November 18th is the First Ever CTEPH Awareness Day

As part of PAH Awareness Month this year November 18th has been assigned as the official CTEPH Awareness Day. This will be the first ever awareness day focused on CTEPH or chronic thromboembolic pulmonary hypertension.

What Makes CTEPH Different from PAH?

CTEPH|clot removed by PTE

CTEPH is caused by chronic blood clots in the lungs that lead to the increased pressure in the pulmonary arteries and the right side of the heart. It is the only form of PAH that can potentially be cured. The possible cure is a surgical procedure called a pulmonary thromboendarterectomy (PTE) that involves opening the chest and removing large areas of clot. This is an intense surgery and there are only a few centers in the country that perform it. Not every patient with CTPEH will qualify for an endarterectomy.

Medications that are approved for PAH are not necessarily indicated for CTEPH. The first medication to be approved by the FDA with a specific indication to be used in CTEPH was Adempas. This medication has been shown to improve exercise capacity and delay clinical worsening in patients with PAH and CTEPH. No other medications approved for PAH have proven to have these positive outcomes in CTEPH patients.

Why Does CTEPH Need Its Own Day?

Raising awareness for chronic thromboembolic pulmonary hypertension is extremely important because it is a potentially curable disease and there is now a specific medication approved to treat it. Currently CTEPH is much more prevalent than recognized meaning patients are walking around with the disease without the proper diagnosis. The goal of CTEPH Awareness Day is to increase awareness so that every patient with a diagnosis of PH is screened for chronic thromboembolic pulmonary hypertension. There are several tests that can be used to look for chronic clots in the lungs including a VQ scan (ventilation perfusion scan) and pulmonary angiography (usually performed at the time of the initial right heart catheterization).

If the diagnosis of CTEPH is missed a patient with a potentially curable disease may be subjected to expensive PAH therapies that have some difficult side effects without improvement. Like all PAH patients if not properly diagnosed and treated CTEPH will progress. Without treatment the chronic clots will block off blood flow to the lungs leading to heart failure and even death.

 3 Ways to Participate in CTEPH Awareness Day

  1. Learn about CTEPH. Visit our CTEPH page to learn more about the disease.
  2. Share your CTEPH story. Speak to friends, family acquaintances, co-workers, etc. about your personal CTEPH story along with information about screening for the disease and treatments.
  3. Use social media. Visit, like, follow, and share organizations on social media sites that are devoted to raising awareness about CTEPH.

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