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Patients with pulmonary hypertension who undergo anesthesia and surgery have high morbidity and mortality. Recent advances in our understanding of pulmonary hypertension and its therapy provide an opportunity to improve outcomes.Pulmonary hypertension can be classified into several subtypes, each with its own causes, pathophysiology, and therapy. Echocardiography remains a critical aspect of the evaluation of patients with pulmonary hypertension, but estimation of right ventricular systolic pressure is often inaccurate. Inhaled vasodilators can produce selective and potent pulmonary vasodilation.The cause of pulmonary hypertension should be defined in perioperative patients with pulmonary hypertension, and therapy should be optimized prior to anesthesia. Pulmonary artery catheterization may be required to confirm the presence of pulmonary hypertension and its severity. The focus of anesthetic management is to maintain right ventricular cardiac output and avoid systemic hypotension. Inhaled vasodilators such as nitric oxide and prostacyclin can be life-saving when perioperative right heart failure occurs due to exacerbation of pulmonary hypertension.