Objectives:High-resolution CT (HRCT) guides clinical diagnosis in systemic autoimmune rheumatic disease (SARD) associated interstitial lung disease (ILD). Histologic confirmation by lung biopsy is not commonly done. We aimed to evaluate HRCT-explant pathology agreement in transplanted SARD-ILD patients. Methods:We retrospectively analyzed all idiopathic inflammatory myopathy (IIM), systemic sclerosis (SSc) and rheumatoid arthritis (RA)-ILD patients who underwent double lung transplant at two Canadian centres from 2012 to 2024. HRCT-histology agreement was assessed using weighted kappa (κ) statistics. Results were visualized via alluvial step-by-step diagrams. Results:Among 82 patients (22 IIM, 32 SSc, 28 RA) who underwent lung transplant, RA patients were older, more often smokers and less frequently female than IIM/SSc. IIM patients most commonly required emergency transplantation. Initial HRCT showed NSIP predominance in IIM (59%) and SSc (81%), while UIP dominated in RA (57%). Explant pathology revealed NSIP as the primary pattern in SSc (62%) and UIP in RA (50%). IIM explants exhibited diverse histopathology without dominance. Using weighted κ analysis, we observed moderate HRCT-histology concordance in RA (κ = 0.46, P = 0.002), fair but non-significant agreement in SSc (κ = 0.31, P = 0.06), and no meaningful correlation in IIM (κ = 0.07, P = 0.6). Anti-MDA5+ IIM patients universally developed rapidly progressive ILD, progressing to diffuse alveolar damage or end-stage fibrosis despite initial NSIP on HRCT. Conclusion:SSc-ILD and RA-ILD demonstrated fair-to-moderate HRCT-histology correlation, whereas IIM-ILD shows marked histologic heterogeneity independent of imaging patterns. Anti-MDA5+ disease consistently exhibited aggressive progression, underscoring the limitations of HRCT in predicting IIM-ILD pathology.

OBJECTIVES:To describe clinical characteristics and post-lung transplant outcomes of patients with idiopathic inflammatory myopathies (IIM), SSc and idiopathic pulmonary fibrosis (IPF). METHODS:We retrospectively analysed interstitial lung disease (ILD) patients with IIM (n = 22), SSc (n = 32) and IPF (n = 64) who underwent lung transplantation (2012-24) at two Canadian centres, Vancouver and Montréal. RESULTS:Among IIM patients, 41% were clinically amyopathic at presentation, and 45% had anti-melanoma differentiation-associated protein 5 (anti-MDA5) DM, all with rapid progressive (RP)-ILD, 32% anti-synthetase syndrome, 14% overlap myositis and 9% other DM. In SSc, 88% had pulmonary hypertension (PH) (31% severe) and 78% had oesophageal dysmotility. IIM patients required more frequent pre-transplant intensive care unit (ICU) admission and emergency transplantation. Post-transplant, IIM patients had longer ICU/hospital stays. There were no significant differences in 1-year survival, survival at last follow-up (median: 2.8 years for IIM, 2.5 years for SSc and 3.6 years for IPF), incidence of chronic lung allograft dysfunction or malignancy. Subgroup analyses of IIM [stratified by transplant urgency, extracorporeal membrane oxygenation (ECMO) support and amyopathy] and SSc (stratified by severe PH, oesophageal dysmotility and transplant urgency) showed no significant differences in long-term survival. No autoimmune disease recurrence was observed. CONCLUSION:Despite their underlying autoimmune diseases, post-transplant survival outcomes of selected IIM and SSc patients did not differ significantly from those with IPF. IIM patients with RP-ILD necessitating emergency transplantation and ECMO support exhibited survival similar to those without such complications. However, their more complex pre- and post-transplant courses emphasize the necessity for individualized lung transplant strategies and a multidisciplinary management approach.

ABSTRACT Background Mycobacterium abscessus complex is known to cause life‐threatening disease in lung transplantation (LT) recipients (LTRs). Therefore, many centers consider the presence of M. abscessus as a relative contraindication to LT. Methods We conducted a multicenter retrospective cohort study including all 4 LT centers in Canada. All LTRs transplanted in 2006–2016 with at least one respiratory sample positive for M. abscessus prior or posttransplantation were included. Pulmonary disease was defined using the American Thoracic Society criteria. Minimal follow‐up duration was 5 years after transplantation. Results Among 2181 LTRs across Canada, 14 had M. abscessus disease after LT (incidence rate 1.1/1000 transplant‐years) and 6 had pulmonary colonization (incidence rate 0.5/1000 transplant‐years). Fifteen patients had M. abscessus cultured before LT. Among those with pretransplant M. abscessus , 53% (8/15) developed M. abscessus infection posttransplant (6 disease, 2 colonization). Posttransplant disease was less frequent among those with microbiologic eradication before transplant (0% vs. 85.7%, p = 0.001). LTRs with pretransplant M. abscessus had comparable survival to those without M. abscessus (log rank p = 0.37). Among patients with pretransplant infection, mortality was higher in those who did not achieve microbiological eradication before transplantation (57.1% vs. 0%, p = 0.03) and those who developed disease after transplantation (66.7% vs. 0%, p = 0.01). Conclusions M. abscessus is an uncommon cause of infection among LTRs. Survival among those with pretransplant M. abscessus infection was similar to that observed in noninfected LTRs. However, lack of eradication before transplantation was associated with higher posttransplant disease and mortality.

Rationale Lung transplant recipients require regular monitoring post-transplant. Frequent spirometry assessments facilitate early detection of potential complications including allograft dysfunction and infection, with most centers offering remote home spirometry. Recent advancements in connected home spirometry devices, integrated with digital health applications, now enable seamless data collection and real-time tracking of spirometry, offering the potential for improved monitoring of lung health status, adherence and patient user experience. We sought to evaluate patients’ adherence and gather patient and healthcare providers opinions on digitally-enabled home spirometry as part of a pilot home monitoring program for lung transplant recipients. Methods 40 Lung transplant recipients were enrolled in a remote monitoring program (patient-facing app + Bluetooth-connected spirometer) between August 2023 and Oct 2024. Patient-recorded data were visible to clinicians in real time via a secure browser-based portal. Patients were instructed to complete spirometry measurements daily. In October 2024, a patient opinion questionnaire was provided to all enrolled patients and one to healthcare providers. We analysed the results from these questionnaire and patients’ adherence to home spirometry. Results 39 patients (97.5%) provided ≥1 spirometry measurement and were included in the analysis of adherance. 9 patients (23%) acheived >80% of days they were enrolled. 14 patients (35%) responded to the patient opinion questionnaire, with their results summarised in the chart below. All patients indicated they would continue using the app with the Bluetooth spirometer and recommend it to other lung transplant patients. 6 healthcare providers responded to the questionnaire. Responses to the four structured questions were as follows: Preference for using the monitoring platform: Yes 66.7% (n=4), No 16.7% (n=1), No preference 16.7% (n=1).Ease of use: Easy 50% (n=3), Very easy 33.3% (n=2), Difficult 16.7% (n=1).Usefulness for managing post-transplant care: Yes 100% (n=6).Support for continued use vs. conventional spirometry post-pilot: Yes 83.3% (n=5), No 16.7% (n=1). Conclusions Most respondents expressed positive experiences with the patient-facing app and Bluetooth-connected spirometer, with 100% indicating they would continue using it and recommend it to others. Adherence to daily spirometry was substantially lower than expected, highlighting that lung transplant centers should be aware that despite careful instructions, one cannot assume patient adherence. These findings also indicate strong provider support for the application and spirometer, for its usability and potential to enhance patient management in the post-transplant period.

Pulmonary hypertension (PH) is a serious, progressive, and often fatal clinical-physiologic syndrome. PH is defined by elevated mean pulmonary artery pressure (PAP), typically complicated by right ventricular (RV) failure in advanced stages, and affected patients suffer symptoms, disability, and premature mortality. PH has been a focus of basic and clinical research since the 1950s, with important contributions by many cardiopulmonary clinicians and scientists in multiple Canadian institutions. The history of PH in Canada starts from the earliest reports of electrocardiogram (EKG) diagnosis of PH and care of children with PH associated with congenital heart disease. Innovative basic science research greatly expanded our understanding of the pathobiology of PH, and subsequent clinical translational research advanced our ability to diagnose, assess, and treat patients with PH-targeted medications and surgical approaches, especially for WHO group 1 pulmonary arterial hypertension (PAH) and WHO group 4 chronic thromboembolic PH (CTEPH). Moreover, development of a network of PH expert centers spanning across Canada supported national collaborative excellence in education, clinical care and research. These centers and treatments have helped to significantly prolong and improve the lives of Canadian patients affected by PH. Furthermore, grassroots efforts by patients and their caregivers to support others similarly affected by PH led to development of a national PH community supported by the nonprofit Pulmonary Hypertension Association (PHA) of Canada. True to the spirit of the "History of Respiratory Medicine in Canada", conceived by Louis-Philippe Boulet, this article captures the history and excellence of Canadian contributions to the field of PH.

OBJECTIVE:Interstitial pneumonia with autoimmune features (IPAF) describes patients with interstitial lung disease (ILD) and autoimmune features without meeting criteria for a specific rheumatic disease. No longitudinal data exist on post-transplant outcomes in IPAF patients. We compared baseline demographics, pre-transplant characteristics and post-transplant outcomes between IPAF and idiopathic pulmonary fibrosis (IPF) patients undergoing double lung transplantation. METHODS:We retrospectively analysed lung transplant recipients with ILD in British Columbia between 1 January 2014 and 30 April 2024. Diagnoses of IPAF and IPF were made by multidisciplinary review. Continuous variables were analysed using the Mann-Whitney U test, categorical variables with Fisher's exact test, and survival using Kaplan-Meier analysis. RESULTS:We identified 20 IPAF and 64 IPF patients. IPAF patients were more likely female (50% vs 17%, P = 0.006), on pre-transplant immunosuppression (60% vs 6.3%, P < 0.001) and were less likely to receive antifibrotics (20% vs 64%, P < 0.001). No difference was seen in 1-year or cumulative survival, though survival curves diverged over time favouring IPAF. Post-transplant lung function, acute rejection, infection-related hospitalization, malignancy and chronic lung allograft dysfunction (CLAD) were similar, with non-usual interstitial pneumonia (UIP) IPAF exhibiting a survival advantage over IPF (100% vs 66%, P = 0.044). Explant pathology revealed more UIP patterns in IPF, while IPAF showed more non-specific interstitial pneumonia (NSIP) or unclassifiable patterns. CONCLUSIONS:Post-transplant survival, lung function and complication rates were comparable between IPAF and IPF patients at one year and the last follow-up. This is the first study to report both short- and long-term lung transplant outcomes in IPAF patients.

BACKGROUND: Cytomegalovirus (CMV)-seronegative lung transplant recipients (LTRs) with seropositive donors (CMV D+/R-) have the highest mortality of all CMV serostatuses. Due to immunosenescence and other factors, we hypothesized CMV D+/R- status might disproportionately impact older LTRs. Thus, we investigated whether recipient age modified the relationship between donor CMV status and mortality among CMV-seronegative LTRs. METHODS: Adult, CMV-seronegative first-time lung -only recipients were identified through the Scientific Registry of Transplant Recipients between May 2005 and December 2019. We used adjusted multivariable Cox regression to assess the relationship of donor CMV status and death. Interaction between recipient age and donor CMV was assessed via likelihood ratio testing of nested Cox models and by the relative excess risk due to interaction (RERI) and attributable proportion (AP) of joint effects. RESULTS: We identified 11,136 CMV-seronegative LTRs. The median age was 59 years; 65.2% were male, with leading transplant indication of idiopathic pulmonary fibrosis (35.6%); and 60.8% were CMV D+/R-. In multivariable modeling, CMV D+/R- status was associated with 27% increased hazard of death (adjusted hazard ratio: 1.27, 95% confidence interval: 1.21-1.34) compared to CMV D-/R-. Recipient age >= 60 years significantly modified the relationship between donor CMV-seropositive status and mortality on the additive scale, including RERI 0.24 and AP 11.4% ( p = 0.001), that is, the interaction increased hazard of death by 0.24 and explained 11.4% of mortality in older CMV D+ recipients. CONCLUSIONS: Among CMV-seronegative LTRs, donor CMV-seropositive status confers higher risk of posttransplant mortality, which is amplified in older recipients. Future studies should define optimal strategies for CMV prevention and management in older D+/R- LTRs. J Heart Lung Transplant 2024;43:615-625 (c) 2024 International Society for Heart and Lung Transplantation. All rights reserved.

Chronic lung allograft dysfunction (CLAD) is the most common cause of mortality in lung transplant recipients after the 1st year of transplantation. CLAD has traditionally been classified into two distinct obstructive and restrictive forms: bronchiolitis obliterans syndrome and restrictive allograft syndrome. However, CLAD may manifest with a spectrum of imaging and pathologic findings and a combination of obstructive and restrictive physiologic abnormalities. Although the initial CT manifestations of CLAD may be nonspecific, the progression of findings at follow-up should signal the possibility of CLAD and may be present on imaging studies prior to the development of functional abnormalities of the lung allograft. This review encompasses the evolution of CT findings in CLAD, with emphasis on the underlying pathogenesis and pathologic condition, to enhance understanding of imaging findings. The purpose of this article is to familiarize the radiologist with the initial and follow-up CT findings of the obstructive, restrictive, and mixed forms of CLAD, for which early diagnosis and treatment may result in improved survival. Supplemental material is available for this article. © RSNA, 2021.

Atrial septal defects are one of the most frequently diagnosed congenital heart defects in adulthood. The presence of concurrent moderate or severe pulmonary arterial hypertension without Eisenmenger syndrome at the time of diagnosis can make for a challenging clinical scenario. There is continually evolving literature to determine the ideal approach to this subset of patients. Here we aim to review the clinical presentation, history, medical therapy, and closure options for atrial septal defects-pulmonary arterial hypertension with predominant left-to-right shunting, in the absence of Eisenmenger syndrome.

Background: Centralized care models are often used for rare diseases like pulmonary hypertension (PH). It is unknown how living in a rural or remote area influences outcomes. Methods: We identified all patients from our PH database who carried a diagnosis of WHO Group 1 or WHO Group 4 PH. Using Canadian postal code data, patients were classified as living in a rural area; or a small, medium or large community size. The commute time from patient residence to our clinic was determined using mapping software. We compared baseline catheterization data according to community size and commute time. At follow up, we evaluated the association between community size and commute time with prognostic parameters of functional class, walk distance and echocardiography. Results: Of the 342 patients identified, 72(21%) patients lived in rural areas, while 26(8%), 49(14%) and 195(57%) resided in small, medium and large population centres, respectively. The commute time was 1 h for 160(47%), 1?3 h for 62(18%), and 3 h for 120(35%). There was no association seen for any catheterization parameter by either community size or commute time. At last follow up, there was no association between any prognostic parameter and community size or commute time. Conclusions: We found no association between community size or commute time with severity of illness at diagnosis, or markers of prognosis at follow up. This suggests that patients who reside in rural or remote environments are not experiencing deficiencies in care compared to urban patients. ? 2021 Elsevier B.V. All rights reserved.

The annual report of the International Society for Heart and Lung Transplantation (ISHLT) Thoracic Organ Transplant Registry (Registry) published in the October issue of this Journal T focuses on the transplant recipient. 1 Chambers DC Perch M Zuckermann A et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-eighth adult lung transplantation report - 2021; focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1060-1072 Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar , 2 Hayes D.J Harhay M.O. Cherikh W.S. et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-fourth Pediatric Lung Transplantation Report — 2021; focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1023-1034 Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar , 3 Khush KK Hsich E Potena L et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-eighth adult heart transplantation report - 2021; Focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1035-1049 Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar , 4 Singh TP Cherikh WS Hsich E et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-fourth pediatric heart transplantation report - 2021; focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1050-1059 Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar The analyses highlight the changes that have taken place over the years in recipient selection. A common theme that emerged is increasing willingness of the clinical teams to transplant recipients with higher risk characteristics, such as higher age, increased immunologic risk, ongoing extracorporeal support and presence of comorbidities including diabetes mellitus, obesity, or history of cancer. 1 Chambers DC Perch M Zuckermann A et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-eighth adult lung transplantation report - 2021; focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1060-1072 Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar , 2 Hayes D.J Harhay M.O. Cherikh W.S. et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-fourth Pediatric Lung Transplantation Report — 2021; focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1023-1034 Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar , 3 Khush KK Hsich E Potena L et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-eighth adult heart transplantation report - 2021; Focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1035-1049 Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar , 4 Singh TP Cherikh WS Hsich E et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-fourth pediatric heart transplantation report - 2021; focus on recipient characteristics. J Heart Lung Transplant. 2021; 40: 1050-1059 Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar Despite this, survival after transplant has consistently increased for most patient groups receiving heart or lung transplant.

BACKGROUND:Combined post- and precapillary pulmonary hypertension (CpcPH) portends poor outcomes in pulmonary hypertension related to left heart disease (PH-LHD). While recent evidence does not support the use of targeted pulmonary arterial hypertension (PAH) therapy in PH-LHD, there is a lack of clinical data on their use in CpcPH. We evaluated the outcomes in patients with CpcPH treated with PAH therapies.METHODS:Retrospectively, 50 patients meeting hemodynamic criteria of CpcPH and started on PAH-targeted drugs were identified. Fifty age- and gender-matched PAH patients were chosen as controls. We evaluated the change in 6-minute walk distance, World Health Organization functional class (FC), tricuspid annular plane systolic excursion, BNP or NT-proBNP, and pulmonary artery systolic pressure at 3, 6, 12, and 24 months of follow-up.RESULTS:After adjusting for age and gender, there was no improvement in World Health Organization FC in CpcPH over 2 years (odds ratio of change to FC I/II 1.01, 95% CI: 0.98-1.04). There was no significant improvement in 6-minute walk distance (β coefficient 0.21, 95% CI: -0.98 to 1.4), reduction in BNP/NT-proBNP (β coefficient -12.16, 95% CI: -30.68 to 6.37), increase in tricuspid annular plane systolic excursion (β coefficient 0.074, 95% CI: 0.010-0.139), or decrease in pulmonary artery systolic pressure (0.996, 95% CI: 0.991-1.011) in CpcPH with therapy. There was higher mortality in CpcPH compared to PAH on treatment (24% vs 4%, P = .003).CONCLUSIONS:There were no improvements in symptoms, exercise capacity, or echocardiographic parameters with PAH-targeted therapy in CpcPH. Further studies into potential treatments benefiting this population are needed.

Canadian lung transplant centers currently use a subjective and dichotomous “Status” ranking to prioritize waitlisted patients for lung transplantation. The lung allocation score (LAS) is an objective composite score derived from clinical parameters associated with both waitlist and post‐transplant survival. We performed a retrospective cohort study to determine whether clinical judgment (Status) or LAS better predicted waitlist mortality. All adult patients listed for lung transplantation between 2007 and 2012 at three Canadian lung transplant programs were included. Status and LAS were compared in their ability to predict waitlist mortality using Cox proportional hazards models and C‐statistics. Status and LAS were available for 1122 patients. Status 2 patients had a higher LAS compared to Status 1 patients (mean 40.8 (4.4) vs 34.6 (12.5), P = .0001). Higher LAS was associated with higher risk of waitlist mortality (HR 1.06 per unit LAS, 95% CI 1.05, 1.07, P < .001). LAS predicted waitlist mortality better than Status (C‐statistic 0.689 vs 0.674). Patients classified as Status 2 and LAS ≥ 37 had the worst survival awaiting transplant, HR of 8.94 (95% CI 5.97, 13.37). LAS predicted waitlist mortality better than Status; however, the best predictor of waitlist mortality may be a combination of both LAS and clinical judgment.

The COVID-19 pandemic has necessitated rapid changes in healthcare delivery. Historically, uptake of virtual health care (VH) has been low, especially in specialized clinics; for example, 0.5% of all billable services were provided virtually in Canada in 2015.1 Prior to March 2020, fewer than 5% of all patient visits in our clinic were by VH utilizing our health authority's approved platform; however, this platform required the patient to attend a remote healthcare facility, clearly undesirable during the pandemic. We trialed various platforms including FaceTime® and Zoom®. Barriers existed with both platforms such as specific device requirements, poor technology literacy, and/or privacy concerns. Ultimately, our clinic adopted a web-based platform (Doxy.me®) that allows for videoconferencing with end-to-end encryption between healthcare provider (HCP) and patient. Over the span of 2 weeks, our clinic transitioned to providing more than 90% of patient care utilizing VH, while maintaining usual clinic capacity of approximately 10 post-transplant patient visits per day. Patients transplanted within the previous 3 months and patients with unstable respiratory symptoms were prioritized for in-person visits. Our clinic cares for >300 lung transplant recipients over an extended geography in western Canada; all patients are eligible for VH. Usual clinic workflow was modified to adapt to VH (Table 1). Human resource modifications were negligible after implementation of VH; our clinic has 3.5 transplant coordinators (RNs), 1 MD per clinic day, and 1 PharmD; no additional support staff was needed during or after implementation. Post-visit satisfaction surveys adapted from Sidhu et al2 were completed immediately following VH visits, these surveys are Research Ethics Board exempt. After 6 weeks of data collection, 157 physician surveys were returned; 70% of visits have taken place by videoconference with the remaining 30% by telephone. Physicians reported being satisfied or very satisfied with VH over 90% of time. Physician dissatisfaction was often a result of missing or incomplete blood work or imaging. Technical problems interfered with care goals in only 2.5% of visits. In the same time period, 45 patient surveys were received, 71% from patients who live more than 150 kilometers from the clinic. Compared to usual care, 91% of patients reported that the virtual visit was as good as or better than in-person visit. Fear of substandard care with VH compared to usual care, and lack of social interaction with other clinic patients and staff were identified by multiple patients as drawbacks of VH. The median estimated out-of-pocket expense saved per VH visit compared with in-person visits was CAN $75 per patient (range $0-$1250), and the median estimated amount of time saved was 9 hours (range 0-92 hours). Many barriers exist to successful implementation of VH for lung transplant patients given their frequent need for blood work, imaging, and pulmonary function testing or spirometry. In our case, these challenges were mostly mitigated by a provincial electronic medical record for laboratory and radiology reporting, allowing patients to have testing performed in their local areas. Clinic spirometry and pulmonary function testing were deferred when possible at the discretion of the transplant physician. Virtual health care has been shown to be effective for management of a number of conditions including lung transplant follow-up.2,3 However, concerns with privacy continue to be a consideration, as does the inability for physicians to physically examine the patient, which may lead to suboptimal care. While this has not been demonstrated in the literature, few, if any, studies have been powered to capture this type of information. There is no literature evaluating the outcomes of rapid implementation of VH in lung transplant patients as necessitated by the COVID-19 pandemic. Virtual health care will play a greater role in post-transplant care even after the COVID-19 pandemic. Patients, HCPs, and health systems are likely to demand increased access to VH to reduce cost and time spent accessing care, and to improve the overall patient experience. The greatest challenges associated with VH at this time appear to be technological barriers including inadequate technological infrastructure as well as poor technology literacy.4 Investment in improved VH infrastructure is needed to enable system-wide implementation. Our preliminary data demonstrate that VH can be rapidly implemented in lung transplant clinics with a high degree of patient and physician satisfaction. Research is ongoing to identify the optimal patients and circumstances for whom VH-based post-transplant

Background: Gaucher disease type 1 (GD1) is a lysosomal storage disease rarely resulting in end stage pulmonary hypertension (PH) and interstitial lung disease. There have only been two previous case reports of patients with GD1 receiving lung transplants. Case presentation: We report a case of successful bilateral sequential lung transplantation in a patient with end-stage GD1-related PH. Prior to transplant, the patient was on enzyme replacement therapy with imiglucerase and pulmonary vasodilator therapy with bosentan, sildenafil and epoprostenol. The patient had pre-transplant comorbidities of prior splenectomy and osteopenia. She underwent bilateral sequential lung transplantation with basiliximab, methylprednisolone and mycophenolate mofetil induction. Her explanted lungs demonstrated severe pulmonary arterial hypertensive changes, but no Gaucher cells. She was maintained on MMF, tacrolimus, prednisone, imiglucerase and warfarin post-transplant. Her post-transplant course was complicated by hemorrhagic shock, prolonged support with extracorporeal membrane oxygenation, and acute renal failure requiring dialysis. Despite these complications, the patient was discharged and is doing well nine months post-transplantation. Conclusions: This is one of only three reported cases of lung transplantation in patients with GD1. Each case has involved previously splenectomised, female patients with GD1. This is the first to report transplantation in a patient with severe PH and no pulmonary parenchymal disease. As evidenced in our patient, long term treatment with imiglucerase may eliminate the Gaucher cells in the lungs. The PH in these patients is most consistent with pulmonary arterial hypertension, raising the question of whether this should be reclassified as WHO Group 1 PH.

BackgroundUntil recently, lung transplantation was not considered in patients with human immunodeficiency virus (HIV). HIV seropositive patients with suppressed viral loads can now expect long-term survival with the advent of highly active antiretroviral therapies (HAART); however, HIV remains a relative contraindication to lung transplantation. We describe, to our knowledge, the first HIV seropositive lung transplant recipient in Canada. We also review the literature of previously reported cases of solid-organ transplantation in patients with HIV with a focus on immunosuppression considerations.Case presentationA 48-year old man received a bilateral lung transplant for a diagnosis of desquamative interstitial pneumonia (DIP) attributed to cigarette and cannabis smoking. His control of HIV infection pre-transplant was excellent on HAART, and he had no other contraindications to lung transplantation. The patient underwent bilateral lung transplantation using basiliximab, methylprednisolone, and mycophenolate mofetil (MMF) as induction immunosuppression. He was maintained on MMF, prednisone, and tacrolimus thereafter, and restarted his HAART regimen immediately post-operatively. His post-transplant course was complicated by Grade A1 minimal acute cellular rejection, as well as an enterovirus/rhinovirus graft infection. Despite these complications, his functional status and control of HIV infection remain excellent 24months post-transplant.ConclusionsOur patient is one of only several HIV seropositive lung transplant recipients reported globally. With growing acceptance of transplantation in this population, there is a need for clarification of prognosis post-transplantation, as well as optimal immunosuppression regimens for these patients. This case report adds to the recent literature that suggests HIV seropositivity should not be considered a contraindication to lung transplantation, and that post-transplant patients with HIV can be managed safely with basiliximab, tacrolimus, MMF and prednisone.