pulmonary disorders
Pulmonary Hypertension
Prognosis for Pulmonary Hypertension
Five-year survival for treated patients is about 50%. However, some patient registries suggest lower mortality (eg, 20 to 30% at 3 to 5 years in the French registry and 10 to 30% at 1 to 3 years in the REVEAL registry), presumably because currently available treatments are superior. Indicators of a poorer prognosis include Lack of response to vasodilators Hypoxemia Reduced overall physical functioning Low 6-minute walk distance High plasma levels of NT-pro-BNP or BNP Echocardiographic indicators of right heart systolic dysfunction (eg, a tricuspid annular plane systolic excursion of<1.6 cm, dilated right ventricle, flattened interventricular septum with paradoxical septal motion, and pericardial effusion) Right heart catheterization showing low cardiac output, very high mean pulmonary artery pressures, and/or high right atrial pressures Patients with systemic sclerosis, sickle cell disease, or HIV infection with pulmonary arterial hypertension (PAH) have a worse prognosis than those without PAH. For example, patients with sickle cell disease and PAH have a 40% 4-year mortality rate. 