TY - JOUR
T1 - Pulmonary arterial hypertension in a patient with β-thalassemia intermedia and reversal with infusion epoprostenol then transition to oral calcium channel blocker therapy
T2 - Review of literature
AU - Ussavarungsi, Kamonpun
AU - Burger, Charles D.
N1 - Publisher Copyright:
© 2014 by the Pulmonary Vascular Research Institute. All rights reserved.
PY - 2014/9
Y1 - 2014/9
N2 - Pulmonary arterial hypertension (PAH) is a potentially life-threatening complication of thalassemia. A sexagenarian with β-thalassemia intermedia presented with new-onset dyspnea and syncope. Right heart catheterization confirmed severe PAH. Her functional class IV symptoms and severely elevated mean pulmonary artery pressure prompted the initiation of continuous epoprostenol therapy. Clinical follow-up documented significant improvement in functional class, 6-minute walk distance, and right ventricular size and function as well as pulmonary arterial pressure on echocardiogram. At the patient’s request, epoprostenol was down-titrated and eventually discontinued. The patient was then safely transitioned to nifedipine therapy after verification of vasoresponsiveness.
AB - Pulmonary arterial hypertension (PAH) is a potentially life-threatening complication of thalassemia. A sexagenarian with β-thalassemia intermedia presented with new-onset dyspnea and syncope. Right heart catheterization confirmed severe PAH. Her functional class IV symptoms and severely elevated mean pulmonary artery pressure prompted the initiation of continuous epoprostenol therapy. Clinical follow-up documented significant improvement in functional class, 6-minute walk distance, and right ventricular size and function as well as pulmonary arterial pressure on echocardiogram. At the patient’s request, epoprostenol was down-titrated and eventually discontinued. The patient was then safely transitioned to nifedipine therapy after verification of vasoresponsiveness.
KW - Epoprostenol treatment
KW - Pulmonary arterial hypertension
KW - Pulmonary hypertension
KW - β-thalassemia intermedia
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U2 - 10.1086/677367
DO - 10.1086/677367
M3 - Review article
AN - SCOPUS:85026337274
SN - 2045-8932
VL - 4
SP - 520
EP - 526
JO - Pulmonary Circulation
JF - Pulmonary Circulation
IS - 3
ER -