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The Challenges of Launching a CTEPH Program

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Manreet Kanwar, MD
Allegheny General Hospital
Pittsburgh, PA, USA

Chronic thromboembolic pulmonary hypertension (CTEPH) continues to present a diagnostic and therapeutic challenge for both patients and physicians - with issues ranging from delay in appropriate diagnosis, inadequate use of screening tools and determination of appropriate treatment strategy. Even though CTEPH remains a rare disease, the delivery of its care has been transformed from experts at highly specialized centers to a broad spectrum of providers with varying degrees of expertise, leading to non-uniformity of care. As a result, early access to expert centers and assurances of optimal patient-care have become increasingly relevant concerns.

The University of California at San Diego (UCSD) continues to lead the expertise in care for patients with CTEPH worldwide. At Allegheny General Hospital (AGH, Pittsburgh), we started our advanced pulmonary hypertension (PH) program over a decade ago. As the PH program grew, we realized the gap for access to treatment options for CTEPH patients who could not travel across the country for consideration of surgery for were considered ineligible for curative pulmonary thromboendarterectomy (PTE). As a result, we decided to invest in the mission to become a treatment center for CTEPH, offering a multidisciplinary team approach to it's diagnosis and management. Now that our CTEPH program is three years since inception, the experience of launching a competitive program in a region with multiple PH referral centers has been quite a learning experience!

As our cardiothoracic surgeon, anesthesia and critical care team, radiologists and interventional cardiologists embarked on enhancing their training for PTE surgery, balloon angioplasty and post-operative care, the medical half of the team focused on the diagnostic evaluation and patient screening for surgical candidacy. Patients who were to undergo PTE surgery at AGH were carefully chosen and in the first 2 years, any patient deemed high risk was referred to UCSD for surgery.

In launching this program, our biggest challenge lay in the fact that this is a referral based practice and we continue to see significant delays in time to diagnosis. Although the role of medical therapy in CTEPH is clearly limited to inoperable patients or those with persistent PH post-surgery, community practitioners are increasingly using the drugs as first line treatment and referring patients if there is inadequate response to therapy. Many patients are deemed inoperable without being given the benefit of doubt of being evaluated by a CTEPH center. Another major issue continues to be the relative dependence on CTA (over a V/Q scan) as a screening tool for CTEPH. Although an excellent tool for acute PE, chronic thromboembolic burden may be missed by a radiologist who is not specifically looking for it in a CTA. For the patients who do get a V/Q scan, there is often confusion on what a 'matched defect' or the interpretation of 'low probability for acute PE' means in terms of CTEPH. After having been misled by interpretation of these scans from outside hospitals, it is tempting to try and repeat all the studies within our center - but this has to be balanced by risk of radiation and contrast to patients as well utilization of health care dollars. Coordinating travel and dealing with out of state insurance has been another time consuming factor. And last, but not the least, the challenge of marketing our program in the north east US has proven to be trickier than we thought!

We have had great clinical success with our first 20 PTE patients. This has only been made possible by utilizing a multidisciplinary, team based approach to this complex disease. We hope to continue to contribute to the first US CTEPH registry launched by UCSD last year. This registry has been created with the mission to promote a greater understanding of the prevalence, pathophysiology, evaluation, and treatment of patients with CTEPH through shared information, education, and collaborative investigation among PH centers of excellence throughout the U.S. Wish us luck! ■

Disclosure Statement: The author has no conflicts of interest to disclose.

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