Pulmonary hypertension (PH) is a condition characterised by high blood pressure in the lungs that leads to significant strain and pressure on the heart. If untreated, the condition can progress to right heart failure and eventual death.
According to the pulmonary hypertension research society, there are currently over 250,000 people living with the condition in the UK. In the US, over 40,000 people are newly diagnosed with the disease each year. While most people will experience symptoms like extreme fatigue and shortness of breath during everyday activities, for some the symptoms can be much more debilitating. Those affected should seek medical help as soon as possible to ensure they receive the best possible treatment for their condition.
What is the Cause of Pulmonary Hypertension?
The primary cause of PH is unknown, however, it has been linked to a variety of factors including:
- Polycystic kidney disease (PKD) – if you have PKD, your risk of developing PH is up to 50%
- Porcupine malformation – if you have this malformation, your risk of developing PH is up to 15%
- Obstructive sleep apnea (OSA) – if you have this condition, your risk of developing PH is up to 10%
- Mesothelioma – this is a type of cancer related to asbestos exposure, and is associated with PH in about 5% of cases
What are the Signs & Symptoms of Pulmonary Hypertension?
The most common sign & symptom of PH is persistent shortness of breath. If you’ve ever had a heart condition, you’ll be familiar with the feeling of shortness of breath as a symptom of the condition. For those with newly diagnosed PH, the shortness of breath can be just as debilitating as the condition itself. The British Journal of Medicine reports that about 60% of patients experience some level of shortness of breath. Those with more severe cases often find themselves unable to leave their beds. The condition also commonly manifests in other ways like swollen feet and ankles, and extreme fatigue.
How is Pulmonary Hypertension Diagnosed?
Your GP will be able to diagnose PH based on your history and a physical examination. They may also wish to conduct a blood test to check for elevated levels of B-type natriuretic peptide (BNP). Elevated levels of BNP are linked to increased risk of PH, and are therefore of prognostic value. In addition to this, your GP may order an echocardiogram to visualise the condition and measure the pressure in the heart. This test involves the use of ultrasound to examine the heart function and structure, and can be useful in detecting mild cases of PH. Echocardiograms are also used to follow up on patients to ensure there’s no relapse of the condition.
Pulmonary Hypertension Treatment: The Initial Step
After diagnosis, your GP will introduce you to the medical community for pulmonary hypertension treatment. In the UK, the pulmonary hypertension research society publishes guidelines for the treatment of the condition. The guidelines recommend:
- The initial step is treatment with a licensed drug called sildenafil (Viagra) – this is because sildenafil is known to reduce the severity of PH-related symptoms like shortness of breath and fatigue
- Tricuspid valve replacement – this is a procedure where the valve connecting the two upper chambers of the heart is replaced, eliminating any obstruction to blood flow. In most cases, this surgery is performed in conjunction with a heart-valve replacement
- Matching siblings – if you have a sibling who’s also been diagnosed with PH, and you want to know your chances of inheriting the condition, a DNA test is available to help determine your risk
What Are the Long-Term Consequences of Pulmonary Hypertension?
While there is no cure for PH, there are treatment options available to ease the condition and give patients with the disease a better quality of life. In the short term, people experience improved quality of life after treatment with sildenafil (Viagra). However, the drug is not without its side effects, and as a result, it’s only recommended for people with mild PH. The guidelines state that people should not take the drug if they experience effects like headaches, upset stomach, and blurred vision. Your GP will be able to advise you on what dosage to take and whether or not these side effects are worth the benefits of the treatment.
Pulmonary Hypertension and Kidney Disease: The Linked Conditions
People with PH are at a significantly increased risk of developing kidney disease. According to the UK pulmonary hypertension research society, about 15-20% of patients will go on to develop some level of kidney disease. This is primarily due to the high blood pressure in the lungs, which results in increased strain and pressure on the kidneys. If you have a history of kidney disease in your family, you’re at particular risk of developing PH. About 50% of people with kidney disease also have PH, and those with end-stage kidney disease are almost certain to develop the condition.
Is Pulmonary Hypertension Life-threatening?
People with PH are at a significantly increased risk of developing heart failure and premature death. According to the UK pulmonary hypertension research society, about half of patients will experience some level of heart failure within three years of diagnosis. People with severe cases of PH may develop right heart failure, wherein the right ventricle of the heart functions inadequately resulting in fatigue, dizziness, and shortness of breath. If this continues and results in decreased output of the left ventricle, the patient may experience fluid congestion and be at risk of sudden cardiovascular collapse. In such cases, treatment with a defibrillator may be necessary to restore normal sinus rhythm and ensure the patient’s survival.
According to the pulmonary hypertension guidelines, early diagnosis and treatment of PH is key to preventing associated complications. These guidelines recommend an annual echocardiogram for patients with the condition to ensure there’s no recurrence of the disease. In addition to this, people with PH should be made aware of the link between their condition and kidney disease, and be screened for signs of the latter if they have the former.