EP Monthly - By Long B & Koyfman A - December 23, 2016
"Pulmonary hypertension is a condition with significant morbidity and mortality. Primary pulmonary hypertension (just one group of the condition) is rare with 5 to 15 cases per 1 million adults, though patients with the disease accounted for 64,400 ED visits over a 5-year period in one study. Patients are often on complex medication regimens, and managing these patients is difficult due to the heterogeneous nature of the disease. Unfortunately, despite the potential for severe disease, up to 21% of patients have symptoms over two years before diagnosis. These patients in particular have greater functional disability and risk of mortality...
Summary
Pulmonary hypertension is a rare disease with high morbidity and mortality. Five groups are present, based on the underlying disease. Ask the patient about prior pulmonary vasodilator treatment, chest pain, exertional dyspnea, and syncope. Key studies include cardiac biomarkers, electrolytes, and imaging (chest X-ray, bedside US, and chest CT). Management requires focus on treating the underlying cause of the exacerbation, consulting pulmonology, continuing pulmonary vasodilator treatment, optimizing volume, optimizing RV systolic function and perfusion, and rapidly treating dysrhythmias. Patients with negative prior workups for cardiovascular/pulmonary complaints should be referred for pulmonology follow-up and echocardiogram."