Snapshot A 37-year-old woman is referred to the pulmonologist’s office from her PCP for worsening shortness of breath. She reports that she has had increasing dyspnea on exertion and fatigue for the past 3 years, but it has gotten much worse in the last 2 months. She used to walk around the neighborhood with her friends but now gets short of breath with occasional chest pain just walking around the house. Her past medical history is otherwise completely negative. On physical exam, she has a JVP of 13 cm H2O, a loud P2, an S3, a right-sided heave, a liver palpable 3 cm below the costal margin, and bilateral leg edema. She is sent for a trans-thoracic echocardiogram which estimates a high pulmonary artery systolic pressure and shows right ventricular hypertrophy and right heart failure. The diagnosis is confirmed with a right heart catheterization and she is started on epoprostenol. Introduction Clinical definition login to view 3 more bullets Epidemiology login to view 7 more bullets Primary Etiologies of Pulmonary Hypertension Etiology Key Points Idiopathic pulmonary arterial hypertension (IPAH) By definition, the cause is unknown Hereditary pulmonary arterial hypertension (HPAH) Mutation in the BMPR-2 gene acts normally to inhibit smooth muscle growth Mnemonic: Blocker of Muscle PRoliferation More common in women (9:1) Poor prognosis Secondary Etiologies of Pulmonary Hypertension Etiology (secondary to) Key Points Autoimmune disease Systemic sclerosis > SLE >> RA Inflammation of vessel wall leads to scarring and stiff vessels Drugs Direct pulmonary vasoconstriction e.g., cocaine or methamphetamine Infection Schistosomiasis leading cause of pulmonary hypertension in endemic areas HIV Left-to-right shunt Increased flow to pulmonary system e.g., from congenital heart defects Left heart disease Decreased cardiac output backs blood into pulmonary circuit e.g., left heart failure or mitral valvular disease Lung disease/hypoxia Decreased oxygen delivery leads to hypoxemic pulmonary vasoconstriction e.g., COPD, obstructive sleep apnea, obesity hypoventilation syndrome, or high altitudes Thromboembolic disease Chronic, recurrent micro-emboli decrease the cross-sectional area of pulmonary vessels Unclear/miscellaneous e.g., carcinoid syndrome, sarcoidosis, vasculitis, or metabolic disorders Pathogenesis login to view 9 more bullets Associated conditions login to view 5 more bullets Prognosis login to view 5 more bullets Presentation Symptoms login to view 8 more bullets Physical examination login to view 7 more bullets Imaging Echocardiogram login to view 5 more bullets Chest radiograph login to view 2 more bullets CT angiography login to view 1 more bullet Studies Labs login to view 2 more bullets Diagnostic procedures login to view 3 more bullets Diagnosis login to view 3 more bullets Differential Congestive heart failure (without PH) login to view 4 more bullets Coronary artery disease login to view 4 more bullets Liver disease login to view 4 more bullets Treatment Management approach login to view 2 more bullets Medical login to view 24 more bullets Complications Cor pulmonale login to view 1 more bullet Chronic hypoxemic respiratory failure