Chronic thromboembolic pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2800-2804
Author(s):  
Irene M. Lang ◽  
Walter Klepetko ◽  
Hiromi Matsubara
Keyword(s):  
Pulmonary Hypertension ◽  
Gold Standard ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Vascular Remodelling ◽  
Pulmonary Endarterectomy ◽  
Medical Treatments ◽  
Group 4 ◽  
Major Vessel ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions represent group 4 in the pulmonary hypertension classification. Angiosarcoma, other intravascular tumours, arteritides, congenital pulmonary arteries stenoses, and parasites (hydatidosis) remain rare differential diagnoses within group 4. CTEPH comprises an occlusive vascular remodelling process of major vessel pulmonary thromboembolism in combination with a pulmonary arteriopathy at the pre-capillary level. The gold standard treatment has been pulmonary endarterectomy that is able to restore normal pulmonary haemodynamics at rest. Because about half of the patients are felt to be unsuited for pulmonary endarterectomy, medical treatments and balloon pulmonary angioplasty have gained grounds in the management of patients with CTEPH.

Chronic thromboembolic pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2800-2804
Author(s):  
Irene M. Lang ◽  
Walter Klepetko ◽  
Hiromi Matsubara
Keyword(s):  
Pulmonary Hypertension ◽  
Gold Standard ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Vascular Remodelling ◽  
Pulmonary Endarterectomy ◽  
Medical Treatments ◽  
Group 4 ◽  
Major Vessel ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions represent group 4 in the pulmonary hypertension classification. Angiosarcoma, other intravascular tumours, arteritides, congenital pulmonary arteries stenoses, and parasites (hydatidosis) remain rare differential diagnoses within group 4. CTEPH comprises an occlusive vascular remodelling process of major vessel pulmonary thromboembolism in combination with a pulmonary arteriopathy at the pre-capillary level. The gold standard treatment has been pulmonary endarterectomy that is able to restore normal pulmonary haemodynamics at rest. Because about half of the patients are felt to be unsuited for pulmonary endarterectomy, medical treatments and balloon pulmonary angioplasty have gained grounds in the management of patients with CTEPH.

Pulmonary Endarterectomy: Assessment of Operability, Surgical Description, and Post-op Care

2014 ◽  
Vol 12 (4) ◽  
pp. 186-192 ◽  
Author(s):  
David Poch ◽  
Victor Pretorius
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Artery Wedge Pressure ◽  
Pulmonary Endarterectomy ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Wedge Pressure

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a mean pulmonary artery pressure ≥25 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg in the presence of occlusive thrombi within the pulmonary arteries. Surgical pulmonary thromboendarterectomy (PTE) is considered the best treatment option for CTEPH.

scholarly journals Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension

2017 ◽  
Vol 26 (143) ◽  
pp. 160111 ◽  
Author(s):  
David Jenkins ◽  
Michael Madani ◽  
Elie Fadel ◽  
Andrea Maria D'Armini ◽  
Eckhard Mayer
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Algorithm ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
European Respiratory Society ◽  
Complex Procedure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Definition Of ◽  
European Society Of Cardiology

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature.Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are <5% and survival is >90% at 1 year and >70% at 10 years. However, PEA, is a complex procedure and relies on a multidisciplinary CTEPH team led by an experienced surgeon to decide on an individual's operability, which is determined primarily by lesion location and the haemodynamic parameters. Therefore, treatment of patients with CTEPH depends largely on subjective judgements of eligibility for surgery by the CTEPH team.Other controversies discussed in this article include eligibility for PEA versus balloon pulmonary angioplasty, the new treatment algorithm in the European Society of Cardiology/European Respiratory Society guidelines and the definition of an “expert centre” for the management of this condition.

scholarly journals Case report: Life-saving pulmonary endarterectomy for a young female with primary antiphospholipid syndrome complicated by severe chronic thromboembolic pulmonary hypertension

2018 ◽  
Vol 24 (1) ◽  
pp. 1-8
Author(s):  
Eglė Palevičiūtė ◽  
Radvilė Malickaitė ◽  
Mindaugas Matačiūnas ◽  
Virginija Šileikienė ◽  
Lina Kryžauskaitė ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Antiphospholipid Syndrome ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Favourable Outcome ◽  
Surgical Removal ◽  
Severe Thrombocytopenia ◽  
Primary Antiphospholipid Syndrome ◽  
Pulmonary Endarterectomy ◽  
Thromboembolic Pulmonary Hypertension

Abstract Background: Prothrombotic state and impaired clot dissolution can contribute to the occurrence of chronic thromboembolic pulmonary hypertension in primary antiphospholipid syndrome. Pulmonary endarterectomy - the surgical removal of the organized thromboembolic material from the proximal pulmonary arteries - is the procedure of choice and potentially a curative option for patients with chronic thromboembolic pulmonary hypertension, including patients with antiphospholipid syndrome. We report an exceptionally severe and complicated case with favourable outcome. Case presentation: We present a case of a successful high risk pulmonary endarterectomy in a 29-year-old female with primary antiphospholipid syndrome and end-stage chronic thromboembolic pulmonary hypertension. Despite highly complicated perioperative course an impressive improvement in symptoms and functional status was achieved. Conclusion: We hope that this complicated but successfully managed case of a combination of two rare diseases will arouse earlier suspicion and timely diagnosis for such patients and will encourage physicians to promptly refer the suitable patients to a pulmonary endarterectomy team. It is important to remember that severe thrombocytopenia may occur in patients with antiphospholipid syndrome

scholarly journals Pulmonary endarterectomy: the potentially curative treatment for patients with chronic thromboembolic pulmonary hypertension

2015 ◽  
Vol 24 (136) ◽  
pp. 263-271 ◽  
Author(s):  
David Jenkins
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Resistance ◽  
Circulatory Arrest ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Ventricular Dysfunction ◽  
Operative Management ◽  
Deep Hypothermic Circulatory Arrest ◽  
Pulmonary Endarterectomy ◽  
Thromboembolic Pulmonary Hypertension

Pulmonary endarterectomy (PEA) is the treatment of choice to relieve pulmonary artery obstruction in patients with chronic thromboembolic pulmonary hypertension (CTEPH). It is a complex surgical procedure with a simple principle: removal of obstructive thromboembolic material from the pulmonary arteries in order to reduce pulmonary vascular resistance, relieve pulmonary hypertension (PH) and alleviate right ventricular dysfunction. In the majority of patients there is symptomatic and prognostic benefit. However, not all patients with CTEPH are suitable for treatment with PEA. Operability assessment is not always easy, being largely subjective and based on experience. It is therefore important that all patients are referred to an experienced CTEPH centre for careful evaluation of suitability for surgery. The most common reason for inoperability is distal vasculopathy accounting for a high proportion of the vascular resistance. Surgery requires cardiopulmonary bypass and periods of deep hypothermic circulatory arrest. Complications include reperfusion lung injury and persistent PH. However, with careful patient selection, surgical technique and post-operative management, PEA is a highly effective treatment with mortality rates <5% at experienced centres. Patients who are unsuitable for surgery may be candidates for medical therapy.

scholarly journals PULMONARY ENDARTERECTOMY FOR CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION

2021 ◽  
Vol 54 (2) ◽  
pp. 126-131
Author(s):  
Tahir Iqbal ◽  
Azam Jan ◽  
Naseer Ahmed ◽  
Amir Muhammad ◽  
Sayed Mumtaz Shah ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Cardiopulmonary Bypass ◽  
Circulatory Arrest ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Medical Institute ◽  
Pulmonary Endarterectomy ◽  
Operative Outcome ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious complication of unresolved pulmonary embolism. CTEPH is a potentially curable disease and the treatment of choice is pulmonary endarterectomy (PEA) with complete clearance of pulmonary arteries being the principle of surgery. The surgery is performed under circulatory arrest during cardiopulmonary bypass circulation. We report 3 cases of CTEPH in 2018-2019 at department of cardiothoracic surgery, Rehman Medical Institute, Peshawar. They were detected by echocardiography (TTE) and confirmed by CTPA. Pulmonary endarterectomy was performed with good peri-operative outcome and significant improvement of hemodynamics.

scholarly journals Microvascular disease in chronic thromboembolic pulmonary hypertension: a role for pulmonary veins and systemic vasculature

2014 ◽  
Vol 44 (5) ◽  
pp. 1275-1288 ◽  
Author(s):  
Peter Dorfmüller ◽  
Sven Günther ◽  
Maria-Rosa Ghigna ◽  
Vincent Thomas de Montpréville ◽  
David Boulate ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Veins ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Microvascular Disease ◽  
Pulmonary Endarterectomy ◽  
Functional Importance ◽  
Lung Histology ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of

Limited numbers of operated patients with chronic thromboembolic pulmonary hypertension (CTEPH) are refractory to pulmonary endarterectomy (PEA) and experience persistent pulmonary hypertension (PH).We retrospectively assessed lung histology available from nine patients with persistent PH (ineffective PEA (inPEA) group) and from eight patients transplanted for distal CTEPH inaccessible by PEA (noPEA group). Microscopically observed peculiarities were compared with the histology of a recently developed CTEPH model in piglets. Pre-interventional clinical/haemodynamic data and medical history of patients from the inPEA and noPEA groups were collected and analysed.Conspicuous remodelling of small pulmonary arteries/arterioles, septal veins and pre-septal venules, including focal capillary haemangiomatosis, as well as pronounced hypertrophy and enlargement of bronchial systemic vessels, were the predominant pattern in histology from both groups. Most findings were reproduced in our porcine CTEPH model. Ink injection experiments unmasked abundant venular involvement in so-called small vessel or microvascular disease, as well as post-capillary bronchopulmonary shunting in human and experimental CTEPH.Microvascular disease is partly due to post-capillary remodelling in human and experimental CTEPH and appears to be related to bronchial-to-pulmonary venous shunting. Further studies are needed to clinically assess the functional importance of this finding.

P4675The utility of additional balloon pulmonary angioplasty for residual hypoxemia in normohemodynamic chronic thromboembolic pulmonary hypertension patients after invasive treatments

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
H Shimokawahara ◽  
H Matsubara ◽  
K Hayashi ◽  
M Tsuji ◽  
T Nishihara ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Multivariate Logistic Regression Analysis ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
Initial Series ◽  
Oxygen Inhalation ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Vascular Obstruction

Abstract Background Balloon pulmonary angioplasty (BPA) improves hemodynamics, symptoms and exercise capacity in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy (PEA). However, certain patients still have hypoxemia after BPA or PEA despite normalization of hemodynamics. In CTEPH, hypoxemia is related increased dead space ventilation caused by vascular obstruction. Purpose This study was aimed to clarify whether additional BPA can improve hypoxemia of CTEPH patients after normalization of hemodynamics. Methods A total of 335 patients who underwent initial series of BPA in our institute were followed up. Sixty-four patients with mean pulmonary artery pressure (mPAP) <30mmHg and percutaneous oxygen saturation (SpO2) <95% without oxygen inhalation at more than 6 months after the initial series of BPA and of patients who could reevaluate hemodynamics and oxygenation after additional BPA were enrolled. These patients were divided into two groups with or without additional BPA procedures. Change of hemodynamics and SpO2 were retrospectively investigated. Results Thirty-three of 64 patients underwent additional BPA procedures. Patients' age was older in BPA group than those in non-BPA group (71.3±10.4 vs. 66.5±9.4 years old, p=0.02). mPAP and pulmonary vascular resistance (PVR) was significantly higher in BPA group (mPAP: 23.9±3.2 vs. 20.7±3.8 mmHg, p=0.001, PVR: 4.2±1.2 vs. 3.5±1.4 wood unit, p=0.03, respectively). Among the 1.8±1.4 BPA procedures per person, total 6.6±3.8 segmental pulmonary arteries per person were treated. While no obvious improvements were observed in non-BPA group, PVR and SpO2 in BPA group were significantly improved (4.2±1.2 to 3.7±1.3 wood unit, p=0.002, 90.7±3.1% to 94.1±3.6%, p<0.001, respectively). In the multivariate logistic regression analysis, additional BPA procedures were associated with further improvement of SpO2 (hazard ratio, 3.7; 95% confidence interval, 1.2–11.5; P=0.02). Conclusions Additional BPA procedure was associated with improvement of hypoxemia in CTEPH patients after normalization of hemodynamics. Treating as many lesions as possible in BPA might relieve the patients' residual dyspnea.

scholarly journals EXPRESS: Chronic thromboembolic pulmonary hypertension following pulmonary embolism with a right ventricular thrombus: A report of two cases

2021 ◽  
pp. 204589402110136
Author(s):  
Tailong Zhang ◽  
Weitao Liang ◽  
Longrong Bian ◽  
Zhong Wu
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Imaging Features ◽  
Pulmonary Endarterectomy ◽  
Right Heart ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Right ◽  
Cardiac Masses

Right heart thrombus (RHT) accompanied by chronic thromboembolic pulmonary hypertension (CTEPH) is a rare entity. RHT may develop in the peripheral veins or in situ within the right heart chambers. The diagnosis of RHT is challenging, since its symptoms are typically non-specific and its imaging features resemble those of cardiac masses. Here, we report two cases of RHT with CTEPH that presented as right ventricular masses initially. Both patients underwent simultaneous pulmonary endarterectomy (PEA) and resection of the ventricular thrombi. Thus, when mass-like features are confirmed by imaging, RHT should be suspected in patients with CTEPH, and simultaneous RHT resection is required along with PEA.

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