Chronic Thromboembolic Pulmonary Hypertension (CTEPH)


Epidemiology


Risk/Etiologic Factors


Physiology


Diagnosis

Arterial Blood Gas (ABG) (see Arterial Blood Gas, [[Arterial Blood Gas]])

Electrocardiogram (EKG) (see Electrocardiogram, [[Electrocardiogram]])

Chest X-Ray (see xxxx, [[xxxx]])

Echocardiogram (see Echocardiogram, [[Echocardiogram]])

Swan-Ganz Catheter (see Swan-Ganz Catheter, [[Swan-Ganz Catheter]])

Cardiac Catheterization (see Cardiac Catheterization, [[Cardiac Catheterization]])

Lower Extremity Venous Doppler Ultrasound (see Lower Extremity Venous Doppler Ultrasound, [[Lower Extremity Venous Doppler Ultrasound]])

CT Pulmonary Angiogram (CTPA) (see Computed Tomography Pulmonary Angiogram, [[Computed Tomography Pulmonary Angiogram]])

Gadolinium-Enhanced Magnetic Resonance Pulmonary Angiogram (MRA) (see Magnetic Resonance Pulmonary Angiogram, [[Magnetic Resonance Pulmonary Angiogram]])

Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests, [[Pulmonary Function Tests]])

Exercise Test (see Exercise Test, [[Exercise Test]])

Ventilation/Perfusion (V/Q) Scan (see Ventilation-Perfusion Scan, [[Ventilation-Perfusion Scan]])

Pulmonary Artery Angiogram (see Pulmonary Artery Angiogram, [[Pulmonary Artery Angiogram]])

Pulmonary Artery Angioscopy (see Pulmonary Artery Angioscopy, [[Pulmonary Artery Angioscopy]])

Open Lung biopsy


Clinical Manifestations

General Comments

Cardiovascular Manifestations

Pulmonary Manifestations


Treatment

General Measures

  • Tobacco Smoking Cessation (see Tobacco, [[Tobacco]]): crucial
  • Supplemental Oxygen (see Oxygen, [[Oxygen]]): as required
  • Pulmonary Rehabilitation (see Pulmonary Rehabilitation, [[Pulmonary Rehabilitation]]): as required
  • Vaccinations

Operable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Anticoagulation

  • Indicated

Pulmonary Thrombendartereectomy (PTE)

  • Indications: resting PVR >300, clot in main/lobar/segmental arteries, and no significant coexisting disease
  • Technique
    • Pulmonary Thrombendarterectomy via Median Sternotomy with Cardiopulmonary Bypass (see xxxx, [[xxxx]])
  • Surgical Mortality Rate: 9% (all deaths in NYHA class 4 cases)
  • Clinical Efficacy: progressive improvement occurs for 6-9 mo post-PTE
  • Complications
    • Inability to Come Off CPB: may occur in cases where PVR does not fall after clot removal or where there is concomitant small vessel pulmonary vascular disease
    • Reperfusion Pulmonary Edema
    • Persistent Pulmonary Hypertension Post-PTE: see below

Prostacyclin Analogues

  • Epoprostenol (PGI2, Prostacyclin, Flolan, Veletri) (see Epoprostenol, [[Epoprostenol]]): probably no clinical benefit [MEDLINE]

Riociguat (Adempas) (see Riociguat, [[Riociguat]])

  • Use of Riociguat as a Bridge to PTE Surgery Has Not Been Studied

Persistent Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Post-PTE

Anticoagulation

  • Indicated

Prostacyclin Analogues

  • Indications: preferred agents for severe (functional class IV) persistent pulmonary hypertension in CTEPH post-PTE surgery
  • Epoprostenol (PGI2, Prostacyclin, Flolan, Veletri) (see Epoprostenol, [[Epoprostenol]])
  • Inhaled Iloprost (Ilomedin, Ventavis) (see Iloprost, [[Iloprost]])
  • Treprostinil (Remodulin, Tyvaso) (see Treprostinil, [[Treprostinil]])

Endothelin Receptor Antagonists (see Endothelin Receptor Antagonists, [[Endothelin Receptor Antagonists]])

  • Agents
    • Bosentan (Tracleer) (see Bosentan, [[Bosentan]])
      • BENEFiT Trial of Bosentan in CTEPH (2008) [MEDLINE]: in subset of patients with persistent pulmonary hypertension after PTE surgery, bosentan improved hemodynamics (PVR), but had no effect on exercise capacity

Riociguat (Adempas) (see Riociguat, [[Riociguat]])

  • Indications: preferred agent for persistent pulmonary hypertension in CTEPH post-PTE surgery (particularly in those with functional class II-III disease)
  • CHEST-1 Study of Riociguat in Patients with Inoperable CTEPH/Persistent or Recurrent CTEPH After Pulmonary Thrombendarterectomy (PTE) Surgery (2013) [MEDLINE]
    • Riociguat Improved Exercise Capacity (6-Minute Walk Test)
    • Riociguat Improved Pulmonary Vascular Resistance (PVR)
    • Riociguat Decreased NT Pro-BNP Levels
    • Riociguat Improved WHO Functional Class

Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Anticoagulation

  • Indicated

Prostacyclin Analogues

  • Epoprostenol (PGI2, Prostacyclin, Flolan, Veletri) (see Epoprostenol, [[Epoprostenol]])
  • Inhaled Iloprost (Ilomedin, Ventavis) (see Iloprost, [[Iloprost]])
  • Treprostinil (Remodulin, Tyvaso) (see Treprostinil, [[Treprostinil]])

Endothelin Receptor Antagonists (see Endothelin Receptor Antagonists, [[Endothelin Receptor Antagonists]])

  • Agents
    • Ambrisentan (Letairis) (see Ambrisentan, [[Ambrisentan]])
    • Bosentan (Tracleer) (see Bosentan, [[Bosentan]])
      • BENEFiT Trial of Bosentan in CTEPH (2008) [MEDLINE]: bosentan improved hemodynamics, but had no effect on exercise capacity
      • Systematic Review/Meta-Analysis of Bosentan in CTEPH (2010) [MEDLINE]: bosentan improved hemodynamics and probably exercise capacity
    • Macitentan (Opsumit) (see Macitentan, [[Macitentan]])

Phosphodiesterase Type 5 Inhibitors (see Phosphodiesterase Type 5 Inhibitors, [[Phosphodiesterase Type 5 Inhibitors]])

  • Sildenafil (Viagra, Revatio) (see Sildenafil, [[Sildenafil]])
    • Trial of Sildenafil in CTEPH (2008) [MEDLINE]: sildenafil improved 6-minute walk distance, activity and symptom components of quality of life, cardiac index, PVR, and NT-proBNP
  • Tadalafil (Adcirca, Cialis) (see Tadalafil, [[Tadalafil]])

Riociguat (Adempas) (see Riociguat, [[Riociguat]])

  • Indications: preferred agent for inoperable CTEPH with functional class II-III
  • Pharmacology: riociguat increases the sensitivity of the guanylate cyclase receptor to nitric oxide, a pulmonary vasodilator
  • CHEST-1 Study of Riociguat in Patients with Inoperable CTEPH/Persistent or Recurrent CTEPH After Pulmonary Thrombendarterectomy (PTE) Surgery (2013) [MEDLINE]
    • Riociguat Improved Exercise Capacity (6-Minute Walk Test)
    • Riociguat Improved Pulmonary Vascular Resistance (PVR)
    • Riociguat Decreased NT Pro-BNP Levels
    • Riociguat Improved WHO Functional Class

Balloon Pulmonary Angioplasty

  • Indications
    • Non-Surgical CTEPH
  • Clinical Efficacy: improves pulmonary blood flow distribution, RV afterload, and 6-minute walk distances

References

Treatment