Pulmonary Hypertension

Pulmonary hypertension is a rare condition of high blood pressure in the blood vessels of the lungs as a result of various disorders. The pressure in the pulmonary artery leading from the right side of heart to the lungs rises above normal levels and imposes a big strain on the right side of the heart. The right side of the heart can become so enlarged and weakened that it eventually fails leading to poor quality of life and death.

Primary pulmonary hypertension (PPH) — which is pulmonary hypertension that isn’t a side effect of (or secondary to) another condition such as emphysema or lupus — strikes only between one and two people per million.

Pulmonary hypertension is classified into five different types:

  • Pulmonary Arterial Hypertension (PAH) — This form affects the blood vessels in the lungs that carry blood from the heart into the lungs where it picks up oxygen. This category is subdivided into two types:
    1. Primary pulmonary hypertension (PPH), which can occur for no discernable reason or is inherited (10% of cases). It is very rare. It most often occurs in young adults and is more than twice as common in women as men.
    2. PAH related to exposure to toxins including diet drugs such as fenphen; street drugs including cocaine and methamphetamine; HIV; collagen vascular diseases including sclerodermalupus and rheumatoid arthritis; chronic liver disease; and congenital heart diseases.
  • Pulmonary Venous Hypertension (PVH) — This form is caused by diseases of the left side of the heart, such as heart failure or mitral valve disease. This can increase pulmonary artery blood pressure but usually doesn’t become severe PAH.
  • Respiratory System — Pulmonary hypertension can be associated with diseases of the respiratory system including interstitial lung disease, emphysema, asthmatic bronchitis, sleep apnea and chronic exposure to high altitude.
  • Chronic Blood Clots — Blood clots in the lung blood vessels.
  • Blood Vessel Disorders — Pulmonary hypertension due to disorders directly affecting the blood vessels in the lungs such as parasites, or inflammation of the blood vessels.

There are no specific signs and symptoms in the early stages of the disease. The symptoms that may occur — such as shortness of breath, fatigue and chest pain — also are common to many other conditions. Other symptoms that may occur include dizziness, swollen ankles and legs, fainting and a bluish cast to lips and skin.

Over time, pulmonary arterial hypertension can damage your heart to the point of danger and result in complications that can interfere with your daily life. Complications from pulmonary hypertension include:

  • Enlarged right ventricle
  • Blood clots

Treatment is determined by whether the PH is arterial, venous, hypoxic, thromboembolic, or miscellaneous. Since pulmonary venous hypertension is synonymous with congestive heart failure, the treatment is to optimize left ventricular function by the use of medications or to repair/replace the mitral valve or aortic valve. Lifestyle changes, digoxin, diuretics, oral anticoagulants, and oxygen therapy are considered conventional therapy for pulmonary hypertension.