Clinical Worsening During Long-Term Follow-Up in Inoperable Chronic Thromboembolic Pulmonary Hypertension

Lung ◽  
2011 ◽  
Vol 190 (2) ◽  
pp. 161-167 ◽  
Author(s):  
Bastiaan E. Scholzel ◽  
Martijn C. Post ◽  
H. W. Thijs Plokker ◽  
Repke J. Snijder
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Long Term Follow Up ◽  
Thromboembolic Pulmonary Hypertension ◽  
Clinical Worsening

scholarly journals Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 53 (6) ◽  
pp. 1802004 ◽  
Author(s):  
Marc Humbert ◽  
Harrison W. Farber ◽  
Hossein-Ardeschir Ghofrani ◽  
Raymond L. Benza ◽  
Dennis Busse ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Risk Stratification ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Low Risk ◽  
Risk Stratification Model ◽  
Risk Stratum ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Clinical Worsening

BackgroundCurrent pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension (PAH) in the French, Swedish and COMPERA registries. Our objective was to investigate the three abbreviated risk stratification methods for patients with mostly prevalent PAH and chronic thromboembolic pulmonary hypertension (CTEPH), in patients from the PATENT-1/2 and CHEST-1/2 studies of riociguat.MethodsRisk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.ResultsWith all three methods, riociguat improved risk group/strata in patients with PAH after 12 weeks. The French non-invasive and Swedish/COMPERA methods discriminated prognosis for survival and clinical worsening-free survival at both baseline and follow-up. Furthermore, patients achieving one or more low-risk criteria or a low-risk stratum at follow-up had a significantly reduced risk of death and clinical worsening compared with patients achieving no low-risk criteria or an intermediate-risk stratum. Similar results were obtained in patients with inoperable or persistent/recurrent CTEPH.ConclusionsThis analysis confirms and extends the results of the registry analyses, supporting the value of goal-oriented treatment in PAH. Further assessment of these methods in patients with CTEPH is warranted.

scholarly journals Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report

2017 ◽  
Vol 87 (3) ◽  
Author(s):  
Paolo Ruggeri ◽  
Federica Lo Bello ◽  
Francesco Nucera ◽  
Michele Gaeta ◽  
Francesco Monaco ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Nephrotic Syndrome ◽  
Main Pulmonary Artery ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Exercise Intolerance ◽  
Artery Thrombosis ◽  
Stable Conditions ◽  
Thromboembolic Pulmonary Hypertension

We present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atypical resection of the left upper pulmonary lobe and the starting of long term anticoagulation the patient was discharged but did not attend the planned follow up visits until one year later when he was seen again for severe dyspnea and exercise intolerance. At this time chronic thromboembolic pulmonary hypertension (CTEPH) was diagnosed by lung perfusion scintigraphy and right heart catheterization. He initially refused the surgical treatment but, after six months, for the presence of worsening dyspnea was referred for bilateral pulmonary endarterectomy followed by a cardio-thoracic rehabilitation program. After a follow-up of seven years the patient is alive and in stable conditions. NS and hyperhomocysteinemia are both known risk factors for pulmonary embolism (PE), but their association with CTEPH is extremely rare. We discuss here the possible mechanisms linking these conditions. CTEPH must be suspected in any patient with NS, with or without hyperhomocysteinemia, and unexplained dyspnea.

Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension

1997 ◽  
Vol 5 (1) ◽  
pp. 35-38
Author(s):  
Gao Chang Qing ◽  
Rodolfo Barragan Garcia
Keyword(s):  
Pulmonary Hypertension ◽  
Median Sternotomy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Thromboendarterectomy ◽  
Pulmonary Hemodynamic ◽  
Major Vessel ◽  
Thromboembolic Pulmonary Hypertension ◽  
Hemodynamic Improvement

We describe our experience of treating chronic major vessel thromboembolic pulmonary hypertension with pulmonary thromboendarterectomy. Thromboendarterectomy was performed in 15 patients through a median sternotomy incision using extracorporeal circulation and deep hypothermia. Postoperatively mean pulmonary artery pressure, mean pulmonary vascular resistance, and cardiac index were significantly improved (p < 0.001). Four patients died during surgery, mainly from perfusion edema (operative mortality 27%). The 11 surviving patients received long-term anticoagulant therapy and no incidence of thromboembolism has occurred during the follow-up period. All have shown sustained pulmonary hemodynamic improvement; however, there were 2 late deaths from cardiac failure. We conclude that thromboendarterectomy is an effective treatment for chronic major vessel thromboembolic pulmonary hypertension.

scholarly journals INITIAL RESULTS AND LONG-TERM FOLLOW-UP OF PERCUTANEOUS MITRAL VALVULOPLASTY IN PATIENTS WITH PULMONARY HYPERTENSION

2017 ◽  
Vol 69 (11) ◽  
pp. 1259
Author(s):  
Rafael A. Meneguz-Moreno ◽  
Jose de Ribamar Costa ◽  
Auristela Ramos ◽  
Nisia Gomes ◽  
Zilda Meneghelo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Mitral Valvuloplasty ◽  
Long Term Follow Up ◽  
Initial Results

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.

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