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Chronic Thromboembolic Pulmonary Hypertension

CTEPH

Chronic thromboembolic pulmonary hypertension implies prolonged narrowing or complete obstruction of pulmonary vessels, which is most likely due to a non-resolving thrombi in the setting of a previous pulmonary embolism. It is a rare entity in clinical practice, but more than 50% of patients suffering from this condition are asymptomatic. Imaging studies are required for diagnosis, whereas surgical removal of clots from occluded vessels is the mainstay of therapy.

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Presentation

In general, patients complain of progressive dyspnea, particularly on exertion, together with hemopstysis, fatigue, syncope, palpitations or edema (all signs of right heart failure) [3]. Schistosomiasis can have an acute and chronic course. A severe inflammatory reaction to the parasite that causes nocturnal fever, headaches, dyspnea and myalgia in the setting of acute disease (also known as Katayama syndrome), whereas an asthma-like disease is seen in chronic forms [4]. In both acute and chronic disease, pulmonary vascular disease and cor pulmonale (right heart failure) may develop and lead to CTEPH [4].

Fatigue
  • Dyspnea and fatigue were the most common presenting symptoms. History of pulmonary embolism was present in 82%. Of patients, 18 (67%) were assessed as operable, of whom 10 (55%) finally underwent pulmonary endarterectomy (PEA).[ncbi.nlm.nih.gov]
  • Symptoms of CTEPH are progressive dyspnea, hemoptysis and right-heart failure signs such as fatigue, weakness and edema. Additional complaints depend on the underlying cause.[symptoma.com]
  • , or near fainting. 4) class IV: symptoms at rest (such as dyspnea and/or fatigue) and inability to carry out any physical activity without symptoms (e.g. may faint especially while bending over with their heads lowered).[clinicaltrials.gov]
  • Changes in the pulmonary arteries restrict blood flow, leading to a rise in blood pressure. 2,3 The most common symptoms of CTEPH are similar to those of PH, including shortness of breath with exercise, fatigue, or weakness.[cteph.com]
  • The commonest presenting symptoms are dyspnea, reduced exercise tolerance and fatigue, common to a range of cardiopulmonary diseases which result in a diagnosis of CTEPH frequently being delayed.[minervamedica.it]
Weakness
  • Being short of breath, especially during exercise Feeling tired Feeling weak Feeling pain or pounding in the chest (the pounding is called palpitations) Swelling of the legs due to fluid retention ( edema ) Developing cyanosis (blue color to fingers and[my.clevelandclinic.org]
  • Changes in the pulmonary arteries restrict blood flow, leading to a rise in blood pressure. 2,3 The most common symptoms of CTEPH are similar to those of PH, including shortness of breath with exercise, fatigue, or weakness.[cteph.com]
  • Symptoms of CTEPH are progressive dyspnea, hemoptysis and right-heart failure signs such as fatigue, weakness and edema. Additional complaints depend on the underlying cause.[symptoma.com]
  • The most common presenting symptom in patients with PAH is persistent dyspnoea when exercising, while chest pain, fatigue, weakness, light-headedness and syncope may also occur [ 37 ].[doi.org]
  • Baseline PVR 1000 dynes.s.cm 5 was a weak predictor of 1-, and 3- year survival (Fisher's exact test p 0.05, p 0.06).[doi.org]
Chest Discomfort
  • We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema.[ncbi.nlm.nih.gov]
  • We describe the case of a 43-years old with a history of progressive shortness of breath, hemoptysis, chest discomfort and syncope. Echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension.[ncbi.nlm.nih.gov]
Fever
  • For e.g. schistosomiasis is manifested by nocturnal fever and malaise several weeks after exposure to contaminated waters in endemic areas (the Caribbean island, Brazil, China, the Middle east and sub-Saharan Africa).[symptoma.com]
Dyspnea
  • CTEPH must be suspected in any patient with NS, with or without hyperhomocysteinemia, and unexplained dyspnea.[ncbi.nlm.nih.gov]
  • The Borg dyspnea index rates the severity of dyspnea (difficult or labored breathing) on a scale from 0 ('Nothing at all') to 10 ('Very, very severe - maximal'). A decrease in the Borg dyspnea index indicates an improvement.[clinicaltrials.gov]
  • In 2014, IVCF occlusion and marked collateral circulation were noted during an examination for transient dyspnea; however, his warfarin level was within the therapeutic range for 88.9% of the time from 2003 to 2014.[ncbi.nlm.nih.gov]
  • After three sessions of balloon pulmonary angioplasty, his pulmonary arterial pressure decreased and his symptom of dyspnea on exertion and desaturation were improved.[ncbi.nlm.nih.gov]
  • A 35-year-old man with a six-month history of progressive exertional dyspnea was referred to our institution.[ncbi.nlm.nih.gov]
Hemoptysis
  • A systematic review revealed 21 studies on the underlying pathology in 1,844 patients with moderate to severe hemoptysis.[ncbi.nlm.nih.gov]
  • We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema.[ncbi.nlm.nih.gov]
  • We describe the case of a 43-years old with a history of progressive shortness of breath, hemoptysis, chest discomfort and syncope. Echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension.[ncbi.nlm.nih.gov]
  • Patients were divided into two groups: patients with lung injury including asymptomatic lung infiltration on computed tomography (CT) images or mild hemoptysis (group L, n 9) and no complications (group N, n 10).[ncbi.nlm.nih.gov]
  • A 12-year-old boy initially complained of hemoptysis, cough and shortness of breath with exertion, any symptoms regarding NS such as edema were not presented.[ncbi.nlm.nih.gov]
Pleural Effusion
  • We report CTEPH in a 58-year-old male who had pleurisy with a small haemorrhagic pleural effusion three months ago. The six-month course of anti-coagulation therapy failed to resolve thromboemboli completely or improve pulmonary hypertension.[ncbi.nlm.nih.gov]
  • Complications include haemoptysis, wire injury, vessel dissection, vessel rupture, reperfusion pulmonary oedema, pulmonary parenchymal bleeding and haemorrhagic pleural effusions.[ncbi.nlm.nih.gov]
  • Imaging studies such as plain chest radiography may show enlarged pulmonary trunk, hypertrophy of the right ventricle and pleural effusions, which may be confirmed by echocardiography, a widely recommended procedure for CTEPH evaluation.[symptoma.com]
  • This causes pulmonary edema and pleural effusions.[en.wikipedia.org]
Exertional Dyspnea
  • A 35-year-old man with a six-month history of progressive exertional dyspnea was referred to our institution.[ncbi.nlm.nih.gov]
  • Chronic thromboembolic pulmonary hypertension (CTEPH), an underappreciated cause of severe pulmonary arterial hypertension (PAH), usually presents with progressive exertional dyspnea.[mayoclinic.org]
  • We also excluded patients who had a history of other diseases that are known to be associated with pulmonary hypertension and those who had preexisting exertional dyspnea.[doi.org]
  • Patients with persistent exertional dyspnea after successful PEA still display an abnormal pulmonary hemodynamic response to exercise. 80 PVR in CTEPH can be partitioned into larger arterial (upstream) and small arterial plus venous (downstream) components[doi.org]
Heart Failure
  • The factors that affect patient QoL 1 year after surgery on some SF-36 subscales included the presence of coronary artery disease, COPD, heart failure, residual pulmonary hypertension, and prolonged ventilation, neurological complications in the early[ncbi.nlm.nih.gov]
  • The patient died from right heart failure before the thrombocytopenia could be managed, preventing performance of a pulmonary endarterectomy procedure.[ncbi.nlm.nih.gov]
  • In both acute and chronic disease, pulmonary vascular disease and cor pulmonale (right heart failure) may develop and lead to CTEPH.[symptoma.com]
  • Untreated, this disease leads to progressive right heart failure and death. It develops in approximately 1% to 5% of patients who suffer an acute pulmonary embolism (PE) and has an overall incidence of 3 to 30 per million in the general population.[ncbi.nlm.nih.gov]
  • failure owing to an exhaustion of RV contractile function.[ncbi.nlm.nih.gov]
Chest Pain
  • A 29-year-old man presented with chest pain, decreased exercise capacity, dyspnoea, and haemoptysis. He was triple positive for antiphospholipid antibodies.[ncbi.nlm.nih.gov]
  • As the disease progresses, other symptoms may appear, including: difficulty breathing water retention in the limbs (edema) swelling or bloating of the abdomen chest pain light-headedness We use advanced diagnostic testing to determine whether patients[brighamandwomens.org]
  • If left untreated, CTEPH can cause fatigue, chest pain, and shortness of breath. In later stages, CTEPH can cause fainting and heart failure symptoms.[healthcare.utah.edu]
  • pain Fatigue Palpitations Edema, or fluid collection in the legs or abdomen Fainting Diagnostics Right-heart catheterization Echocardiogram Ventilation-perfusion scan (VQ scan) Pulmonary angiogram – shows blood flow through the lungs Pulmonary function[houstonmethodist.org]
  • The most common presenting symptoms in patients with CTEPH include dyspnoea, oedema, fatigue and chest pain [ 36 ].[doi.org]
Palpitations
  • Being short of breath, especially during exercise Feeling tired Feeling weak Feeling pain or pounding in the chest (the pounding is called palpitations) Swelling of the legs due to fluid retention ( edema ) Developing cyanosis (blue color to fingers and[my.clevelandclinic.org]
  • In general, patients complain of progressive dyspnea, particularly on exertion, together with hemopstysis, fatigue, syncope, palpitations or edema (all signs of right heart failure). Schistosomiasis can have an acute and chronic course.[symptoma.com]
  • […] and heart disease Some surgeries such as hip or knee replacement Bone fractures Family or personal blood clot history Spleen removal Long-term use of intravenous catheters, including those for dialysis Symptoms Shortness of breath Chest pain Fatigue Palpitations[houstonmethodist.org]
  • The most common symptoms of CTEPH include: Fatigue Shortness of breath, especially with exercise Less common symptoms include: Chest pain Heart palpitations or fluttering Dry cough Swelling of the lower extremities or stomach (edema) As the disease progresses[upmc.com]
  • […] obstructive pulmonary disease (COPD) Obesity (overweight) Heart failure Common symptoms include: Shortness of breath, especially with exercise or climbing stairs Fatigue (tiredness) Swelling of the legs Dizziness Tightness in the chest or belly Fainting Palpitations[foundation.chestnet.org]
Cyanosis
  • Being short of breath, especially during exercise Feeling tired Feeling weak Feeling pain or pounding in the chest (the pounding is called palpitations) Swelling of the legs due to fluid retention ( edema ) Developing cyanosis (blue color to fingers and[my.clevelandclinic.org]
Myalgia
  • A severe inflammatory reaction to the parasite that causes nocturnal fever, headaches, dyspnea and myalgia in the setting of acute disease (also known as Katayama syndrome), whereas an asthma-like disease is seen in chronic forms.[symptoma.com]
  • However, there are some adverse effects of this drug such as headache, diarrhea, nausea, back pain, dyspepsia, flushing and myalgia. Soluble guanylate cyclase (sGC) is the intracellular receptor for NO.[en.wikipedia.org]
Microscopic Hematuria
  • Proteinuria and microscopic hematuria also disappeared. Encouragingly, RA and RV dilation, and the pulmonary arterial pressure almost returned to a normal range half a year later, with alleviation of MR.[ncbi.nlm.nih.gov]
Headache
  • AEs were mainly mild to moderate in nature and the most common were headache and dizziness.[doi.org]
  • A severe inflammatory reaction to the parasite that causes nocturnal fever, headaches, dyspnea and myalgia in the setting of acute disease (also known as Katayama syndrome), whereas an asthma-like disease is seen in chronic forms.[symptoma.com]
  • In addition, prostacyclin side effects are common and include jaw pain, leg and foot pain, headache, diarrhea, ascites and flushing.[ecrjournal.com]
  • For one patient in the riociguat group, the adverse events (diarrhea, heartburn, nausea, vomiting, and headache) leading to discontinuation were considered by the investigator to be related to the study drug.[doi.org]
  • However, there are some adverse effects of this drug such as headache, diarrhea, nausea, back pain, dyspepsia, flushing and myalgia. Soluble guanylate cyclase (sGC) is the intracellular receptor for NO.[en.wikipedia.org]
Dizziness
  • […] of breath, especially with exercise Less common symptoms include: Chest pain Heart palpitations or fluttering Dry cough Swelling of the lower extremities or stomach (edema) As the disease progresses, symptoms of light-headedness or exercise-related dizziness[upmc.com]
  • AEs were mainly mild to moderate in nature and the most common were headache and dizziness.[doi.org]
  • […] diseases, including: Asthma Emphysema Chronic obstructive pulmonary disease (COPD) Obesity (overweight) Heart failure Common symptoms include: Shortness of breath, especially with exercise or climbing stairs Fatigue (tiredness) Swelling of the legs Dizziness[foundation.chestnet.org]
  • […] bleeding (3.7%), pulmonary reperfusion edema (13.5%) 90 Life-long treatment, evidence currently based on a single positive trial, only approved for nonoperable patients and patients with persistent/recurrent PH after PEA; syncope (2%), headache (25%), dizziness[doi.org]

Workup

A thorough patient history may provide vital clues about the underlying cause, for e.g.recent travel to endemic areas for schistosomiasis and exposure to potentially contaminated waters approximately 2-12 weeks prior to the appearance of symptoms [4]. A meticulous physical examination with an emphasis on cardiopulmonary auscultation is equally important. Namely, distended neck veins, ascites, edema and tricuspid regurgitation may reflect right heart failure, whereas peripheral pulmonary bruits (very rare but highly specific for CTEPH) may be observed in approximately 10% of patients [3]. Imaging studies such as plain chest radiography may show enlarged pulmonary trunk, hypertrophy of the right ventricle and pleural effusions [4], which may be confirmed by echocardiography, a widely recommended procedure for CTEPH evaluation [3]. MRI and CT are also frequently used, but ventilation/perfusion (V/Q) lung scintigraphy may show segmental perfusion defects and for this reason, it is considered as the optimal diagnostic procedure [16]. Pulmonary angiography is equally effective in visualizing the level of obstruction and vessel narrowing [9]. In most patients CTEPH is distinguished from PAH by presence of thrombi in major pulmonary vessels, but virtually any part of the circulation may be affected [12]. Apart from their role in diagnosis, these procedures are important in evaluating the possibility of endarterectomy [9].

Right Axis Deviation
  • Electrocardiography Right axis deviation and precordial T wave abnormalities are early signs. Pulmonary function tests Elevated pulmonary artery pressure causes restrictive physiology.[slideplayer.com]
Decreased Platelet Count
  • Platelet transfusions may further decrease platelet count, as it can trigger formation of new antibodies.[ncbi.nlm.nih.gov]
Pleural Effusion
  • We report CTEPH in a 58-year-old male who had pleurisy with a small haemorrhagic pleural effusion three months ago. The six-month course of anti-coagulation therapy failed to resolve thromboemboli completely or improve pulmonary hypertension.[ncbi.nlm.nih.gov]
  • Complications include haemoptysis, wire injury, vessel dissection, vessel rupture, reperfusion pulmonary oedema, pulmonary parenchymal bleeding and haemorrhagic pleural effusions.[ncbi.nlm.nih.gov]
  • Imaging studies such as plain chest radiography may show enlarged pulmonary trunk, hypertrophy of the right ventricle and pleural effusions, which may be confirmed by echocardiography, a widely recommended procedure for CTEPH evaluation.[symptoma.com]
  • This causes pulmonary edema and pleural effusions.[en.wikipedia.org]

Treatment

Several therapeutic strategies exist for management of CTEPH. Pulmonary endarterectomy is a surgical procedure that provides curative effects in many patients (since it enables complete removal of emboli from the occluded vessels) and is now considered as the mainstay of therapy [5]. But because of its characteristics (profound hypothermia, cardiopulmonary bypass and induced cardiac arrest), a mortality rate of 4.4% during surgery is observed [14]. Moreover, it may not be performed in all patients (presence of additional comorbidities, location of emboli in smaller vessels and poor overall condition). It may also be unsuccessful in patients with significant arteriopathy of smaller vessels, which is why alternative interventions are advocated. Anticoagulant therapy, prostanoids, endothelin receptor antagonists, or phosphodiesterase-5-inhibitors are pharmacological agents most frequently used in these patients, but their efficacy requires further research [17]. As a last resort, lung transplantation may be performed [17].

Prognosis

If CTEPH is left untreated, the estimated survival is about three years [1]. Moreover, the majority of patients are diagnosed in advanced stages of the disease [1], when little can be done in terms of treatment, which is why an early diagnosis is detrimental.

Etiology

Several conditions have been associated with PAH in literature, including Schistosomiasis (caused by a nematode worm Schistosoma mansoni, S. hematobium or S.japonicum) [7], idiopathic pulmonary arterial hypertension (IPAH), anorexigen exposure and sleep apnea [1] [8]. In all diseases, CTEPH stems from occlusion of pulmonary vessels by thromboemboli that generate increased pressure and resistance. Pulmonary embolism (PE) is, in fact, the condition that predisposes patients to CTEPH, which may develop in any of the mentioned conditions through various pathophysiological mechanisms. However, why do these emboli persist after months or even years remains unclear. Current studies have determined that some patients possess clotting factor aberrations, but in the majority of patients, the exact mechanism is yet to be determined [9].

Epidemiology

Studies have determined that CTEPH develops in approximately 4,000 individuals in the United States every year [1], whereas overall incidence rates after pulmonary embolism are up to 3%-4% after 2 years [2] [6]. PE is, by far, the most significant risk factor for CTEPH as approximately 50% of patients report acute pulmonary thromboembolism in their recent history [10]. Schistosomiasis is an infection affecting more than 200 million individuals over the world, but its prevalence is highest in developing countries and certain geographical areas [7]. The Caribbean countries, the eastern part of South America (Brazil), sub-Saharan Africa, China and the Middle east are considered to be endemic regions of this parasite and travel to these areas is considered as an important risk factor [4]. Approximately 8% of individuals suffering from this disease develop PAH [7], but other reports suggest that the prevalence of PAH is as high as 20% [4]. For unknown reasons, CTEPH is more frequently encountered in females [1]. An additional risk factor is the presence of lupus anticoagulant [11], an antibody against phospholipids that promotes coagulation [11], while splenectomy, ventriculoatrial shunts, inflammatory bowel disease and osteomyelitis have also been determined as conditions that increase the susceptibility toward CTEPH [12].

Sex distribution
Age distribution

Pathophysiology

Although the exact model of disease in CTEPH is incompletely understood, current theories implicate various etiologic factors that impair the coagulatory cascade [13]. Numerous diseases have been described as potential triggers of pulmonary arterial hypertension, defined as mean pressure in the pulmonary artery ≥ 25 mmHg at rest or > 30 mmHg during exercise [1]. In virtually all patients, the predisposing condition that leads to CTEPH is pulmonary embolism, in which formation of a thrombus occludes pulmonary vessels and causes increased pressure. Usually, the thrombus dissolves completely, but in some individuals, this process is not carried out in full [14]. The exact mechanism remains unknown, but it is established that up to 10% of patients have some abnormality of the coagulation system, such as protein C or S deficiency and impairment of the fibrin clotting system [14]. Procoagulatory states in the setting of increased factor VIII values were identified as well [15]. Additionally, some patients develop repetitive pulmonary embolism, which further contributes to persistence of clots in the pulmonary vasculature [14]. In the setting of schistosomiasis, the life cycle of the parasite starts by shedding from the initial host (local population) through feces and urine into water, infection of various snail species and subsequent invasion of human hosts through breaks in the skin [4]. Once the parasite spreads through the lymphatic system and into the lungs, it replicates and disseminates throughout the gastrointestinal tract and the liver, eventually forming an embolus in many blood vessels, including pulmonary arteries [4]. Regardless of the pathophysiological mechanism, the end-result in CTEH is remodeling of the emboli that narrows or obstructs the major arteries of the pulmonary system, leading to dysfunction of the right ventricle and increased pulmonary vascular resistance, eventually causing cardiac insufficiency [10].

Prevention

Although prevention of venous thromboembolism through management of the underlying disease may be a prudent strategy, studies have determined that more than 60% of CTEPH patients do not exhibit any symptoms suggestive of venous disease [6]. For this reason, wide-scale use of echocardiography may be highly effective as a tool for an early diagnosis, especially in the setting of known schistosomiasis infection [7]. Additionally, long-term monitoring and prophylaxis of patients who developed pulmonary embolism and increased awareness of possible CTEPH is detrimental for prevention [2].

Summary

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as persistent pulmonary arterial hypertension (PAH) for three consecutive months after an episode of pulmonary thromboembolism [1]. Although CTEPH is a rarely encountered phenomenon in clinical practice, it is often unrecognized and associated with a substantial risk of mortality [2]. Numerous diseases are known to cause PAH and thus predispose to CTEPH, including schistosomiasis, sleep apnea, exposure to anorexigen (an appetite suppressant) and various collagenoses [1], whereas idiopathic pulmonary arterial hypertension (IPAH) is also an important cause. In the presence of these conditions, intraluminal organization of a thrombus and concomitant stenosis as a result of fibrous tissue accumulation leads to increased resistance in the pulmonary vasculature [3]. In an attempt to overcome this adverse circulatory event, the right ventricle enlarges and increases its contractile force, but because the vessel lumen is persistently narrowed or completely occluded, failure of the right heart eventually ensues [3]. Symptoms of CTEPH are progressive dyspnea, hemoptysis and right-heart failure signs such as fatigue, weakness and edema [3]. Additional complaints depend on the underlying cause. For e.g. schistosomiasis is manifested by nocturnal fever and malaise several weeks after exposure to contaminated waters in endemic areas (the Caribbean island, Brazil, China, the Middle east and sub-Saharan Africa) [4]. To make the diagnosis, a thorough approach consisting of a properly obtained patient history and a detailed physical examination is vital. Initial signs of heart failure (tricuspid regurgitation, distended neck veins, or ascites) are important clinical findings that can guide the physician toward the diagnosis [3], which is confirmed by various imaging studies. Plain radiography, echocardiography, computed tomography (CT), magnetic resonance imaging (MRI), lung scintigraphy and angiography are all indicated when CTEPH is suspected, to solidify the diagnosis and to determine the optimal therapeutic strategy.The procedure of choice (when possible) is pulmonary endarterectomy (PEA), comprising of surgical removal of emboli from pulmonary vessels. Despite a small, but significant risk of death during surgery (4.4%), this procedure has curative effects in the vast majority of patients and can profoundly improve the quality of life [5]. If CTEPH is left untreated, the disease is universally fatal within a few years, which is why an early diagnosis must be made. This may be difficult, however, as more than 60% of patients show no symptoms of previous thromboembolic disease [6], implying the necessity for more detailed regular check-ups and conduction of screening strategies.

Patient Information

Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition that develops due to narrowing and obstruction of blood vessels in the lungs, most commonly following pulmonary embolism (PE), where a blood clot gets "stuck" in one or more vessels. Because clots block the passage of blood, the heart generates higher pressures to overcome this obstacle, leading to increased pressure inside the blood vessels of the lungs, medically known as pulmonary hypertension (PAH). For yet unknown reasons, CTEPH develops months or even years after the initial episode of PE and factors that promote incomplete resolution of the clot are yet to be discovered. In addition to PE, conditions such as sleep apnea, schistosomiasis (a parasitic infection), exposure to anorexigen (a suppressant of appetite) and idiopathic pulmonary hypertension (IPAH) are known causes of CTEPH. It is estimated that approximately 4,000 cases develop in the United States every year and a strong predilection toward female gender was established. Risk factors that promote coagulatory states (and thus impair resolution of previously formed blood clots) include osteomyelitis, inflammatory bowel disease (IBD), presence of ventriculoatrial shunts and spleen removal. The diagnosis may be attained with great difficulty, as many patients report no obvious signs of thromboembolic disease. Progressive heavy breathing (dyspnea), coughing of blood (hemoptysis) and symptoms that reflect failure of the right side of the heart due to prolonged efforts to combat blood vessel occlusion, such as fatigue, malaise, palpitations and syncope are most frequently encountered. Imaging studies, including plain chest X-ray, computed tomography (CT scan), magnetic resonance imaging (MRI) and echocardiography are all useful in solidifying the diagnosis, but the two most important procedures are CT-guided angiography and lung scintigraphy, which allow direct visualization of blood flow through the lungs and identification of the exact site of occlusion. This is quite important, as the optimal therapeutic strategy is surgical removal of clots from the occluded arteries, known as pulmonary endarterectomy. Because this type of surgery mandates cessation of heart function and marked reduction of body temperature (as low as 20 degrees Celsius), a very small number of patients (around 4%) do not survive this procedure, but its efficacy that may even result in complete cure significantly outweighs the risks. An early diagnosis is essential for patients sufferning from CTEPH, primarily to obtain a much better prognosis, as the condition is fatal within a few years if left untreated.

References

Article

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  2. Pengo V, Lensing AWA, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004; 350:2257-2264.
  3. Hoeper MM, Mayer E, Simonneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113:2011-20.
  4. Butrous, Ghofrani HA. Pulmonary Vascular Disease in the Developing World. Circulation. 2008;118:1758-1766.
  5. Corsico AG, D'Armini AM, Cerveri I, et al. Long-term outcome after pulmonary endarterectomy. Am J Respir Crit Care Med. 2008;178:419-424.
  6. Tapson VF, Humbert M. Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc. 2006;3:564-567.
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  8. Natali D, Girerd B, Montani D, Soubrier F, Simonneau G, Humbert M, et al. Pulmonary arterial hypertension in a patient with Cowden syndrome and anorexigen exposure. Chest. 2011;140(4):1066-1068.
  9. Dartevelle P, Fadel E, Mussot S, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2004;23:637-468.
  10. Morris TA, Marsh JJ, Chiles PG, et al. Fibrin derived from patients with chronic thromboembolic pulmonary hypertension is resistant to lysis. Am J Respir Crit Care Med. 2006;173:1270.
  11. Auger WR, Permpikul P, Moser KM. Lupus anticoagulant, heparin use, and thrombocytopenia in patients with chronic thromboembolic pulmonary hypertension: a preliminary report. Am J Med. 1995;99:392.
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  15. Bonderman D, Turecek PL, Jakowitsch J, et al. High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension. Thromb Haemost. 2003;90:372.
  16. Dong C, Zhou M, Liu D, Long X, Guo T, Kong X. Diagnostic Accuracy of Computed Tomography for Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-Analysis. PLoS ONE. 2015;10(4):e0126985.
  17. Rubin LW, Humbert M, Klepetko W, et al. Current and future management of chronic thromboembolic pulmonary hypertension. Proc Am Thoracic SOc. 2006;3:601-607.

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Last updated: 2019-07-11 19:55