Chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension
Other namesCTEPH
SpecialtyPulmonology, cardiology
Usual onset63 years (median)[1]
DurationLong term
Risk factorsSplenectomy, inflammatory bowel disease, chronic thyroid hormone replacement, blood types other than O, malignancy, infected ventriculo-atrial shunt, permanent intravenous leads [1][2][3][4][5]
Diagnostic methodInvasively measured mean pulmonary arterial pressure combined with specialist imaging [6]
TreatmentPulmonary endarterectomy, Balloon pulmonary angioplasty, medical treatment [6]
Frequency5 cases per million[7]

Chronic thromboembolic pulmonary hypertension (CTEPH) is a long-term disease caused by a blockage in the blood vessels that deliver blood from the heart to the lungs (the pulmonary arterial tree). These blockages cause increased resistance to flow in the pulmonary arterial tree which in turn leads to rise in pressure in these arteries (pulmonary hypertension). The blockages either result from organised (or hardened) blood clots that usually originate from the deep veins of the lower limbs of the body (thromboembolism) and lodge in the pulmonary arterial tree after passing through the right side of the heart. The blockages may also result from scar tissue that forms at the site where the clot has damaged the endothelial lining of the pulmonary arteries, causing permanent fibrous obstruction (blood flow blockage).[8] Most patients have a combination of microvascular (small vessel) and macrovascular (large vessel) obstruction. Some patients may present with normal or near-normal pulmonary pressures at rest despite symptomatic disease. These patients are labelled as having chronic thromboembolic disease (CTED).[6]

Diagnosis is made after at least three months of effective blood thinning to discern this condition from subacute pulmonary embolism. Diagnostic findings for CTEPH are:[6]

  1. Invasively (i.e., in the blood) measured mean pulmonary arterial pressure (mPAP) ≥25 mmHg;
  2. Mismatched perfusion defects on lung ventilation/perfusion (V/Q) scan and specific diagnostic signs for CTEPH seen by multidetector computed tomography angiography (MDCT), magnetic resonance imaging (MRI) or conventional pulmonary cineangiography (PAG), such as ring-like stenoses, webs/slits, chronic total occlusions (pouch lesions, or tapered lesions) and tortuous lesions.
  1. ^ a b Cite error: The named reference Pepke-Zaba_2011 was invoked but never defined (see the help page).
  2. ^ Cite error: The named reference Bonderman_2005 was invoked but never defined (see the help page).
  3. ^ Cite error: The named reference Bonderman_2009 was invoked but never defined (see the help page).
  4. ^ Cite error: The named reference Bonderman_2009b was invoked but never defined (see the help page).
  5. ^ Cite error: The named reference Lang_2013 was invoked but never defined (see the help page).
  6. ^ a b c d Galiè, Nazzareno; Humbert, Marc; Vachiery, Jean-Luc; Gibbs, Simon; Lang, Irene; Torbicki, Adam; Simonneau, Gérald; Peacock, Andrew; Vonk Noordegraaf, Anton (2016-01-01). "2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)". European Heart Journal. 37 (1): 67–119. doi:10.1093/eurheartj/ehv317. ISSN 1522-9645. PMID 26320113.
  7. ^ Cite error: The named reference Pepke-Zaba_2013 was invoked but never defined (see the help page).
  8. ^ Kim, Nick H. (August 2016). "Group 4 Pulmonary Hypertension: Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Pathophysiology, and Treatment". Cardiology Clinics. 34 (3): 435–441. doi:10.1016/j.ccl.2016.04.011. ISSN 1558-2264. PMID 27443139.