Clinical presentation, differential diagnosis, and vasodilator testing of pulmonary hypertension

Clinicians should be cognizant of the symptoms and risk factors associated with pulmonary hypertension (PH). While known PH poses significant therapeutic challenges, occult PH carries the added potential for unanticipated complications when treating concurrent medical illnesses. PH may occur with underlying medical conditions and risk factors or may occur de novo as idiopathic pulmonary arterial hypertension (IPAH). Symptoms of PH are frequently attributed to more common conditions, and their nonspecific nature and insidious onset may lead to delay in presentation, evaluation, and diagnosis. Initial symptoms are dyspnea, fatigue, chest pain, and palpitations. Lower extremity edema, presyncope, and syncope are symptoms of more advanced disease. Thorough evaluation of symptoms and identification of patients with risk factors for PH are critical in making a timely diagnosis. History and physical examination can identify patients with suspected PH. Further testing is necessary for definitive diagnosis, classification, assessment of severity, and guiding therapeutic decisions.