Pulmonary thromboendarterectomy: The Marie Lannelongue Hospital experience.

Background: Targeted medical therapy and balloon pulmonary angioplasty (BPA) entered the field of chronic thromboembolic pulmonary hypertension (CTEPH) treatment in the early 2010's. Multimodal therapy is emerging as the new gold standard for CTEPH management. Whether this change of paradigm impacted early outcomes of pulmonary endarterectomy (PEA) remains unknown. Our aim is to report our surgical experience in the era of CTEPH multimodal management. Methods: Patients who underwent PEA between 2016 and 2020 were included in the study. Early outcomes were described and compared between three groups of patients: PEA alone, PEA after targeted medical therapy induction and PEA after BPA. Results: A total of 418 patients, 225 males and 193 females, with a mean age of 59±14 years were included in the study. 336 patients underwent PEA alone, 69 after medical targeted therapy induction and 13 after unilateral BPA. Baseline preoperative pulmonary vascular resistance [4.99 (IQR, 1.71-8.48), 6.21 (IQR, 4.37-8.1), 5.03 (IQR, 4.44-7.19) wood units (WU), P=0.230, respectively] and PEA effectiveness [% decrease mean pulmonary artery pressure (mPAP), 24 (IQR, 7-42), 25 (IQR, 7-35), 23 (IQR, 3-29), P=0.580] did not differ between groups. Compared to PEA alone and PEA+BPA, the medical therapy induction group represented the most challenging group with higher baseline mPAP (45±10 vs. 42±11 and 43±11 mmHg, P=0.047), longer circulatory arrest time (30.1±15 vs. 26.6±10 and 19.6±6 min, P=0.005), higher post-PEA extracorporeal membrane oxygenation use (20.6% vs. 8.7 and 9.1%, P=0.004), higher duration on mechanical ventilation [4 (IQR, 1-12) vs. 1 (IQR, 0.5-5) and 2 (IQR, 1-3) days, P=0.005], higher complication rate (85.5% vs. 74.6% and 76.9%, P=0.052) and higher 90-day mortality (13% vs. 3.9% and 0%, P=0.002). Compared to PEA and PEA+ medical therapy induction groups, patients in the BPA induction group were older [72 (IQR, 62-76) vs. 60 (IQR, 48-69) and 62 (IQR, 52-72) years, P=0.005], and underwent shorter cardiopulmonary bypass (191.9±47.9 vs. 222±107.2 and 236.8±46.4 min, P<0.001), aortic cross clamping (54.8±21 vs. 82.7±31.4 and 80.1±32.9 min, P=0.002) and circulatory arrest time (19.6±6.2 vs. 26.6±10.8 and 30.1±15.1 min, P=0.008). Conclusions: Multimodal therapy approach to CTEPH patients did not affect effectiveness of PEA. Medical therapy and BPA could act in synergy with surgery to treat more challenging patients.

Transplantation for pulmonary arterial hypertension with congenital heart disease: Impact on outcomes of the current therapeutic approach including a high-priority allocation program.

Patients with end-stage pulmonary arterial hypertension due to congenital heart disease have limited access to heart-lung transplantation or double-lung transplantation. We aimed to assess the effects of a high-priority allocation program established in France in 2007. We conducted a retrospective study to compare waitlist and posttransplantation outcomes before versus after implementation of the high-priority allocation program. We included 67 consecutive patients (mean age at listing, 33.2 ± 10.5 years) with pulmonary arterial hypertension due to congenital heart disease listed for heart-lung transplantation or double-lung transplantation from 1997 to 2016. At one month, the incidences of transplantation and death before transplantation were 3.5% and 24.6% in 1997-2006, 4.8% and 4.9% for patients on the regular list in 2007-2016, and 41.2% and 7.4% for patients listed under the high-priority allocation program (p < .001 and p = .0001, respectively). Overall survival was higher in patients listed in 2007-2016 (84.2% and 61.2% at 1 and 10 years vs. 36.8% and 22.1%, p = .0001). Increased incidence of transplantation, decreased waiting list mortality, and improved early and long-term outcomes were observed in patients with pulmonary arterial hypertension due to congenital heart disease listed for transplantation in the recent era, characterized by implementation of a high-priority allocation program.

Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the European CTEPH registry.

BACKGROUND: Women are more susceptible than men to several forms of pulmonary hypertension, but have better survival. Sparse data are available on chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: We investigated sex-specific differences in the clinical presentation of CTEPH, performance of pulmonary endarterectomy (PEA), and survival. RESULTS: Women constituted one-half of the study population of the European CTEPH registry (N = 679) and were characterized by a lower prevalence of some cardiovascular risk factors, including prior acute coronary syndrome, smoking habit, and chronic obstructive pulmonary disease, but more prevalent obesity, cancer, and thyroid diseases. The median age was 62 (interquartile ratio, 50-73) years in women and 63 (interquartile ratio, 53-70) in men. Women underwent PEA less often than men (54% vs 65%), especially at low-volume centers (48% vs 61%), and were exposed to fewer additional cardiac procedures, notably coronary artery bypass graft surgery (0.5% vs 9.5%). The prevalence of specific reasons for not being operated, including patient's refusal and the proportion of proximal vs distal lesions, did not differ between sexes. A total of 57 (17.0%) deaths in women and 70 (20.7%) in men were recorded over long-term follow-up. Female sex was positively associated with long-term survival (adjusted hazard ratio, 0.66; 95% confidence interval, 0.46-0.94). Short-term mortality was identical in the two groups. CONCLUSIONS: Women with CTEPH underwent PEA less frequently than men, especially at low-volume centers. Furthermore, they had a lower prevalence of cardiovascular risk factors and were less often exposed to additional cardiac surgery procedures. Women had better long-term survival.

Autologous Tracheal Replacement: Surgical Technique and Outcomes.

Finding a good and durable substitute to trachea and proximal airways has remained the holy grail for thoracic surgeons for many decades. Autologous tracheal reconstruction using armed forearm free flap with rib cartilage achieved satisfactory results in managing extended tracheal lesions without the need for synthetic materials or immunosuppression. This well-vascularized and rigid neo trachea limits postoperative airway collapse, mediastinal infection, and ischemic airway issues, and achieves long-term functional benefit and prolonged survival. Further improvement is needed to deal with the lack of mucociliary clearance for longer airway replacement involving trachea and bronchial bifurcation.

Factors predicting outcome after pulmonary endarterectomy.

OBJECTIVE: Few studies have reported predictive factors of outcome after pulmonary endarterectomy (PEA) in chronic thromboembolic pulmonary hypertension. The purpose of this study was to determine factors influencing mortality and predictors of hemodynamic improvement after PEA. METHODS: A total of 383 consecutive patients who underwent PEA between January 2005 and December 2009 were retrospectively reviewed. Among them, 150 were fully reevaluated 7.5±1 months after PEA by NYHA class, 6-minute walk distance (6MWD), percentage of predicted carbon monoxide transfer factor (TLCO) and right heart catheterisation. RESULTS: Mortality rates at 1 month, 1 year and 3 years were 2.8%, 6.9% and 7.5%, respectively. Preoperative pulmonary vascular resistance (PVR) independently predicted 1-month, 1- and 3-year mortality and age predicted mortality at 1 year and 3 years. Significant improvement in NYHA class and 6MWD were observed and PVR decreased from 773±353 to 307±221 dyn.sec.cm-5 (p<0.001). In 96 patients (64%), PVR decreased by at least 50% and/or was reduced to lower than 250 dyn.sec.cm-5. Preoperative cardiac output (CO) and TLCO predicted hemodynamic improvement. CONCLUSION: PEA is associated with an excellent long-term survival and a marked improvement in clinical status and hemodynamics. Some preoperative factors including PVR, CO and TLCO can predict postoperative outcomes.

Ewing Sarcoma of the Chest Wall: Prognostic Factors of Multimodal Therapy Including En Bloc Resection.

BACKGROUND: Radiotherapy has long been the treatment of choice for local control of Ewing sarcoma of the chest wall (ESCW). However, there is debate regarding the use of surgery versus radiotherapy. The objective of this study was to identify risk factors that may affect long-term outcomes of nonmetastatic ESCW treated with preoperative chemotherapy (CT) followed by en bloc resection and adjuvant CT or chemoradiation. METHODS: Between 1996 and 2014, 30 patients with a median age of 25 years (SD ± 8.9 years) were treated at Marie-Lannelongue Hospital in Le Plessis-Robinson, France. Adjuvant therapy was used in 27 patients: CT for 6, chemoradiation for 20, and radiotherapy for 1. Patients' demographics, treatment data, tumor features, and outcomes were collected. RESULTS: In this cohort of patients who received multimodal therapy, including neoadjuvant CT and en bloc resection, there was no postoperative mortality. Eight patients (27%) experienced postoperative complications. Resection included at least one rib (n = 27) and the sternum (n = 1) or the spine (n = 8). Negative and microscopic disease resections were achieved in 28 and 2 patients, respectively. Tumor viability (TV) was ≤5% in 18 patients (60%). In patients with TV >5% at definitive histologic examination, adjuvant chemoradiation was associated with a better long-term outcome than was treatment with adjuvant CT alone. The 5-year overall survival and disease-free survival rates were 60.7% and 41.0%, respectively, with a median survival of 87 months. By univariate analysis, TV >5% and pleural extension at diagnosis were associated with poorer long-term survival (p < 0.05). CONCLUSIONS: Multimodality treatment of ESCW, including neoadjuvant CT followed by en bloc resection and adjuvant CT or chemoradiation, is associated with excellent long-term outcomes.

Tracheal replacement.

Tracheal reconstruction is one of the greatest challenges in thoracic surgery when direct end-to-end anastomosis is impossible or after this procedure has failed. The main indications for tracheal reconstruction include malignant tumours (squamous cell carcinoma, adenoid cystic carcinoma), tracheoesophageal fistula, trauma, unsuccessful surgical results for benign diseases and congenital stenosis. Tracheal substitutes can be classified into five types: 1) synthetic prosthesis; 2) allografts; 3) tracheal transplantation; 4) tissue engineering; and 5) autologous tissue composite. The ideal tracheal substitute is still unclear, but some techniques have shown promising clinical results. This article reviews the advantages and limitations of each technique used over the past few decades in clinical practice. The main limitation seems to be the capacity for tracheal tissue regeneration. The physiopathology behind this has yet to be fully understood. Research on stem cells sparked much interest and was thought to be a revolutionary technique; however, the poor long-term results of this approach highlight that there is a long way to go in this research field. Currently, an autologous tissue composite, with or without a tracheal allograft, is the only long-term working solution for every aetiology, despite its technical complexity and setbacks.

Impact of High-Priority Allocation on Lung and Heart-Lung Transplantation for Pulmonary Hypertension.

BACKGROUND: Since 2006 and 2007, patients in France with severe pulmonary hypertension (PH) who are at imminent risk of death, despite optimal treatment in the intensive care unit, are placed on a high-priority list (HPL) for heart-lung transplantation (HLT) or double-lung transplantation (DLT). We assessed the effect of this approach on the waiting list and outcomes after transplantation. METHODS: We conducted a single-center, retrospective, before-and-after study of consecutive patients with severe group 1, 1', or 4 PH listed for DLT or HLT between 2000 and 2013 (ie, 6 years before and 6 years after HPL implementation). RESULTS: We included 234 patients. HPL implementation resulted in a significant decrease of the cumulative incidence of death on the waiting list at 1 and 2 years (p < 0.0001). The cumulative incidence of transplantation increased significantly from 48% to 76% after 2 years (p < 0.0001). Overall survival after transplantation was not significantly different between the pre-HPL and post-HPL era. In the HPL period, patients on the regular list who received a transplant had a nonsignificant trend toward improved overall survival compared with those on the HPL who received a transplant (at 1, 2, 3, and 5 years: 85%, 77%, 72%, and 72% vs 67%, 61%, 58%, and 50%; p = 0.053). Finally, survival after listing improved significantly after HPL implementation (at 1, 2, 3, and 5 years: 69%, 62%, 58%, and 54% vs 54%, 45%, 34%, and 26% before the HPL; p < 0.001). CONCLUSIONS: HPL implementation was followed by higher survival of PH patients after registration on the DLT or HLT waiting list and by a higher cumulative incidence of transplantation among waiting-list patients.

Abnormal pulmonary endothelial cells may underlie the enigmatic pathogenesis of chronic thromboembolic pulmonary hypertension.

BACKGROUND: Chronic thromboembolic pulmonary hypertension results from chronic mechanical obstruction of the pulmonary arteries after acute venous thromboembolism. However, the mechanisms that result in the progression from unresolved thrombus to fibrotic vascular remodeling are unknown. We hypothesized that pulmonary artery endothelial cells contribute to this phenomenon via paracrine growth factor and cytokine signaling. METHODS: Using enzyme-linked immunosorbent assay and cell migration assays, we investigated the circulating growth factors and cytokines of chronic thromboembolic pulmonary hypertension patients as well as the cross talk between pulmonary endothelial cells and pulmonary artery smooth muscle cells and monocytes from patients with chronic thromboembolic pulmonary hypertension in vitro. RESULTS: Culture medium from the pulmonary endothelial cells of chronic thromboembolic pulmonary hypertension patients contained higher levels of growth factors (fibroblast growth factor 2), inflammatory cytokines (interleukin 1ß, interleukin 6, monocyte chemoattractant protein 1), and cell adhesion molecules (vascular cell adhesion molecule 1 and intercellular adhesion molecule 1). Furthermore, exposure to the culture medium of pulmonary endothelial cells from patients with chronic thromboembolic pulmonary hypertension elicited marked pulmonary artery smooth muscle cell growth and monocyte migration. CONCLUSIONS: These findings implicate pulmonary endothelial cells as key regulators of pulmonary artery smooth muscle cell and monocyte behavior in chronic thromboembolic pulmonary hypertension and suggest a potential mechanism for the progression from unresolved thrombus to fibrotic vascular remodeling.

Osteopontin and thrombospondin-1 play opposite roles in promoting tumor aggressiveness of primary resected non-small cell lung cancer.

BACKGROUND: Osteopontin (OPN) and thrombospondin-1 (TSP-1) are extracellular matrix proteins secreted by stromal and tumor cells. These proteins appear to have a key role in the tumor microenvironment for cancer development and metastasis. There is little information regarding the prognostic value of the combination of these two proteins in human cancers. Our aim was to clarify clinical significance and prognostic value of each circulating protein and their combination in primary resected non-small cell lung cancer (NSCLC) patients. METHODS: We retrospectively reviewed 171 patients with NSCLC following curative intent surgery from January to December of 2012. Preoperative serums, demographics, clinical and pathological data and molecular profiling were analyzed. Pre-treatment OPN and TSP-1 serum levels were measured by ELISA. Tissue protein expression in primary tumor samples was determined by immunohistochemical analysis. RESULTS: OPN and TSP-1 serum levels were inversely correlated with survival rates. For each 50 units increment of serum OPN, an increased risk of metastasis by 69 % (unadjusted HR 1.69, 95 % CI 1.12-2.56, p = 0.01) and an increased risk of death by 95 % (unadjusted HR 1.95, 95 % CI 1.15-3.32, p = 0.01) were observed. Conversely, for each 10 units increment in TSP-1, the risk of death was decreased by 85 % (unadjusted HR 0.15, 95 % CI 0.03-0.89; p = 0.04). No statistically significant correlation was found between TSP-1 serum level and distant metastasis-free survival (p = 0.2). On multivariate analysis, OPN and TSP-1 serum levels were independent prognostic factors of overall survival (HR 1.71, 95 % CI 1.04-2.82, p = 0.04 for an increase of 50 ng/mL in OPN; HR 0.18, 95 % CI 0.04-0.87, p = 0.03 for an increase of 10 ng/mL in TSP-1). In addition, the combination of OPN and TSP-1 serum levels remained an independent prognostic factor for overall survival (HR 1.31, 95 % CI 1.03-1.67, p = 0.03 for an increase of 6 ng/mL in OPN/TSP-1 ratio). CONCLUSIONS: Our results show that pre-treatment OPN and TSP-1 serum levels may reflect the aggressiveness of the tumor and might serve as prognostic markers in patients with primary resected NSCLC.

Outcomes After Resection of T4 Non-Small Cell Lung Cancer Using Cardiopulmonary Bypass.

BACKGROUND: Complete, en bloc resection offers the greatest chance of long-term survival in T4 non-small cell lung cancer (NSCLC). The use of cardiopulmonary bypass (CPB) to achieve an en bloc resection is controversial because of potentially increased bleeding, lung dysfunction, and tumor dissemination. We reviewed our institutional experience to assess CPB's effect on survival. METHODS: All patients who underwent resection for T4 NSCLC at our institution between 1980 and 2013 were retrospectively reviewed and stratified according to whether they did (CPB group, n = 20) or did not (No CPB group, n = 355) undergo CPB. Primary outcomes of interest were overall and disease-free survival and perioperative complications. RESULTS: Baseline characteristics and medical therapy were similar between the groups. Median overall survival for all patients was 31 months, with 1-, 3-, 5-, and 10-year survival of 73%, 47%, 40%, and 26%, respectively. Median disease-free survival for all patients was 19 months, with 1-, 3-, 5-, and 10-year disease-free survival of 61%, 40%, 33%, and 21%, respectively. No difference was found in overall or disease-free survival at 1, 3, 5, and 10 years between the No CPB and CPB groups (p = 0.89 and p = 0.88). In addition, no differences were found in the rates of major perioperative complications. CONCLUSIONS: The use of CPB allows for complete, en bloc resection in otherwise inoperable patients with T4 NSCLC and offers similar overall and disease-free survival to patients resected without CPB. All thoracic surgeons who manage T4 NSCLC should consider the use of CPB if it is necessary to achieve a complete, en bloc resection.

Extracorporeal Life Support After Pulmonary Endarterectomy as a Bridge to Recovery or Transplantation: Lessons From 31 Consecutive Patients.

BACKGROUND: Extracorporeal life support (ECLS) can be used to sustain patients having cardiorespiratory failure after pulmonary endarterectomy (PEA). We aimed to assess outcomes and to identify factors associated with short-term survival among patients who required ECLS after PEA. METHODS: We reviewed the charts of consecutive patients who required ECLS after PEA between 2005 and 2013 at our institution. Patients with failed PEA were scheduled for heart-lung transplantation, and patients with potentially reversible hemodynamic or respiratory failure were given appropriate supportive care until recovery. RESULTS: Of the 829 patients who underwent PEA, 31 (3.7%) required postoperative ECLS. Of these, 23 continued to receive support, and 8 were listed for heart-lung transplantation during ECLS. Overall inhospital survival was 48.4% (15 of 31). Of patients listed for transplantation, 2 died while on support; 4 of the 6 patients undergoing transplantation lived to hospital discharge. Of the 23 supportive care patients, 11 (47.8%) were alive at hospital discharge. The factors associated with survival were younger age (p = 0.02), larger post-PEA decrease in mean pulmonary artery pressure (p = 0.020), lower post-PEA total pulmonary resistance (p = 0.008), and pure respiratory failure related to reperfusion edema or airway bleeding (p = 0.003). CONCLUSIONS: Extracorporeal life support may be useful to support patients with complications after PEA either to recovery or to salvage transplantation.

Operability assessment in CTEPH: Lessons from the CHEST-1 study.

Pulmonary endarterectomy is the gold standard treatment for chronic thromboembolic pulmonary hypertension and is potentially curative, although some patients are unsuitable for pulmonary endarterectomy and require alternative management. Lack of standardized assessment of pulmonary endarterectomy eligibility risks suboptimal treatment in some patients. We discuss the implications for future clinical trials and practice of a unique operability assessment in patients who have chronic thromboembolic pulmonary hypertension and were initially screened for inclusion in the CHEST-1 (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase Stimulator Trial-1) study. The CHEST-1 study evaluated riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy. Screened patients who were initially considered "inoperable" underwent central independent adjudication by a committee of experienced surgeons, or local adjudication in collaboration with an experienced surgeon. Operability decisions were based on accessibility of thrombi and the association between pulmonary vascular resistance (PVR) and the extent of obstruction, using pulmonary angiography/computed tomography with ventilation/perfusion scintigraphy as the minimum diagnostic tests. Of 446 patients screened for CHEST-1, a total of 188 and 124 underwent central and local adjudication, respectively, after being initially considered to be "inoperable." After a second assessment by an experienced surgeon, 69 of these 312 "inoperable" patients were deemed operable. Rigorous measures in CHEST-1 guaranteed that only technically inoperable patients, or patients who had persistent/recurrent pulmonary hypertension, were enrolled, thus ensuring that only patients for whom surgery was not an option were enrolled. This study design sets new standards for future clinical trials and practice in CTEPH, helping to ensure that patients who have CTEPH receive optimal treatment.

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.

BACKGROUND: Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension. METHODS AND RESULTS: A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1.49-11.62; P=0.0065 and HR, 4.76; 95% CI, 1.76-12.88; P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. CONCLUSIONS: The long-term prognosis of operated patients currently is excellent and better than the outcome of not-operated patients.

Chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension (PH) characterized by the persistence of thromboembolic obstructing the pulmonary arteries as an organized tissue and the presence of a variable small vessel arteriopathy. The consequence is an increase in pulmonary vascular resistance resulting in progressive right heart failure. CTEPH is classified as group IV pulmonary hypertension according to the WHO classification of pulmonary hypertension. CTEPH is defined as precapillary pulmonary hypertension (mean pulmonary artery pressure ≥ 25 mmHg with a pulmonary capillary wedge pressure ≤ 15 mmHg) associated with mismatched perfusion defects on ventilation-perfusion lung scan and signs of chronic thromboembolic disease on computed tomography pulmonary angiogram and/or conventional pulmonary angiography, in a patient who received at least 3 months of therapeutic anticoagulation. CTEPH as a direct consequence of symptomatic pulmonary embolism (PE) is rare, and a significant number of CTEPH cases develop in the absence of history of PE. Thus, CTEPH should be considered in any patient with unexplained PH. Splenectomy, chronic inflammatory conditions such as inflammatory bowel disease, indwelling catheters and cardiac pacemakers have been identified as associated conditions increasing the risk of CTEPH. Ventilation-perfusion scan (V/Q) is the best test available for establishing the thromboembolic nature of PH. When CTEPH is suspected, patients should be referred to expert centres where pulmonary angiography, right heart catheterization and high-resolution CT scan will be performed to confirm the diagnosis and to assess the operability. Pulmonary endarterectomy (PEA) remains the gold standard treatment for CTEPH when organized thrombi involve the main, lobar or segmental arteries. This operation should only be performed by experienced surgeons in specialized centres. For inoperable patients, current ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension recommend the use of riociguat and say that off-label use of drugs approved for PAH and pulmonary angioplasty may be considered in expert centres.

Hybrid treatment of an aortic pseudoaneurysm arising at the innominate artery junction secondary to superior vena cava stenting.

Pseudoaneurysm of the innominate artery secondary to superior vena cava stenting has never been reported. We report the case of a 42-year-old woman previously treated for a Masaoka stage III thymoma with superior vena cava replacement through median sternotomy followed by adjuvant radiation therapy. Four years later, the patient came back with a large pseudoaneurysm at the junction of the innominate artery and ascending aorta. To avoid resternotomy, endovascular deployment of a stent graft in the ascending aorta with a periscope stenting in the left common carotid artery after axilloaxillary bypass was performed to treat this aortic pseudoaneurysm.

Validation of a scoring system to predict recurrence of resected solitary fibrous tumors of the pleura.

BACKGROUND: Solitary fibrous tumors of the pleura (SFTPs) are infrequent neoplasms with no standardized criteria to predict risk of recurrence after curative surgery. The aim of the present study is to validate a recently proposed recurrence score in a large European cohort of patients with SFTP. METHODS: Validation of a previously published scoring system was assessed in a population of 113 patients who underwent complete resection of SFTPs. Patients were scored according to the pleural origin, morphology, size, hypercellularity, presence of necrosis or hemorrhage, and number of mitoses in their SFTPs. Receiver operating characteristic curves were plotted for the score. Time to recurrence analysis was performed using the Kaplan-Meier and Cox proportional hazards methods. RESULTS: After a mean follow-up of 13.2 ± 7.3 years, there were nine recurrences (8.0%). Score performance to predict recurrence was as follows: sensitivity = 78%, specificity = 74%, positive likelihood ratio = 3.0, and negative likelihood ratio = 0.3. A cutoff of 3 points was used to classify 79 patients (69.9%) at low risk and 34 patients (30.1%) at high risk for recurrence. A high-risk classification was significantly associated with more recurrences during follow-up (P = .004), worse overall survival (P = .0008), more extensive lung resections (P = .001), and the use of adjuvant therapies (P = .009). The present score outperformed England's criteria (P = .049) and de Perrot classification (P < .001) when predicting SFTP recurrence. CONCLUSIONS: The proposed scoring system, which combines common clinical and histologic features of resected SFTPs, remains predictive of recurrence in a separate patient population. The simple score may guide the postoperative surveillance of this uncommon tumor.

En Bloc Resection of Pulmonary Sulcus Non-small Cell Lung Cancer Invading the Spine: A Systematic Literature Review and Pooled Data Analysis.

OBJECTIVE: To conduct a systematic literature review and pooled data analysis focusing on outcome after en bloc resection of pulmonary sulcus non-small cell lung cancer (NSCLC) invading the spine. BACKGROUND: This rare type of NSCLC has historically been considered unresectable and fatal. Nowadays, carefully selected patients can be cured when treated surgically within a multimodality concept. METHODS: The MEDLINE database was searched using the PubMed engine to retrieve relevant articles. Corresponding authors were contacted, and shared data were pooled and analyzed. RESULTS: Search strategy yielded 134 articles. Six were relevant and nonduplicative. Four authors shared updated data on 135 patients. All tumors were resected en bloc with the lung, chest wall, and spine. Induction was administered in 85 patients (63%) and consisted of chemotherapy (n = 32), radiation (n = 1), or concurrent chemoradiation (n = 52). Spine resections included total (n = 23), hemi- (n = 94), and partial (n = 18) vertebrectomies. R0 resection was achieved in 120 patients (89%). Adjuvant treatment was administered to 70 patients (52%) and included chemotherapy (n = 16), radiotherapy (n = 22), or chemoradiation (n = 32). Overall, 3-, 5-, and 10-year survival rates were 57%, 43%, and 27%, respectively. Univariate analysis identified the type of resection (R0 vs R1/R2, P < 0.001) as significant prognostic factor among the variables tested (age, histology, pT/pN, type of induction/adjuvant treatment, type of lung/spine resection). CONCLUSIONS: Multimodality therapy including en bloc resection for pulmonary sulcus NSCLC invading the spine provides excellent long-term survival in selected patients. This result establishes a benchmark against which the effects of new treatments can be compared in the future.

Subclavian artery resection and reconstruction for thoracic inlet neoplasms.

BACKGROUND: To update the long-term outcomes after subclavian artery (SA) resection and reconstruction during surgery for thoracic inlet (TI) cancer through the anterior transclavicular approach. METHODS: Between 1985 and 2014, 85 patients (60 men and 25 women; mean age, 52 years) underwent en bloc resection of thoracic-inlet non-small cell lung cancer (NSCLC) (n=69), sarcoma (n=11), breast carcinoma (n=3) or thyroid carcinoma (n=2) involving the SA. L-shaped transclavicular cervicothoracotomy was performed, with posterolateral thoracotomy in 18 patients or a posterior midline approach in 15 patients. Resection extended to the chest wall (>2 ribs, n=60), lung (n=76), and spine (n=15). Revascularization was by end-to-end anastomosis (n=48), polytetrafluoroethylene (PTFE) graft interposition (n=28), subclavian-to-common carotid artery transposition (n=8), or grafting of the autologous superficial femoral artery in an anterolateral thigh free flap (n=1). Complete R0 resection was achieved in 75 patients and microscopic R1 resection in 10 patients. Postoperative radiation therapy was given to 51 patients. RESULTS: There were no cases of postoperative death, neurological sequelae, graft infection or occlusion, or limb ischemia. Postoperative morbidity consisted of pneumonia (n=16), phrenic nerve palsy (n=2), recurrent nerve palsy (n=4), bleeding (n=4), acute pulmonary embolism (n=1), cerebrospinal fluid leakage (n=1), chylothorax (n=1), and wound infection (n=2). Five-year survival and disease-free survival rates were 32% and 22%, respectively. Long-term survival was not observed after R1 resection. CONCLUSIONS: Subclavian arteries invaded by TI malignancies can be safely resected and reconstructed through the anterior transclavicular approach, with good long-term survival provided complete R0 resection is achieved.