RESUMO
After presenting to the emergency department with severe chest pain, this patient was thought to be experiencing acute myocardial infarction until imaging tests revealed apical ballooning syndrome.
RESUMO
Pulmonary arterial hypertension used to be considered an ultimately fatal disease despite the formerly available therapy. It is a disease characterized by progressive elevation of pulmonary vascular resistance and pulmonary arterial pressure, eventually resulting in right ventricular failure and death. The purpose of this article is to review the basic pathophysiologic processes involved in pulmonary arterial hypertension, to discuss patient presentation, classification, and diagnostic workup. Regarding treatment, all patients with PAH should be considered for standard therapy with anticoagulation, oxygen, and diuretics for management of right heart failure. Calcium channel blockers are only indicated for patients with a positive acute vasoreactivity test. Patients with a negative vasoreactivity test and considered low risk can be treated with oral agents such as endothelin receptor antagonists or PDE-5 inhibitor. Patients at high risk should be treated with prostacyclin analogs. Finally, a brief mention of new and future potential therapeutic strategies is also included.