ABSTRACT
Pulmonary arterial hypertension (PAH) is characterized by progressive remodeling of small pulmonary arteries leading to elevated pulmonary vascular resistance and right-heart failure. PAH patients may suffer from acute right-heart failure requiring management in the intensive care unit (ICU). These episodes of decompensated right ventricular failure necessitate close monitoring, and specialized management may include high-dose intravenous diuretics, as well as inotropic and vasoconstrictive drugs. Many studies have evaluated acute right-heart failure in the absence of preexisting pulmonary hypertension (mainly acute pulmonary embolism), and guidelines are available in this setting. By contrast, very few studies have investigated acute heart failure in PAH patients. Despite the apparent similarities with other causes of acute right-heart failure, the physiological mechanisms might be different in PAH, because of previous adaptation of the right ventricle to chronic elevation of pulmonary vascular resistance and that of the left ventricle to dilated and hypokinetic right ventricle. In the absence of comprehensive studies in PAH, precise therapeutic end points remain elusive. This chapter will focus on practical management and predictors of outcome of acute worsening of PAH.
