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

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.

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.

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.

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.

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 The changing landscape of chronic thromboembolic pulmonary hypertension management

2017 ◽  
Vol 26 (146) ◽  
pp. 170105 ◽  
Author(s):  
Michael Madani ◽  
Takeshi Ogo ◽  
Gérald Simonneau
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Options ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Standard Of Care ◽  
Surgical Removal ◽  
Pulmonary Endarterectomy ◽  
Current Standard ◽  
Beneficial Impact ◽  
Background Therapy ◽  
Thromboembolic Pulmonary Hypertension

For patients with chronic thromboembolic pulmonary hypertension (CTEPH), the current standard of care involves surgical removal of fibro-thrombotic obstructions by pulmonary endarterectomy. While this approach has excellent outcomes, significant proportions of patients are not eligible for surgery or suffer from persistent/recurrent pulmonary hypertension after the procedure. The availability of balloon pulmonary angioplasty and the approval of the first medical therapy for use in CTEPH have significantly improved the outlook for patients ineligible for pulmonary endarterectomy. In this comprehensive review, we discuss the latest developments in the rapidly evolving field of CTEPH. These include improvements in imaging modalities and advances in surgical and interventional techniques, which have broadened the range of patients who may benefit from such procedures. The efficacy and safety of targeted medical therapies in CTEPH patients are also discussed, particularly the encouraging data from the recent MERIT-1 trial, which demonstrated the beneficial impact of using macitentan to treat patients with inoperable CTEPH, including those on background therapy. As the treatment options for CTEPH improve, hybrid management involving more than one intervention in the same patient may become a viable option in the near future.

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.

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