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This chapter explores the epidemiology, clinical presentation, and evaluation of patients with suspected pulmonary hypertension (PH). Right heart catheterization remains the gold standard for diagnosing PH. Accurate assessment of hemodynamics, in particular the pulmonary capillary wedge pressure, is critical to accurately make the diagnosis and to differentiate pre- from postcapillary forms of PH. All patients with pulmonary arterial hypertension (PAH) should also undergo pulmonary vasoreactivity testing on initial catheterization, most commonly performed with inhaled nitric oxide. It is also reasonable to perform a full oxygen saturation run in this population to rule out congenital heart disease as an etiology of PAH. The chapter concludes with a brief discussion on advanced therapies for the treatment of pulmonary arterial hypertension and several illustrative case examples.
KeywordsPulmonary hypertension Pulmonary arterial hypertension Pulmonary vascular resistance Vasoreactivity Congenital heart disease
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