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Chronic Thromboembolic Pulmonary Hypertension (CTEPH) - Scan For It!

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Manreet Kanwar, MD
ISHLT Junior Faculty & Trainee Council Vice Chair
Allegheny Health Network
Pittsburgh, Pennsylvania, USA

One of the first patients I was assigned in the Internal Medicine residency clinic as an intern was a 23-year-old woman who had initially presented with worsening shortness of breath. Her work-up revealed multiple bilateral chronic pulmonary emboli and severe pulmonary hypertension. This was my first exposure to a patient with CTEPH—and I remember feeling quite overwhelmed when I realized how sick she was, and how little I knew of how to manage her! Under the guidance of my clinic preceptor, we referred her to the University of Michigan, our local tertiary care center. Through Vallerie McLaughlin's detailed clinic notes, I learned more about this disease than I ever had read about in a medicine book. The patient was then referred to the University of San Diego for pulmonary endarterectomy but unfortunately was felt to be an inoperable candidate. I remember thinking, 'so what else can we offer her', only to realize that we had pretty much run out of options. She developed progressive right ventricular failure in spite of pulmonary vasodilator therapy and died before the age of 25. This incident left me wondering if I would ever get to see this terrible disease being conquered. And ten years later, having witnessed the exciting new developments in the CTEPH management, I can confidently say that we are getting very close! But the key to successfully managing these patients remains in early diagnosis and timely referral to experienced CTEPH centers.

CTEPH is a progressive disease which develops when thromboembolic material obstructs pulmonary artery branches causing a rise in pulmonary arterial pressure. It is quite rare but likely under-diagnosed, with an estimated 2-year incidence of 3.8% after first-time PE. However up to 60% of CTEPH patients have no history of PE. Whereas an abnormal echocardiogram usually triggers further evaluation, VQ scan (and not CTA) remains the preferred test for screening for CTEPH. Current guidelines suggest that patients with acute PE showing signs of PH or RV dysfunction at any time during their hospital stay should receive a follow-up echocardiography after discharge (usually after 3-6 months) to determine whether or not PH has resolved. A hemodynamic assessment with right heart catheterization is critical along with a high quality pulmonary angiogram to confirm the anatomy and location of clot burden. Once confirmed, patients should be assessed for pulmonary endarterectomy operability. There is an ever-increasing role of PAH targeted medical therapy in CTEPH management, particularly in those considered inoperable or with residual PH post operatively (but not as a replacement for PEA). Riociguat is an oral soluble guanylate cyclase stimulator that was recently approved for inoperable CTEPH patients.

The European CTEPH registry data has helped guide the field of CTEPH forward and a US registry is in the process of being established. The forthcoming ISHLT meeting in San Diego (April 2014) and the International CTEPH Association conference in Paris (June 2014) will provide more/ new information in this exciting time of advancements in challenging this disease.

So, whenever I get the opportunity to diagnose and manage a CTEPH patient, I think back 10 years ago and feel more empowered with the tools and knowledge we have to tackle this disease now, instead of feeling overwhelmed by the grim prognosis.

Disclosure Statement: The author has no disclosures to report.

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