Case-based session will examine expanding options for CTEPH management

Treatment options for the management of chronic thromboembolic pulmonary hypertension (CTEPH) have expanded over the last decade, according to Jean M. Elwing, MD, FCCP, Director of the Pulmonary Hypertension Program at University of Cincinnati College of Medicine.

During Wednesday’s Approach to the CTEPH Patient: Choosing the RIGHT Management Strategy session at 3:30 pm CT, Dr. Elwing and colleagues will present four cases of CTEPH that require utilization of these expanded management strategies.

“Current therapeutic options for CTEPH include our gold standard surgical intervention with pulmonary thromboendarterectomy,” Dr. Elwing said. “But now we can also offer balloon angioplasty for patients with more distal chronic thromboembolic disease or patients with a high-risk medical history that does not allow for surgical intervention.”

For patients who are not candidates for surgery or balloon angioplasty, she said they can be offered medical management.

Riociguat is a soluble guanylate cyclase stimulatory that was approved by the US Food and Drug Administration for the medical treatment of pulmonary arterial hypertension and CTEPH in 2013. The drug is approved for patients who are not surgical candidates or those who have persistent pulmonary hypertension after surgical intervention.

“In the past, if a patient was not a candidate for surgery or was not fully recovered after surgery—in terms of pulmonary hypertension—we had little to offer them that had any scientific basis for use,” Dr. Elwing said. “Now, we are not only able to treat patients with the standard surgical intervention, but we are able to offer them treatment with medication and balloon angioplasty.”

Dr. Elwing will open the case-based session with an overview of inoperable CTEPH. She will also discuss the approval of riociguat and review other medications that have been evaluated but had negative studies for the treatment of CTEPH.

William R. Auger, MD, FCCP, Professor Emeritus at the University of California, San Diego, will present a case illustrating when surgery is the best option for a patient. This will contrast with the presentation by Gustavo A. Heresi, MD, of Cleveland Clinic, who will discuss when catheter-based intervention is the best approach.

Finally, Belinda R. Rivera-Lebron, MD, FCCP, of the University of Pittsburgh School of Medicine, will present a case discussing how to manage patients with CTEPH after surgery and when medical therapy may still be required.

Despite the growing options for management of CTEPH, Dr. Elwing said that the condition remains underdiagnosed.

“CTEPH is not well recognized because of multiple factors,” she said.

Often patients present with nonspecific symptoms such as shortness of breath, fatigue, and chest discomfort, which may delay evaluation. Also, patients who have a history of pulmonary embolism may feel better for several months after a PE, but then start to feel worse again. Because of this initial improvement, it may not be recognized that worsening symptoms could be due to chronic pulmonary emboli and associated pulmonary hypertension.

Another pitfall relates to imaging, Dr. Elwing said.

“Many times, we miss CTEPH on standard CT scan with contrast, and we have to look at other imaging modalities, like ventilation perfusion scanning, to prompt us to examine things further,” she explained.

Finally, CTEPH goes undetected because half of people who develop CTEPH do not have a known history of pulmonary embolism, which may delay the correct workup.

“In these cases, you really have to have a high clinical index of suspicion to reach the right diagnosis,” Dr. Elwing said.

By tuning in for these presentations, Dr. Elwing said she hopes that participants will increase their awareness of CTEPH and the multiple available treatment modalities.

This session is supported in part by an unrestricted educational grant from United Therapeutics Corporation.

This session is supported in part by an educational grant from Actelion Pharmaceuticals US, Inc., a Janssen Pharmaceutical Company of Johnson & Johnson.