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Pulmonary hypertension is a rare lung disease characterised by the narrowing of blood vessels that carry blood from the heart to the lungs. This narrowing makes it difficult for blood to flow through the vessels, resulting in abnormally high blood pressure. The increased pressure puts a strain on the right ventricle of the heart, which is responsible for pumping blood through the pulmonary arteries. Over time, the right ventricle can enlarge and lead to right heart failure.
Pulmonary Hypertension Symptoms
Symptoms of pulmonary hypertension are not usually observed until the condition has progressed. Symptoms of pulmonary hypertension include:
- Early symptoms: Shortness of breath during everyday activities, fatigue, dizziness, fainting spells, swelling in the ankles, abdomen, or legs, bluish lips and skin, chest pain, and high blood pressure.
- Advanced stage symptoms: Passing out or dizziness, irregular heartbeat, shortness of breath during exercise, racing pulse, difficulty in breathing even at rest.
Pulmonary Hypertension Causes
Causes of pulmonary hypertension include:
- Underlying heart conditions: Pulmonary hypertension can be caused by congenital heart defect, such as atrial septal defect or ventricular septal defect, which affect the normal blood flow in the heart and lungs.
- Lung conditions: Certain lung diseases like chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, emphysema, or lung tumours can lead to pulmonary hypertension by affecting the normal functioning of the lungs and blood vessels.
- Genetic factors: In some cases, pulmonary hypertension can be inherited, meaning it can run in families. If a family member has or has pulmonary hypertension, there may be an increased risk for other family members to develop the condition.
- Living at high altitudes: People living in high-altitude areas may develop pulmonary hypertension due to the lower oxygen levels in the environment, which can put a strain on the pulmonary blood vessels.
- Rheumatic disorders: Autoimmune conditions like scleroderma or systemic lupus erythematosus (lupus) can lead to inflammation and damage to the blood vessels, including those in the lungs, contributing to pulmonary hypertension.
- Previous use of Fen-Phen: The use of the appetite suppressant drug combination known as Fen-Phen (fenfluramine/phentermine) has been associated with an increased risk of developing pulmonary hypertension. Although this medication has been withdrawn from the market, its effects may still manifest years after use.
Pulmonary Hypertension Diagnosis
Diagnosing pulmonary hypertension (PH) requires a comprehensive approach to rule out other similar conditions and take into account various factors influencing the disease and its symptoms. The diagnosis involves:
- Medical history: A thorough review of your medical history, including any past or current conditions, symptoms, and risk factors.
- Physical exam: A physical examination by a healthcare professional to assess signs and symptoms associated with PH, such as abnormal heart sounds or fluid retention.
- Symptom description: A detailed description of your symptoms, such as shortness of breath, chest pain, fatigue, and dizziness.
- Blood tests: Blood tests may be conducted to measure specific markers related to PH and rule out other potential causes.
- Chest X-ray: It can help evaluate the heart and lungs for any abnormalities.
- Doppler echocardiogram: This non-invasive test uses sound waves to create images of the heart and measure the blood flow to assess the structure and function of the heart and detect any signs of PH.
- Walk test: A six-minute walk test measures how far you can walk in six minutes and can provide information about exercise capacity and oxygen levels.
- Polysomnogram or overnight oximetry: Sleep studies may be conducted to assess breathing patterns and oxygen levels during sleep, as certain sleep disorders can contribute to PH.
- Pulmonary function test: This test evaluates lung function, including lung capacity and the ability to move air in and out of the lungs.
- Ventilation-perfusion scan: This nuclear medicine scan assesses lung ventilation and blood flow to detect any abnormalities.
- Chest CT scan: It provides detailed images of the heart, lungs, and blood vessels to identify any structural abnormalities.
- Right heart catheterisation: This invasive procedure involves threading a catheter into the right side of the heart to directly measure blood pressure and assess the severity of PH.
- Pulmonary angiogram: This imaging test uses a contrast dye injected into the pulmonary arteries to visualise blood flow and detect any blockages or abnormalities.
Treatment of Pulmonary Hypertension
Treatment of Pulmonary Hypertension includes:
- Riociguat: The first approved medication for PH, indicated for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension. It restores nitric oxide levels to counteract pathophysiological manifestations, particularly in dyssynchronous PH.
Other medications:
- Endothelin receptor antagonists: Drugs that block the effects of endothelin, a substance that narrows blood vessels.
- Phosphodiesterase-5 inhibitors: Medications that relax and widen blood vessels.
- Prostacyclin analogues: Synthetic versions of prostacyclin, a natural substance that dilates blood vessels.
- Soluble guanylate cyclase stimulators: Medications that stimulate an enzyme involved in relaxing blood vessels.
Surgical therapies:
- Pulmonary endarterectomy: A surgical procedure to remove blood clots from the arteries in the lungs.
- Atrial septostomy: A minimally invasive procedure to create a hole between the upper chambers of the heart to relieve pressure.
Pulmonary Hypertension Prevention
To prevent pulmonary hypertension and maintain heart and lung health, you can follow these strategies:
Pulmonary Hypertension Complications
Complications of pulmonary hypertension are-
- Right-Sided Heart Enlargement (Cor Pulmonale)
- Increased risk of blood clots in the small arteries in the lungs
- Bleeding in the lungs
- Irregular heartbeats leading to arrhythmias
- Life-threatening for a developing baby if it occurs in a pregnant woman
Book an appointment today to manage your hypertension! Our team of experts is ready to help you take control of your health.
Reviewed & Updated On
Reviewed by Dr. Munesh Tomar, Director - Paediatric Cardiology, Paediatric (Ped) Cardiology, Cardiac Sciences, Cardiology on 22-May-2023.
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