What is Pulmonary Hypertension?

We’ve talked about some ways in which lupus can affect the heart: its inflammation can cause pericarditis; a painful inflammation of the lining around the heart, lupus patients are at an increased risk for development of Coronary Artery Disease, dyslipidema and many other heart-related problems.

Pulmonary Hypertension is not to be confused with high blood pressure, the systemic type in which your doctor measures your blood pressure with a cuff around your arm. For the purposes of this discussion, we speak of PULMONARY hypertension, or HIGH BLOOD PRESSURE IN THE LUNGS and treatment for it involves sophisticated treatment by subspecialists- after a through diagnostic workup. The World Health Organization has chosen to classify 5 types of Pulmonary Hypertension into Groups:

Group 1. Pulmonary Arterial Hypertension-the Pulmonary Artery becomes narrowed, so the pressure inside this artery becomes high.

Group 2. Pulmonary Hypertension due to left heart disease. This is often seen when stiff LEFT-SIDED HEART vessels don’t completely relax. A good example might be one which is caused by left ventricular diastolic dysfunction.

Group 3. Pulmonary Hypertension due to lung disease- the diseases here include COPD, mainly empyhsema and chronic bronchitis, as well as any disease that leads to scarring of the lungs, like pulmonary fibrosis and sleep-disordered breathing. In this instance (sleep-disordered breathing) CPAP for Obstructive Sleep Apnea is necessary.

Group 4. Pulmonary Hypertension due to blood clots in the lungs: this is sometimes called Chronic Thromboembolic Pulmonary Hypertension and in it, (blood clots) form within the lungs causing pulmonary hypertension by blocking the flow of blood through the pulmonary arteries., not only raising the blood pressure in the pulmonary artery, but since blood flow is blocked, more clots form.

Group 5. blood and other rare disorders that result in Pulmonary Hypertension-or Chronic Thromboembolic Pulmonary Hypertension. For an unknown reason, blood clots form in the lungs and in addidtion to the formation of clots which create more clots, these clots increase the pressure inside the pulmonary arteries.

The diagnosis of Pulmonary Hyptertension is often one made during a work-up of shortness of breath. Shortness of breath can have multiple causes and unless diagnosed and treated by subspecialists early, the shortness of breath of Pulmonary Hypertension  can have a poor outcome.

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