Snapshot A 48-year-old woman presents to the emergency department with worsening exertional dyspnea over the course of a few weeks. She reports mild chest pain when climbing the stairs or walking a few blocks. She has also noticed some swelling in both of her legs. Medical history is remarkable for sarcoidosis and she denies any alcohol, cigarette, or illicit drug use. Physical examination is notable for a loud P2 heart sound, bilateral lower extremity pitting edema, and jugular venous distension. Troponin and B-natruietic peptide are not elevated. An ECG demonstrates right axis deviation. She is admitted to the cardiac step down unit where she undergoes a right heart catheterization, which demonstrates a mean pulmonary arterial pressure of 67 mmHg at rest. Introduction Definition a state of increased mean pulmonary arterial pressure ≥ 25 mmHg (at rest) in the absence of lung or left-sided heart disease Etiology Primary pulmonary arterial hypertension idiopathic familial connective tissue disease (e.g., systemic lupus erythematosus and rheumatoid arthritis) HIV congenital left-to-right shunt Left heart disease (e.g., mitral stenosis, mitral regurgitation, atrial or ventricular (diastolic or systolic) dysfunction) Lung disease (e.g., chronic obstructive pulmonary disease, sleep apnea, and interstitial lung disease) Chronic thromboembolic disease (e.g., pulmonary embolism) Sarcoidosis Malignancy compressing pulmonary vessels Presentation Symptoms exertional dyspnea, chest pain, and syncope fatigue and lethargy Physical examination loud P2 on auscultation right ventricular heave right-sided 4th heart sound murmurs holosystolic murmur of tricuspid regurgitation systolic ejection murmur diastolic pulmonic regurgitation (in severe cases) Imaging Echocardiogram estimates pulmonary artery systolic pressure evaluates the right ventricle size, thickness, and function also evaluates the left ventricular function and valvular function Radiography of the chest may demonstrate central pulmonary arterial dilatation loss of peripheral blood vessels may find right atrial and ventricular enlargement (suggestive of advanced disease) Studies ECG can demonstrate right ventricular hypertrophy (e.g., right axis deviation) Right heart catheterization confirms the diagnosis of pulmonary hypertension mean pulmonary artery pressure is ≥ 25 mmHg at rest (8-20 mmHg at rest is considered normal) vasoreactivity testing can be performed this involves administering a short-acting vasodilator followed by measuring the hemodynamics of the pulmonary artery agents that are used include nitric oxide epoprostenol adenosine diltiazem (in patients with positive vasoreactivity testing) Differential Myocardial infarction differentiating factors left heart catheterization will demonstrate a blockage in the coronary vessels Treatment Conservative continuous long-term oxygen therapy indication to maintain adequate oxygenation Medical note that there are a myriad of causes of pulmonary hypertension treatment should be aimed at treating and optimizing the underlying condition (e.g., anticoagulation in patients with recurrent pulmonary embolisms) vasoactive agents indication pulmonary hypertension targeted therapy medication options calcium channel blockers prostacyclin pathway agonists (e.g., epoprostenol, treprostinil, and iloprost) endothelin receptor antagonists (e.g., bosentan, macitentan, and ambrisentan) nitric oxide-cyclic GMP enhancer (e.g., sildenafil and tadalafil) Complications Right ventricular dysfunction Right heart failure Arrhythmia Prognosis Can result in right-sided heart failure