Pulmonary Hypertension

Pulmonary Hypertension
A rare lung disorder, primary pulmonary hypertension (PPH) is high blood pressure in the lungs. This condition is marked by a narrowing of the blood vessels in the lungs, which causes the pressure in the pulmonary arteries (structures that carry blood from your body to the lungs to be oxygenated) to rise above normal levels. Primary pulmonary hypertension is a chronic disease that can lead to heart failure if left untreated.  

What Causes Primary Pulmonary Hypertension? 

While medical professionals have not designated an exact cause of primary pulmonary hypertension, there are some possible links to: 

  • Genetics or gene defects 
  • Certain heart and lung diseases, including COPD and aortic valve disease 
  • Prior use of the diet drug “fen-phen” (dexfenfluramine and phentermine) 

If not related to the presence of another condition, the cause of primary pulmonary hypertension is oftentimes unknown.  

Signs & Symptoms of Primary Pulmonary Hypertension 

The signs of primary pulmonary hypertension can develop slowly over time, and individuals with this condition may experience symptoms differently.  

Signs to look out for include: 

  • Fatigue 
  • Breathing difficulties or shortness of breath 
  • Chest pain (angina) 
  • Dizziness or passing out 
  • Irregular heartbeat or racing pulse 

As the disease worsens, carrying out regular activities may become more difficult. 

Diagnosing Primary Pulmonary Hypertension 

To diagnose primary pulmonary hypertension, your doctor will conduct a physical examination and ask about your medical history and symptoms. Next, additional testing will help rule out other possible causes and conditions.  

Tests used to diagnose PPH include: 

  • Imaging scans such as a chest X-ray or CT scan 
  • An electrocardiogram (ECG) to record the strength and timing of the heart rhythm 
  • An echocardiogram, a test that uses sound waves to check the chambers and valves of the heart 
  • Pulmonary function tests (PFTs) to check for other lung conditions 
  • A pulmonary angiogram or ventilation perfusion scan (V/Q scan) to look for blood clot blockages 
  • Blood tests to check how other organs are functioning and rule out additional infections 

How is Primary Pulmonary Hypertension Treated? 

While there is no exact cure for primary pulmonary hypertension, seeking early diagnosis and treatment is critical to manage symptoms and keep the disease from worsening.  

Possible treatments may include: 

  • Medications, including anticoagulants, diuretics and vasodilator drugs 
  • Oxygen therapy to make breathing easier 
  • Surgical therapies, such as a lung or heart-lung transplant, in severe cases of PPH 

If you exhibit any of the signs of primary pulmonary hypertension, seeking appropriate medical care early is key. At Tampa General Hospital, our lung and pulmonary specialists facilitate effective and personalized treatment of PPH and other pulmonary disorders. We are also a highly ranked lung transplant hospital serving patients with severe lung conditions.