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Pulmonary arterial hypertension is associated with tyrosine kinase inhibitors used in the treatment of chronic myeloid leukemia. Dasatinib is a known cause of drug-induced pulmonary arterial hypertension. There have been case reports linking Bosutinib with deterioration of pre-existing pulmonary arterial hypertension. Here, we present a case of a 37-year-old woman with chronic myeloid leukemia treated with Bosutinib who was diagnosed with pulmonary arterial hypertension. Prior to Bosutinib, she had received Dasatinib without documented cardiopulmonary toxicity. Withdrawal of Bosutinib led to partial reversal of pulmonary arterial hypertension, and with the addition of pulmonary arterial hypertension-targeted treatment, there was near normalization of hemodynamics.

To perform a systematic review and meta-analysis assessing the safety and efficacy of balloon pulmonary angioplasty (BPA) in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH).Systematic literature searches were performed from inception to June 2022 to identify studies assessing BPA for CTEPH. Outcomes of interest included the following functional and hemodynamic measures: (a) six-minute walk distance (6MWD), (b) New York Heart Association (NYHA) status, (c) World Health Organization (WHO)-Functional Class status, (d) cardiac index (CI), (e) mean pulmonary artery pressure (mPAP), (f) mean right atrial pressure (mRAP), and (g) pulmonary vascular resistance (PVR). Subgroup analysis was also performed for BPA in post-pulmonary endarterectomy (PEA) patients. All reported BPA-related complications were also recorded. Forty unique studies with a total of 1763 patients were identified for meta-analysis.All functional and hemodynamic parameters improved significantly following BPA; 6MWD increased 70 m (95% CI 58-82; P 0.001), NYHA class improved by - 0.9 classes (95% CI - 1.0 to - 0.8; P 0.001), WHO-FC class improved by - 1 classes ((95% CI - 1.2 to - 0.9; P 0.001), CI increased 0.26 L/min/mBPA represents a safe and effective treatment option for select individuals with CTEPH with significant improvements in hemodynamic parameters, improved exercise tolerance and a relatively low risk of major complications.

BACKGROUND: Pulmonary endarterectomy (PEA) in severe chronic thromboembolic pulmonary hypertension (CTEPH) is associated with higher risks. However, recent evidence suggests that these risks may be mitigated with the use of extracorporeal membrane oxygenation (ECMO). METHODS: We performed a retrospective analysis of 401 consecutive patients undergoing PEA at the Toronto General Hospital between August 2005 and March 2020. Patients with severe CTEPH defined by pulmonary vascular resistance (PVR) >1,000 dynes.s.cm(–5) at the time of diagnosis were compared to those with PVR 1,000 dynes.s.cm(–5). A greater proportion of patients with PVR >1,000 dynes.s.cm(–5) was treated with targeted pulmonary hypertension (PH) medical therapy (38% vs. 18%, P1,000 dynes.s.cm(–5) compared to those with PVR 1,000 dynes.s.cm(–5) decreased from 10.3% in 2005–2013 to 1.6% in 2014–2020 (P=0.05), while the hospital mortality in patients with PVR 1,000 dynes.s.cm(–5) compared to 78% in patients with PVR

Pulmonary hypertension (PH) defined as a mean pulmonary arterial pressure ≥25 mmHg is a common complication in hemodialysis occurring in up to 58% of patients. PH is classified according to its etiology. We report in a patient with severe PH of mixed etiology (connective tissue disease and left-sided heart failure) who improved after initiation of intensive home hemodialysis. We postulated that the use of a frequent mode of hemodialysis may ameliorate PH via enhanced volume control. Thereby, an intensive hemodialysis schedule may be preferred renal replacement modality in this patient population.

Determine the long-term outcome and need for additional therapy after pulmonary endarterectomy (PEA) for segmental chronic thromboembolic pulmonary hypertension.Retrospective analysis of a prospective cohort of 401 consecutive Canadian patients undergoing PEA between August 2005 and March 2020 in Toronto. The outcome of segmental disease defined as Jamieson type 3 was compared with more proximal disease defined as Jamieson type 1 and 2. The cohort was divided into 3 intervals to analyze the trend over time: 2005-2010, 2011-2015, and 2016-2020.Type 3 disease accounted for 41% of patients undergoing PEA durig 2016-2020 compared with 7% in 2006-2010. Total pulmonary vascular resistance improved by 505 ± 485 dynes/s/cmPEA achieved excellent early and long-term results in segmental chronic thromboembolic pulmonary hypertension. However, patients with segmental disease are at increased risk of requiring additional therapy after PEA and should be carefully monitored.

Purpose: Initial combination therapy with ambrisentan and tadalafil has been demonstrated superior to either agent alone in pulmonary arterial hypertension (PAH). More recently, the OPTIMA trial showed efficacy of another combination of endothelin receptor antagonist and phosphodiesterase 5-inhibitor, macitentan and tadalafil, as initial therapy for PAH. The objective of this study was to assess the effectiveness, tolerability, and safety of macitentan and tadalafil in a real-world clinical setting.Methods: This single centre, retrospective cohort study identified adult patients newly diagnosed with PAH between January 2014 and December 2017 who were started on macitentan and tadalafil. Patients were retrospectively followed for one year. Effectiveness was evaluated via change from baseline in disease risk profile based on a validated score incorporating World Health Organization functional class, 6-minute walk distance (6MWD), B-type natriuretic peptide (BNP), and hemodynamics on follow-up right heart catheterization. Secondary endpoints included change in 6MWD, BNP, and hemodynamic variables. Drug tolerability and adverse events were assessed.Results: The cohort included 46 patients, 8 of whom (17%) did not tolerate and discontinued either macitentan or tadalafil. Median time to follow-up was 161 days (IQR 72). 42% of patients with an initially moderate or high risk disease profile improved to low risk. Secondary endpoints showed a reduction in the geometric mean of pulmonary vascular resistance of 45% (95% CI 29, 57%) and improvement in 6MWD of 88m (95% CI 27, 148m).Conclusion: In a real-world setting, macitentan and tadalafil as initial combination therapy for PAH was well tolerated and yielded clinical benefit.

Patients with chronic thromboembolic pulmonary hypertension (CTEPH) and decompensated right heart failure (DRHF) have worse outcomes after pulmonary endarterectomy (PEA). We reviewed the role of central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge to recovery after PEA in these patients.Of 388 consecutive patients undergoing PEA, 40 (10.3%) were admitted with DRHF before PEA. This group was compared to the remaining 348 patients undergoing PEA (elective group). We also compared 2 periods: 2005-2013 (n = 120) and 2014-2019 (n = 268) after which early central VA-ECMO was introduced as a strategy to manage difficulty weaning from cardiopulmonary bypass (CPB).The proportion of patients with DRHF remained similar between the first and second period (13% vs 9%, p = .2). The number of VA-ECMO bridge to recovery increased from 0.8% in 2005-2013 to 6.3% in 2014-2019 (p = .02). In the second period, 29% of DRHF patients were transitioned intraoperatively from CPB to central VA-ECMO for a median duration of 3 (2-7) days. After the introduction of central VA-ECMO as a bridge to recovery, the hospital mortality in patients with DRHF dropped from 31% in 2005-2013 to 4% in 2014-2019 (p = .03). In the long-term, the functional recovery and survival after discharged from hospital was similar between the DRHF group and the elective group. However, at 5 years, DRHF patients more frequently required PH targeted medical therapy (45% vs 20% in the elective group, p = .002).Central VA-ECMO as a bridge to recovery is an important treatment strategy that can decrease hospital mortality in patients with DRHF and lead to excellent long-term outcome.

Background Radiological assessment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) is critical to decide whether patients should be treated with pulmonary endarterectomy (PEA). Although computed tomography pulmonary angiography (CTPA) is increasingly used for decision making in CTEPH, the value of CTPA to predict surgical findings and outcome has never been explored. Methods We retrospectively reviewed 100 consecutive patients with high-quality CTPA undergoing PEA for CTEPH between May 2015 and December 2017. The most proximal level of disease in the pulmonary artery on CTPA was classified by two blinded radiologists as level 1 (main pulmonary artery), 2a (lobar pulmonary artery), 2b (origin of basal segmental pulmonary artery), 3 (segmental pulmonary artery) or 4 (predominantly subsegmental pulmonary artery). Results CTPA demonstrated level 1 in 20%, level 2a in 43%, level 2b in 11%, level 3 in 23% and level 4 in 3%. A majority of males presented with level 1 (55%) and level 2 (57%), and a majority of females (83%) with level 3 (p=0.01). Levels 3 and 4 were associated with longer duration of circulatory arrest (p=0.03) and higher frequency of Jamieson type III disease at surgery (p, High-quality CTPA provides accurate evaluation of CTEPH patients. The level of disease on CTPA can predict surgical difficulty and the need for targeted PH therapy after PEA. https://bit.ly/317ZBSu

Background Pulmonary endarterectomy (PEA) is a curative procedure for patients with chronic thromboembolic pulmonary hypertension. Body composition and exercise capacity have been associated with adverse outcomes in patients undergoing cardiothoracic surgeries, but their significance with PEA is unclear. We evaluated the association of body composition and 6-minute walk distance (6MWD) with disease severity, hospital length of stay, discharge disposition and post-operative functional recovery. Methods Retrospective, single-center cohort study of patients who underwent PEA (Jan/2014-Dec/2017). Body composition (skeletal muscle mass and adiposity cross sectional area, body mass index) was quantified using thoracic computed tomography with Slice-O-Matic software. Association of body composition measures and 6MWD with clinical outcomes was evaluated using multivariable regression models. Results 127 patients (58±14 years; 42% males, Body Mass Index: 31±7 kg/m2, 6MWD: 361±165 m) were included. Muscle and 6MWD were associated with disease severity measures. Of those surviving hospitalization (n=125), a greater 6MWD was associated with a shorter hospital stay (1.9 median days per 100m, p Conclusions Exercise capacity was a more prognositc marker of PEA outcomes compared to body composition. Future research is needed to explore pre-PEA rehabilitation strategies.

Systemic sclerosis, or scleroderma, is a connective tissue disease that causes fibrosis of the skin and potentially internal organs (1). The most common lung findings in those with scleroderma are interstitial lung disease and pulmonary hypertension (1). Here we describe a 58-year-old man with scleroderma, interstitial lung disease and pulmonary arterial hypertension. He presented with atypical pulmonary manifestations and an acute kidney injury caused by a new diagnosis of ANCA-associated vasculitis.

A 40-year-old woman was referred to pulmonology after presenting with dyspnoea and self-limiting haemoptysis. Chest CT revealed diffuse ground glass opacities and small thin-walled cysts. Bronchoalveolar lavage cultures were negative and cytology revealed haemosiderin-laden macrophages. Transthoracic echocardiogram was normal. Connective tissue disease and vasculitis work-up were negative. Vascular endothelial growth factor-D level was indeterminate. Lung function was normal. She underwent video-assisted thoracoscopic lung biopsy. In addition to findings consistent with lymphangioleiomyomatosis, histopathological examination identified haemosiderosis without capillaritis, confirming a diagnosis of diffuse alveolar haemorrhage in the context of the associated clinical and radiographic features. Follow-up imaging after 5 months showed resolution of the diffuse ground glass opacities. Pharmacotherapy with sirolimus was not initiated due to absence of deterioration in pulmonary function. Diffuse alveolar haemorrhage in patients with lymphangioleiomyomatosis is a rare but important presentation. The few previously reported cases progressed to respiratory failure requiring mechanical ventilation.

Background and objective In this study, we evaluated survival in rheumatoid arthritis-associated pulmonary arterial hypertension (RA-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH) patients, and evaluate differences in disease severity and treatment. Methods We conducted a retrospective cohort study of RA-PAH and IPAH at the University Health Network Pulmonary Hypertension Programme, Toronto, Canada. The primary outcome was time to all-cause mortality. We evaluated survival using Kaplan–Meier curves. Using a propensity score-matched cohort, we used Cox proportional hazards models to estimate survival. Results Screening 1385 patients identified 18 RA-PAH and 155 IPAH patients. RA-PAH patients had an older median age of onset (64.0 vs 53.7 years) and lower baseline mean pulmonary arterial pressure (mPAP) (41 vs 50 mm Hg, P = 0.02). RA-PAH patients tended to have a higher proportion of females (83% vs 70%, relative risk 0.55, 95% confidence interval (CI): 0.19–1.57), lower proportion with baseline World Health Organization functional class III/IV (39% vs 52%), lower median baseline brain natriuretic peptide (58.4 vs 95.0 pg/mL) and longer baseline 6-min walk distance (440 vs 397 m). There were 35 deaths, 2/18 (11%) RA-PAH patients and 33/155 (21%) IPAH patients. The unadjusted 1-year survival was 93% for RA-PAH and 94% for IPAH. In the matched cohort, there were seven deaths: 2/18 (11%) RA-PAH and 5/18 (28%) IPAH patients, hazard ratio 1.53 (95% CI: 0.15–2.84). Separation of survival curves did not achieve statistical significance, log–rank 0.56. Conclusions Compared with IPAH patients, RA-PAH patients have an older age of onset and lower baseline mPAP. RA-PAH patients have comparable survival to IPAH patients.

Purpose Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). The outcome after PEA for disease located in the segmental pulmonary arteries (PA) remains unclear. Methods We reviewed 282 consecutive patients undergoing PEA in our institution between 01/2005 and 12/2017. Level of disease was defined surgically by the most proximal extension as level 1 (main PA), level 2 (lobar-interlobar PA), level 3 (proximal segmental PA), and level 4 (distal segmental-subsegmental PA). Results The proportion of patients with level 3 (n=66) and 4 (n=28) disease increased from 4% to 24% and from 0% to 16%, respectively, between 2005-2008 and 2015-2017. The proportion of females was higher in level 3 and 4 compared to level 1 and 2 (70% vs 41%, p 1,200 Dynes.s.cm−5 was higher in level 1 (38%) and 4 (39%) than in level 2 (21%) and 3 (21%) (p=0.02). Total circulatory arrest time (min) was longer in level 4 (48±16) than in level 1 (42±11), 2 (39±14) and 3 (41±15) (p=0.02). The 30-day mortality ranged between 1.3% in level 1 and 3.6% in level 4 (p=0.8). Mean PA pressures (mmHg) significantly improved postoperatively from 50±12 to 23±7 in level 1 (p 1,200 Dynes.s.cm−5 (p=0.01) were independent predictor for targeted PH therapy after PEA, while sex was not (p=0.1). Conclusion PEA in segmental disease provides significant reduction in PA pressures and is associated with excellent long-term outcomes.

Chronic thromboembolic pulmonary hypertension (CTEPH) is still largely underdiagnosed in the general population. Although transthoracic echocardiogram (TTE) is recommended to screen for CTEPH, it may not detect patients with chronic thromboembolic disease (CTED) and mild or exercise-induced pulmonary hypertension (PH) who could also benefit from pulmonary endarterectomy (PEA).All patients referred to our CTEPH program with persistent mismatched perfusion defects on ventilation-perfusion (VQ) scan between January 2005 and June 2015 were divided into three groups according to TTE and right heart catheterization (RHC) as follows: (1) typical CTEPH group (PH on RHC and TTE), (2) TTE-negative (neg) CTEPH group (PH on RHC, but not TTE), or (3) CTED group (no PH on RHC and TTE).Of 225 patients with abnormal VQ scans, 188 (84%) had typical CTEPH, 15 had TTE-neg CTEPH, and 22 had CTED. PEA was performed in 179 patients (80%). Reasons for exclusion in CTEPH patients included primarily distal disease (n = 11) and comorbidities (n = 10). In contrast, the absence of functional limitation was the main cause of exclusion in CTED patients (75% versus 3% in CTEPH patients, p0.0001). The 90-day mortality rate after PEA was 4% in the typical CTEPH group and 0% in the TTE-neg CTEPH and CTED groups. Pulmonary arterial pressures and functional class significantly improved after PEA in all three groups.Patients with mild CTEPH can benefit from PEA, but may not be detected by TTE. Symptomatic patients with functional limitation and persistent mismatched perfusion defects on VQ scan should undergo further investigations with pulmonary angiogram and RHC.

Rosai Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that typically presents as painless cervical adenopathy. Occasionally, Rosai Dorfman disease may involve extranodal sites and it can be associated with constitutional symptoms. In this report, we present a patient with progressive dyspnea on exertion, obstruction on spirometry, and paratracheal soft tissue thickening extending to the proximal bronchi and hila. After extensive radiologic and interventional measures, including multiple biopsies, the diagnosis was established on pathology showing characteristic features of Rosai Dorfman disease in a paratracheal lymph node and features of extranodal Rosai Dorfman disease with extensive fibrosis in the paratracheal mass. In addition, the involved paratracheal lymph node demonstrated focally increased IgG4-positive plasma cells, a finding that was not present in the tracheal mass. A review of intrathoracic manifestations of Rosai Dorfman disease including the proposed causes and causative triggers is presented. The histological features and the current understanding of the management and prognosis are also discussed. This unusual presentation highlights the need to consider histiocytic disorders in the differential diagnosis of paratracheal disease.

Background The impact of male sex as a determinant of health outcomes in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is controversial. The primary objective of this study was to evaluate the effect of sex on survival in patients with SSc-PAH. The secondary objectives were to evaluate the effect of sex on age of PAH diagnosis, time from SSc diagnosis to PAH diagnosis, and SSc disease manifestations. Methods Sex-based disparities were evaluated in a cohort of SSc-PAH patients with a primary outcome of time from PAH diagnosis to all-cause mortality. Secondary outcomes were differences in age of diagnosis, disease duration, and SSc manifestations. Survival differences were evaluated using Kaplan-Meier and Cox proportional hazard models. Results We identified 378 SSc-PAH (58 males, 320 females) patients, with a female:male ratio of 5.5:1. Males had a shorter mean ± standard deviation time from SSc diagnosis to PAH diagnosis (1.7 ± 14 versus 5.5 ± 14.2 years); shorter PAH duration (3.5 ± 3.1 versus 4.7 ± 4.2 years), increased frequency of renal crisis (19 % versus 8 %, relative risk (RR) 2.33, 95 %CI 1.22, 4.46), interstitial lung disease (67 % versus 48 %, RR 1.41, 95 %CI 1.14, 1.74), and diffuse subtype (40 % versus 22 %, RR 1.84, 95 %CI 1.26, 2.69). Males appeared to have decreased 1-, 2-, 3-, and 5-year survival (83.2 %, 68.7 %, 53.2 %, 45.6 %) compared to females (85.7 %, 75.7 %, 66.4 %, 57.4 %). However, there was no difference in mortality between sexes (HR 1.43 (95 %CI 0.97, 2.13). Conclusions Sex disparities appear to exist in the frequency of PAH, time to PAH diagnosis, PAH disease duration and SSc disease burden. However, male sex does not independently impact SSc-PAH survival.

Background Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (PH). However, this surgery remains performed in few experienced centres only. The goal of the study is to review our overall experience since the implementation of our program in August 2005. Methods Review all patients referred to our program between August 2005 and July 2011. Results Among 84 consecutive patients referred to our program, 52 patients underwent elective PEA and 6 emergency PEA. After PEA, 74% patients were extubated within 2 days, 71% were discharged from the intensive care unit within 4 days and 64% were discharged from hospital within 15 days. One patient undergoing elective surgery and 2 patients undergoing emergency surgery died within 30 days of surgery for an operative mortality of 1.9% after elective pulmonary endarterectomy and an overall operative mortality of 5.2%, when the 6 emergency operative cases were included. The total pulmonary vascular resistance decreased from 965 ± 445 to 383 ± 162 dynes per second per cm −5 and was associated with significant improvement in World Health Organization/New York Heart Association (WHO/NYHA) functional class, 6 minutes walk distance, echocardiographic findings, and brain natriuretic peptide level at 6 months after PEA. After a median follow-up of 23 months (1-65 months), 3 patients had to be started on targeted PH therapy for deterioration of their (WHO/NYHA) functional class. Conclusions Elective PEA can be performed with limited risk, and results in excellent early and long-term outcome. All patients diagnosed with chronic thromboembolic PH should be referred for consideration of PEA in a specialized centre.

Blood transfusion requirements were reviewed for a consecutive series of 25 patients undergoing elective pulmonary endarterectomy (PEA) between August 2005 and March 2009 in our institution. Patients were divided into two groups based on the implementation of a conservative blood transfusion algorithm that combined antifibrinolytic therapy, intraoperative blood sequestration, blood salvage and lack of correction of coagulation parameters in the absence of ongoing bleeding. Despite similar perioperative coagulation profiles in the two groups, the introduction of a conservative blood transfusion algorithm was associated with a significant increase in the number of patients receiving no homologous blood products. Of 16 patients who underwent surgery after the introduction of the algorithm, nine (56%) required no homologous blood products and five (31%) required one or two units of homologous red blood cells only. The international normalized ratio normalized within six to 12 hours after discontinuation of cardiopulmonary bypass without transfusion of fresh frozen plasma or platelets in 13 of the 16 patients. In conclusion, a conservative blood transfusion strategy allows PEA to be safely performed with no or minimal blood product transfusions in a majority of patients despite deep hypothermic circulatory arrest.

OBJECTIVES Extracorporeal life support (ECLS) for rescue after pulmonary endarterectomy (PEA) has become a viable option. This study aims to present a single-centre experience looking at the indications and outcome of ECLS after PEA. METHODS Retrospective analysis of all patients undergoing PEA from January 2008 to January 2015 in our institution. RESULTS Among 144 consecutive patients undergoing PEA for chronic thromboembolic pulmonary hypertension, 6 (4%) received ECLS postoperatively for right ventricular (RV) failure (n = 3), severe hypoxaemia (n = 2) and haemorrhagic pulmonary oedema (n = 1). ECLS configuration was central veno-arterial (cVA) in 3 patients, peripheral VA (pVA) in 1 and veno-venous (VV) in 2. One patient with cVA was switched to VV after 5 days. Overall ECLS duration ranged between 3 and 39 (median 5) days. ECLS patients had higher preoperative total pulmonary vascular resistance (TPR) compared with non-ECLS patients (1477 ± 671 vs 954 ± 462 Dynes.s.cm(-5), P = 0.009) and more frequently required hospital admission for RV failure before surgery (50 vs 9%, P = 0.02). The overall in-hospital mortality rate for all patients was 2% (3/144), including one ECLS patient on pVA. The remaining 5 ECLS patients (83%) were discharged from the hospital and are alive after a median follow-up of 11 (range 6-27) months. Two ECLS patients (40%) are on therapy for residual PH compared with 13 (10%) in the non-ECLS patients (P = 0.09). CONCLUSIONS ECLS is a safe and important rescue option after PEA. The use of ECLS may expand eligibility for PEA by allowing sicker patients to undergo surgery.

Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.

Venous thromboembolism (VTE) is one of the most common and easily preventable complications following surgical procedures. Anticoagulant prophylaxis has been shown to be highly effective, safe and cost-effective in the prevention of VTE in a large number of surgical groups and should be routinely utilized in these patients. Mechanical prophylaxis should be reserved primarily for the small proportion of patients with a very high bleeding risk. In general surgery, and major gynecologic or urologic surgery, low dose heparin or low molecular weight heparin are recommended options. For hip and knee arthroplasty and hip fracture surgery, the recommended options are low molecular weight heparin, fondaparinux, and dose-adjusted warfarin. Almost all trauma and critical care patients should receive thromboprophylaxis, although individual prophylaxis decisions should be based on the thrombosis and bleeding risks, both of which may change over time. Anticoagulant thromboprophylaxis can be implemented concomitantly with regional anesthesia and continuous epidural analgesia as long as a few basic principles are followed and caution is used.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH. To our knowledge, PAH associated with adult HLH has only been described in the literature once before. PAH may now be a potential differential diagnosis for patients with HLH who present with respiratory symptoms.

The outcome of patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) after urgent hospitalization for decompensated right heart failure (DRHF) remains unclear.Among 120 consecutive patients undergoing PEA, 16 (13%) presented with a history of urgent hospitalization for DRHF (severe RHF group). This group was compared with the remaining 104 patients presenting with total peripheral vascular resistance (TPR)1,200 dynes · sec · cm(-5) (n = 78, control group) and1,200 dynes · sec · cm(-5) (n = 26, high TPR group).DRHF occurred predominantly in patients with TPR1,200 dynes · sec · cm(-5) (prevalence of 32% vs 5% in patients with TPR1,200 dynes · sec · cm(-5), p0.0001). The overall in-hospital mortality after PEA was 4% (n = 5). All deaths occurred in patients with TPR1,200 dynes · sec · cm(-5) and DRHF. The proportion of patients with residual PH immediately after surgery was higher in the high TPR group (50%) and severe RHF group (56%) compared with the control group (29%; p = 0.04). In multivariate analysis, risk factors for residual PH after surgery were TPR1,200 dynes · sec · cm(-5), Jamieson disease Type III-IV, and female gender. A history of DRHF by itself was not a risk factor for residual PH after PEA. The overall 5-year survival was 87%. After a median follow-up of 20 months, the proportion of patients receiving medical therapy for residual PH was higher in patients with post-operative mean pulmonary artery pressure ≥35 mm Hg (61% vs 9%, p = 0.0007).DRHF occurs more frequently in patients with TPR1,200 dynes · sec · cm(-5), increasing the operative risk in these patients. The outcome of patients with high TPR in the absence of DRHF is excellent. However, patients with residual mean pulmonary artery pressure ≥35 mm Hg frequently receive pulmonary hypertension therapy after PEA.

Depatrment of Respirology, University of Toronto, Toronto General Hospital, Toronto, Ontario Correspondence: Dr Dmitry Rozenberg, Depatrment of Respirology, Toronto General Hospital, 9N 965 – 585 University Avenue, Toronto, Ontario M5G 2N2. Telephone 416-340-4591, fax 416-340-3109, e-mail dmitry.rozenberg@uhn.ca A 41-year-old woman presented with a one-year history of progressive dyspnea, early satiety and abdominal cramping. A clinical diagnosis of lymphangioleiomyomatosis (LAM) was made based on a confirmed right-sided chylous effusion (milky, exudative and triglyceride level of 52.6 mmol/L) and characteristic cystic changes on a highresolution computed tomography scan of the chest (Figure 1). A large left-sided retroperitoneal lymphangioleiomyoma supported the diagnosis (Figure 2), with no renal angiomyolipoma or clinical evidence of tuberous sclerosis. She was started on sirolimus therapy, which resolved her dyspnea and abdominal symptoms within six months. Her forced expiratory volume in 1 s (FEV1) (1.6 L) and FVC (2.1 L) had markedly improved during this time interval to 2.6 L and 4.2 L, respectively. Her chest x-ray demonstrated almost complete resolution of the right pleural effusion (Figure 3) and the lymphangioleiomyoma had also decreased in size (Figure 4). The patient remains clinically stable on sirolimus with few side effects and her FEV1 and FVC on therapy at nine months increased to 3.3 L (96%) and 4.8 L (118%), respectively. The present case is a dramatic example of a chylous pleural effusion and massive intra-abdominal lymphangioleiomyoma responding to sirolimus therapy. images in respiratory meDicine

In this study, we evaluated survival in rheumatoid arthritis-associated pulmonary arterial hypertension (RA-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH) patients, and evaluate differences in disease severity and treatment.We conducted a retrospective cohort study of RA-PAH and IPAH at the University Health Network Pulmonary Hypertension Programme, Toronto, Canada. The primary outcome was time to all-cause mortality. We evaluated survival using Kaplan-Meier curves. Using a propensity score-matched cohort, we used Cox proportional hazards models to estimate survival.Screening 1385 patients identified 18 RA-PAH and 155 IPAH patients. RA-PAH patients had an older median age of onset (64.0 vs 53.7 years) and lower baseline mean pulmonary arterial pressure (mPAP) (41 vs 50 mm Hg, P = 0.02). RA-PAH patients tended to have a higher proportion of females (83% vs 70%, relative risk 0.55, 95% confidence interval (CI): 0.19-1.57), lower proportion with baseline World Health Organization functional class III/IV (39% vs 52%), lower median baseline brain natriuretic peptide (58.4 vs 95.0 pg/mL) and longer baseline 6-min walk distance (440 vs 397 m). There were 35 deaths, 2/18 (11%) RA-PAH patients and 33/155 (21%) IPAH patients. The unadjusted 1-year survival was 93% for RA-PAH and 94% for IPAH. In the matched cohort, there were seven deaths: 2/18 (11%) RA-PAH and 5/18 (28%) IPAH patients, hazard ratio 1.53 (95% CI: 0.15-2.84). Separation of survival curves did not achieve statistical significance, log-rank 0.56.Compared with IPAH patients, RA-PAH patients have an older age of onset and lower baseline mPAP. RA-PAH patients have comparable survival to IPAH patients.

Despite a major reduction in pulmonary vascular resistance (PVR), patients with chronic thromboembolic pulmonary hypertension (CTEPH) do not always return to functional New York Heart Association (NYHA) class I after pulmonary endarterectomy (PEA). We hypothesized that residual abnormal compliance (Cp) after PEA is associated with incomplete functional recovery despite major improvement in PVR.The Cp of 34 consecutive patients with CTEPH was assessed before and after PEA. Cp was defined as stroke volume over pulse pressure and was divided into three groups:2.0 mL/mm Hg, 2.0 to 3.9 mL/mm Hg, and ≥ 4 mL/mm Hg. To establish predicted Cp after PEA, we collected an age- and gender-matched control group.Before PEA, Cp was2.0 mL/mm Hg in 82% (n = 28) of the patients. After PEA, Cp improved to 2.0 to 3.9 mL/mm Hg in 11 patients and to ≥ 4.0 mL/mm Hg in 14 patients. Residual Cp2.0 mL/mm Hg was associated with delayed extubation and prolonged hospital stay. At 3 months' follow-up, 13 patients (93%) with postoperative Cp ≥ 4.0 mL/mm Hg returned to NYHA class I, whereas 45% with Cp of 2.0 to 3.9 mL/mm Hg and 25% with Cp2.0 mL/mm Hg returned to NYHA class I. In multivariate analysis, postoperative Cp ≥ 4.0 mL/mm Hg was the only predictor of functional recovery to NYHA class I. The group of patients with postoperative Cp ≥ 4.0 mL/mm Hg was also the only group to achieve hemodynamic parameters similar to those of their matched control subjects.Postoperative Cp is an important predictor of recovery after PEA. Residual vascular stiffness after PEA can be associated with persistent functional limitation and lack of complete remodeling of the right ventricle.

Objective.—To determine if there is an association between migraines and intrapulmonary right-to-left shunt. Background.—Several studies have described an association between migraines and intracardiac right-to-left shunt. Methods.—Patients with hereditary hemorrhagic telangiectasia (HHT) were retrospectively recruited from the Toronto Hereditary Hemorrhagic Telangiectasia Center Clinical Database. All patients had been prospectively, systematically asked about a history of migraines and systematically screened for pulmonary and cerebral arteriovenous malformations (AVMs). All patients with a definite diagnosis of HHT, assessed during a 2-year period (February 1997 to April 1999), were included. Univariate analyses and logistic regression were performed, for migraine as the dependent variable and the following independent variables: age, sex, pulmonary AVMs, and cerebral AVMs. Results.—Of the 200 patients assessed during the 2-year period, 124 (62%) had a definite diagnosis of HHT and were included in the analysis. Eighty (65%) were females. Forty-seven (38%) of the HHT patients had a history of migraine, of whom 38 (81%) had migraine with aura. The prevalence of migraine was greater in patients with pulmonary AVMs (46%) compared to patients without pulmonary AVMs (33%), OR = 1.7 (0.8 to 3.6), though this did not reach statistical significance (P= .14). Pulmonary AVMs were significantly associated with migraine (OR = 2.4, 95% CI = 1.1 to 5.5, P= .04), after adjustment for age and sex, using logistic regression. Conclusions.—There is a significant association between intrapulmonary right-to-left shunt and migraine.

A 19-year-old woman presented to her local emergency department with acute chest pain and shortness of breath. She denied any cough, haemoptysis, fever or leg swelling. Her past medical history was significant for premature birth at 28 weeks gestational age. She required surgical correction of a right clubfoot as an infant. In addition, she had intestinal malrotation complicated by caecal volvulus and perforation treated with laparotomy at age 13, and an ovarian cystectomy performed at age 17. There was no family history of pneumothorax or respiratory disease. She was a non-smoker on no medications. On examination, she was tachycardic, tachypnoeic and had an oxygen saturation in the low 80s. Her respiratory exam demonstrated decreased air entry to bilateral lung bases. A …