High blood pressure

Disease Description (Causes and Symptoms)

• Definition: Pulmonary hypertension is a condition in which the pressure in the blood vessels leading to the lungs (pulmonary arteries) is elevated. It is defined when the mean pulmonary artery pressure is 25 mmHg or higher.
• Pathophysiology: Mainly due to increased pulmonary vascular resistance caused by constriction, wall hypertrophy, intimal proliferation, and thrombotic pulmonary vascular lesions in small pulmonary vessels with a diameter of ≤1 mm, resulting in right ventricular overload and progression to right-sided heart failure. In some patients, it occurs secondarily due to pulmonary thromboembolic lesions or underlying heart or lung disease.
• Main causes: Unknown cause (primary/idiopathic), hereditary predisposition, medications (e.g., some appetite suppressants and hormone medications), connective tissue disease, congenital heart disease, chronic lung disease, pulmonary embolism/thrombosis, liver disease, and others.
• Symptoms: In the early stages, shortness of breath (worsening with exertion), fatigue, dizziness or fainting, chest pain, hemoptysis, peripheral edema, hoarseness, and others. Symptoms progress gradually, and it often takes an average of approximately 2.5 years to reach a diagnosis.
• Epidemiology (summary): Rare, occurring in approximately 2 people per million population (estimated at approximately 1,500 people in Korea). It is most common in people in their 20s–40s and occurs more often in women (female-to-male ratio approximately 1.7:1).

Treatment Methods (Standard Treatment, Medications, Procedures, Lifestyle Management, and Precautions)

• Diagnostic basics: Right heart catheterization (cardiac catheterization) is the standard for measuring pulmonary artery pressure and performing an acute vasoreactivity test. The cause is classified using echocardiography, V/Q scanning, chest CT, pulmonary function tests, and blood tests (including autoimmune and liver function tests).
• Drug treatment (standard targeted therapy)
  • Vasodilators (including calcium channel blockers): Useful for the small number of patients who respond to an acute vasoreactivity test.
  • Prostacyclin agents (e.g., epoprostenol or its analogues): Available in intravenous, subcutaneous, inhaled, and oral formulations; important for improving exercise capacity, symptoms, and survival. A key treatment for patients with severe disease.
  • Endothelin receptor antagonists (ERAs; e.g., bosentan): Used to improve symptoms and prognosis.
  • PDE-5 inhibitors (sildenafil and tadalafil) and guanylate cyclase stimulators (riociguat): Used to promote vasodilation and improve symptoms.
  • Anticoagulants: Used in some patients to inhibit the progression of thrombotic lesions in the pulmonary vessels (individualized treatment).
  • Supportive therapy: Oxygen therapy (correction of hypoxia), diuretics (edema control), and other symptomatic treatments.
• Procedures and surgery
  • For pulmonary thromboembolic causes, embolus removal may be possible (through surgery or a catheter-based procedure).
  • In advanced cases that cannot be controlled with medication, lung or heart-lung transplantation may be considered (the 2-year post-transplant survival rate is approximately 60%).
• Other
  • Acute inhaled nitric oxide (NO), among others, has been reported, but there are limitations to routine use.
• Precautions (Lifestyle Management and Contraindications)
  • Pregnancy can worsen pulmonary artery pressure, so contraception is generally recommended; oral contraceptives containing estrogen are contraindicated. In many situations, termination of pregnancy is recommended if pregnancy occurs (individual assessment is necessary).
  • Avoid excessive physical activity and strenuous exercise, high altitudes (air travel and mountain climbing), and exposure to smoking or secondhand smoke.
  • Use caution with medications that may reduce cardiac output, such as barbiturate sleeping pills.
  • Treatment medications (including prostacyclins, ERAs, and PDE-5 inhibitors) have medication-specific adverse effects and interactions, so regular monitoring is necessary (including liver enzyme levels, blood pressure, and blood tests).
  • Vaccination (including influenza and pneumococcal vaccines) is recommended, as respiratory infections may trigger worsening.

Checklist Before an Outpatient Visit (Items to Prepare and Things to Confirm)

• Items to prepare/bring
  • A list of all medications currently being taken (including prescription drugs, over-the-counter drugs, supplements, and hormone medications)
  • Previous test results and imaging reports (including echocardiography, chest CT, V/Q scan, pulmonary function tests, and right heart catheterization results) or the relevant CD
  • A record of recent symptoms and their progression (when shortness of breath began and its pattern of worsening, fainting or presyncopal symptoms, changes in exercise capacity, weight changes, leg swelling, hemoptysis, and others)
  • Information on the possibility of pregnancy and contraceptive use, smoking history, and occupational exposures
• Main items the doctor will check during the visit
  • When symptoms worsened and triggering factors (exercise, altitude, infection, and others)
  • Symptoms suspected to be adverse effects of medications (dizziness, hypotension, liver-related symptoms, and others)
  • Activity level of shortness of breath (e.g., changes in 6-minute walk distance) and degree of physical limitation

Examples of General Questions to Ask the Doctor

• "What is my pulmonary artery pressure, and how well is my right ventricle functioning?"
• "What standard treatment options are recommended, and what effects and adverse effects can be expected?"
• "Did I undergo an acute vasoreactivity test? What are the treatment implications of the result?"
• "I am planning a pregnancy. What safe contraceptive methods or management plan would apply if I become pregnant?"
• "When might lung transplantation or referral to a specialized center be necessary?"

Examples of Disease-Specific Questions

• "Was I a responder in the acute vasodilation (vasoreactivity) test performed during right heart catheterization? How will the result affect my long-term treatment plan, such as the use of calcium channel blockers?"
• "Which should be prioritized for me among prostacyclin (intravenous, inhaled, or oral), endothelin antagonists, and PDE-5 inhibitors? What is the basis for choosing each medication, and what lifestyle inconveniences can be expected, such as administration methods and monitoring requirements?"