Despite significant advancements in pharmacological treatment, interventional and surgical options are still viable treatments for patients with pulmonary arterial hypertension (PAH), particularly idiopathic PAH. Herein, we review the interventional and surgical treatments for PAH. Atrial septostomy and the Potts shunt can be useful bridging tools for lung transplantation (Ltx), which remains the final surgical treatment among patients who are refractory to any other kind of therapy. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) remains the ultimate bridging therapy for patients with severe PAH. More importantly, VA-ECMO plays a crucial role during Ltx and provides necessary left ventricular conditioning during the initial postoperative period. Pulmonary denervation may potentially be a new way to ensure better transplant-free survival among patients with the aforementioned disease. However, high-quality randomized controlled trials are needed. As established, obtaining the Eisenmenger physiology among patients with severe pulmonary hypertension by creating artificial defects is associated with improved survival. However, right-to-left shunting may be harmful after Ltx. Closure of the artificially created defects may carry some risk associated with cardiac surgery, especially among patients with Potts shunts. In conclusion, PAH requires an interdisciplinary approach using pharmacological, interventional, and surgical modalities.
Background: In Poland, the clinical characteristics and outcomes of patients with COVID-19 requiring extracorporeal membrane oxygenation (ECMO) remain unknown. This study aimed to answer these unknowns by analyzing data collected from high-volume ECMO centers willing to participate in this project. Methods: This retrospective, multicenter cohort study was completed between March 1, 2020, and May 31, 2021 (15 months). Data from all patients treated with ECMO for COVID-19 were analyzed. Pre-ECMO laboratory and treatment data were compared between non-survivors and survivors. Independent predictors for death in the intensive care unit (ICU) were identified. Results: There were 171 patients admitted to participating centers requiring ECMO for refractory hypoxemia due to COVID-19 during the defined time period. A total of 158 patients (mean age: 46.3 ± 9.8 years) were analyzed, and 13 patients were still requiring ECMO at the end of the observation period. Most patients (88%) were treated after October 1, 2020, 77.8% were transferred to ECMO centers from another facility, and 31% were transferred on extracorporeal life support. The mean duration of ECMO therapy was 18.0 ± 13.5 days. The crude ICU mortality rate was 74.1%. In the group of 41 survivors, 37 patients were successfully weaned from ECMO support and four patients underwent a successful lung transplant. In-hospital death was independently associated with pre-ECMO lactate level (OR 2.10 per 1 mmol/L, p = 0.017) and BMI (OR 1.47 per 5 kg/m2, p = 0.050). Conclusions: The ICU mortality rate among patients requiring ECMO for COVID-19 in Poland was high. In-hospital death was independently associated with increased pre-ECMO lactate levels and BMI.
29 May 2019 in International Journal of Occupational Medicine and Environmental Health
Objectives: Lung transplantation not only saves a patient’s life but also creates the opportunity for becoming more self-reliant and getting back to work. The aim of this single center study was to assess the prospects of employment, as well as its influence on the quality of life and...
Patients with pulmonary arterial hypertension (PAH) become candidates for lung or lung and heart transplantation when the maximum specific therapy is no longer effective. The most difficult challenge is choosing one of the above options in the event of symptoms of right ventricular failure. Here, we present two female patients with PAH: (1) a 21-year-old patient with Eisenmenger syndrome, caused by a congenital defect—patent ductus arteriosus (PDA); and (2) a 39-year-old patient with idiopathic PAH and coexistent PDA. Their common denominator is PDA and the hybrid surgery performed: double lung transplantation with simultaneous PDA closure. The operation was performed after pharmacological bridging (conditioning) to transplantation that lasted for 33 and 70 days, respectively. In both cases, PDA closure effectiveness was 100%. Both patients survived the operation (100%); however, patient no. 1 died on the 2nd postoperative day due to multi-organ failure; while patient no. 2 was discharged home in full health. The authors did not find a similar description of the operation in the available literature and PubMed database. Hence, we propose this new treatment method for its effectiveness and applicability proven in our practice.
Birt–Hogg–Dubé Syndrome (BHDS) is a rare autosomal dominant disorder associated with mutations in the Foliculin gene (FLCN), characterized by skin lesions, renal tumors (often malignant or leading to malignancy), and lung cysts, often leading to spontaneous pneumothorax. Lung function is generally preserved in these patients, which is why lung transplantation (LTx) has not been performed in this population to date. Below, we present the case of a 15-year-old boy with BHDS, with extensive cystic lung disease, complicated by recurrent pneumothorax (requiring pleurodesis) and, in his case, progressive respiratory failure, who ultimately underwent double sequential lung transplantation (DLTx). Postoperative management included treatment of humoral rejection and treatment of SARS-CoV-2 infection, in addition to typical immunosuppression. To our knowledge, this is the first DLTx for BHDS reported worldwide. The patient underwent a double lung transplant due to a life-threatening recurrent pneumothorax. This case highlights the importance of multidisciplinary care and demonstrates the feasibility of transplantation in advanced cases of BHDS (despite prior pleurodesis).
Objectives: This study aims to describe the experience of using ECMO on various patients who require ECMO support during the entire perioperative period of lung transplantation. ECMO has several roles: it can bridge patients to transplantation, improve lung graft function in case of primary graft dysfunction after transplantation, improve left ventricle function after transplantation in patients with primary pulmonary hypertension, and manage COVID-19 patients who are awaiting LuTx or undergoing LTx. Methods: We present 6-year results from a high-volume lung transplant center (219 cases/6 years, >50 cases/2022). We used ECMO in 56 cases (25.6%) of all lung transplants between 2018 and 2023. Results: The one-year survival rate of patients transplanted on ECMO was 85.7%. We applied all advanced ECMO techniques, such as bridging to transplantation on ECMO (n = 15, early survival 66.7%) and left ventricular conditioning after LuTx with VA-ECMO (n = 12, 60-day and one-year survival 85.1% and 53%, respectively). We also bridged patients with COVID-19 to transplantation and transplanted them from ECMO (n = 9, early survival 55%). Conclusions: This article shows possible applications of ECMO therapy for various indications in lung transplant patients and, along with data from other publications, it demonstrates that ECMO can improve survival and outcomes for patients with respiratory failure, primary pulmonary hypertension, and COVID-19. The COVID-19 pandemic highlighted new utilization of ECMO, demonstrating its usefulness and importance in critical care medicine. Further research into capabilities of the ECMO system may expand the knowledge about its role in lung transplantation and future treatments.