[Pulmonary hypertension in patient with beta-thalassemia major].

Abstract

We report a case of pulmonary hypertension (PH) in a 35-year old patient with beta-thalassemia major; he had commenced blood transfusions after the age of 4 years and had been splenectomised at the age of 6 years. PH clinical presentation was not uncommon. Hemodynamic study revealed precapillary PH with high cardiac output; vasodilators agents led to significant pulmonary responsiveness. In beta-thalassemia, whereas congestive heart failure is common and due to cardiac hemosiderin deposition, PH appears to be non rare but its etiopathogenic mechanism remain unclear and probably non univoqual. Hypoxemia as well as hemodynamic changes related to chronic anemia including increased pulmonary flow might play an important role. Management should include blood transfusions to correct anemia, the indication and the choice of vasodilator agents need to be evaluated.

• Authors: J M Mérault, R Escamilla, B Degano, J Guittard, C Hermant, M Krempf
• Journal: Revue des maladies respiratoires (Rev Mal Respir) Vol. 17 Issue 3 Pg. 689-91 (Jun 2000) ISSN: 0761-8425 [Print] France
• Vernacular Title: Hypertension artérielle pulmonaire chez un patient porteur d'une bêta-thalassémie majeure.
• PMID: 10951965 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Vasodilator Agents

Topics

• Adult
• Blood Transfusion
• Echocardiography, Doppler
• Hemodynamics
• Humans
• Hypertension, Pulmonary (diagnosis, drug therapy, etiology, physiopathology)
• Male
• Pulmonary Circulation
• Tomography, X-Ray Computed
• Vasodilator Agents (therapeutic use)
• beta-Thalassemia (blood, complications, genetics, therapy)