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CTEPH Diagnosis

CTEPH diagnosis is challenging

Difficult to distinguish from other forms of PH1

Common symptoms of CTEPH include progressive dyspnea on exertion, rapid exhaustion, and fatigue. These symptoms and the clinical course of CTEPH can be indistinguishable from other forms of severe pulmonary hypertension (PH).1 If symptoms, signs, and history suggest PH, patients should be urgently referred for further assessments.

 

Can present without history of PE1,2

Patients with CTEPH can present without a history of pulmonary embolism (PE), but estimates vary.1,2 An international prospective registry including 679 patients showed that 75% of patients with CTEPH had a history of acute PE.2

 

Conversely, Lang reported in 2004 that of 142 consecutive CTEPH patients, 90 (63%) had no history of acute PE.3

 

Symptoms can be delayed1

CTEPH can progress quickly, though some patients may experience a “honeymoon period” of months to years between their acute PE and clinical signs of CTEPH1

  • Signs of right-heart failure occur late in the course of the disease1

 

Diagnosis

Diagnosis of CTEPH requires comprehensive evaluation4

  • Echocardiography is used in the initial assessment of suspected PH5

  • Ventilation/perfusion (V/Q) scan is the preferred and recommended screening test for CTEPH4

    • A diagnosis of CTEPH may be confirmed by the presence of a mismatched wedge-shaped perfusion defect5

    • Diagnosis of CTEPH can be supported by characteristic findings during multislice CT angiography, including a mosaic perfusion pattern, dilatation of proximal pulmonary arteries and right heart chambers, and the presence of vascular stenosis or obstruction5

  • Right heart catheterization is used for hemodynamic evaluation to confirm the presence of PH and to provide prognostic information5

  • Referral to specialized centers for testing to define anatomical locations and extent of obstructions and to quantify the degree of PH is essential6

  • The sequence of these tests may vary according to the factors suggestive of PH7

diagnosis algorithm for CTEPH

V/Q is the
recommended test for CTEPH

Watch Raymond L. Benza, MD, of Allegheny Health Network, Pittsburgh, review the roles of ventilation/perfusion scans and computed tomographic pulmonary angiography in CTEPH

References:

1. Hoeper M, Mayer E, Simmoneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113(16):2011-2020. 2. Pepke-Zaba J, Delcroix M, Lang I, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011;124(18):1973-1981. 3. Lang IM. Chronic thromboembolic pulmonary hypertension – not so rare at all. N Engl J Med. 2004;350:2236-2238. 4. Galiè N, Humbert M, Vachiery JL, et al; ESC Scientific Document Group. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67-119. 5. Jenkins D, Mayer E, Screaton N, Madani M. State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management. Eur Respir Rev. 2012;21(123):32-39. 6. Piazza G and Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364:351-360. 7. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Circulation. 2009;119(116):2250-2294.