Staying Healthy: Understanding Pulmonary Hypertension

Just as hypertension refers to high blood pressure, pulmonary (lung) hypertension is high blood pressure that is unique because it affects the arteries in the lungs and right side (pulmonary circulatory system) side of the heart. Pulmonary hypertension happens due to arteries and vessels becoming damaged. This thickens or scars the inside of the vessel walls making it hard for normal blood flow through the heart. The increased pressure causes the right side of the heart to work less effectively and can lead to heart damage or a heart attack. There is no cure for pulmonary hypertension at this time, however, research has helped to improved treatment options, and with treatment, it may be possible to control some symptoms of pulmonary hypertension and delay the progression of the disease.

Causes of Pulmonary Hypertension

Pulmonary hypertension causes an increase in blood pressure as the heart attempts to send blood from the right ventricle through the pulmonary artery due to scarring or damage within the artery and vessel walls that causes a thickening or stiffness to the pulmonary artery.

In rare cases, the cause of this damage is unknown (idiopathic pulmonary hypertension). Some people may inherit a genetic factor increasing their risk for pulmonary hypertension so for this reason, if anyone in your immediate family has been diagnosed with idiopathic hypertension, you should speak to your doctor about careful screening and monitoring. Most cases of idiopathic hypertension have no identifiable cause.

More common is secondary pulmonary hypertension. This is caused by a number of various factors such as an underlying medical condition, some medications, or trauma of some sort. Some common medical conditions known to increase the risk of secondary pulmonary hypertension include any lung diseases such as COPD, emphysema, or cystic fibrosis. Sickle cell anemia, chronic liver disease, sleep disorders such as sleep apnea, heart failure, heart disease, and AIDS all are documented risk factors. Certain lifestyle choices may also put you at risk for pulmonary hypertension such as hiking or climbing in high altitudes (greater than 8 thousand feet) without proper training or the use of cocaine or other stimulant drugs.

  • Pulmonary hypertension – This PubMed description of pulmonary hypertension includes alternate terms used to describe this condition. Clear Signs & Symptoms of the disease are included along with recommendations for when to call your doctor.
  • Mayo Clinic: Pulmonary Hypertension – The Mayo Clinic provides a full description and explanation of pulmonary hypertension. An explanation of differences between Idiopathic pulmonary hypertension (no known cause) and Secondary pulmonary hypertension (caused by an underlying medical problem) is also found here. Mayo Clinic also provides a helpful checklist to prepare for a doctor’s appointment if you believe you have pulmonary hypertension.
  • MedicineNet: Pulmonary Hypertension – MedicineNet begins explaining pulmonary hypertension by explaining the pulmonary arteries and the normal mechanics of pulmonary (lung) circulation. There are links from this site to assist in finding a cardiologist and forums for patient discussions with others diagnosed with pulmonary hypertension.
  • Getting to Know Your Pulmonary Hypertension – This page asks the question, would a change in diet help? Several dietary changes may help reduce fluid and other symptoms of pulmonary hypertension. Low sodium, attention to particular foods such as green leafy vegetables if you aren’t on an anticoagulant such as Coumadin or Warfarin, or possible nutritional supplements. Such changes should always be done only under direction of your doctor if you have been diagnosed with pulmonary hypertension.
  • Pulmonary Hypertension in Sickle Cell Disease: Study – According to this study, Pulmonary Hypertension is often a complication of Sickle Cell Disease also known as Sickle Cell Anemia.

Signs & Symptoms of Pulmonary Hypertension

As with the more common systemic hypertension, pulmonary hypertension often has no signs or symptoms in the early stages. Since it is a chronic progressive disease, eventually symptoms arise but may initially be misdiagnosed or regarded as part of another medical condition already being treated. 

Common symptoms of pulmonary hypertension may include shortness of breath while attempting to exercise. This may progress to shortness of breath with any exertion such as normal walking from one room to another and may progress to shortness of breath at rest. The poor circulation occurring with pulmonary hypertension often causes other signs and symptoms such dizziness, fainting, fatigue, chest pains and quickened heartbeats, swelling in ankles and abdomen and a bluish tinge (cyanosis) to skin, especially the lips and mouth area. Not everyone will experience the same symptoms, it is important to report any new symptoms to your doctor for evaluation.

  • Updated Clinical Classification of Pulmonary Hypertension – This is an update of classification involving clinical pulmonary hypertension. It looks at the way pulmonary hypertension is involved in other medical conditions and recommends a clinical approach for diagnosis, treatment, and intervention.
  • Prevalence and Risk Factors of Pulmonary Hypertension – According to the Cleveland Medical Clinic statistics, worldwide risk of pulmonary hypertension in women is approximately one and a half times more in women than in men. However, in the United States, women are nearly four times as likely to be diagnosed with pulmonary hypertension. The causes for this increased risk compared to women in other countries are unclear.

Diagnosis Pulmonary Hypertension

Because pulmonary hypertension often has no obvious signs or symptoms in the early stages, it usually will not be found during a routine physical exam. Signs and symptoms of pulmonary hypertension are also seen with several more common lung and heart conditions which may lead to a misdiagnosis. Several tests may be needed to rule out other conditions before a diagnosis of pulmonary hypertension is reached. Pulmonary hypertension is often diagnosed after extensive damage to the lung vessels has occurred.

Some tests your doctor may order to diagnose or rule out pulmonary hypertension include echocardiograms and x-rays to check for the size and function of your heart. A specialized echocardiogram (transesophageal echocardiogram) that uses flexible tubing placed down the throat to the esophagus may be used to obtain a cleared echocardiogram picture. After evaluating the echocardiogram results, a heart catheterization (heart cath) may be ordered. A heart cath is the best way to examine closely the vessels and function in the right ventricles and is the most reliable test used to diagnose pulmonary hypertension.

To obtain a close examination of your lungs and especially the pulmonary artery, other tests focused on the lungs may also be ordered. These tests include pulmonary function tests and a perfusion lung scan. These check air and blood flow within the lungs. A ventilation scan may be done at the same time as the perfusion lung scan is done. Other imagining tests may include a CT scan (computerized tomography), MRI (magnetic resonance imaging) to focus on areas of vessels, and lung involvement. Genetic testing or a lung biopsy may also be ordered based on family history or suspicion of an underlying cause that may have led to secondary pulmonary hypertension.

  • Diagnosis and Treatment of Pulmonary Hypertension – This American Academy of Family Physicians site gives specific instances of pulmonary hypertension diagnosis and the underlying medical conditions that pulmonary hypertension is often associated with. 
  • How serious is Pulmonary Hypertension? – There is no cure for pulmonary hypertension but with early diagnosis and treatment a better prognosis is possible now than in previous years. Every individual is different; prognosis depends in part on the underlying cause of pulmonary hypertension and other medical conditions.
  • Persistent Pulmonary Hypertension of the Newborn (PPHN) – PPHN is a form of pulmonary hypertension in newborns. Though rare, this is a life-threatening condition requiring immediate treatment. Full details about this form of pulmonary hypertension are found at this site along with access to free information if you or someone you know has a newborn with this condition.
  • Pulmonary Hypertension – Research Screening Study – This is the site to find information from the National Institutes of Health (NIH) and their need for people to take part in a research study. Information obtained from the study could help to expand the understanding and treatment of pulmonary hypertension. Visit this site for more information.


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