Surgery for fibrosing mediastinitis with severe pulmonary hypertension due to pulmonary venous stenosis.

A 21-year-old woman with severe pulmonary hypertension and circulatory collapse was referred to our hospital for possible lung transplantation with extracorporeal membrane oxygenation support. Computed tomography revealed severe stenosis of all 4 pulmonary veins, and fibrosing mediastinitis was suspected. Surgical reconstruction of the pulmonary veins was performed, and extracorporeal membrane oxygenation support was weaned off. After surgery, pulmonary vascular resistance normalized. This successful case demonstrates that surgical pulmonary venous reconstruction is an important treatment for fibrosing mediastinitis induced by pulmonary venous stenosis and pulmonary hypertension.

Total Anomalous Pulmonary Venous Connection: Surgical Outcomes and Risk Factors for Postoperative Pulmonary Vein Obstruction.

Background: Total anomalous pulmonary venous connection (TAPVC) is a fatal congenital cardiac anomaly that requires urgent surgical intervention. The development of postoperative pulmonary vein obstruction (PVO) negatively impacts long-term survival. Objectives: The present study aimed to evaluate the surgical outcomes of TAPVC repair and risk factors associated with postoperative PVO. Methods: Patients who underwent primary TAPVC repair at our institute between 2004 and 2022 were retrospectively enrolled, and those with right atrial isomerism and single ventricle physiology were excluded. Factors associated with survival and postoperative PVO were analyzed. Results: A total of 116 patients were enrolled in the present study. The early mortality rate was 6.9%. Nineteen patients (16.4%) developed postoperative PVO within a median time of 59 days of the primary repair, of whom 10 were successfully relieved without any recurrent obstruction. In long-term follow-up, patients with postoperative PVO had significantly lower long-term survival rates than those without postoperative PVO [57.9%, 95% confidence interval (CI) = 34.8-79.5%; vs. 90.4%, 95% CI = 83-96.6% at 10 years, p < 0.001]. Risk factors for postoperative PVO development included lower body weight, younger age, preoperative mechanical ventilation, preoperative inotrope use, and emergency operation. Conclusions: Postoperative PVO was significantly associated with a higher long-term mortality rate after primary TAPVC repair, with the risk being higher in patients with critical preoperative status. The long-term outcome was good for patients in whom the obstruction was successfully relieved. Early detection and prompt intervention for postoperative PVO after TAPVC repair can improve overall survival in these patients.

Association between SLICC/ACR damage index and outcomes for lupus patients after cardiac valve surgery.

Systemic lupus erythematosus (SLE) is associated with multi-organ damage including cardiac valve, which may need valvular operation. However, methods for outcome prediction and prosthetic valve selection are unclear in SLE patients undergoing cardiac valve surgery. Twenty-five SLE patients receiving valvular operation in a single institute between 2002 and 2020 were enrolled. Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR damage index, SDI) was applied to evaluate the damage severity. Clinical outcomes were compared between patients with different SDI. The hospital survival rate was 88%, and long-term survival rate was 59.5% and 40.2% at 5 and 10 years. The median SDI was 4 (interquartile range 3-6) in our study, patients were then grouped into higher SDI (defined as SDI ≥ 5, n = 11) and lower SDI group (defined as SDI < 5, n = 14). The in-hospital survival rate (72.2% vs 100%, P = 0.074) and 5-year survival rate (18.2% vs 92.9%, P < 0.001) were lower in higher SDI group, compared to lower SDI group. SDI score was associated with long-term outcome for SLE patients receiving cardiac valve surgery. SDI ≥ 5 was associated with very poor long-term outcomes. This finding implicates that xenograft might be a reasonable choice for SLE patients with SDI ≥ 5.

Correlation of tumor spread through air spaces and clinicopathological characteristics in surgically resected lung adenocarcinomas.

OBJECTIVES: Tumor spread through air spaces (STAS) has recently been reported as a novel invasive pattern in lung adenocarcinoma, but the correlation between other clinicopathological and genetic profiles has not been well studied. The aim of this study was to investigate these correlations in patients with surgically resected lung adenocarcinoma. MATERIALS AND METHODS: Five hundred consecutive patients with lung adenocarcinoma who underwent curative lung tumor resection and with available STAS profile were reviewed retrospectively from January to December 2016. The correlations of STAS presence and clinicopathological and genetic characteristics were analyzed. RESULTS: One hundred thirty-four patients (26.8%) had positive STAS. The pathological stage of these patients was adenocarcinoma in situ, IA, IB, II, and III in 25 (5%), 343 (68.6%), 63 (12.6%), 29 (5.8%), and 40 (8%), respectively. Multivariate analysis showed that the presence of STAS was significantly correlated to higher T (p = 0.001) and N (p = 0.032) stages, moderate/poor differentiation (p = 0.001), and the presence of lymphovascular invasion (p = 0.001). Although positive epidermal growth factor receptor mutation and non-lepidic histologic subtypes were correlated with the presence of STAS in the univariate analysis, they were not significantly correlated with the presence of STAS in the multivariate analysis (p = 0.676 and 0.286, respectively). CONCLUSIONS: STAS was significantly correlated with several invasive clinicopathological characteristics in surgically resected lung adenocarcinoma. Based on our results and current evidence, the presence of STAS may be considered as a staging profile in future staging system.