Dr. Raina describes the manifestations of pulmonary hypertension, CTEPH, and various treatment options available for the often underlooked condition.
The diagnosis of pulmonary hypertension can often be difficult in relation to hypertension, due to nonspecific symptoms and the multitude of risk factors.
In an interview with HCPLive, Amresh Raina, MD, Alleghany Health Network, described the various facets of the diagnosis process. Raina has specialty training in pulmonary hypertension and currently serves as the Director of the Advanced Heart Failure and Pulmonary Hypertension program at his institution.
Raina highlighted the signs and symptoms suggestive of pulmonary hypertension, from shortness of breath, chest tightness, and lightheadeness.
"In patients who have specific risk factors for the development of pulmonary hypertension, they are suggestive," Raina said.
The risk factors of the disease may include family history, exposure to specific drugs and toxins, and underlying disorders, such as autoimmune or connective tissue disorders, Raina noted. He additionally described both non-invasive and invasive procedures to determine if a patient has pulmonary hypertension
Raina went on to discuss chronic thromboembolic pulmonary hypertension, or CTEPH, and the various symptoms stemming from blood clots in the lungs. Other risk factors included cancer, thyroid abnormalities, and even a removed spleen.
"Obviously, the other risk factor is for having a blood clot that was never recognized in the first place," he added. "About 20% of patients who develop CTEPH have actually never had a symptomatic blood clot. It only presents much later with chronic symptoms of shortness of breath or swelling, etc."
Raina noted the treatment options for CTEPH, with a particuarly highlight on pulmonary thromboendarterectomy (PTE) surgery. The invasive surgery is only performed in select centers across the country, of which his institution is one.
"That is the gold standard surgery in people who are operative candidates, and it can be potentially curative, which is why it's important to refer patients to a center that has surgical capabilities and expertise," Raina said.
Medications such as pulmonary vasodilator therapy may additionally play a role in patients who are inoperable or suffer from recurrent CTEPH after these procedures.
When looking at the numbers of patients with pulmonary embolisms, it is more than 500,000 per year, according to Raina. But, the number of PTE surgeries is only 2000 - 3000 a year. The incidence of CTEPH is about 4 - 6% after a pulmonary embolism, which Raina reasons is missing a lot of potential CTEPH.
"I think what I would highlight is that patients should be screened for CTEPH after a pulmonary embolism with an accurate scan, especially if they have an extensive or large pulmonary embolism, or they have risk factors for the development of CTEPH such as an implanted device, a vascular catheter, a splenectomy, etc," Raina concluded.