Summary about Disease
Pulmonary hypertension (PH) is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. In pulmonary hypertension, the blood vessels that carry blood from the heart to the lungs become narrowed, blocked or destroyed. This makes it harder for blood to flow through the lungs, and raises the pressure within the pulmonary arteries. As the pressure increases, the heart's right ventricle must work harder to pump blood through the lungs, eventually causing the heart muscle to weaken and fail.
Symptoms
Symptoms of pulmonary hypertension can be subtle at first and may not be recognized for years. As the disease progresses, symptoms usually worsen. Common symptoms include:
Shortness of breath, especially during exercise
Fatigue
Dizziness or fainting spells
Chest pain
Swelling in the ankles, legs and eventually in the abdomen (edema)
Bluish color to the lips and skin (cyanosis)
Racing or pounding heartbeat
Causes
Pulmonary hypertension can be caused by a variety of factors, including:
Idiopathic pulmonary arterial hypertension (IPAH): The cause is unknown.
Heritable pulmonary arterial hypertension (HPAH): Caused by genetic mutations.
Associated with other conditions: Such as congenital heart defects, connective tissue diseases (scleroderma, lupus), HIV infection, liver disease (cirrhosis), sleep apnea, chronic thromboembolic pulmonary hypertension (CTEPH), sickle cell anemia, and certain medications or toxins.
Left heart disease: When the left side of the heart isn't working well, it can cause blood to back up into the lungs, increasing pressure in the pulmonary arteries.
Lung diseases: Conditions like COPD and pulmonary fibrosis can also lead to pulmonary hypertension.
Medicine Used
There is no cure for pulmonary hypertension, but medications can help manage symptoms and slow the progression of the disease. Medications used to treat pulmonary hypertension include:
Vasodilators: These medications relax and widen the blood vessels in the lungs, making it easier for blood to flow. Examples include prostacyclin analogs (epoprostenol, treprostinil, iloprost), endothelin receptor antagonists (bosentan, ambrisentan, macitentan), and phosphodiesterase-5 (PDE5) inhibitors (sildenafil, tadalafil).
Calcium channel blockers: In some cases, these can help relax the blood vessels in the lungs.
Diuretics: Help to remove excess fluid from the body, reducing swelling.
Anticoagulants: Prevent blood clots, which can worsen pulmonary hypertension.
Oxygen therapy: Can help to improve blood oxygen levels.
Digoxin: Helps the heart beat stronger and more efficiently.
Is Communicable
Pulmonary hypertension is not communicable. It is not an infectious disease and cannot be spread from person to person.
Precautions
Precautions for individuals with pulmonary hypertension include:
Following your doctor's treatment plan: Taking medications as prescribed and attending regular checkups.
Avoiding strenuous activity: Restricting activities that cause shortness of breath or fatigue.
Avoiding high altitudes: Lower oxygen levels at high altitudes can worsen symptoms.
Avoiding pregnancy: Pregnancy can be dangerous for women with pulmonary hypertension.
Getting vaccinated: Against the flu and pneumonia to prevent respiratory infections.
Quitting smoking: Smoking damages the lungs and can worsen pulmonary hypertension.
Managing underlying conditions: If pulmonary hypertension is caused by another condition, such as sleep apnea or HIV, it's important to manage those conditions effectively.
Maintaining a healthy weight: Obesity can put extra strain on the heart and lungs.
How long does an outbreak last?
Pulmonary hypertension is not an infectious disease that causes outbreaks. It is a chronic condition, meaning it is long-lasting.
How is it diagnosed?
Pulmonary hypertension can be difficult to diagnose early because the symptoms are similar to those of other conditions. Diagnostic tests may include:
Echocardiogram: An ultrasound of the heart to assess the pressure in the pulmonary arteries and the function of the heart.
Right heart catheterization: A catheter is inserted into a blood vessel in the neck or groin and guided into the right side of the heart and pulmonary arteries to directly measure the pressure. This is the most accurate test for diagnosing pulmonary hypertension.
Pulmonary function tests: Measure lung capacity and airflow.
Chest X-ray: Can show enlargement of the pulmonary arteries or right ventricle.
CT scan of the chest: Provides detailed images of the lungs and blood vessels.
Pulmonary angiogram: Uses dye to highlight the pulmonary arteries and look for blockages.
Blood tests: Can help rule out other conditions and assess overall health.
Sleep Study: Tests for sleep apnea.
Timeline of Symptoms
The timeline of symptoms in pulmonary hypertension can vary greatly from person to person.
Early Stage: Often subtle and may include shortness of breath with exertion, fatigue, and dizziness. These symptoms may be mild and easily dismissed.
Progressive Stage: As the disease progresses, symptoms become more pronounced and frequent. Shortness of breath occurs with less exertion, and fatigue becomes more debilitating. Other symptoms, such as chest pain, swelling in the legs and ankles, and bluish skin, may develop.
Advanced Stage: In the advanced stages, symptoms are severe and can occur even at rest. The person may experience severe shortness of breath, chest pain, fainting spells, and significant swelling. Heart failure may develop.
Important Considerations
Early diagnosis and treatment are crucial to improving the prognosis of pulmonary hypertension.
Pulmonary hypertension is a serious condition that can significantly impact quality of life.
Patients with pulmonary hypertension should be managed by a team of specialists, including a pulmonologist, cardiologist, and other healthcare professionals.
Support groups and other resources can provide valuable information and support for patients and their families.
Clinical trials are ongoing to develop new and more effective treatments for pulmonary hypertension.