Case 2 depiction. In a transfusion-dependent patient with HbE β thalassemia (case 2), splenectomy was followed by discontinuation of regular transfusions. Fifteen years later, severe pulmonary arterial hypertension and congestive heart failure developed. These pathological changes were reversed by resumption of transfusions (arrow) and supportive care. LVEF%, left ventricular ejection fraction (percent); PASP, pulmonary artery systolic pressure.