Pulmonary Hypertension and Hypoxia

Pulmonary hypertension (PH) is a rare but life-threatening disease that affects the pulmonary arteries, making them narrow and thick. The vessels, which are responsible for transporting blood from the right heart ventricle to the lungs, become obstructed which leads to high blood pressure in the lungs. In the long-term, pulmonary hypertension may result in enlargement of the heart, weakening of the heart, right heart failure, or death.

Shortness of breath, fatigue, dizziness or fainting spells, pressure or pain in the chest, swelling (edema) in the ankles, legs and abdomen (ascites), bluish color in the lips and skin (cyanosis), and irregular heartbeat are among the most common symptoms. Though there is no cure for the disease, there are treatments that can help ease the symptoms and increase life expectancy. The disease can result in a series of minor or severe complications that affect overall health.

Development of Hypoxia-Related Pulmonary Hypertension

In some cases, pulmonary hypertension is a idiopathic disease, which means the causes are not fully understood. In other cases, it occurs as result of other medical conditions. In both cases it can cause numerous complications. Hypoxia is a condition that occurs when body tissues do not receive enough oxygen to properly function. Because of the lack of oxygen, hypoxia is a life-threatening condition which can result in damage to the heart, lungs, brain, liver, and other organs.

Characteristics of Pulmonary Hypertension and Hypoxia

Hypoxia is a fast occurrence that provokes severe consequences in just minutes. Symptoms characteristic of hypoxia include skin color changes, confusion, coughing, fast heart rate, fast breathing, shortness of breath, sweating and wheezing. There are similarities between the symptoms experienced by patients with pulmonary hypertension and by those who have hypoxia. In the case of hypoxia-related pulmonary hypertension, the symptoms are exacerbated.

Classification of Pulmonary Hypertension and Hypoxia

The study “Hypoxia- and non-hypoxia-related pulmonary hypertension — Established and new therapies” focused on the development and treatment of hypoxia-related pulmonary hypertension. The study also explained that pulmonary hypertension associated with hypoxia is included in a different classification by the World Health Organization. It is in the same class as PH related to chronic obstructive pulmonary disease (COPD), interstitial lung diseases, sleep disorder breathing, chronic exposure to high altitude, and some rare neonatal diseases.

Treatment for Hypoxia-Related Pulmonary Hypertension

According to the same study, based on the guidelines issued by the American College of Chest Physicians (ACCP) and by the European Society of Cardiology (ESC), the treatment for pulmonary hypertension focuses on pulmonary vasoconstriction, vascular obstruction by recurrent embolism and/or local thrombosis, and vascular remodelling.

In order to best treat patients who have pulmonary hypertension with hypoxia, physicians need to know that both diseases are present.

Patients may benefit from treatment with antiproliferative substances or vasodilator therapies that decrease pulmonary vascular resistance without worsening gas exchange through the induction of ventilation and perfusion mismatch.

A Conversation With Rare Disease Advocates