scholarly journals A Healthy Young Lady with ‘Unprovoked’ Persistent Bilateral Pulmonary Embolism - Why?

2021 ◽  
Vol 5 (0-2) ◽  
pp. 25
Author(s):  
Muhammad Farid Bin Mohd Fauad ◽  
Hazlyna Baharuddin ◽  
Mohd Arif Mohd Zim ◽  
Bushra Johari
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Clinical Manifestations ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Normal Lung ◽  
Exertional Dyspnoea ◽  
Strain Pattern ◽  
Right Ventricular Strain

Pulmonary embolism (PE) was reported in about 9% patients with antiphospholipid syndrome (APS). Seronegative APS is an entity which demonstrates clinical manifestations highly suggestive of APS but persistently negative APS antibodies. A 31-year-old lady presented with a two-month history of exertional dyspnoea. She had two consecutive miscarriages at 12 and 14 weeks, previously. Physical examination revealed a thin lady who was tachycardic, tachypneic, hypoxic but normotensive. There was a loud P2 without signs of heart failure. Investigations revealed a type 1 respiratory failure, sinus tachycardia with right ventricular strain pattern, cardiomegaly with normal lung fields, and dilated right atrium and right ventricle with increased in pulmonary arterial pressure of 70mmHg from echocardiography. CT pulmonary angiography (CTPA) confirmed the presence of PE over bilateral pulmonary arteries. She continued to have exertional dyspnoea and was readmitted 9 months later with worsening dyspnoea. APS antibodies performed during both admissions were negative. Seronegative APS was diagnosed. Interestingly, two CTPAs performed at 6 months and 9 months after initial presentation revealed persistent bilateral pulmonary embolism. The provoking factors for PE should be sought because ‘unprovoked’ PE especially in young individuals need further attention. APS, including seronegative APS, should be considered. Persistence of symptoms of PE also warrants further attention as chronic thromboembolic pulmonary hypertension (CTEPH) may be the cause. Currently, there are available medical and surgical treatment of CTEPH, therefore establishing its diagnosis is important and it is best performed in pulmonary hypertension expert centre.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S25

scholarly journals Chronic Thromboembolic Pulmonary Hypertension Medical Management

2021 ◽  
Vol 17 (2) ◽  
pp. e28-e32
Author(s):  
Ryan Logue ◽  
Zeenat Safdar
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Management ◽  
Soluble Guanylate Cyclase ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Standard Of Care ◽  
Pulmonary Angiography ◽  
Thromboembolic Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase Stimulator

Chronic thromboembolic pulmonary hypertension (CTEPH) is a common long-term complication of pulmonary embolism characterized by thromboembolic obstruction of the pulmonary arteries, vascular arteriopathy, vascular remodeling, and ultimately pulmonary hypertension (PH). Although pulmonary endarterectomy (PEA) surgery is the standard of care, approximately 40% of patients in the international CTEPH registry were deemed inoperable. In addition to lifelong anticoagulation, the cornerstone of PH-specific medical management is riociguat, a soluble guanylate cyclase stimulator. Medical management should be started early in CTEPH patients and may be used as a bridge to PEA surgery or balloon pulmonary angiography. Medical management is indicated for inoperable CTEPH patients and patients who have recurrence of PH after PEA surgery.

scholarly journals Clinical Updates on the Diagnosis and Management of Chronic Thromboembolic Pulmonary Hypertension

2020 ◽  
Vol 49 (5) ◽  
pp. 320-330
Author(s):  
Wen Ruan ◽  
Jonathan JL Yap ◽  
Kevin KH Quah ◽  
Foong Koon Cheah ◽  
Ghee Chee Phuah ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Residual Disease ◽  
Treatment Options ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Pulmonary Endarterectomy ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Cardinal Symptom

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a known sequela after acute pulmonary embolism (PE). It is a debilitating disease, and potentially fatal if left untreated. This review provides a clinically relevant overview of the disease and discusses the usefulness and limitations of the various investigational and treatment options. Methods: A PubMed search on articles relevant to pulmonary embolism, pulmonary hypertension, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, and balloon pulmonary angioplasty were performed. A total of 68 articles were found to be relevant and were reviewed. Results: CTEPH occurs as a result of non-resolution of thrombotic material, with subsequent fibrosis and scarring of the pulmonary arteries. Risk factors have been identified, but the underlying mechanisms have yet to be fully elucidated. The cardinal symptom of CTEPH is dyspnoea on exertion, but the diagnosis is often challenging due to lack of awareness. The ventilation/perfusion scan is recommended for screening for CTEPH, with other modalities (eg. dual energy computed tomography pulmonary angiography) also being utilised in expert centres. Conventional pulmonary angiography with right heart catherisation is important in the final diagnosis of CTEPH. Conclusion: Operability assessment by a multidisciplinary team is crucial for the management of CTEPH, as pulmonary endarterectomy (PEA) remains the guideline recommended treatment and has the best chance of cure. For inoperable patients or those with residual disease post-PEA, medical therapy or balloon pulmonary angioplasty are potential treatment options. Keywords: Balloon pulmonary angioplasty, Chronic thromboembolic pulmonary hypertension, Pulmonary embolism, Pulmonary endarterectomy, Pulmonary hypertension

Chronic Thromboembolic Pulmonary Hypertension: When to Suspect It, When to Refer for Surgery

2003 ◽  
Vol 2 (1) ◽  
pp. 4-9 ◽  
Author(s):  
Kim M. Kerr ◽  
Peter F. Fedullo ◽  
William R. Auger
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Acute Pulmonary Embolism ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The United States ◽  
Thromboembolic Disease ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Chronic Thromboembolic Disease

Chronic thromboembolic obstruction of the major pulmonary arteries is an underrecognized sequela of acute pulmonary embolism. Depending on the burden and location of thrombus, as well as on the duration of vessel obstruction, chronic thromboembolic disease may lead to pulmonary hypertension and cor pulmonale. Chronic thromboembolic disease affects an estimated 500 to 2500 patients each year in the United States, roughly 0.1 to 0.5 percent of patients who survive acute pulmonary embolism. Consequently, while this disease is uncommon, chronic thromboembolic pulmonary hypertension (CTEPH) is not rare, and should be considered in patients with unexplained dyspnea, as it is potentially correctible with pulmonary thromboendarterectomy.1

scholarly journals From pulmonary embolism to chronic thromboembolic pulmonary hypertension: risk factors

2021 ◽  
Vol 25 (3) ◽  
pp. 11
Author(s):  
O. Ya. Vasiltseva ◽  
A. G. Edemskiy ◽  
D. S. Grankin ◽  
E. N. Kliver ◽  
A. M. Chernyavskiy
Keyword(s):  
Risk Factors ◽  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Recurrence Risk ◽  
National Guidelines ◽  
Thromboembolic Pulmonary Hypertension

<p>Chronic thromboembolic pulmonary hypertension is a long-term consequence of acute pulmonary embolism. Gradual obstruction of the pulmonary arteries and secondary changes in the pulmonary microcirculation over time cause progressive increases in pulmonary vascular resistance and pulmonary artery pressure that can result in severe right heart failure. This article provides an overview of pulmonary embolism and chronic thromboembolic pulmonary hypertension scientific literature and national guidelines. We focus on disease and recurrence risk factors and outline future directions of research to improve short- and long-term patient outcomes.</p><p>Received 17 January 2021. Revised 1 March 2021. Accepted 19 April 2021.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> The authors declare no conflicts of interests.</p><p><strong>Contribution of the authors</strong><br />Conception and study design: A.M. Chernyavskiy, A.G. Edemskiy, D.S. Grankin, E.N. Kliver<br />Drafting the article: O.Ya. Vasiltseva<br />Critical revision of the article: E.N. Kliver<br />Final approval of the version to be published: O.Ya. Vasiltseva, A.G. Edemskiy, D.S. Grankin, E.N. Kliver, A.M. Chernyavskiy</p>

scholarly journals Molecular Research in Chronic Thromboembolic Pulmonary Hypertension

2019 ◽  
Vol 20 (3) ◽  
pp. 784 ◽  
Author(s):  
Isabelle Opitz ◽  
Michaela Kirschner
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Current Knowledge ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pi3k Signaling ◽  
Molecular Alterations ◽  
Complex Processes ◽  
Thromboembolic Pulmonary Hypertension ◽  
Differential Gene

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a debilitating disease, for which the underlying pathophysiological mechanisms have yet to be fully elucidated. Occurrence of a pulmonary embolism (PE) is a major risk factor for the development of CTEPH, with non-resolution of the thrombus being considered the main cause of CTEPH. Polymorphisms in the α-chain of fibrinogen have been linked to resistance to fibrinolysis in CTEPH patients, and could be responsible for development and disease progression. However, it is likely that additional genetic predisposition, as well as genetic and molecular alterations occurring as a consequence of tissue remodeling in the pulmonary arteries following a persistent PE, also play an important role in CTEPH. This review summarises the current knowledge regarding genetic differences between CTEPH patients and controls (with or without pulmonary hypertension). Mutations in BMPR2, differential gene and microRNA expression, and the transcription factor FoxO1 have been suggested to be involved in the processes underlying the development of CTEPH. While these studies provide the first indications regarding important dysregulated pathways in CTEPH (e.g., TGF-β and PI3K signaling), additional in-depth investigations are required to fully understand the complex processes leading to CTEPH.

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 Selective Segmental Pulmonary Angiography: Anatomical, Technical and Safety Aspects of a Must-Learn Technique in Times of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension

2021 ◽  
Vol 10 (15) ◽  
pp. 3358
Author(s):  
Maite Velázquez ◽  
Nicolás Maneiro ◽  
Ana Lareo ◽  
Agustín Albarrán ◽  
Sergio Huertas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Imaging Modality ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Subtraction Technique ◽  
Manual Skill ◽  
Appropriate Treatment ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

With the advent of balloon pulmonary angioplasty (BPA) for non-surgical chronic thromboembolic pulmonary hypertension (CTEPH) patients, there is renewed interest in the pulmonary angiography technique. This technique is still the standard imaging modality to confirm CTEPH, which, in addition, helps to determine the most appropriate treatment. Furthermore, learning this technique fulfills two main purposes: to identify BPA candidates and to provide the operator with the catheter handling needed to perform BPA. Operators interested in performing BPA must learn not only the pulmonary arteries’ anatomy, but also which are the best angiographic projections and the most suitable catheters to canalize and display each segmental branch. Unfortunately, this information is scarce in the literature. With this goal, learning the diagnostic pulmonary angiography technique can be a first step on the way to perform BPA. Although there are descriptions on how to perform a pulmonary angiography with balloon-tipped catheters and the digital subtraction technique, this technique does not provide operators with the catheter knowledge and manual skill needed to cannulate each segmental branch. In contrast, learning the conventional selective segmental pulmonary angiography (SSPA) technique provides the operator with this knowledge and skills. In this review, based on the experience of the authors, we describe the pulmonary arteries’ anatomy and detail the practical aspects of the SSPA procedure, with the aim of providing operators with the anatomical and technical knowledge needed to perform BPA. We also summarize the contemporary complications of SSPA in CTEPH patients at a reference center.

P4671A well-defined subgroup of chronic thromboembolic pulmonary hypertension patients are positive for antiphospholipid antibodies

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Denas ◽  
C Chen ◽  
Y Du ◽  
X Jiang ◽  
Y Zhou ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Risk Factor ◽  
Antiphospholipid Antibodies ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Angiography ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Thromboembolic Pulmonary Hypertension

Abstract Introduction Recurrent thromboembolism is a major risk factor for chronic thromboembolic pulmonary hypertension (CTEPH). Antiphospholipid antibodies (aPL) are a recognized risk factor for recurrent thromboembolism, however, their contribution to the development of CTEPH is currently unknown. Purpose The aim of this study was to evaluate the prevalence of antiphospholipid autoantibodies in a large cohort of consecutive patients with CTEPH. Methods Starting May 2013 to December 2018, 297 CTEPHpatients were consecutively enrolled. Diagnosis was confirmed with standardized right heart catheterization, 2D or/and 3D pulmonary angiography in all patients.All patients were also screened for thrombophilia including lupus anticoagulant IgG/IgM anticardiolipin antibodies and IgG/IgM aβ2GPI antibodies. Clinical and demographic characteristics, laboratory profile and hemodynamic data were compared between patients with aPL positive and aPL negative laboratory profiles. Results Overall, 297 consecutive patients with CTEPH were assessed for aPLs. Of these, 23 patients (7.7%) resulted positive for laboratory tests exploring aPL. Among patients with positive aPL, profile 17 (74%) were positive for all three test exploring aPL (LA+, aCL+, aB2GPI+). When compared to CTEPH patients without aPL test positivity, those positive for aPL were significantly younger (30.0 vs 55.6 years, p<0.0001), had a positive history of pulmonary embolism (95.6% vs 65.7%, p=0.003), deep venous thrombosis (78.3% vs 41.9%, p=0.0008) and were more frequently affected by other autoimmune diseases (43.5% vs 2.9%, p<0.0001). Moreover, positive aPL patients showed better hemodynamics on right heart catheterization, had more proximal lesions, and had more frequently level I and II surgical classification (82.6% vs 40.5%, p=0.0002) on pulmonary angiography. Conclusions These findings suggest that young patients with pulmonary embolism should be assessed for aPLs to guide anticoagulation therapy and prevent recurrences. Additionally, younger age, more proximal lesions and less severe hemodynamic profiles make majority patients with APS good candidates for PEA surgery.

scholarly journals Pulmonary hypertension: when the acute event leads to diagnosis. Case report in a patient with Ehlers-Danlos syndrome.

2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Gianmarco Secco ◽  
Anna Giulia Falchi ◽  
Francesco Salinaro ◽  
Chiara Blatti ◽  
Bianca Giacomuzzi Moore ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Favourable Outcome ◽  
Vascular Pathology ◽  
Ehlers Danlos Syndrome ◽  
Thromboembolic Pulmonary Hypertension ◽  
Work Up ◽  
Danlos Syndrome

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a pulmonary vascular pathology caused by the chronic obstruction of the major pulmonary arteries, usually being the consequence of recurrent episodes of pulmonary embolism. Such events are usually unknown; the delay in such a diagnosis can therefore lead to a deterioration of the clinical picture, worsening the overall prognosis.  This is a case of a 55-year-old man who came to the Emergency Room (ER) because of an acute exacerbation of chronic dyspnea, that he experienced for several years, in the absence of an explanatory diagnosis. Acute pulmonary embolism was diagnosed in the setting of the ER, with bedside echography resulting to be crucial to the work up. A multidisciplinary approach allowed proper treatment, management and a favourable outcome.

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