Surgery is the principal therapy but knowing signs and symptoms will help you with CTPH.

The Pulmonary Circulation
The pulmonary circulation is made up of the blood vessels that carry oxygen-rich blood through the lungs (the pulmonary system). As the blood flows though the lungs, it loads up with oxygen and loses the carbon dioxide it is carrying. Then, the oxygen-rich blood returns to the heart through the left upper chamber (atrium), flows into the left lower chamber (ventricle) and out through the aorta to all cells in the body.

Once the oxygen is lost from the blood, the veins carry it back to the heart from all over the body. It flows into the heart’s right atrium. After the atrium is filled with blood, it squeezes (contracts) and sends the blood into the right ventricle. When the right ventricle fills and contracts, it pushes blood into the pulmonary artery. The pulmonary artery goes into the lungs through the pulmonary circulation.

Chronic is a medical term that describes a disease or condition that continues for a period of months or years.

A Pulmonary Thromboembolism refers to the clots that travel from veins in the body (emboli) and clog the arteries in the lungs. Once lodged in the lung arteries, they can cause more clots to form (thrombosis) and add more resistance to the blood flow through the lung.
This increased resistance from the clots increases the pressure inside the lungs. The medical term for increased pressure is Hypertension. Combining these key words gives us the meaning of chronic thromboembolic pulmonary hypertension: High blood pressure in the pulmonary arteries that lasts six months or longer. The condition often happens after there is a pulmonary embolism.

What causes CTEPH?
When the body cannot resorb a pulmonary embolism (PE), it can trigger CTEPH. However, up to half of all patients with CTPEH do not have a history of PE. In 2008, the World Council on Pulmonary Embolism reported that approximately 2 to 4 percent of people with PE develop CTEPH. It is a relatively rare disease. Only about 5,000 people in the United States are diagnosed with CTEPH each year. Because many patients with CTEPH have no history of PE, or don’t know they have PE, it can be overlooked or not suspected.

Risk factors for PE and CTEPH
Risk factors for pulmonary embolism include:
• Long periods of inactivity. This can include bed rest due to surgery or illness, or even a long trip by car or plane.
• Certain types of surgery, such as joint replacement of the hip or knee
• Older age
• Certain diseases, including peripheral artery disease, heart disease and cancer
• Family or personal history of blood clots
• Smoking
• Obesity
• A history of taking synthetic estrogen in the form of birth control pills or hormone replacement therapy
The risk factors for developing CTEPH include:
• Large PE or more than one episode of PE
• Previous surgical removal of the spleen
• Chronic inflammatory diseases, such as inflammatory bowel disease and osteomyelitis
• History of cancer
• Female sex
• Hypercoagulability disorders

What are the symptoms of CTEPH?
Many people who develop pulmonary hypertension after PE go through a so-called “honeymoon period” when they do not have any symptoms. When symptoms develop, they are vague and non-specific in the early stages of the disease. These include:
• Shortness of breath with exercise
• Chest discomfort
• Fatigue

Symptoms of later-stage disease include fainting and signs of right heart failure, such as fluid retention (edema) and blue-tinged fingers and toes (cyanosis).
Some patients with CTEPH never have any early symptoms. Most times, the disease is discovered in these patients when they develop late-stage symptoms or during an autopsy.

CTEPH is a unique and curable form of PH. Unlike PAH, the principal therapy is surgery. Without surgery, patients with CTEPH have a poor prognosis and succumb to right heart failure. Despite advances in CT, the screening test of choice for CTEPH remains a VQ scan. Even a single segmental perfusion defect in a patient with PH or unexplained dyspnea should alert the clinician to the possibility of chronic thromboembolic disease. Pulmonary endarterectomy remains an effective and relatively safe surgery if performed at an experienced center. Current data do not support medical treatment with PAH-approved therapies either in lieu of or as bridge to pulmonary endarterectomy. Whether a subset of patients with predominantly small-vessel disease can benefit from medical therapy is unknown. The difficulty, however, remains in identifying the subset of patients with significant inoperable, small-vessel disease prior to endarterectomy. Until a more objective, reliable preoperative test emerges to assist with predicting postoperative hemodynamic results, all patients with CTEPH should be considered for surgery.

Chronic Thromboembolic Pulmonary Hypertension: Summary
Nick H. Kim, MD
Faculty and Disclosures

Collected by
Connie Dello Buono
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