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| {{Infobox medical condition (new)
| | ==Chronic Thromboembolic Pulmonary Hypertension== |
| |name=Chronic thromboembolic pulmonary hypertension
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| |image=|alt=|caption=|synonyms=CTEPH
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| |field=[[Pulmonology]], [[cardiology]]
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| |onset=63 years (median)<ref name="Pepke-Zaba_2011" />
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| |duration= Long term
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| |types=
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| |causes=
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| |risks= [[Splenectomy]], [[inflammatory bowel disease]], chronic thyroid hormone replacement, [[blood types]] other than O, [[malignancy]], infected ventriculo-atrial shunt, permanent intravenous leads <ref name="Pepke-Zaba_2011" /><ref name="Bonderman_2005" /><ref name="Bonderman_2009" /><ref name="Bonderman_2009b" /><ref name="Lang_2013" />
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| |diagnosis=Invasively measured mean pulmonary arterial pressure combined with specialist imaging <ref name=":Galie_2015" />
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| |differential=
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| |prevention=
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| |treatment = [[Pulmonary thromboendarterectomy|Pulmonary endarterectomy]], [[Balloon pulmonary angioplasty]], medical treatment <ref name=":Galie_2015" />
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| |medication =
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| |prognosis =
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| |frequency = 5 cases per million<ref name="Pepke-Zaba_2013" />
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| |deaths=
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| }}
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| '''Chronic thromboembolic pulmonary hypertension''' ('''CTEPH''') is a long-term disease caused by a [[Vascular occlusion|blockage]] in the blood vessels that deliver blood from the heart to the lungs ([[lung arteries|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 ([[Thrombosis|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 [[endothelium|endothelial lining]] of the pulmonary arteries, causing permanent fibrous obstruction (blood flow blockage).<ref name=":0">{{Cite journal|last=Kim|first=Nick H.|date=August 2016|title=Group 4 Pulmonary Hypertension: Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Pathophysiology, and Treatment|journal=Cardiology Clinics|volume=34|issue=3|pages=435–441|doi=10.1016/j.ccl.2016.04.011|issn=1558-2264|pmid=27443139}}</ref> 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|chronic thromboembolic disease (CTED)]].<ref name=":Galie_2015">{{Cite journal|last=Galiè|first=Nazzareno|last2=Humbert|first2=Marc|last3=Vachiery|first3=Jean-Luc|last4=Gibbs|first4=Simon|last5=Lang|first5=Irene|last6=Torbicki|first6=Adam|last7=Simonneau|first7=Gérald|last8=Peacock|first8=Andrew|last9=Vonk Noordegraaf|first9=Anton|date=2016-01-01|title=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)|journal=European Heart Journal|volume=37|issue=1|pages=67–119|doi=10.1093/eurheartj/ehv317|issn=1522-9645|pmid=26320113|doi-access=free}}</ref>
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| Diagnosis is based on findings obtained after at least 3 months of effective [[Anticoagulant|anticoagulation therapy (blood thinners)]] in order to discriminate this condition from ‘subacute’ pulmonary embolism (blood clot in the lungs, PE). Diagnostic findings for CTEPH are:<ref name=":Galie_2015" />
| | '''Chronic Thromboembolic Pulmonary Hypertension''' (CTEPH) is a form of [[pulmonary hypertension]] caused by chronic obstruction of the [[pulmonary arteries]] due to organized [[blood clots]]. This condition is a serious complication that can arise after an episode of [[pulmonary embolism]], where blood clots travel to the lungs and block the pulmonary arteries. |
| # Invasively (i.e., in the blood) measured mean pulmonary arterial pressure (mPAP) ≥25 mmHg;
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| # 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|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.
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| == Signs and symptoms == | | ==Pathophysiology== |
| Clinical symptoms and signs are often non-specific or absent in early CTEPH, with signs of [[Heart failure#Right-sided failure|right heart failure]] only in advanced disease. The main symptom of CTEPH is exertional breathlessness (shortness of breath during exertion such as exercise), which is unspecific and may often be attributed to other, more common, diseases by physicians. When present, the clinical symptoms of CTEPH may resemble those of acute PE, or of [[Pulmonary hypertension#Causes|idiopathic pulmonary arterial hypertension (iPAH)]]. Leg [[Edema|oedema]] (swelling) and [[Hemoptysis|haemoptysis]] (blood in mucus) occur more often in CTEPH, while [[Syncope (medicine)|syncope]] (fainting) is more common in iPAH.<ref name="Lang_2013" />
| | CTEPH occurs when blood clots that have traveled to the lungs do not dissolve completely and instead become organized into fibrotic tissue. This leads to persistent obstruction of the pulmonary arteries, resulting in increased [[pulmonary vascular resistance]] and elevated [[pulmonary artery pressure]]. Over time, this increased pressure can lead to [[right ventricular hypertrophy]] and eventually [[right heart failure]]. |
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| == Pathogenesis == | | ==Symptoms== |
| People with CTEPH lack traditional thrombosis risk factors.<ref name="Pepke-Zaba_2011">{{Cite journal|last=Pepke-Zaba|first=Joanna|last2=Delcroix|first2=Marion|last3=Lang|first3=Irene|last4=Mayer|first4=Eckhard|last5=Jansa|first5=Pavel|last6=Ambroz|first6=David|last7=Treacy|first7=Carmen|last8=D'Armini|first8=Andrea M.|last9=Morsolini|first9=Marco|date=2011-11-01|title=Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry|journal=Circulation|volume=124|issue=18|pages=1973–1981|doi=10.1161/CIRCULATIONAHA.110.015008|issn=1524-4539|pmid=21969018|doi-access=free}}</ref><ref name="Bonderman_2005">{{Cite journal|last=Bonderman|first=Diana|last2=Jakowitsch|first2=Johannes|last3=Adlbrecht|first3=Christopher|last4=Schemper|first4=Michael|last5=Kyrle|first5=Paul A.|last6=Schönauer|first6=Verena|last7=Exner|first7=Markus|last8=Klepetko|first8=Walter|last9=Kneussl|first9=Meinhard P.|date=March 2005|title=Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension|journal=Thrombosis and Haemostasis|volume=93|issue=3|pages=512–516|doi=10.1160/TH04-10-0657|issn=0340-6245|pmid=15735803}}</ref><ref name="Bonderman_2009">{{Cite journal|last=Bonderman|first=Diana|last2=Skoro-Sajer|first2=Nika|last3=Jakowitsch|first3=Johannes|last4=Adlbrecht|first4=Christopher|last5=Dunkler|first5=Daniela|last6=Taghavi|first6=Sharokh|last7=Klepetko|first7=Walter|last8=Kneussl|first8=Meinhard|last9=Lang|first9=Irene M.|date=2007-04-24|title=Predictors of outcome in chronic thromboembolic pulmonary hypertension|journal=Circulation|volume=115|issue=16|pages=2153–2158|doi=10.1161/CIRCULATIONAHA.106.661041|issn=1524-4539|pmid=17420352|doi-access=free}}</ref><ref name="Bonderman_2009b">{{Cite journal|last=Bonderman|first=D.|last2=Wilkens|first2=H.|last3=Wakounig|first3=S.|last4=Schäfers|first4=H.-J.|last5=Jansa|first5=P.|last6=Lindner|first6=J.|last7=Simkova|first7=I.|last8=Martischnig|first8=A. M.|last9=Dudczak|first9=J.|date=February 2009|title=Risk factors for chronic thromboembolic pulmonary hypertension|journal=The European Respiratory Journal|volume=33|issue=2|pages=325–331|doi=10.1183/09031936.00087608|issn=1399-3003|pmid=18799507|doi-access=free}}</ref><ref name="Lang_2013">{{Cite journal|last=Lang|first=Irene M.|last2=Simonneau|first2=Gérald|last3=Pepke-Zaba|first3=Joanna W.|last4=Mayer|first4=Eckhard|last5=Ambrož|first5=David|last6=Blanco|first6=Isabel|last7=Torbicki|first7=Adam|last8=Mellemkjaer|first8=Sören|last9=Yaici|first9=Azzedine|date=July 2013|title=Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study|journal=Thrombosis and Haemostasis|volume=110|issue=1|pages=83–91|doi=10.1160/TH13-02-0097|issn=0340-6245|pmid=23677493}}</ref> Current understanding is that CTEPH is a result of “inflammatory thrombosis”:<ref name="Lang_2013" /> When pro-thrombotic (blood-clot forming) conditions combine with chronic inflammation and infection, non-resolution of thrombus may ensue. Risk factors for CTEPH include [[splenectomy]], [[inflammatory bowel disease]], chronic thyroid hormone replacement, [[blood types]] other than 0, [[malignancy]], infected ventriculo-atrial shunt and permanent intravenous leads.<ref name="Bonderman_2009b" /><ref name="Lang_2013" />
| | The symptoms of CTEPH are often similar to those of other forms of pulmonary hypertension and can include: |
| | * [[Dyspnea]] (shortness of breath), especially during exertion |
| | * [[Fatigue]] |
| | * [[Chest pain]] |
| | * [[Syncope]] (fainting) |
| | * [[Edema]] (swelling) in the legs and ankles |
| | * [[Cyanosis]] (bluish discoloration of the skin) |
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| == Diagnosis == | | ==Diagnosis== |
| [[File:CTEPH pulmonary artery angiogram (PA angiogram).tif|thumb|Example of a side-selective pulmonary artery angiogram (A and B right pulmonary artery, C and D left pulmonary artery) in digital subtraction technique of a patient with CTEPH. Panels A and C represent anterior-posterior projections, panels B and D represent lateral projections. ]]
| | The diagnosis of CTEPH involves a combination of clinical evaluation, imaging studies, and hemodynamic measurements. Key diagnostic tools include: |
| Early diagnosis still remains a challenge in CTEPH, with a median time of 14 months between symptom onset and diagnosis in expert centres.<ref name="Pepke-Zaba_2013" /> A suspicion of PH is often raised by [[echocardiography]], but an invasive [[Cardiac catheterization|right heart catheterisation]] is required to confirm it.<ref name=":Galie_2015" /> Once PH is diagnosed, the presence of thromboembolic disease requires imaging. The recommended diagnostic algorithm stresses the importance of initial investigation using an echocardiogram and [[Ventilation/perfusion scan|V/Q scan]] and confirmation with right heart catheter and [[Pulmonary angiography|pulmonary angiography (PA)]].<ref name=":0" />
| | * [[Ventilation/perfusion scan]] (V/Q scan) to detect mismatched perfusion defects |
| | * [[Computed tomography pulmonary angiography]] (CTPA) to visualize the pulmonary arteries |
| | * [[Right heart catheterization]] to measure pulmonary artery pressures |
| | * [[Pulmonary angiography]] to confirm the presence of organized thrombi |
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| Both V/Q scanning and modern multidetector [[CT pulmonary angiogram|CT angiography (CTPA)]] may be accurate methods for the detection of CTEPH, with excellent diagnostic efficacy in expert hands (sensitivity, specificity, and accuracy of 100%, 93.7%, and 96.5% for V/Q and 96.1%, 95.2%, and 95.6% for CTPA).<ref>{{Cite journal|last=He|first=Jia|last2=Fang|first2=Wei|last3=Lv|first3=Bin|last4=He|first4=Jian-Guo|last5=Xiong|first5=Chang-Ming|last6=Liu|first6=Zhi-Hong|last7=He|first7=Zuo-Xiang|date=May 2012|title=Diagnosis of chronic thromboembolic pulmonary hypertension: comparison of ventilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography|journal=Nuclear Medicine Communications|volume=33|issue=5|pages=459–463|doi=10.1097/MNM.0b013e32835085d9|issn=1473-5628|pmid=22262242}}</ref> However, CTPA alone cannot exclude the disease, but may help identify pulmonary artery distension resulting in left main coronary artery compression, pulmonary parenchymal lesions (e.g. as complications from previous pulmonary infarctions), and bleeding from bronchial collateral arteries.<ref>{{Cite journal|last=Tunariu|first=Nina|last2=Gibbs|first2=Simon J. R.|last3=Win|first3=Zarni|last4=Gin-Sing|first4=Wendy|last5=Graham|first5=Alison|last6=Gishen|first6=Philip|last7=Al-Nahhas|first7=Adil|date=May 2007|title=Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension|journal=Journal of Nuclear Medicine|volume=48|issue=5|pages=680–684|doi=10.2967/jnumed.106.039438|issn=0161-5505|pmid=17475953|doi-access=free}}</ref> Today, the gold standard imaging remains invasive pulmonary angiography (PAG) using native angiograms or a [[Digital subtraction angiography|digital subtraction technique]].{{citation needed|date=December 2018}}
| | ==Treatment== |
| | The primary treatment for CTEPH is [[pulmonary endarterectomy]] (PEA), a surgical procedure that removes the organized clots from the pulmonary arteries. This surgery can significantly improve symptoms and hemodynamics in eligible patients. For patients who are not candidates for surgery, medical therapies such as [[riociguat]] or [[balloon pulmonary angioplasty]] may be considered. |
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| == Treatment == | | ==Prognosis== |
| Decision making for patients with CTEPH can be complex and needs to be managed by CTEPH teams in expert centres. CTEPH teams comprise cardiologists and pulmonologists with specialist PH training, radiologists, experienced PEA surgeons with a significant caseload of CTEPH patients per year and physicians with percutaneous interventional expertise. Currently, there are three recognised targeted treatment options available including the standard treatment of [[pulmonary thromboendarterectomy|pulmonary endarterectomy (PEA)]]. [[Balloon pulmonary angioplasty]] (BPA) and pulmonary vasodilator drug treatment may be considered for those people that are not suitable for surgery.<ref name=Brenot2017>{{Cite journal|last=Brenot|first=Philippe|last2=Mayer|first2=Eckhard|last3=Ghofrani|first3=Hossein-Ardeschir|last4=Kurzyna|first4=Marcin|last5=Matsubara|first5=Hiromi|last6=Ogo|first6=Takeshi|last7=Meyer|first7=Bernhard C.|last8=Lang|first8=Irene|date=31 March 2017|title=Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension|journal=[[European Respiratory Review]]|language=en|volume=26|issue=143|pages=160119|doi=10.1183/16000617.0119-2016|issn=1600-0617|pmid=28356406|doi-access=free}}</ref>
| | The prognosis for patients with CTEPH varies depending on the severity of the disease and the success of treatment. Surgical intervention with PEA can be curative in many cases, leading to significant improvements in quality of life and survival. However, untreated CTEPH can lead to progressive right heart failure and increased mortality. |
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| Specialist imaging using either magnetic resonance or invasive PA is necessary to determine risks and benefits of interventional treatment with PEA or BPA.<ref name=":0" />
| | ==Related Pages== |
| | * [[Pulmonary hypertension]] |
| | * [[Pulmonary embolism]] |
| | * [[Right heart failure]] |
| | * [[Pulmonary endarterectomy]] |
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| === Medical therapy ===
| | [[Category:Pulmonary diseases]] |
| Standard medical treatment consists of [[Anticoagulant|anticoagulants (blood thinners)]], [[diuretic]]s, and [[Oxygen therapy|oxygen]]. Lifelong anticoagulation is recommended, even after PEA. Routine inferior vena cava filter placement is not recommended.<ref name=":Galie_2015" />
| | [[Category:Cardiovascular diseases]] |
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| In patients with non-operable CTEPH or persistent/recurrent PH after PEA, there is evidence for benefit from pulmonary vasodilator drug treatment.<ref name=":7" /> The microvascular disease component in CTEPH has provided the rationale for off-label use of drugs approved for PAH.<ref name="Pepke-Zaba_2011" /> Currently, only [[riociguat]] (a stimulator of soluble guanylate cyclase) is approved for treatment of adults with inoperable CTEPH or persistent or recurrent CTEPH after surgical treatment.<ref>{{Cite journal|last=Ghofrani|first=Hossein-Ardeschir|last2=D'Armini|first2=Andrea M.|last3=Grimminger|first3=Friedrich|last4=Hoeper|first4=Marius M.|last5=Jansa|first5=Pavel|last6=Kim|first6=Nick H.|last7=Mayer|first7=Eckhard|last8=Simonneau|first8=Gerald|last9=Wilkins|first9=Martin R.|date=2013-07-25|title=Riociguat for the treatment of chronic thromboembolic pulmonary hypertension|journal=The New England Journal of Medicine|volume=369|issue=4|pages=319–329|doi=10.1056/NEJMoa1209657|issn=1533-4406|pmid=23883377|hdl=10044/1/19669|url=http://spiral.imperial.ac.uk/bitstream/10044/1/19669/2/New%20England%20Journal%20of%20Medicine_369_4_2013.pdf}}</ref> Other drug trials are ongoing in patients with inoperable CTEPH, with macitentan recently proving efficacy and safety in MERIT<ref>{{Cite web|url=https://clinicaltrials.gov/ct2/show/NCT02021292|title=Clinical Study to Assess the Efficacy, Safety and Tolerability of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension - Full Text View - ClinicalTrials.gov|website=clinicaltrials.gov|language=en|access-date=2017-07-31}}</ref>
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| === Pulmonary endarterectomy ===
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| [[File:PEA_specimen.tif|thumb|Typical specimen harvested during the surgical procedure of [[pulmonary thromboendarterectomy|pulmonary endarterectomy]] in a patient with chronic thromboembolic pulmonary hypertension.]]
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| [[Pulmonary thromboendarterectomy|Pulmonary endarterectomy (PEA)]] is the gold standard treatment for suitable CTEPH patients. Operability of patients with CTEPH is determined by multiple factors among which surgical accessibility of thrombi and the patient-determined risk-benefit ratio are most important. There is no haemodynamic (e.g., considering pulmonary pressure) or age threshold that should exclude patients from surgery, and concomitant cardiac procedures can be included if necessary. However, only 60% of patients are classified as operable across Europe and Canada.<ref name=":4" />
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| In contrast to surgical embolectomy for acute PE, treatment of CTEPH necessitates a true bilateral endarterectomy (removal of blockage from the blood vessels) through the medial layer of the pulmonary arteries, which is performed under deep [[hypothermia]] (lowering of body temperature) and circulatory arrest (temporary stoppage of blood flow),<ref name=":5">{{Cite journal|last=Madani|first=Michael M.|last2=Auger|first2=William R.|last3=Pretorius|first3=Victor|last4=Sakakibara|first4=Naohide|last5=Kerr|first5=Kim M.|last6=Kim|first6=Nick H.|last7=Fedullo|first7=Peter F.|last8=Jamieson|first8=Stuart W.|date=July 2012|title=Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2,700 patients|journal=The Annals of Thoracic Surgery|volume=94|issue=1|pages=97–103|doi=10.1016/j.athoracsur.2012.04.004|issn=1552-6259|pmid=22626752}}</ref> which is not complicated by cognitive dysfunction.<ref>{{Cite journal|last=Vuylsteke|first=Alain|last2=Sharples|first2=Linda|last3=Charman|first3=Gill|last4=Kneeshaw|first4=John|last5=Tsui|first5=Steven|last6=Dunning|first6=John|last7=Wheaton|first7=Ella|last8=Klein|first8=Andrew|last9=Arrowsmith|first9=Joseph|date=2011-10-15|title=Circulatory arrest versus cerebral perfusion during pulmonary endarterectomy surgery (PEACOG): a randomised controlled trial|journal=Lancet|volume=378|issue=9800|pages=1379–1387|doi=10.1016/S0140-6736(11)61144-6|issn=1474-547X|pmid=22000135}}</ref> The majority of patients experience substantial relief from symptoms and improvement in haemodynamics after PEA.<ref name=":5" /><ref name=":6">{{Cite journal|last=Mayer|first=Eckhard|last2=Jenkins|first2=David|last3=Lindner|first3=Jaroslav|last4=D'Armini|first4=Andrea|last5=Kloek|first5=Jaap|last6=Meyns|first6=Bart|last7=Ilkjaer|first7=Lars Bo|last8=Klepetko|first8=Walter|last9=Delcroix|first9=Marion|date=March 2011|title=Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry|journal=The Journal of Thoracic and Cardiovascular Surgery|volume=141|issue=3|pages=702–710|doi=10.1016/j.jtcvs.2010.11.024|issn=1097-685X|pmid=21335128|doi-access=free}}</ref><ref>{{Cite journal|last=Jenkins|first=D.|last2=Mayer|first2=E.|last3=Screaton|first3=N.|last4=Madani|first4=M.|date=2012-03-01|title=State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management|journal=European Respiratory Review|volume=21|issue=123|pages=32–39|doi=10.1183/09059180.00009211|issn=1600-0617|pmid=22379172|doi-access=free}}</ref> In Europe, in-hospital mortality during PEA is currently 4.7% or lower in high volume single centres.<ref name=":5" /><ref name=":6" />
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| Up to 35% of patients may have persistent/recurrent CTEPH following surgery.<ref name=":7">{{Cite journal|last=Hoeper|first=Marius M.|date=June 2015|title=Pharmacological therapy for patients with chronic thromboembolic pulmonary hypertension|journal=European Respiratory Review|volume=24|issue=136|pages=272–282|doi=10.1183/16000617.00001015|issn=1600-0617|pmid=26028639|doi-access=free}}</ref> The definition of post-PEA PH is still not clear, but some data suggest that 500–590 dynes·s·cm<sup>−5</sup> may represent a [[Vascular resistance|pulmonary vascular resistance (PVR)]] threshold for poor long-term outcome.<ref name=":8">{{Cite journal|last=Skoro-Sajer|first=Nika|last2=Marta|first2=Gabriel|last3=Gerges|first3=Christian|last4=Hlavin|first4=Gerald|last5=Nierlich|first5=Patrick|last6=Taghavi|first6=Shahrokh|last7=Sadushi-Kolici|first7=Roela|last8=Klepetko|first8=Walter|last9=Lang|first9=Irene Marthe|date=February 2014|title=Surgical specimens, haemodynamics and long-term outcomes after pulmonary endarterectomy|journal=Thorax|volume=69|issue=2|pages=116–122|doi=10.1136/thoraxjnl-2013-203746|issn=1468-3296|pmc=3913220|pmid=24052543}}</ref><ref>{{Cite journal|last=Jamieson|first=Stuart W.|last2=Kapelanski|first2=David P.|last3=Sakakibara|first3=Naohide|last4=Manecke|first4=Gerard R.|last5=Thistlethwaite|first5=Patricia A.|last6=Kerr|first6=Kim M.|last7=Channick|first7=Richard N.|last8=Fedullo|first8=Peter F.|last9=Auger|first9=William R.|date=November 2003|title=Pulmonary endarterectomy: experience and lessons learned in 1,500 cases|journal=The Annals of Thoracic Surgery|volume=76|issue=5|pages=1457–1462|issn=0003-4975|pmid=14602267|doi=10.1016/S0003-4975(03)00828-2}}</ref> Recent data from National UK PEA cohort suggests residual PH post PEA only impacts on longer term survival when mPAP is >38 mmHg or PVR >425 dynes·s·cm<sup>−5</sup>.<ref>{{Cite journal|last=Cannon|first=John E.|last2=Su|first2=Li|last3=Kiely|first3=David G.|last4=Page|first4=Kathleen|last5=Toshner|first5=Mark|last6=Swietlik|first6=Emilia|last7=Treacy|first7=Carmen|last8=Ponnaberanam|first8=Anie|last9=Condliffe|first9=Robin|date=2016-05-03|title=Dynamic Risk Stratification of Patient Long-Term Outcome After Pulmonary Endarterectomy: Results From the United Kingdom National Cohort|journal=Circulation|volume=133|issue=18|pages=1761–1771|doi=10.1161/CIRCULATIONAHA.115.019470|issn=1524-4539|pmid=27052413|pmc=5860739}}</ref>
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| Bridging therapy with PAH-targeted drugs, complications and additional procedures during PEA, and residual PH after PEA are associated with worse outcomes. Immediate postoperative PVR is a long-term predictor of prognosis.<ref name=":8" />
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| === Balloon pulmonary angioplasty ===
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| Although BPA technique is still evolving, it may currently be considered for CTEPH patients who are technically non-operable or carry an unfavourable risk-benefit ratio for PEA.<ref name=":Galie_2015" /> The technique has been refined in Japan<ref>{{Cite journal|last=Mizoguchi|first=Hiroki|last2=Ogawa|first2=Aiko|last3=Munemasa|first3=Mitsuru|last4=Mikouchi|first4=Hiroshi|last5=Ito|first5=Hiroshi|last6=Matsubara|first6=Hiromi|date=December 2012|title=Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension|journal=Circulation: Cardiovascular Interventions|volume=5|issue=6|pages=748–755|doi=10.1161/CIRCINTERVENTIONS.112.971077|issn=1941-7632|pmid=23192917|doi-access=free}}</ref> and initial reports have confirmed the safety and efficacy of the technique, based on data showing haemodynamic improvement and recovery of right ventricular function.<ref>{{Cite journal|last=Fukui|first=Shigefumi|last2=Ogo|first2=Takeshi|last3=Morita|first3=Yoshiaki|last4=Tsuji|first4=Akihiro|last5=Tateishi|first5=Emi|last6=Ozaki|first6=Kumi|last7=Sanda|first7=Yoshihiro|last8=Fukuda|first8=Tetsuya|last9=Yasuda|first9=Satoshi|date=May 2014|title=Right ventricular reverse remodelling after balloon pulmonary angioplasty|journal=The European Respiratory Journal|volume=43|issue=5|pages=1394–1402|doi=10.1183/09031936.00012914|issn=1399-3003|pmid=24627536|doi-access=free}}</ref> Research is ongoing.<ref name=Brenot2017/><ref>{{Cite journal|last=Lang|first=Irene Marthe|last2=Madani|first2=Michael|date=2014-08-05|title=Update on chronic thromboembolic pulmonary hypertension|journal=Circulation|volume=130|issue=6|pages=508–518|doi=10.1161/CIRCULATIONAHA.114.009309|issn=1524-4539|pmid=25092279|doi-access=free}}</ref>
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| == Prognosis ==
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| Historically the prognosis for patients with untreated CTEPH was poor, with a 5-year survival of <40% if the mPAP was >40 mmHg at presentation.<ref>{{Cite journal|last=Riedel|first=M.|last2=Stanek|first2=V.|last3=Widimsky|first3=J.|last4=Prerovsky|first4=I.|date=February 1982|title=Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data|journal=Chest|volume=81|issue=2|pages=151–158|issn=0012-3692|pmid=7056079|doi=10.1378/chest.81.2.151}}</ref> More contemporary data from the European CTEPH registry have demonstrated a 70% 3-year survival in patients with CTEPH who do not undergo the surgical procedure of [[Pulmonary thromboendarterectomy|pulmonary endarterectomy (PEA)]].<ref name=":4">{{Cite journal|last=Delcroix|first=Marion|last2=Lang|first2=Irene|last3=Pepke-Zaba|first3=Joanna|last4=Jansa|first4=Pavel|last5=D'Armini|first5=Andrea M.|last6=Snijder|first6=Repke|last7=Bresser|first7=Paul|last8=Torbicki|first8=Adam|last9=Mellemkjaer|first9=Sören|date=2016-03-01|title=Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry|journal=Circulation|volume=133|issue=9|pages=859–871|doi=10.1161/CIRCULATIONAHA.115.016522|issn=1524-4539|pmid=26826181}}</ref> Recent data from an international CTEPH registry demonstrate that mortality in CTEPH is associated with [[New York Heart Association Functional Classification|New York Heart Association (NYHA) functional class]] IV, increased right atrial pressure, and a history of cancer. Furthermore, comorbidities such as coronary disease, [[Heart failure#Left-sided failure|left heart failure]], and [[Chronic obstructive pulmonary disease|chronic obstructive pulmonary disease (COPD)]] are risk factors for mortality.<ref name=":4" />
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| == Epidemiology ==
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| CTEPH is an [[Rare disease|orphan disease]] with an estimated incidence of 5 cases per million,<ref name="Pepke-Zaba_2013">{{Cite journal|last=Pepke-Zaba|first=Joanna|last2=Jansa|first2=Pavel|last3=Kim|first3=Nick H.|last4=Naeije|first4=Robert|last5=Simonneau|first5=Gerald|date=April 2013|title=Chronic thromboembolic pulmonary hypertension: role of medical therapy|journal=The European Respiratory Journal|volume=41|issue=4|pages=985–990|doi=10.1183/09031936.00201612|issn=1399-3003|pmid=23397304|doi-access=free}}</ref> but it is likely that CTEPH is under-diagnosed as symptoms are non-specific. Although a cumulative incidence of CTEPH between 0.1% and 9.1% within the first 2 years after a symptomatic PE has been reported,<ref>{{Cite journal|last=Pengo|first=Vittorio|last2=Lensing|first2=Anthonie W. A.|last3=Prins|first3=Martin H.|last4=Marchiori|first4=Antonio|last5=Davidson|first5=Bruce L.|last6=Tiozzo|first6=Francesca|last7=Albanese|first7=Paolo|last8=Biasiolo|first8=Alessandra|last9=Pegoraro|first9=Cinzia|date=2004-05-27|title=Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism|journal=The New England Journal of Medicine|volume=350|issue=22|pages=2257–2264|doi=10.1056/NEJMoa032274|issn=1533-4406|pmid=15163775}}</ref> it is currently unclear whether acute symptomatic PE begets CTEPH. Routine screening for CTEPH after PE is not recommended because a significant number of CTEPH cases develops in the absence of previous acute symptomatic PE.<ref name=":Galie_2015" /> In addition, approximately 25% of patients with CTEPH do not present with a clinical history of acute PE.<ref name="Pepke-Zaba_2011" /> The median age of patients at diagnosis is 63 years (there is a wide age range, but paediatric cases are rare), and both genders are equally affected.<ref name="Pepke-Zaba_2011" /><ref>{{Cite journal|last=Berger|first=Rolf M. F.|last2=Beghetti|first2=Maurice|last3=Humpl|first3=Tilman|last4=Raskob|first4=Gary E.|last5=Ivy|first5=D. Dunbar|last6=Jing|first6=Zhi-Cheng|last7=Bonnet|first7=Damien|last8=Schulze-Neick|first8=Ingram|last9=Barst|first9=Robyn J.|date=2012-02-11|title=Clinical features of paediatric pulmonary hypertension: a registry study|journal=Lancet|volume=379|issue=9815|pages=537–546|doi=10.1016/S0140-6736(11)61621-8|issn=1474-547X|pmc=3426911|pmid=22240409}}</ref><ref>{{Cite journal|last=Madani|first=Michael M.|last2=Wittine|first2=Lara M.|last3=Auger|first3=William R.|last4=Fedullo|first4=Peter F.|last5=Kerr|first5=Kim M.|last6=Kim|first6=Nick H.|last7=Test|first7=Victor J.|last8=Kriett|first8=Jolene M.|last9=Jamieson|first9=Stuart W.|date=March 2011|title=Chronic thromboembolic pulmonary hypertension in pediatric patients|journal=The Journal of Thoracic and Cardiovascular Surgery|volume=141|issue=3|pages=624–630|doi=10.1016/j.jtcvs.2010.07.010|issn=1097-685X|pmid=20800245}}</ref>
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| == References ==
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| {{reflist}}
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| ==External links==
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| {{Medical resources
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| | DiseasesDB = 10998
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| | ICD10 = {{ICD10|I27.2}}
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| [[Category:Pulmonary heart disease and diseases of pulmonary circulation]] | |
| [[Category:Hypertension]] | |
| {{dictionary-stub1}}
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| ==Chronic thromboembolic pulmonary hypertension==
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| <gallery>
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| File:CTEPH_pulmonary_artery_angiogram_(PA_angiogram).tif|CTEPH pulmonary artery angiogram (PA angiogram)
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| File:PEA_specimen.tif|PEA specimen
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| </gallery>
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Chronic Thromboembolic Pulmonary Hypertension
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a form of pulmonary hypertension caused by chronic obstruction of the pulmonary arteries due to organized blood clots. This condition is a serious complication that can arise after an episode of pulmonary embolism, where blood clots travel to the lungs and block the pulmonary arteries.
Pathophysiology
CTEPH occurs when blood clots that have traveled to the lungs do not dissolve completely and instead become organized into fibrotic tissue. This leads to persistent obstruction of the pulmonary arteries, resulting in increased pulmonary vascular resistance and elevated pulmonary artery pressure. Over time, this increased pressure can lead to right ventricular hypertrophy and eventually right heart failure.
Symptoms
The symptoms of CTEPH are often similar to those of other forms of pulmonary hypertension and can include:
Diagnosis
The diagnosis of CTEPH involves a combination of clinical evaluation, imaging studies, and hemodynamic measurements. Key diagnostic tools include:
Treatment
The primary treatment for CTEPH is pulmonary endarterectomy (PEA), a surgical procedure that removes the organized clots from the pulmonary arteries. This surgery can significantly improve symptoms and hemodynamics in eligible patients. For patients who are not candidates for surgery, medical therapies such as riociguat or balloon pulmonary angioplasty may be considered.
Prognosis
The prognosis for patients with CTEPH varies depending on the severity of the disease and the success of treatment. Surgical intervention with PEA can be curative in many cases, leading to significant improvements in quality of life and survival. However, untreated CTEPH can lead to progressive right heart failure and increased mortality.
Related Pages
