Pulmonary arterial hypertension in a patient with β-thalassemia intermedia and reversal with infusion epoprostenol then transition to oral calcium channel blocker therapy: review of literature

Abstract

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.

Keywords: epoprostenol treatment; pulmonary arterial hypertension; pulmonary hypertension; β-thalassemia intermedia.

Figure 1

Time line of patient’s clinical course. E/e’: ratio of early transmitral flow velocity to early diastolic mitral annulus velocity; LV: left ventricle; LVEF: left ventricular ejection fraction; MPAP: mean pulmonary artery pressure; NYHA: New York Heart Association; RHC: right heart catheterization; RV: right ventricle; RVSP: right ventricular systolic pressure; TAPSE: tricuspid annular plane systolic excursion; TID: 3 times per day; XL: extended release.