Diagnosis and treatment of lung disease associated with alpha one-antitrypsin deficiency: A position statement from the Thoracic Society of Australia and New Zealand.

AATD is a common inherited disorder associated with an increased risk of developing pulmonary emphysema and liver disease. Many people with AATD-associated pulmonary emphysema remain undiagnosed and therefore without access to care and counselling specific to the disease. AAT augmentation therapy is available and consists of i.v. infusions of exogenous AAT protein harvested from pooled blood products. Its clinical efficacy has been the subject of some debate and the use of AAT augmentation therapy was recently permitted by regulators in Australia and New Zealand, although treatment is not presently subsidized by the government in either country. The purpose of this position statement is to review the evidence for diagnosis and treatment of AATD-related lung disease with reference to the Australian and New Zealand population. The clinical efficacy and adverse events of AAT augmentation therapy were evaluated by a systematic review, and the GRADE process was employed to move from evidence to recommendation. Other sections address the wide range of issues to be considered in the care of the individual with AATD-related lung disease: when and how to test for AATD, changing diagnostic techniques, monitoring of progression, disease in heterozygous AATD and pharmacological and non-pharmacological therapy including surgical options for severe disease. Consideration is also given to broader issues in AATD that respiratory healthcare staff may encounter: genetic counselling, patient support groups, monitoring for liver disease and the need to establish national registries for people with AATD in Australia and New Zealand.

A successful aggressive surgical and medical approach to pulmonary artery obstruction due to Mycobacterium abscessus infection post lung transplantation.

Mycobacterium abscessus infection following lung transplantation has historically been associated with poor outcomes. We report a case of bilateral lung retransplantation complicated by obstruction of the right pulmonary artery secondary to M. abscessus mycotic aneurysm. Aggressive surgical management, including reconstruction of the right pulmonary artery, was undertaken with prolonged antimicrobial therapy. Thirty-six months later, antibiotics have been discontinued and the patient has stable soft tissue chest wall disease with good graft function. Mortality and morbidity associated with M. abscessus infection is considerable but this case illustrates that with aggressive early management, outcomes may be favorable.

Feasibility and Safety of a Transthoracic Pneumostoma Airway Bypass in Severe Emphysema Patients.

BACKGROUND: Emphysema is characterised by airflow obstruction, hyperinflation, and resultant dyspnoea. It is worth investigating whether decompression improves lung mechanics and enhances quality of life (QoL). OBJECTIVES: The purpose of this study was to describe the feasibility and safety of creating a transthoracic pneumostoma to enable lung reduction. METHODS: A transthoracic 10-mm diameter Portaero Access Tube (Portaero™, Cupertino, CA, USA) was implanted via a third intercostal space incision in 15 severe emphysema patients [mean age 63 years, forced expiratory volume in 1 s 54% predicted, diffusing capacity for carbon monoxide 31% predicted, residual volume 246% predicted, Six-Minute Walk Test 296 m]. Four weeks later, an 8-mm Portaero Disposable Tube (3-8 cm in length) was substituted and changed daily thereafter. The targeted primary endpoints were a ≥12% increase in forced expiratory volume in 1 s and a decrease of ≥4 points in Saint George's Respiratory Questionnaire score at 6 months. RESULTS: Sixteen procedures were performed on 15 patients, complicated by 1 intercostal haemorrhage, 1 pneumothorax, and universal mild surgical emphysema. Early patency issues were common, but often responded to external endoscopic debridement or argon plasma laser. Three-month patency was achieved in 9 of 15 patients, and 6 of these had long-term patency (mean of 4 years). Patency was associated with potentially useful long-term improvements or stability in spirometry, residual volume, and QoL. However, the primary endpoints were not met at 6 months. CONCLUSION: The creation and maintenance of a transthoracic pneumostoma appears feasible and safe in patients with severe emphysema. Further studies refining patient selection (perhaps via chest computed tomography collateral ventilation and fissure assessments), techniques, and tube materials are suggested.

Anaesthetic and perioperative considerations for extrapleural pneumonectomy and extended pleurectomy/decortication: a scoping review protocol.

INTRODUCTION: Extrapleural pneumonectomy (EPP) and extended pleurectomy/decortication (ePD) are surgical cytoreductive techniques aimed at achieving macroscopic resection in malignant pleural tumours such as pleural mesothelioma, non-mesothelioma pleural malignancies such as thymoma and sarcoma, and rarely for pleural tuberculosis, in a more limited fashion. Despite extensive studies on both surgical techniques and consequences, a significant knowledge gap remains regarding how best to approach the perioperative anaesthesia challenges for EPP and ePD.It is unknown if the risk stratification processes for such surgeries are standardised or what types of functional and dynamic cardiac and pulmonary tests are employed preoperatively to assist in the perioperative risk stratification. Further, it is unknown whether the types of anaesthesia and analgesia techniques employed, and the types of haemodynamic monitoring tools used, impact on outcomes. It is also unknown whether individualised haemodynamic protocols are used to guide the rational use of fluids, vasoactive drugs and inotropes.Finally, there is a dearth of evidence regarding how best to monitor these patients postoperatively or what the most effective enhanced recovery protocols are to best mitigate postoperative complications and accelerate hospital discharge. To increase our knowledge of the perioperative and anaesthetic treatment for patients undergoing EPP/ePD, this scoping review attempts to synthesise the literature and identify these knowledge gaps. METHODS AND ANALYSIS: This scoping review will be conducted in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analysis Extension for Scoping Review Protocols methodology. Electronic databases, OVID Medline, EMBASE and the Cochrane Library, will be systematically searched for relevant literature corresponding to EPP or ePD and perioperative or anaesthetic management. Data will be analysed and summarised descriptively and organised according to the three perioperative stages: preoperative, intraoperative and postoperative factors in clinical care. ETHICS AND DISSEMINATION: Ethics approval was not required. The findings will be disseminated through professional networks, conference presentations and publications in scientific journals.

Predictors of Late Atrial Fibrillation Recurrence After Cardiac Surgery.

BACKGROUND: Although postoperative atrial fibrillation (POAF) frequently occurs early after cardiac surgery, there is a paucity of data evaluating predictors and timing of late atrial fibrillation (AF) recurrence. OBJECTIVES: The authors sought to evaluate predictors of late AF recurrence in patients undergoing cardiac surgery. METHODS: We retrospectively reviewed cardiac surgery patients from 2010 to 2018 with no preoperative diagnosis of AF or atrial flutter. We recorded incidence and timing of late AF recurrence, defined as occurring ≥12 months following surgery. RESULTS: 1,031 patients were included (mean age at surgery 64 ± 12 years, 74% male). Early POAF was recorded in 445 patients (43%). POAF was usually transient, with total AF duration <48 hours in 72% and reversion to sinus rhythm at discharge in 91%. At 4.7 ± 2.4 years follow-up, late AF occurred in 139 patients (14%). Median time to AF recurrence was 4.4 years post-surgery (Q1-Q3: 2.6-6.2 years). Late AF was significantly more likely among patients with early POAF than those without (23% vs 6%; P < 0.001), with highest incidence (38%) in those with POAF duration >48 hours. In a multivariable analysis, early POAF duration >48 hours was a significant predictor of late AF recurrence (HR: 5.9). Surgery type and CHA2DS2-VASc score were not predictive of late AF events. CONCLUSIONS: Post-operative AF episodes of duration ≥48 hours predict recurrent AF episodes over long-term follow-up after cardiac surgery. Implications for arrhythmia surveillance and anticoagulation in patients with longer duration POAF episodes require further study.

Buying time: The use of extracorporeal membrane oxygenation as a bridge to lung transplantation in pediatric patients.

To describe our experience to date of four children with end-stage lung disease who have been bridged with ECMO to successful lung transplantation in our institution. Between March 2006 and June 2012, a total of 21 pediatric patients successfully underwent lung transplantation within The Alfred's lung transplantation program. This included four children who were bridged on ECMO prior to transplantation according to the "ECMO bridge to transplant" protocol and whose clinical notes and outcomes were reviewed. Lung transplantation is an established life-saving treatment for patients with severe lung disease, but remains limited due to scarcity of suitable donor organs. This is a particular issue in the pediatric setting, where the smaller child waits disproportionately longer compared with adult patients for size-matched donor lungs. As ECMO has become more widely accepted, its use as a bridge to lung transplantation in pediatric patients with severe acute lung injury or end-stage chronic lung disease has been considered. The medical notes from the four pediatric patients were retrospectively reviewed. Our report describes excellent short- and medium-term outcomes in a small number of children who have been bridged to transplant on ECMO.

Heart-Lung Transplantation: Technical Modifications to Simplify the Procedure.

Heart-lung transplantation is a mature therapy but has perioperative complications, such as phrenic nerve dysfunction and mediastinal bleeding. We report our technical modifications to simplify the procedure.

Extracorporeal Membrane Oxygenation as a Bridge to Surgical Repair of Postinfarct Ventricular Septal Defect.

Postinfarct ventricular septal defect (PIVSD) is associated with high mortality and the management of these patients has been a challenge with little improvement in outcomes. We commenced a protocol of veno-arterial extracorporeal membrane oxygenation (VA ECMO) for those patients who present in cardiogenic shock with the aim to improve end-organ function before definitive surgical repair to reduce postoperative mortality. This study reviewed the results of this strategy. This was a single-center, retrospective review of all patients who were admitted to our institution with PIVSD in cardiogenic shock from September 2015 to November 2019. Clinical and investigative data were evaluated. Eight patients were referred with PIVSD during this period in cardiogenic shock. One patient had an anterior PIVSD and the other seven had inferior PIVSD. Six patients underwent surgical repair at a median (interquartile range, IQR) of 7 (5-8) days after initiation of VA ECMO. Two patients did not undergo surgical repair. Five patients survived after surgery and one patient died postoperatively due to multiorgan failure. Preoperative use of VA ECMO is a feasible strategy for PIVSD and may improve the results of repair.

Right atrial appendage rupture and cardiac tamponade secondary to blunt trauma.

BACKGROUND: Right atrial appendage rupture from blunt trauma is exceedingly rare, even more so when no other chest wall injuries are found. Very few cases have been documented with respect to survival from such an injury. PURPOSE: To highlight the optimal management of such cases, namely through timely and safe transport to a trauma centre, maintaining a high degree of clinical suspicion for tamponade, early diagnostic ultrasound use, pericardial decompression, haemorrhage control and situational control. CASE PRESENTATION: A case report delineating the diagnostic and therapeutic approach to an individual with right atrial appendage rupture. Subsequent post-operative and convalescent course till hospital discharge.A young male patient involved in a high-speed motor vehicle accident was hypotensive at the scene with altered sensorium. Transport to a trauma centre was delayed due to entrapment and geographical location. An ultrasound done on arrival identified cardiac tamponade, which was successfully treated with an emergent left lateral thoracotomy, pericardial decompression, and haemorrhage control from a ruptured right atrial appendage, with definitive closure in the operating theatre. CONCLUSION: Whilst rare, haemodynamic compromise in the absence of obvious thoracic trauma following high-energy, rapid deceleration mechanisms should raise suspicion for right atrial appendage rupture with pericardial tamponade. Aggressive resuscitation, early diagnostic ultrasound use and urgent pericardial decompression are essential in maximising the likelihood of positive outcomes.

ECMO (extra corporeal membrane oxygenation) in major trauma: A 10 year single centre experience.

Aim To review the indications, complications and outcomes of extracorporeal membrane oxygenation (ECMO) in major trauma patients. Methods Single centre, retrospective, cohort study. Results Over a ten year period, from 13,420 major trauma patients, 11 were identified from our institutional trauma registry as having received ECMO. These patients were predominantly younger (mean 39 +/- 17 years), male (91%) and severely traumatised (median ISS 50, IQR 34 - 54). Veno-venous (VV) ECMO was used predominantly (n = 7, 64%), to treat hypoxic respiratory failure (mean PaO2/FiO2 ratio 69.7 +/- 38.6), secondary to traumatic lung injury. Veno-arterial (VA) ECMO was used less frequently, primarily to treat massive pulmonary embolism following trauma. Major bleeding complications occurred in four patients, however only one patient died from haemorrhage. Heparin free (2/11), delayed (3/11) or low dose heparin (2/11) therapy was frequently utilised. The median time from injury to ECMO initiation was 1 day (IQR 0.5 - 5.5) and median ECMO duration 9 days (IQR 6.5 - 10.5). ECMO was initiated <72 hours in 6 patients, with survival to discharge 67%, compared to 20% in those initiated >72 hours. Overall survival to discharge was 45%, and was higher with VV ECMO (64%), than other configurations (25%). Conclusion ECMO was rarely used in major trauma, the most common indication being severe hypoxaemic respiratory failure secondary to lung injury. In this severely injured cohort, overall survival was poor but better in VV compared to VA and better if initiated early (<72 hours), compared to late.

A donor PaO2/FiO2 < 300 mm Hg does not determine graft function or survival after lung transplantation.

BACKGROUND: A donor arterial PO2/FiO2 (P/F ratio) of less than the 300 threshold would frequently result in either exclusion of the donor or placement of the lungs on ex vivo lung perfusion (EVLP). The aim was to investigate the veracity of the P/F ratio threshold of 300 for donor lung acceptability. METHODS: In 93 brain dead lung donors, arterial blood gases were drawn in the intensive care unit (ICU) just before procurement and each of the 4 donor pulmonary veins in the operating room (OR). No donor lungs were rejected for transplantation based on the last ICU or OR P/F ratio, and EVLP was not used. The recipients were followed up 6 and 12 months following transplantation. RESULTS: There were 93 recipients of bilateral lung transplantation. An arterial P/F ratio of < 300 was largely driven by a low P/F ratio in the lower lobes. There were no differences between the recipients receiving donor lungs where the ICU P/F ratio was < 300 compared with ≥ 300 in the time to extubation, grade of primary graft dysfunction, pulmonary function at 6 and 12 months, and 12-month survival. CONCLUSIONS: From this study:(1) If a donor P/F threshold of 300 was adhered to, 36% would have been rejected, and (2) The donor P/F ratio threshold of 300 is excessively conservative and results in the wastage of donor lungs and the application of unnecessary EVLP.

Survival following traumatic thoracic compartment syndrome managed with VV-ECMO.

Whilst post-traumatic respiratory failure is the most common indication for use of VV-ECMO in trauma patients, its use in traumatic thoracic compartment syndrome is not yet well described. Thoracic compartment syndrome, a rare complication of thoracic trauma, occurs in the setting of chest wall injuries, impaired chest wall compliance, pulmonary contusions and subsequent high ventilatory pressures. This in turn impairs venous return and increases risk of circulatory arrest due to obstructive shock. This case study describes the successful use of VV-ECMO in a young male with thoracic compartment syndrome following severe blunt chest trauma sustained in a high speed motor vehicle crash. Following brief circulatory arrest, thoracic compartment syndrome was relieved during thoracotomy but reoccurred on chest closure. The use of VV-ECMO for oxygenation permitted lower ventilatory pressures, allowing venous return and primary closure of the thoracotomy. The patient subsequently had an excellent functional outcome. This case describes the successful use of VV-ECMO for a novel indication. The indications for ECMO in thoracic trauma patients continue to evolve.

Diagnosis of right atrial mass by perioperative transesophageal echocardiography.

A 67-year-old lady presented with shortness of breath, and was found to have severe triple vessel disease by coronary angiography. Transthoracic echocardiography (TTE) was reported as normal. However a large cystic mass was unexpectedly found in the right atrium by intra-operative transesophageal echocardiography (TOE). This mass was attached to the anterior wall, just by the inter-atrial septum. Flow was demonstrated within the mass by colour flow doppler. The right atrium was opened and the mass excised during cardio-pulmonary bypass. A right coronary artery aneurysm was diagnosed, which was confirmed on histopathological examination. The importance of intra-operative TEE in demonstrating new findings and changing management is highlighted. Coronary artery aneurysm may predispose to embolism or thrombosis if untreated, and may not be seen on angiography or TTE, as in this case.

Sequential unilateral lung volume reduction for emphysema - Stretching the benefit.

Bronchoscopic Lung Volume Reduction (BLVR) and Surgical Lung Volume Reduction (SLVR) and are two different approaches used to remodel severely emphysematous lungs to improve lung function and quality-of-life. We present a case initially referred for lung transplantation, where sequential left upper lobe BLVR and 7 years later right upper lobe SLVR, providing enduring physiological and functional improvement. The potential for sustained benefit via sequential unilateral lung volume reduction is under-appreciated.

Isavuconazole as salvage therapy for mucormycosis.

Mucormycosis carries a high mortality rate with few therapeutic options available. We describe a man with pulmonary/splenic mucormycosis complicating hypoplastic myelodysplastic syndrome on a background of chronic kidney disease, who achieved a complete response with salvage isavuconazole therapy following intolerance of consecutive courses of liposomal amphotericin and posaconazole therapy.

Coronary Vasospasm as an Unexpected Cause of Intraoperative Hemodynamic Instability and Cardiac Arrest.

We present a case of intraoperative coronary artery vasospasm during thoracic surgery causing profound hemodynamic instability. A 68-year-old man undergoing completion right pneumonectomy exhibited intraoperative widespread ST elevation with associated hypotension. Transesophageal echocardiography performed by the anesthetist revealed hypokinetic apical and lateral walls, prompting transportation to the catheterization laboratory, with angiography demonstrating widespread coronary artery spasm. Intracoronary nitroglycerin relieved the vasospasm; however, heparin administration caused significant postoperative bleeding, resulting in cardiac arrest requiring resuscitation and return to the operating room. He ultimately recovered and was discharged to a rehabilitation facility 3 weeks later.

Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis.

This case reports the successful management of chylothorax in a non-transplanted patient with pulmonary lymphangioleiomyomatosis (pLAM). Prolonged initial therapy failed, including total parenteral nutrition, pleural drainage, surgical pleurodesis, and pleurectomy. Commencement of sirolimus 2 mg daily (2 mg alternating days had failed) led to resolution of chylothorax after 20 days. Discontinuation of sirolimus for abdominal surgery led to recurrence of the chylothorax. Reinstitution of sirolimus led to rapid resolution of the effusion, stabilization of lung function, and there has been no recurrence in the ensuing 4 years. We conclude that sirolimus should be considered in the management of pLAM-related chylothorax, perhaps before surgical intervention.

Safety, feasibility, and effect of remote ischemic conditioning in patients undergoing lung transplantation.

BACKGROUND: Primary graft dysfunction (PGD) remains a significant problem after lung transplantation. Data from animal and clinical studies suggest that remote ischemic conditioning (RIC) may reduce ischemia-reperfusion injury in solid organ transplantation. METHODS: A pilot randomized controlled trial of 60 patients undergoing bilateral sequential lung transplantation assessed the utility of RIC in attenuating PGD. Treated recipients underwent 3 cycles of lower limb ischemic conditioning before allograft reperfusion. The primary outcome measure was a comparison of the partial pressure of arterial oxygen/fraction of inspired oxygen ratio (P/F ratio) between treatment groups. RESULTS: No adverse effects of tourniquet application were observed. The mean lowest P/F ratio during the first 24 hours after transplantation was 271.3 mm Hg in the treatment arm vs 256.1 mm Hg in the control arm (p = 0.46). PGD grade and severity and the rate of acute rejection also showed a tendency to favor the treatment arm. Sub-group analysis demonstrated a significant benefit of treatment in patients with a primary diagnosis of restrictive lung disease, a group at high risk for the development of PGD. RIC was not accompanied by systemic release of high-molecular-weight group box 1. Levels of cytokines, high-molecular-weight group box 1, and endogenous secretory receptor for advanced glycation end products peaked within 2 hours after reperfusion and likely reflected donor organ quality rather than an effect of RIC. CONCLUSIONS: RIC did not significantly improve P/F ratios or PGD in this randomized controlled trial. However, encouraging results in this small study warrant a large multicenter trial of RIC in lung transplantation.