What is PPH?

Primary Pulmonary Hypertension, PPH, also known as ‘Unexplained’ Pulmonary Hypertension, is a severe lung and heart disease. It is related to the blood vessels carrying deoxygenated (oxygen deficient) blood from the heart to the lungs which are known as the Pulmonary artery. ‘Hypertension’ means abnormally high blood pressure. A patient suffering from PPH will show high blood pressure in the pulmonary blood vessels. While, Pulmonary artery pressure in a normal person is approximately 14 mmHg (at rest), in a person suffering from PPH the average blood pressure in the pulmonary artery will rise above 25 mmHg (at rest) and above 30 mmHg (during exercise). Generally, blood pressure in the pulmonary artery could rise up to 30% to 50% higher than normal levels. In PPH the blood vessels of the pulmonary artery stiffen, thicken and constrict blood flow and increasing blood pressure. In response to this the blood vessels create more resistance on the flow of blood. This causes the heart to pump blood with even more pressure eventually causing the heart to fail. PPH was first recorded in 1891 but was only recognized in 1951. Even though it is a rare condition, statistics suggest a rise in the number of patients in the last few years especially in young women.

What are the Symptoms of PPH?

Symptoms of PPH include shortness of breath, constant and continuous pain in the chest, fatigue, cough and dizziness. Because these symptoms develop gradually over a long period of time many patients do not seek necessary treatment delaying diagnosis.

What are the Causes for PPH?

There could be several reasons for a rise in blood pressure in the pulmonary artery/vein, such as other lung and heart diseases, tumors etc. But as the name implies, Primary or Unexplained Pulmonary Hypertension occurs for no apparent reason!

However, research carried out by doctors and scientists has uncovered evidence to suggest a clear link between PPH and appetite suppressing diet pills prescribed as a remedy for obesity. Dexfenfluramine, Aminorex fumarate and Fenfluramine are some chemicals which have been identified as possible causes of PPH. These chemicals are the main constituents of diet pills which you might recognize by the names of Redux®, Phen Fen®, and Pondimin®. Even though the side effects of diet pills were identified in Europe, US companies denied these allegations and continued to market them as ‘safe’ weight-loss methods. Therefore, victims can claim compensation from the drug manufacturing companies not only for the expense of treatment, but also for the pain and suffering they go through.

What are the treatments available for PPH?

PPH can be treated using several drugs. However these can be quite expensive. Lung transplants may also be required in some cases. This is very complicated as it is not only very expensive but also because of the difficulty to find a suitable lung transplant donor. Very rarely a heart transplant may also be required. Treatment of PPH is primarily focused on maximizing the lifespan and well being of patients. Thus proper treatment of PPH will grant the patient a longer and happier life.