Do you get short of breath when you bend over to tie your shoelaces?

We call this symptom bendopnoea. This can be an important symptom which suggests significant heart disease and pulmonary hypertension

What is pulmonary hypertension?

This is not the typical elevated blood pressure that most people know about. This is a serious condition when the pressure within the lung arteries is increased.

What are the symptoms of pulmonary hypertension?

These are typically shortness of breath, fatigue, chest pain and at later stages lower limb swelling and distension of the belly.
Many patients present late in the course of the disease because the breathlessness is a common symptom and many have initially attributed their symptoms to the lack of fitness, mild asthma or even anxiety.

How is pulmonary hypertension diagnosed?

This is not easy to diagnose. There can however be some clues which suggests this condition such as an abnormal heart sound on physical examination or ECG changes.

The easiest way to evaluate PH is to use a transthoracic echocardiogram ( ultrasound test) to measure the pressures. This is simple, painless, non invasive and can provide clues. If there is a strong suspicion on echocardiogram, a right heart catheterization study would be needed to evaluate this further.

A right heart catheter is a procedure where a thin balloon tipped catheter is placed within the veins of the thigh. This balloon enables the catheter to float within the veins and into the right heart before reaching the lung arteries. ( In fact, the design of this catheter was inspired by the Spinnaker on a sail boat. )A pressure sensor enables the pressures within the lung arteries to be measured accurately. The whole procedure takes about 45 minutes and is guided by fluroscopy (X-ray).

What happens when I am diagnosed with pulmonary hypertension?

You will undergo a series of test to determine the cause of elevated pressures. These could be due to a variety of diseases.

A comprehensive set of tests could include the following:

  1. Tests to assess the lung: CT scans of the lung and spirometry ( to determine the anatomy and physiology of the lung)
  2. Testing for clots within the lung arteries ( CT scan for pulmonary embolism or Ventilation and perfusion Scintigraphy)
  3. Assessment for autoimmune diseases ( usually blood tests are done for these)
  4. Assessment for cardiac disease with cardiac MRI or transesophageal echocardiogram)
  5. Other rarer disease conditions ( liver disease, blood disorders, immune related disorders, genetic abnormalities, drug related causes)

What happens next?

Depending on the cause of the elevated pulmonary pressures, you will receive treatment to manage these. There are also specific medications that are used to treat pulmonary hypertension.

What is the prognosis of pulmonary hypertension

This is a serious condition that needs to be effectively treated. In the past, there were only limited options of treatment which led to persistent symptoms, poor quality of life as well as a reduction of lifespan. The discovery of several new classes of medications have however changed the outcomes significantly.