Panagiotis, Tsiapras, Dimitris, Papadopoulou, Varvara, Diamantidis, Michael D., Fotiou, Paraskevi, Demerouti, Eftychia, Voudris, Vassilis
Format: Article
Language:English
Subjects:
Angioplasty
Angioplasty, Balloon
Blood diseases
Cardiology
Chronic Disease
Health risk assessment
Hematology
Hemolytic anemia
Humans
Hypertension
Hypertension, Pulmonary - surgery
Medicine
Medicine & Public Health
Morbidity
Patients
Pulmonary arteries
Pulmonary artery
Pulmonary Artery - surgery
Pulmonary hypertension
Splenectomy
Surgery
Thalassemia
Thalassemia - therapy
Thromboembolism
Thromboembolism - physiopathology
Treatment Outcome
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Summary: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.
ISSN:0929-5305
1573-742X
DOI:10.1007/s11239-018-1720-4