WHAT IS CTEPH?

CTEPH is a form of pulmonary hypertension (PH) and is designated as WHO Group 4. CTEPH is defined as1-3:

  • Pulmonary vascular resistance (PVR) >300 dyn-sec-cm-5
  • Mean pulmonary arterial pressure (mPAP) >25 mm Hg
  • Pulmonary capillary wedge pressure (PCWP) ≤15 mm Hg
  • In the presence of multiple chronic/organized occlusive thrombi/emboli in the elastic pulmonary arteries (main, lobar, segmental, subsegmental) after at least 3 months of effective anticoagulation

CTEPH=chronic thromboembolic pulmonary hypertension; WHO=World Health Organization

EVALUATING CTEPH PATIENTS FOR TREATMENT

All patients should be evaluated by CTEPH experts for pulmonary thromboendarterectomy (PTE) surgery.4

flow chart
OPERABLE CTEPH
INOPERABLE CTEPH
SEE INOPERABLE CTEPH
SEE OPERABLE CTEPH
PTE
After PTE surgery, patients should be followed in CTEPH centers,
with at least one hemodynamic assessment to be considered at 6–12 months after the procedure.3

Adempas is the first and only FDA-approved therapy to treat adults with inoperable or persistent/recurrent CTEPH. Adempas was predominantly studied in WHO functional class II-III patients.1

V/Q=ventilation/perfusion

bgteaser
bgteaser
pathogenesis
PATHOGENESIS

CTEPH may be caused by either single or recurrent pulmonary emboli that incompletely resolve, resulting in endothelialized residua that obstruct or significantly narrow pulmonary arteries.8

artery

CTEPH results from persistent macrovascular obstruction, often associated with vasoconstrictor response, which leads to a secondary small vessel arteriopathy. Small vessel arteriopathy is characterized by medial hypertrophy, intimal proliferation and thickening, microthrombi formation, and plexiform lesions.8

WHO
WHO

WHO PH CLASSIFICATION9

Identify which WHO group your PH patients are in.

I.PAH

II.PH due to left heart disease

III.PH due to lung disease and/or hypoxia

IV.CTEPH

V.PH with unclear multifactorial mechanism

 

Adempas is approved for adults with PAH (WHO Group 1) and inoperable or persistent/recurrent CTEPH (WHO Group 4) after surgery. Adempas has been studied predominantly in WHO functional class II-III patients.1

PAH=pulmonary arterial hypertension; PH=pulmonary hypertension

Speed Dial Icon

Learn about efficacy and safety.

Find out how Adempas can help here.

MORE IMPORTANT SAFETY INFORMATION LESS IMPORTANT SAFETY INFORMATION
References:
  1. Adempas Prescribing Information. Whippany, NJ. Bayer Pharmaceuticals Inc., 2018.
  2. Ghofrani HA, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369(4):319-329.
  3. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67-119.
  4. Hoeper MM, Barberà JA, Channick RN, et al. Diagnosis, assessment, and treatment of non-pulmonary arterial hypertension pulmonary hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S85-96.
  5. Freed DH, et al. Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg. 2011;141(2):383-387.
  6. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D92–D99.
  7. Condliffe R, Kiely DG, Gibbs SR, et al. Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2008;177(10):1122-1127.
  8. Piazza G, Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364(4):351-360.
  9. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D34–D41

CTEPH FACTS