scholarly journals Chronic thromboembolic pulmonary hypertension: anticoagulation and beyond

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 478-484
Author(s):  
Karlyn A. Martin ◽  
Michael J. Cuttica
Keyword(s):  
Pulmonary Hypertension ◽  
Residual Disease ◽  
Rare Complication ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
High Morbidity ◽  
Functional Evaluation ◽  
Pulmonary Thromboendarterectomy ◽  
Pulmonary Vasodilator ◽  
Hemodynamic Assessment ◽  
Thromboembolic Pulmonary Hypertension

Abstract Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication in pulmonary embolism (PE) survivors, characterized by chronic vascular occlusion and pulmonary hypertension. The identification and diagnosis of CTEPH requires a stepwise approach, starting with symptom evaluation, functional evaluation, screening imaging, and progressing to interventional hemodynamic assessment. On the backbone of anticoagulation, CTEPH management necessitates a multidisciplinary approach. Surgical pulmonary thromboendarterectomy (PTE) is the only potentially curative option. In nonoperable disease or residual disease after PTE, interventional balloon pulmonary angioplasty and/or pulmonary-vasodilator therapies can be offered, in collaboration with interventional and vascular pulmonary colleagues. As it is a disease that can cause high morbidity and mortality, CTEPH requires a high index of suspicion to diagnose and treat in patients following PE.

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.

Pulmonary thromboendarterectomy is a curative resolution for chronic thromboembolic pulmonary hypertension associated with antiphospholipid syndrome: a retrospective cohort study

Lupus ◽  
2018 ◽  
Vol 27 (14) ◽  
pp. 2206-2214 ◽  
Author(s):  
C Li ◽  
J Zhao ◽  
S Liu ◽  
W Song ◽  
J Zhu ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Cohort Study ◽  
Cardiac Function ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Short Term ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and life-threatening condition with poor prognosis in patients with antiphospholipid syndrome (APS). Pulmonary thromboendarterectomy (PTE) is the optimal surgical option for CTEPH. Objectives This retrospective cohort study aimed to evaluate the efficacy and risk of PTE in patients with APS-associated CTEPH. Methods Consecutive patients with APS-associated CTEPH diagnosed between January 2012 and September 2017 at Peking Union Medical College Hospital were retrospectively evaluated. Demographics, clinical manifestations, antiphospholipid antibody (aPL) profiles, and pulmonary arterial hypertension–targeted medications were collected. Deterioration of cardiac function and death were chosen as the endpoints, in order to assess the effect of PTE on short-term and long-term prognoses (evaluated by the change of cardiac function after treatment and cardiac deterioration or death in the follow-up, respectively). Results A total of 20 patients with APS-associated CTEPH were enrolled, and eight patients underwent PTE. Chi-square test ( p = 0.01) and Kaplan–Meier curves (log rank test, p = 0.04) showed that there were statistically significant differences in both short-term and long-term prognoses between patients with and without PTE. Conclusion These results provide strong evidence that PTE is a curative resolution in patients with APS-associated CTEPH. Following a full specialized and multidisciplinary risk-benefit evaluation to limit the risk of thrombosis or bleeding and to manage possible thrombocytopenia, PTE is at least a temporal curative resolution for CTEPH complicated with APS.

scholarly journals The soluble guanylate cyclase stimulator riociguat is the first-line therapy for chronic thromboembolic pulmonary hypertension patients

Kardiologiia ◽  
2020 ◽  
Vol 60 (8) ◽  
pp. 115-123
Author(s):  
Z. S. Valieva ◽  
S. E. Gratsianskaya ◽  
T. V. Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Guanylate Cyclase ◽  
Soluble Guanylate Cyclase ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
First Line ◽  
Pulmonary Thromboendarterectomy ◽  
First Line Therapy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Microcirculation

Chronic thromboembolic pulmonary hypertension (CTEPH) is a precapillary type of pulmonary hypertension with chronic obstruction of large and medium branches of pulmonary arteries along with secondary alterations in pulmonary microcirculation, which cause progressive increases in pulmonary vascular resistance and pulmonary arterial pressure and ensuing severe right heart dysfunction and heart failure. Pulmonary thromboendarterectomy (PTE) is the treatment of choice for CTEPH; however, this procedure is available not for all patients. Although the surgery performed in the conditions of centers with advanced experience generally shows good results, up to 40% of patients are technically inoperable or PTE is associated with a high risk of complications. At present, riociguat, the only officially approved drug from the class of soluble guanylate cyclase stimulators, is considered as a first-line treatment for inoperable and residual forms of STEPH. Introduction of riociguat to clinical practice can be called a real breakthrough in the treatment of patients with STEPH who cannot undergo PTE or those with relapse or persistent STEPH after the surgery.

Chronic thromboembolic pulmonary hypertension secondary to implantable cardioverter defibrillator lead thrombus in a patient with Brugada syndrome: a rare complication requiring a multidisciplinary approach

2020 ◽  
Vol 13 (5) ◽  
pp. e234549
Author(s):  
Sofia Alegria ◽  
Filipa Ferreira ◽  
Débora Repolho ◽  
Maria José Loureiro
Keyword(s):  
Pulmonary Hypertension ◽  
Implantable Cardioverter Defibrillator ◽  
Brugada Syndrome ◽  
Rare Complication ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
Heart Team ◽  
Cardioverter Defibrillator ◽  
Thromboembolic Pulmonary Hypertension ◽  
Appropriate Shocks

We report the case of a 57-year-old male patient with prior syncope associated with sustained ventricular tachycardia in the setting of Brugada syndrome, who was submitted to implantation of a cardioverter defibrillator for secondary prevention. During follow-up, he presented a significant increase in lead impedance, and a transthoracic echocardiogram showed a mass attached to the lead. He was started on oral anticoagulation after infective endocarditis was excluded but nevertheless suffered repeated episodes of pulmonary embolism that led to severe chronic thromboembolic pulmonary hypertension. After heart team discussion, he was referred to pulmonary endarterectomy and replacement of the implantable cardioverter defibrillator with a subcutaneous device. This led to significant improvement of functional class and normalisation of pulmonary haemodynamics. More recently, he suffered syncope in the setting of ventricular fibrillation with appropriate shocks and was started on quinidine without further recurrence of arrhythmic episodes.

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