Pulmonary hypertension has become an increasingly identified comorbidity to numerous common disease processes and portends a very poor prognosis. Recent advancements in clinical recognition, innovative imaging modalities, and new classification schemes have shed new light on our understanding of this condition and advanced our treatment options. The principal subtype, pulmonary arterial hypertension, is a rare, progressive, and fatal condition manifest by elevated blood pressure in the pulmonary circulation as a result of increased pulmonary vascular resistance (PVR). Elevated pulmonary blood pressure increases the afterload of the right ventricle creating strain, which can progress to right ventricular failure and death. This condition is manifest by clinical symptoms including dyspnea, fatigue, and chest pain along with signs of elevated pulmonary pressures and right heart dysfunction. The clinical evaluation of pulmonary hypertension includes numerous invasive and noninvasive testing including blood work, imaging modalities, and right heart catheterization. Treatment of pulmonary hypertension includes both supportive care therapeutics and targeted pulmonary vasodilators. While supportive care with diuretics, oxygen, anticoagulation, and inotropic agents remain commonplace, newer agents including prostacyclin analogs, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and guanylate cyclase stimulators have clearly shown safety and efficacy in numerous clinical trials. Recent years have produced substantial progress in our management of pulmonary hypertension; still much needs to be done in order to identify novel targets that may have far-reaching impact on overall morbidity and mortality.
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology(all)