scholarly journals Pulmonary Endarterectomy in Patients with Antiphospholipid Syndrome-Associated Chronic Thromboembolic Pulmonary Hypertension

Author(s):  
Serpil Tas ◽  
◽  
Arzu Antal ◽  
Ali Fuad Durusoy ◽  
Mehmed Yanartas ◽  
...  
2018 ◽  
Vol 24 (1) ◽  
pp. 1-8
Author(s):  
Eglė Palevičiūtė ◽  
Radvilė Malickaitė ◽  
Mindaugas Matačiūnas ◽  
Virginija Šileikienė ◽  
Lina Kryžauskaitė ◽  
...  

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


2014 ◽  
Vol 12 (4) ◽  
pp. 186-192 ◽  
Author(s):  
David Poch ◽  
Victor Pretorius

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.


2021 ◽  
pp. 204589402110136
Author(s):  
Tailong Zhang ◽  
Weitao Liang ◽  
Longrong Bian ◽  
Zhong Wu

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.


2020 ◽  
Vol 22 (Supplement_F) ◽  
pp. F30-F37
Author(s):  
Stepan Havranek ◽  
Zdenka Fingrova ◽  
David Ambroz ◽  
Pavel Jansa ◽  
Jan Kuchar ◽  
...  

Abstract Atrial fibrillation (AF) and atrial tachycardia (AT) are frequently observed in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who were treated with pulmonary endarterectomy (PEA). Their prevalence and impact on prognosis of patients are not known. We analysed the prevalence of AF/AT and the clinical outcome in 197 patients with CTEPH treated with PEA (median age 62; interquartile range 53–68 years; 62% males). The prevalence of AF/AT was 29% (57 patients). Compared to patients without arrhythmia, the subjects with AF/AT were older [60 (50–67) vs. 62 (57–70) years], manifested an increased size of the left atrium [39 (35–44) vs. 45 (40–50) mm], had a reduced 6-min walking distance [411 (321–506) vs. 340 (254–460) m], and higher pulmonary artery systolic pressure after PEA [38 (30–47) vs. 45 (38–71) mmHg], all results with P-value <0.05. During the follow-up with a median 4.2 (1.6–6.3) years, 45 (23%) patients died. In a multivariate Cox regression model only the male gender [hazard ratio (HR) 2.27, 95% confidence interval (CI) 1.15–4.50], a reduced 6-min walking distance (HR 3.67, 95% CI 1.74–7.73), and an increased New York Heart Association class (HR 8.56, 95% CI 4.17–17.60) were associated with mortality (P < 0.05). The prevalence of AF/AT in patients with CTEPH treated with PEA is high. Arrhythmias are associated with reduced functional capacity but not with mortality.


2018 ◽  
Vol 37 (2) ◽  
pp. 250-258 ◽  
Author(s):  
Mareike Lankeit ◽  
Valentin Krieg ◽  
Lukas Hobohm ◽  
Sebastian Kölmel ◽  
Christoph Liebetrau ◽  
...  

2010 ◽  
Vol 299 (4) ◽  
pp. H1083-H1091 ◽  
Author(s):  
Joost Lumens ◽  
Daniel G. Blanchard ◽  
Theo Arts ◽  
Ehtisham Mahmud ◽  
Tammo Delhaas

Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with abnormal left ventricular (LV) filling hemodynamics [mitral early passive filling wave velocity/late active filling wave velocity ( E/ A) < 1]. Pulmonary endarterectomy (PEA) acutely reduces pulmonary vascular resistance, resulting in an increase of mitral E/ A. The abolishment of leftward septal bulging and an increase in right ventricular (RV) output are thought to be responsible for the increase of mitral E/ A. In this study, we quantified the separate effects of leftward septal bulging and RV output on LV hemodynamics. In 39 CTEPH patients who underwent PEA, transmitral flow velocities and RV hemodynamic data were obtained pre- and postoperatively. A mathematical model describing the mechanics of ventricular interaction was fitted to the preoperative average values of cardiac output (CO; 4.4 l/min), mean pulmonary artery pressure (mPAP; 50 mmHg), mitral E/ A (0.74), and mean left atrial pressure (mLAP; 9.8 mmHg). Starting from this preoperative reference state with leftward septal bulging, PEA was simulated by changing mPAP and CO to average postoperative values (28 mmHg and 5.7 l/min, respectively). Simulated and postoperatively measured data on E/ A (1.27 vs. 1.48), mLAP (12.6 vs. 11.5 mmHg), and septal curvature (both rightward) were consistent. When an exclusive decrease of mPAP was simulated, mitral E/ A increased 26%, mLAP decreased 16%, and septal curvature became rightward. When an exclusive increase of CO was simulated, mitral E/ A increased 53% and mLAP increased 62%, whereas leftward septal bulging persisted. Thus, our simulations suggest that the increase of mitral E/ A with PEA is caused two-thirds by an increase of RV output and one-third by the abolishment of leftward septal bulging.


Sign in / Sign up

Export Citation Format

Share Document