Chronic thromboembolic pulmonary hypertension

Last updated
Chronic thromboembolic pulmonary hypertension
Other namesCTEPH
Specialty Pulmonology, cardiology
Usual onset63 years (median) [1]
DurationLong term
Risk factors Splenectomy, 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 method Invasively measured mean pulmonary arterial pressure combined with specialist imaging [6]
Treatment Pulmonary 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]

Contents

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.

Signs and symptoms

Clinical symptoms and signs are often non-specific or absent in early CTEPH, with signs of 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 idiopathic pulmonary arterial hypertension (iPAH). Leg oedema (swelling) and haemoptysis (blood in mucus) occur more often in CTEPH, while syncope (fainting) is more common in iPAH. [5]

Pathogenesis

People with CTEPH lack traditional thrombosis risk factors. [1] [2] [3] [4] [5] Current understanding is that CTEPH is a result of “inflammatory thrombosis”: [5] 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. [4] [5]

Diagnosis

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. CTEPH pulmonary artery angiogram (PA angiogram).tif
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. 

Early diagnosis still remains a challenge in CTEPH, with a median time of 14 months between symptom onset and diagnosis in expert centres. [7] A suspicion of PH is often raised by echocardiography, but an invasive right heart catheterisation is required to confirm it. [6] 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 V/Q scan and confirmation with right heart catheter and pulmonary angiography (PA). [8]

Both V/Q scanning and modern multidetector 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). [9] 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. [10]

Treatment

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 endarterectomy (PEA). Balloon pulmonary angioplasty (BPA) and pulmonary vasodilator drug treatment may be considered for those people that are not suitable for surgery. [11]

Specialist imaging using either magnetic resonance or invasive PA is necessary to determine risks and benefits of interventional treatment with PEA or BPA. [8]

Medical therapy

Standard medical treatment consists of anticoagulants (blood thinners), diuretics, and oxygen. Lifelong anticoagulation is recommended, even after PEA. Routine inferior vena cava filter placement is not recommended. [6]

In patients with non-operable CTEPH or persistent/recurrent PH after PEA, there is evidence for benefit from pulmonary vasodilator drug treatment. [12] The microvascular disease component in CTEPH has provided the rationale for off-label use of drugs approved for PAH. [1] 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. [13] Other drug trials are ongoing in patients with inoperable CTEPH, with macitentan recently proving efficacy and safety in MERIT [14]

Pulmonary endarterectomy

Typical specimen harvested during the surgical procedure of pulmonary endarterectomy in a patient with chronic thromboembolic pulmonary hypertension. PEA specimen.tif
Typical specimen harvested during the surgical procedure of pulmonary endarterectomy in a patient with chronic thromboembolic pulmonary hypertension.

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. About 60% of patients are classified as operable across Europe and Canada. [15]

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), [16] which is not complicated by cognitive dysfunction. [17] The majority of patients experience substantial relief from symptoms and improvement in haemodynamics after PEA. [16] [18] [19] In Europe, in-hospital mortality during PEA is currently 4.7% or lower in high volume single centres. [16] [18]

Up to 35% of patients may have persistent/recurrent CTEPH following surgery. [12] The definition of post-PEA PH is still not clear, but some data suggest that 500–590 dynes·s·cm−5 may represent a pulmonary vascular resistance (PVR) threshold for poor long-term outcome. [20] [21] 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−5. [22]

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. [20]

Balloon pulmonary angioplasty

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. [6] The technique has been refined in Japan [23] and initial reports have confirmed the safety and efficacy of the technique, based on data showing haemodynamic improvement and recovery of right ventricular function. [24] Research is ongoing. [11] [25]

Prognosis

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. [26] 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 endarterectomy (PEA). [15] Recent data from an international CTEPH registry demonstrate that mortality in CTEPH is associated with New York Heart Association (NYHA) functional class IV, increased right atrial pressure, and a history of cancer. Furthermore, comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease (COPD) are risk factors for mortality. [15]

Epidemiology

CTEPH is an orphan disease with an estimated incidence of 5 cases per million, [7] 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, [27] 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. [6] In addition, approximately 25% of patients with CTEPH do not present with a clinical history of acute PE. [1] 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. [1] [28] [29]

Related Research Articles

<span class="mw-page-title-main">Respiratory failure</span> Inadequate gas exchange by the respiratory system

Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise in arterial carbon dioxide levels is called hypercapnia. Respiratory failure is classified as either Type 1 or Type 2, based on whether there is a high carbon dioxide level, and can be acute or chronic. In clinical trials, the definition of respiratory failure usually includes increased respiratory rate, abnormal blood gases, and evidence of increased work of breathing. Respiratory failure causes an altered state of consciousness due to ischemia in the brain.

<span class="mw-page-title-main">Pulmonary embolism</span> Blockage of an artery in the lungs

Pulmonary embolism (PE) is a blockage of an artery in the lungs by a substance that has moved from elsewhere in the body through the bloodstream (embolism). Symptoms of a PE may include shortness of breath, chest pain particularly upon breathing in, and coughing up blood. Symptoms of a blood clot in the leg may also be present, such as a red, warm, swollen, and painful leg. Signs of a PE include low blood oxygen levels, rapid breathing, rapid heart rate, and sometimes a mild fever. Severe cases can lead to passing out, abnormally low blood pressure, obstructive shock, and sudden death.

<span class="mw-page-title-main">Heart failure</span> Failure of the heart to provide sufficient blood flow

Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome caused by an impairment in the heart's ability to fill with and pump blood. Although symptoms vary based on which side of the heart is affected, HF typically presents with shortness of breath, excessive fatigue, and bilateral leg swelling. The severity of the heart failure is mainly decided based on ejection fraction and also measured by the severity of symptoms. Other conditions that have symptoms similar to heart failure include obesity, kidney failure, liver disease, anemia, and thyroid disease.

<span class="mw-page-title-main">Pulmonary heart disease</span> Medical condition

Pulmonary heart disease, also known as cor pulmonale, is the enlargement and failure of the right ventricle of the heart as a response to increased vascular resistance or high blood pressure in the lungs.

<span class="mw-page-title-main">Vascular surgery</span> Medical specialty, operative procedures for the treatment of vascular disorders

Vascular surgery is a surgical subspecialty in which vascular diseases involving the arteries, veins, or lymphatic vessels, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiovascular surgery where it refined the management of just the vessels, no longer treating the heart or other organs. Modern vascular surgery includes open surgery techniques, endovascular techniques and medical management of vascular diseases - unlike the parent specialities. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons also are called to assist other physicians to carry out surgery near vessels, or to salvage vascular injuries that include hemorrhage control, dissection, occlusion or simply for safe exposure of vascular structures.

<span class="mw-page-title-main">Pulmonary hypertension</span> Increased blood pressure in lung arteries

Pulmonary hypertension is a condition of increased blood pressure in the arteries of the lungs. Symptoms include shortness of breath, fainting, tiredness, chest pain, swelling of the legs, and a fast heartbeat. The condition may make it difficult to exercise. Onset is typically gradual. According to the definition at the 6th World Symposium of Pulmonary Hypertension in 2018, a patient is deemed to have pulmonary hypertension if the pulmonary mean arterial pressure is greater than 20mmHg at rest, revised down from a purely arbitrary 25mmHg, and pulmonary vascular resistance (PVR) greater than 3 Wood units.

<span class="mw-page-title-main">Endarterectomy</span> Surgical procedure

Endarterectomy is a surgical procedure to remove the atheromatous plaque material, or blockage, in the lining of an artery constricted by the buildup of deposits. It is carried out by separating the plaque from the arterial wall.

<span class="mw-page-title-main">Pulmonary angiography</span>

Pulmonary angiography is a medical fluoroscopic procedure used to visualize the pulmonary arteries and much less frequently, the pulmonary veins. It is a minimally invasive procedure performed most frequently by an interventional radiologist or interventional cardiologist to visualise the arteries of the lungs.

<span class="mw-page-title-main">Pulmonary fibrosis</span> Disease that causes scarring of the lungs

Pulmonary fibrosis is a condition in which the lungs become scarred over time. Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing. Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer.

<span class="mw-page-title-main">Bronchial artery</span> Blood vessels supplying the lungs

In human anatomy, the bronchial arteries supply the lungs with oxygenated blood, and nutrition. Although there is much variation, there are usually two bronchial arteries that run to the left lung, and one to the right lung, and are a vital part of the respiratory system.

<span class="mw-page-title-main">Respiratory disease</span> Disease of the respiratory system

Respiratory diseases, or lung diseases, are pathological conditions affecting the organs and tissues that make gas exchange difficult in air-breathing animals. They include conditions of the respiratory tract including the trachea, bronchi, bronchioles, alveoli, pleurae, pleural cavity, the nerves and muscles of respiration. Respiratory diseases range from mild and self-limiting, such as the common cold, influenza, and pharyngitis to life-threatening diseases such as bacterial pneumonia, pulmonary embolism, tuberculosis, acute asthma, lung cancer, and severe acute respiratory syndromes, such as COVID-19. Respiratory diseases can be classified in many different ways, including by the organ or tissue involved, by the type and pattern of associated signs and symptoms, or by the cause of the disease.

<span class="mw-page-title-main">Pulmonary thromboendarterectomy</span>

In thoracic surgery, a pulmonary thromboendarterectomy (PTE), also referred to as pulmonary endarterectomy (PEA), is an operation that removes organized clotted blood (thrombus) from the pulmonary arteries, which supply blood to the lungs.

Portopulmonary hypertension (PPH) is defined by the coexistence of portal and pulmonary hypertension. PPH is a serious complication of liver disease, present in 0.25 to 4% of all patients with cirrhosis. Once an absolute contraindication to liver transplantation, it is no longer, thanks to rapid advances in the treatment of this condition. Today, PPH is comorbid in 4-6% of those referred for a liver transplant.

<span class="mw-page-title-main">CT pulmonary angiogram</span> Medical imaging of blood flow between heart and lungs

A CT pulmonary angiogram (CTPA) is a medical diagnostic test that employs computed tomography (CT) angiography to obtain an image of the pulmonary arteries. Its main use is to diagnose pulmonary embolism (PE). It is a preferred choice of imaging in the diagnosis of PE due to its minimally invasive nature for the patient, whose only requirement for the scan is an intravenous line.

<span class="mw-page-title-main">Riociguat</span> Chemical compound

Riociguat, sold under the brand name Adempas, is a medication by Bayer that is a stimulator of soluble guanylate cyclase (sGC). It is used to treat two forms of pulmonary hypertension (PH): chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH). Riociguat constitutes the first drug of the class of sGC stimulators. The drug has a half-life of 12 hours and will decrease dyspnea associated with pulmonary arterial hypertension.

<span class="mw-page-title-main">Chronic pulmonary aspergillosis</span> Fungal infection

Chronic pulmonary aspergillosis is a long-term fungal infection caused by members of the genus Aspergillus—most commonly Aspergillusfumigatus. The term describes several disease presentations with considerable overlap, ranging from an aspergilloma—a clump of Aspergillus mold in the lungs—through to a subacute, invasive form known as chronic necrotizing pulmonary aspergillosis which affects people whose immune system is weakened. Many people affected by chronic pulmonary aspergillosis have an underlying lung disease, most commonly tuberculosis, allergic bronchopulmonary aspergillosis, asthma, or lung cancer.

<span class="mw-page-title-main">Pulmonary venoocclusive disease</span> Medical condition

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. The disease is progressive and fatal, with median survival of about 2 years from the time of diagnosis to death. The definitive therapy is lung transplantation.

<span class="mw-page-title-main">Stuart W. Jamieson</span> British surgeon

Stuart William Jamieson is a British cardiothoracic surgeon, specialising in pulmonary thromboendarterectomy (PTE), a surgical procedure performed to remove organized clotted blood (thrombus) from pulmonary arteries in people with chronic thromboembolic pulmonary hypertension (CTEPH).

<span class="mw-page-title-main">Balloon pulmonary angioplasty</span> Medical procedure

Balloon pulmonary angioplasty (BPA) is an emerging minimally invasive procedure to treat chronic thromboembolic pulmonary hypertension (CTEPH) in people who are not suitable for pulmonary thromboendarterectomy (PTE) or still have residual pulmonary hypertension and areas of narrowing in the pulmonary arterial tree following previous PTE.

<span class="mw-page-title-main">Pulmonary artery agenesis</span>

Pulmonary artery agenesis refers to a rare congenital absence of pulmonary artery due to a malformation in the sixth aortic arch. It can occur bilaterally, with both left and right pulmonary arteries being absent, or unilaterally, the absence of either left or right pulmonary artery (UAPA). About 67% of UAPA occurs isolated in the right lung. The absence of pulmonary artery can be an isolated disorder, or accompanied by other related lesions, most commonly Tetralogy of Fallot.

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  24. ↑ Fukui, Shigefumi; Ogo, Takeshi; Morita, Yoshiaki; Tsuji, Akihiro; Tateishi, Emi; Ozaki, Kumi; Sanda, Yoshihiro; Fukuda, Tetsuya; Yasuda, Satoshi (May 2014). "Right ventricular reverse remodelling after balloon pulmonary angioplasty". The European Respiratory Journal. 43 (5): 1394–1402. doi: 10.1183/09031936.00012914 . ISSN   1399-3003. PMID   24627536.
  25. ↑ Lang, Irene Marthe; Madani, Michael (2014-08-05). "Update on chronic thromboembolic pulmonary hypertension". Circulation. 130 (6): 508–518. doi: 10.1161/CIRCULATIONAHA.114.009309 . ISSN   1524-4539. PMID   25092279.
  26. ↑ Riedel, M.; Stanek, V.; Widimsky, J.; Prerovsky, I. (February 1982). "Longterm follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data". Chest. 81 (2): 151–158. doi:10.1378/chest.81.2.151. ISSN   0012-3692. PMID   7056079.
  27. ↑ Pengo, Vittorio; Lensing, Anthonie W. A.; Prins, Martin H.; Marchiori, Antonio; Davidson, Bruce L.; Tiozzo, Francesca; Albanese, Paolo; Biasiolo, Alessandra; Pegoraro, Cinzia (2004-05-27). "Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism". The New England Journal of Medicine. 350 (22): 2257–2264. doi:10.1056/NEJMoa032274. hdl: 11577/2438406 . ISSN   1533-4406. PMID   15163775.
  28. ↑ Berger, Rolf M. F.; Beghetti, Maurice; Humpl, Tilman; Raskob, Gary E.; Ivy, D. Dunbar; Jing, Zhi-Cheng; Bonnet, Damien; Schulze-Neick, Ingram; Barst, Robyn J. (2012-02-11). "Clinical features of paediatric pulmonary hypertension: a registry study". Lancet. 379 (9815): 537–546. doi:10.1016/S0140-6736(11)61621-8. ISSN   1474-547X. PMC   3426911 . PMID   22240409.
  29. ↑ Madani, Michael M.; Wittine, Lara M.; Auger, William R.; Fedullo, Peter F.; Kerr, Kim M.; Kim, Nick H.; Test, Victor J.; Kriett, Jolene M.; Jamieson, Stuart W. (March 2011). "Chronic thromboembolic pulmonary hypertension in pediatric patients". The Journal of Thoracic and Cardiovascular Surgery. 141 (3): 624–630. doi: 10.1016/j.jtcvs.2010.07.010 . ISSN   1097-685X. PMID   20800245.
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