What is Pulmonary Hypertension?

You have just been diagnosed with Pulmonary Hypertension (PH) but you don't know what it is exactly and your scared. You doctor has most likely told you that this disease is progressive and there is no cure at the moment. Now what? What was the doctor saying I had again? Check out below for further clarification on what PH is....

What is PH?

Pulmonary hypertension is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and may become life threatening.

What are the symptoms?

Symptoms of pulmonary hypertension include shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells and fainting.

What kinds of PH are out there?

Idiopathic Pulmonary Hypertension (Formally known as Primary Pulmonary Hypertension)
When pulmonary hypertension occurs in the absence of a known cause, it is referred to as idiopathic pulmonary arterial hypertension (IPAH). This term should not be construed to mean that because it has a single name it is a single disease. There are likely many unknown causes of IPAH. IPAH is extremely rare, occurring in about two persons per million population per year.

Secondary Pulmonary Hypertension
Secondary pulmonary hypertension means the cause is known. A common cause of secondary PH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus erythematosus (SLE). Congenital heart diseases that cause shunting of extra blood through the lungs like ventricular and atrial septal defects, chronic pulmonary thromboembolism (old blood clots in the pulmonary artery), HIV infection, liver disease and diet drugs like fenfluramine and dexfenfluramine are also causes of pulmonary hypertension.

Diagnosis and Rates of Survival

Pulmonary hypertension is frequently misdiagnosed and has often progressed to late stage by the time it is accurately diagnosed. Pulmonary hypertension has been historically chronic and incurable with a poor survival rate. However, new treatments are available which have significantly improved prognosis. Recent data indicate that the length of survival is continuing to improve, with some patients able to manage the disorder for 15 to 20 years or longer.

Information provided from PHA (Pulmonary Hypertension Association)