Pulmonary Hypertension – definition, types and symptoms

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What is Pulmonary Hypertension ?

Any reference to the word pulmonary is associated with the lungs, and this serious heart condition, also known as pulmonary arterial hypertension (PAH), is a type of high blood pressure that occurs less frequently than the more well known form of arterial hypertension, and is uniquely associated with the lungs and the right hand side of the heart. It also affects more women than men – you are up to nine times more likely to develop pulmonary hypertension if you are female.

This form of hypertension is characterised by the familiar problem of arterial blockage, or narrowing, similar to the causes of atherosclerosis. However, pulmonary hypertension affects the very small arteries that are found in the lungs, known as the pulmonary arteries. When the heart beats, the right chamber, or ventricle, is the section of the heart that pumps blood through the lungs. If you are suffering from pulmonary hypertension, the arterial blockages cause raised pressure within the pulmonary arteries that in turn causes resistance to the pumping action of the heart muscle, and hence puts a strain on the organ. As a result of this, the blood pressure is raised, and over time this causes excessive loading of the heart that can ultimately damage it, or even cause complete heart failure. 

There is no cure for this heart disease, but there are treatments that can reduce the symptoms, and possibly prevent the worsening of the condition that otherwise could worsen, and could lead to death.

Different types of pulmonary hypertension

Primary pulmonary hypertension (PPH)

This term is applied to the condition of pulmonary hypertension where there is no obvious cause for the elevated blood pressure found in the lungs. It is also known as unexplained pulmonary hypertension, ideopathic pulmonary hypertension (IPH) or even ideopathic pulmonary arterial hypertension (IPAH). There may be a genetic root cause for this heart complaint that can only be confirmed by careful medical investigation and diagnosis. However most cases do not reveal a confirmed hereditary link.  

Secondary pulmonary hypertension

Some cases of pulmonary hypertension may develop from another simultaneously occurring medical condition. This more frequent type of pulmonary hypertension is known as secondary pulmonary hypertension, and it is more commonly encountered than idiopathic pulmonary hypertension. There are numerous factors that that may lead to secondary pulmonary hypertension including :

• Blood clots in the lungs (pulmonary emboli)
• Chronic obstructive pulmonary diseases, such as emphysema
• Connective tissue disorders, such as scleroderma
• Sleep apnea
• Congenital heart disease
• Sickle cell anemia
• Chronic liver disease (cirrhosis)
• AIDS
• Lupus
• Lung diseases such as pulmonary fibrosis, a condition that causes scarring in the tissue between the lungs’ air sacs (interstitium)
• Left-sided heart failure

Symptoms of pulmonary hypertension

This heart condition is notoriously difficult to diagnose. Many of the following symptoms develop slowly, and may be difficult to differentiate from other non-related indications. Ultimately though, without appropriate diagnosis and treatment, these symptoms will worsen until the sufferer is forced to seek medical attention :-

• Chest pain, or feeling of pressure within the chest cavity
• Breathlessnes (dyspnea), either whilst resting or during physical exertion 
• Excessive tiredness or fatigue
• Fainting or feelings of dizziness (syncope)
• Swollen ankles (edema), legs or abdomen (ascites)
• Blue coloration of the the skin and lips (cyanosis)
• Arrythmia – an excessive or irregular heart rate