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Pulmonary Hypertension (PH) is a common but complex and often misunderstood disease. It is different from a simpler Hypertension because, in Systemic Hypertension or increased blood pressure, there is a significant increase in blood pressure in the arteries throughout the body whereas, in Pulmonary Hypertension, blood pressure is elevated in the lungs.

The blood vessels of the lungs are terribly affected in Pulmonary Hypertension as they become stiff and narrow. As a result, the right side of the heart has to work harder to pump the required amount of blood.

This condition gradually worsens and can be fatal. Some common symptoms of Pulmonary Hypertension are:

  • Fatigue
  • Chest Pain
  • Pressure on chest
  • Weight loss
  • Edema (Swelling) in ankles, legs, and ascites (abdomen)
  • Bluish lips and skin (cyanosis)
  • Heart palpitations

There are different forms of Pulmonary Hypertension so it is crucial for acute patients or patients who are newly diagnosed with this complex disease to find a Pulmonary Hypertension specialist who is capable of finding the exact reason for Pulmonary Hypertension. A treatment plan should be developed to cure the specific type of diseases.

Causes of Pulmonary Hypertension

There are several causes of Pulmonary Hypertension and the causes are classified into five groups.

Group 1: Pulmonary Arterial Hypertension

Causes of Pulmonary Arterial Hypertension include:

  • Idiopathic Arterial Hypertension means unknown cause
  • A genetic mutation that is passed by a family member
  • Overuse of prescription diet drugs or illegal drugs such as methamphetamine
  • Congenital heart disease that is present at birth
  • Connective tissue disorder including lupus and scleroderma
  • HIV disease or chronic liver disease

Group 2 – Pulmonary Hypertension caused by Left-Sided Heart Disease

Cause of this complexed form of the disease include:

  • Left-side heart valve disease including a mitral valve or aortic valve
  • Inability or failure of the lower left heart chamber or left ventricle

Group 3 – Lung Disease that results in Pulmonary Hypertension

Causes of this include:

  • Obstructive and recurrent sleep apnea
  • Pulmonary Fibrosis – resulted by scarring in the tissues between air sacs lungs (interstitium)
  • Chronic Obstructive Pulmonary Disease (COPD)
  • Long-term exposure in high altitudes in a patient with a higher risk of pulmonary hypertension

Group 4 – Pulmonary Hypertension caused by Chronic Blood Clots

  • Causes of this disease include the likes of:
  • Clotting disorders
  • Chronic blood clots present in the lung (pulmonary emboli)

Group 5 – Pulmonary Hypertension triggered by any other health conditions

Some unknown factors play their part in triggering Pulmonary Hypertension, some of the common ones are:

  • Kidney Disease
  • Tumours that are pressed against pulmonary arteries
  • Blood Disorders that include polycythemia vera and thrombocythemia
  • Inflammatory diseases including vasculitis and sarcoidosis
  • Metabolic Disorders such as glycogen storage disease

EXERCISE-INDUCED PULMONARY HYPERTENSION

Exercise-induced Pulmonary Hypertension is defined as higher than normal mPAP during exercise. It is associated with decreased exercise strength and capacity and poor prognosis.

Patients who are trying to lose weight with extensive exercise are not at risk of Pulmonary Hypertension but those who have shown symptoms should avoid doing strenuous exercise.

Pulmonary Hypertension is a contributing factor in weight fluctuation. Physical activity is challenging for patients particularly because of shortness of breath and fatigue.

Patients sometimes also go through unusual weight loss that can be because of increased heart rate and decreased lung function. In case of a weight loss, doctors recommend taking more calories than required in a healthy adult.

TREATMENT

Almost three decades ago, there was no cure for Pulmonary Hypertension. Research and studies over the years have made it possible to have 14 Pulmonary Hypertension specific treatments while more are in the developmental phase.

Pulmonary Hypertension specific drugs are now available in the market.

PEOPLE WHO ARE MOST AT RISK

Although Pulmonary Hypertension is a condition that may affect people of all ages and ethnic backgrounds. However, most commonly it is reported in young women of childbearing years. The disease is threatening because it has a history of being incurable with a poor survival rate.

Proper and timely diagnosis can alleviate symptoms and improve the quality of life of Pulmonary Hypertension patients. The solution is hidden in finding a Pulmonary Hypertension Specialist who pursues immediate treatment without any delay.

PULMONARY HYPERTENSION SPECIALISTS

Correct diagnosis on an average takes two years because of the mixed symptoms. The disease progresses exponentially during this time and it becomes more difficult to manage and treat it. Some patients do not survive long enough for getting properly diagnosed and treated.

General Physicians are not able to diagnose Pulmonary Hypertension. Physicians who can diagnose the disease are unaware of the progress of the illness. Some physicians also make diagnoses without using the right heart catheterization that causes further harm to the patient.

A Pulmonary Hypertension Specialist is an expert physician who has got special training in Pulmonary Hypertension at a well-recognized Pulmonary Hypertension Centre. Most commonly, Pulmonary Hypertension Specialists are Pulmonologists or Cardiologists as both diseases are interlinked.

These specialists are committed to treating Pulmonary Hypertension patients. General Physicians or doctors who diagnose Pulmonary Hypertension in any patient should refer them to Pulmonary Hypertension Specialists. It can save the patient and the progression of the disease. Knowing what kind of doctors diagnose and treat pulmonary hypertension is important and is the first step after getting diagnosed with this disease.

DIAGNOSIS OF PULMONARY HYPERTENSION

Whenever a doctor suspects Pulmonary Hypertension, he/she may recommend the following diagnostic tests:

Chest X-Ray – An image of the heart, blood vessels and lungs is taken to see if the heart is enlarged. Lung scarring is evident in advanced stages of Pulmonary Hypertension causing shortness of breath.

Electrocardiography – Commonly known as EKG is carried out to measure the electrical activity of the heart. It helps in figuring any irregular heartbeat.

Right, Heart Catheterization – It is done to measure the blood flow, particularly in the pulmonary arteries and heart. It helps in blood pressure determination.

Echocardiography – It uses sound waves to produce a moving image of the heart. It helps in estimating the pressure in the pulmonary arteries and the ability of the heart in pumping blood.

SEVERITY OF DISEASE

Pulmonary Hypertension is a fatal disease. It can cause serious complications in patients even during the course of treatment. A Pulmonary Hypertension Specialist uses the result of the diagnostic test to rank the severity of the disease. The severity is ranked from class 1 to class 4.

  • Class 1 patients show no visible symptoms and can perform normal activity.
  • Class 2 patients show symptoms during regular physical activity.
  • Class 3 patients experience visible symptoms with slight physical activity.
  • Class 4 patients complain about symptoms while resting and severe symptoms during physical activity.

CONCLUSION

New researches are being conducted on a regular basis to find out the practically possible cure and mode of treatment for Pulmonary Hypertension. Timely medical intervention can make it possible for patients to live with the disease without any complication.