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CTEPH FACTS

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

WHAT IS CTEPH?

CTEPH is a form of pulmonary hypertension (PH) and is designated as WHO Group 4. Diagnosis of CTEPH is based on the following after 3 months of anticoagulation1-3

  • PVR ≥3 WU or 240 dynseccm-5
  • Mean pulmonary arterial pressure (mPAP) >20 mm Hg
  • Pulmonary artery wedge pressure (PAWP) ≤15 mm Hg
  • The presence of multiple chronic/organized occlusive thrombi/emboli in the elastic pulmonary arteries as indicated by mismatched perfusion defects on lung scan and specific signs of occlusion on imaging

CTEPH=chronic thromboembolic pulmonary hypertension; WHO=World Health Organization; WU=Wood units.

EVALUATING CTEPH PATIENTS FOR TREATMENT

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

Flowchart for CTEPH patient evaluation
Surgical knife

Regular follow-up is recommended, including invasive assessment with
RHC 3-6 months after the intervention to support consideration of a multimodal treatment approach.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.

Beaker icon in red background

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

CTEPH artery icon in red background

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 PH CLASSIFICATION3,9

Identify which WHO group your PH patients are in.

I.PAH

II.PH associated with left heart disease

III.PH associated with lung disease and/or hypoxia

IV.ICTEPH

V.PH with unclear and/or 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.

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CTEPH FACTS

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

WHAT IS CTEPH?

CTEPH is a form of pulmonary hypertension (PH) and is designated as WHO Group 4. Diagnosis of CTEPH is based on the following after 3 months of anticoagulation1-3

  • PVR ≥3 WU or 240 dynseccm-5
  • Mean pulmonary arterial pressure (mPAP) >20 mm Hg
  • Pulmonary artery wedge pressure (PAWP) ≤15 mm Hg
  • The presence of multiple chronic/ organized occlusive thrombi/emboli in the elastic pulmonary arteries as indicated by mismatched perfusion defects on lung scan and specific signs of occlusion on imaging

CTEPH=chronic thromboembolic pulmonary hypertension; WHO=World Health Organization; WU=Wood units.

EVALUATING CTEPH PATIENTS FOR TREATMENT

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

Flowchart for CTEPH patient evaluation
Flowchart for CTEPH patient evaluation
SEE INOPERABLE CTEPH SEE OPERABLE CTEPH
Surgical knife

Regular follow-up is recommended, including invasive assessment with RHC 3-6 months after the intervention to support consideration of a multimodal treatment approach.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.

Beaker icon in red background

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

CTEPH artery icon in red background

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 PH CLASSIFICATION3,9

Identify which WHO group your PH patients are in.

I.PAH

II.PH associated with left heart disease

III.PH associated with lung disease and/or hypoxia

IV.ICTEPH

V.PH with unclear and/or 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.

 
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References:
  1. Adempas Prescribing Information. Whippany, NJ. Bayer Pharmaceuticals Inc., 2021.
  2. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):180913.
  3. Humbert M, Kovacs G, Hoeper M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;00:1-114.
  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.