Pulmonary Hypertension: Learn about Symptoms and Diagnosis

Pulmonary Hypertension is high pressure in the Pulmonary artery (lungs arteries).  It is a chronic condition with no cure, but treatment options are increasing. Pulmonary hypertension can be difficult to diagnose due to slow onset of symptoms. There are different types of pulmonary hypertension and have common symptoms are shortness of breath, chest pain and leg swelling.

What are Pulmonary Arteries?

Pulmonary arteries carry blood from right side of the heart to lungs. The normal mean pressure in these arteries is 12-16 mm Hg. The severity of pulmonary hypertension is based on degree of mean Pressure elevation in the pulmonary artery.

• Mild: 25- 40 mm Hg
• Moderate: 41-55 mm Hg
• Severe: More than 55mm Hg

Diagnosis of Pulmonary Hypertension

Initially, diagnosis is suspected based on symptoms of shortness of breath, chest pain, and leg swelling. Several tests are done to confirm the diagnosis and cause of pulmonary hypertension.

• Echocardiogram: it is a simple and non- invasive test. It is an excellent screening test and gives an estimate of Pulmonary artery pressures. This test also evaluates the left side of the heart, leaky valves or damage of the right side of the heart. This test is not 100% accurate so further tests are usually done to confirm the diagnosis. No treatment is started based on echocardiogram alone.
• Right heart Catheterization: In this test tube is inserted via groin, neck, or arm to measure pressures in the right side of the heart and pulmonary artery.
  Tests done to evaluate causes of pulmonary hypertension
• Chest X ray, Pulmonary function test: To rule out COPD
• Sleep Study: To rule out sleep apnea
• CT scan of the lungs: To rule out blood clot in the lungs.
• Blood tests: For evaluation of rheumatic disease like lupus, RA, Scleroderma

Types and Causes of Pulmonary Hypertension

According to WHO, Pulmonary Hypertension has been divided into several types based on similarity in their pathophysiology (causation of disease) and respond to treatment. It is important to accurately classify Pulmonary hypertension to ensure accurate treatment.

Group I (Pulmonary Arterial Hypertension): Idiopathic, Familial, HIV, Scleroderma, drugs, toxins, Congenital heart disease, Sickle cell diseas

Group II (Pulmonary Venous Hypertension): Pulmonary venous hypertension due to left-sided heart disease.

Group III (Hypoxia): COPD, Interstitial lung disease, Sleep apnea

Group IV (Embolic): Chronic thromboembolic disease (Blood clot in the lungs)

Group V (Miscellaneous): Sarcoidosis, Compression of the pulmonary artery by tumor

Learn more about the different types of pulmonary hypertension.

Symptoms of Pulmonary Hypertension

    The symptoms are very slow onset and can be confused with other medical conditions. It usually leads to delayed diagnosis.

•   Gradual onset of Shortness of breath
•     Fatigue
•     Chest pain
•     Dizziness or passing out
•     Leg swelling
•     Raynaud’s phenomenon (Fingers and toes turning blue in cold)

Complications of Pulmonary Hypertension

If left untreated the Pulmonary artery pressures will continue to rise and causing increased pressure on the right side of the heart causing Right ventricle failure.

Treatment of Pulmonary Hypertension

     The treatment will depend on the type of pulmonary Hypertension.  Treatment is often aimed at:

• Lifestyle modifications: Reduce salt intake, increase physical activity, limit alcohol, quit smoking
• Treating the underlying cause like COPD
• Specific Medications to lower Pressure in the lungs (Vasodilator therapy for Pulmonary Hypertension)
• Water Pills to help with symptoms
• Surgery like lung transplant may be needed in specific cases.
• Oxygen may be needed for the majority of the cases.

FAQ for Newly Diagnosed PH Patients

1. Could pulmonary hypertension run in the family?

Yes, pulmonary hypertension can be inherited or passed over from the parents to the children. However, it is relatively uncommon, and only a small percentage of PH gene carriers will develop the disease. You can find out if you carry this gene through genetic testing.

2. What specialization of doctors expertly treat pulmonary hypertension?

Pulmonary hypertension is rare. However, it’s a severe disease, and the expert doctors who handle this medical case are pulmonologists and cardiologists since they’re capable off determining the best treatments for PH or combination of therapies available. In some cases, a rheumatologist also treats patients with pulmonary hypertension. They are familiar with the medications of PH and their side-effects.

3. What is the possible outcome for someone with pulmonary hypertension?

Every patient with pulmonary hypertension is different, so answering this question is not easy. The outlook for patients with pulmonary hypertension depends on different factors, such as the severity of disease and response to treatment. Nevertheless, a wide range of therapies and treatments are available to help improve a patient’s quality of life. With proper treatment, a lot of patients live for many years.

4. How will pulmonary hypertension affect a person’s life?

It isn’t easy to imagine the future following PH diagnosis. But, there are many patients who try to develop positive coping strategies to handle the physical, mental, and emotional aspects of having the disease. Many PH patients live happy, fulfilling lives. You can make minor changes in your daily activities and listen to your body, such as getting rest and adequate sleep when you need to. Ask your doctor about taking supplemental oxygen as a part of a backup plan.

Prognosis

Pulmonary Hypertension is a progressive disease with a significant affect on longevity and quality of life. There are treatments available with proven benefit in improving the quality of life and exercise capacity.

If suspected to have Pulmonary hypertension you may be referred to Pulmonary Hypertension Specialist – Cardiologist or Pulmonologist or Rheumatologist.

Conclusion

Now you are aware of pulmonary hypertension. The basic information provided above will make you more informed so that you can make better health decisions. Seek a doctor’s advice for a more in-depth and tailor-fit patient education about pulmonary hypertension.

Resources:

• https://phassociation.org/
• www.who.org