ABSTRACT
Pulmonary angiosarcoma (PAS) is a malignant tumor of the vascular wall of mesenchymal origin. PAS is rare and has unknown etiology and poor prognosis. Depending on the location, angiosarcoma can lead to serious obstructive and embolic complications, as well as severe pulmonary hypertension and right ventricular heart failure. Patients with PAS are often mistakenly diagnosed with pulmonary embolism, chronic thromboembolic pulmonary hypertension, or lung tumors. Here, we present a clinical case of combined treatment of PAS.
Subject(s)
Hemangiosarcoma , Hypertension, Pulmonary , Pulmonary Embolism , Chronic Disease , Endarterectomy , Hemangiosarcoma/complications , Hemangiosarcoma/diagnosis , Hemangiosarcoma/surgery , Humans , Hypertension, Pulmonary/etiologyABSTRACT
BACKGROUND: Pulmonary artery denervation (PADN) procedure has not been applied to patients with residual chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary endarterectomy (PEA). OBJECTIVES: This study sought to assess the safety and efficacy of PADN using remote magnetic navigation in patients with residual CTEPH after PEA. METHODS: Fifty patients with residual CTEPH despite medical therapy at least 6 months after PEA, who had mean pulmonary artery pressure ≥25 mm Hg or pulmonary vascular resistance (PVR) > 400 dynâ§sâ§cm-5 based on right heart catheterization were randomized to treatment with PADN (PADN group; n = 25) using remote magnetic navigation for ablation or medical therapy with riociguat (MED group; n = 25). In the MED group, a sham procedure with mapping but no ablation was performed. The primary endpoint was PVR at 12 months after randomization. Key secondary endpoint included 6-min walk test. RESULTS: After PADN procedure, 2 patients (1 in each group) developed groin hematoma that resolved without any consequences. At 12 months, mean PVR reduction was 258 ± 135 dynâ§sâ§cm-5 in the PADN group versus 149 ± 73 dynâ§sâ§cm-5 in the MED group, mean between-group difference was 109 dynâ§sâ§cm-5 (95% confidence interval: 45 to 171; p = 0.001). The 6-min walk test distance was significantly increased in the PADN group as compared to distance in the MED group (470 ± 84 m vs. 399 ± 116 m, respectively; p = 0.03). CONCLUSIONS: PADN in patients with residual CTEPH resulted in substantial reduction of PVR at 12 months of follow-up, accompanied by improved 6-min walk test.
Subject(s)
Denervation , Endarterectomy , Hypertension, Pulmonary , Pulmonary Artery , Pulmonary Embolism/complications , Pyrazoles/administration & dosage , Pyrimidines/administration & dosage , Cardiac Catheterization/methods , Denervation/instrumentation , Denervation/methods , Endarterectomy/adverse effects , Endarterectomy/methods , Enzyme Activators/administration & dosage , Female , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/therapy , Male , Middle Aged , Pulmonary Artery/innervation , Pulmonary Artery/surgery , Pulmonary Wedge Pressure/physiology , Surgery, Computer-Assisted/methods , Treatment Outcome , Vascular Resistance/physiology , Walk Test/methodsABSTRACT
PURPOSE: To assess the dynamic of various health-related quality of life (HRQoL) parameters 3 years after pulmonary thromboendarterectomy (PTE), and to identify factors affecting HRQoL parameters in patients with chronic thromboembolic pulmonary hypertension (CTEPH) in the long-term follow-up after surgery. METHODS: This prospective cohort study included 128 patients with CTEPH before and after the PTE (3 year follow-up). The HRQoL was examined using the Short-Form 36 Health Survey Questionnaire (SF-36). RESULTS: In patients with CTEPH 3 years after PTE, a significant improvement in all the HRQoL parameters. The summary indicators of the physical and mental components of health remained at the same level as 1 year after the PTE and did not exceed 50 points. The residual pulmonary hypertension was a leading factor limiting parameters of physical and mental health 3 years after a PTE. In addition, the parameters of physical activity were adversely affected by age and the age-adjusted Charlson Comorbidity Index. CONCLUSIONS: In the study group of patients with CTEPH, PTE contributes to a significant improvement in all HRQoL parameters, which observed both 1 year and 3 years after surgery. The leading factor adversely affecting the physical and emotional components of health in the long-term period after PTE was residual pulmonary hypertension recorded in the early postoperative period. In addition, some physical HRQoL parameters are affected by age and age-adjusted Charlson Comorbidity Index.
Subject(s)
Hypertension, Pulmonary/surgery , Pulmonary Embolism/surgery , Adult , Chronic Disease , Cohort Studies , Female , Humans , Hypertension, Pulmonary/psychology , Male , Middle Aged , Prospective Studies , Pulmonary Embolism/psychology , Quality of Life/psychology , Treatment OutcomeSubject(s)
Carotid Artery Diseases/surgery , Coronary Artery Bypass , Coronary Artery Disease/surgery , Stents , Aged , Brain/diagnostic imaging , Brain/pathology , Brain Ischemia/diagnostic imaging , Brain Ischemia/etiology , Cardiopulmonary Bypass/adverse effects , Carotid Arteries/pathology , Carotid Artery Diseases/complications , Coronary Artery Bypass/adverse effects , Coronary Artery Disease/complications , Female , Follow-Up Studies , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Postoperative Complications/diagnostic imagingABSTRACT
BACKGROUND: Pulmonary thromboendarterectomy (PTE) is a recognized treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH), with good long-term hemodynamic results. The aim of this study was to test the hypothesis that the dynamics of health-related quality of life (QoL), 1 year after PTE, depend both on the clinical determinants associated with the underlying disease and the adverse events occurring in the early postoperative period. METHODS: This prospective cohort study included 136 patients with CTEPH. All patients before and after PTE (1-year follow-up) were examined in terms of QoL, by using the Short Form 36 Health Survey Questionnaire. RESULTS: One year after PTE, a significant improvement in the QoL of patients was observed in all subscales of the Short Form 36 Health Survey Questionnaire, except in the general health scale. Multivariate analysis showed that the predictors adversely affecting the dynamics of QoL after PTE were the presence of comorbidities (such as chronic obstructive pulmonary disease [COPD] and coronary artery disease) and early postoperative complications (specific ones for this category of patients are residual pulmonary hypertension and neurological complications, and nonspecific ones are atrial fibrillation and heart failure). CONCLUSIONS: The determinants that affect the dynamics of QoL 1 year after PTE in patients with CTEPH were the presence of comorbidities (COPD and coronary artery disease) and adverse events in the early postoperative period (residual pulmonary hypertension, neurological complications, atrial fibrillation, and heart failure).
Subject(s)
Endarterectomy , Hypertension, Pulmonary/surgery , Pulmonary Embolism/surgery , Quality of Life , Adult , Chronic Disease , Comorbidity , Endarterectomy/adverse effects , Female , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/psychology , Linear Models , Male , Middle Aged , Multivariate Analysis , Postoperative Complications/psychology , Prospective Studies , Pulmonary Embolism/diagnosis , Pulmonary Embolism/physiopathology , Pulmonary Embolism/psychology , Risk Factors , Surveys and Questionnaires , Time Factors , Treatment OutcomeABSTRACT
One of the most severe and frequent complication of pulmonary thromboendarterectomy is reperfusion pulmonary oedema. The only effective treatment for this complication is extracorporeal membrane oxygenation. A case of successful treatment of reperfusion pulmonary oedema with prolonged veno-arterial extracorporeal membrane oxygenation complicated by several episodes of bleeding and surgical site infection is presented.
