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1.  Long-term survival in idiopathic pulmonary arterial hypertension associated with massive pulmonary artery dilation 
The present report describes two patients with long-term survival after being diagnosed with idiopathic pulmonary arterial hypertension more than 20 years earlier. Both patients were treated with calcium channel blockers for several years and are currently maintained on bosentan, an oral endothelin receptor antagonist. Severe dilation of the main pulmonary arteries is present in both patients and may be related to long-term survival with idiopathic pulmonary arterial hypertension.
PMCID: PMC3328880  PMID: 21766084
Idiopathic pulmonary arterial hypertension; IPAH; Pulmonary artery dilation; Survival
2.  Sitaxsentan-induced acute severe hepatitis treated with glucocorticoid therapy 
Endothelin receptor antagonists are commonly used in the treatment of pulmonary hypertension. Sitaxsentan, a selective endothelin A receptor blocker, induces a mild transaminitis in approximately 3% to 5% of patients, but rarely an acute severe hepatitis. A case involving a 61-year-old female with sitaxsentan-induced acute severe liver failure is presented. Depite withdrawal of therapy, her liver tests failed to improve. After six weeks of monitoring, the patient was administered high-dose corticosteroids, with a good clinical and biochemical response. While endothelin receptor antagonists are postulated to cause hepatitis by inhibition of a bile salt transporter pump, an immune-mediated or idiosyncratic mechanism should be considered.
PMCID: PMC3298840  PMID: 22332138
Endothelin receptor antagonists; Hepatits; Sitaxsentan
3.  Exacerbation of pulmonary fibrosis following single lung transplantation 
Acute exacerbations of interstitial lung disease present as clinical deteriorations, with progressive hypoxemia and parenchymal consolidation not related to infection, heart failure or thromboembolic disease. Following single lung transplantation, patients receive maintenance immunosuppression, which could mitigate the development of acute exacerbations in the native lung. A 66-year-old man with fibrotic, nonspecific interstitial pneumonitis presented with fever, hypoxemia and parenchymal consolidation limited to the native lung four years after single lung transplantation. Investigations were negative for infection, heart failure and thromboembolic disease. The patient worsened over the course of one week despite broad-spectrum antimicrobial therapy, but subsequently improved promptly with augmentation of prednisone dosed to 50 mg daily and addition of N-acetylcysteine. Hence, the patient fulfilled the criteria for a diagnosis of an acute exacerbation of pulmonary fibrosis in his native lung. Clinicians should consider acute exacerbation of parenchymal lung disease of the native lung in the differential diagnosis of progressive respiratory deterioration following single lung transplantation for pulmonary fibrosis.
PMCID: PMC3299038  PMID: 22332139
Acute exacerbation; Lung transplantation; Nonspecific interstitial pneumonitis; Pulmonary fibrosis
4.  Assessing response to therapy in idiopathic pulmonary arterial hypertension: A consensus survey of Canadian pulmonary hypertension physicians 
Advances in the field of pulmonary hypertension have led to the emergence of several treatment options for patients with idiopathic pulmonary arterial hypertension aimed at slowing disease progression and improving quality of life. These advances, however, have progressed more rapidly than our understanding of how to use these novel therapies clinically. Although there are no current therapy guidelines or evidence-based recommendations directing therapy, clinicians must make many decisions as to when and how to re-evaluate patients after treatment initiation, to define an adequate response and to plan ongoing management. This survey-based study assessed the attitudes of Canadian physicians caring for patients with idiopathic pulmonary arterial hypertension, and aimed to generate a consensus opinion regarding the methods of reassessment and therapeutic goals after treatment initiation.
Many treatment options are now available for patients with idiopathic pulmonary arterial hypertension (IPAH). Data regarding the optimal combination of therapies are lacking, as is consensus on how to assess response to therapy and when to change therapeutic regimens.
To gather the opinions of Canadian pulmonary hypertension (PH) experts regarding standard practice in the care of IPAH patients after therapy is initiated.
Canadian PH physicians were surveyed using short questionnaires to assess their opinions and practices in the care of IPAH patients. A Delphi forecasting approach was used to gain consensus among Canadian physicians on the most important clinical parameters to consider when assessing patients after the initiation of therapy.
Twenty-six of 37 Canadian PH experts who were invited to participate completed the study. All endorsed the use of combination therapy for IPAH patients despite the lack of universal provincial coverage for this approach. By consensus, WHO functional class, 6 min walk distance and hospitalization for right heart failure were the most important clinical parameters. The most highly rated physical examination parameters were jugular venous pressure, peripheral edema, the presence of ascites and body weight.
The overall approach to care of IPAH patients is similar across PH centres in Canada. A limited number of clinical and physical examination parameters were considered to be most important to reassess patients after therapy is initiated. These parameters, along with definition of threshold values, will facilitate the development of standard practice guidelines for IPAH patients in Canada.
PMCID: PMC3205105  PMID: 22059182
Health survey; Pulmonary hypertension; Therapy
5.  Burkholderia gladioli – a predictor of poor outcome in cystic fibrosis patients who receive lung transplants? A case of locally invasive rhinosinusitis and persistent bacteremia in a 36-year-old lung transplant recipient with cystic fibrosis 
Traditionally, Burkholderia gladioli has been regarded primarily as a plant pathogen; however, it is increasingly being reported to cause illness in humans. B gladioli is closely related to a complex that is well known to cause severe illness in cystic fibrosis patients, in whom poor transplant outcomes have been reported. This report describes a lung transplant recipient who died from severe rhinosinusitis due to B gladioli infection in the context of post-transplant immunosuppression.
There have been very few reports describing postlung transplant outcomes in patients’ infected/colonized with Burkholderia gladioli pretransplant. A case involving a lung transplant recipient with cystic fibrosis who ultimately died as a result of severe rhinosinusitis due to B gladioli infection in the context of postlung transplant immunosuppression is reported.
PMCID: PMC3205109  PMID: 22059186
Bacteremia; Case report; Cystic fibrosis; Lung transplant; Opportunistic infection
6.  Transition from intravenous epoprostenol to oral or subcutaneous therapy in pulmonary arterial hypertension: A retrospective case series and systematic literature review 
Intravenous epoprostenol, a prostaglandin analogue, has been a mainstay of therapy for patients with advanced pulmonary arterial hypertension (PAH) since the early 1990s. This medication has multiple side effects, and sudden discontinuation is potentially associated with severe sequelae. Several recent case series have described the transition from intravenous to newer oral or subcutaneous therapies. A case series detailing the authors’ experience with such transitions, and a systematic lierature review is presented.
All consecutive PAH patients seen at the Vancouver Pulmonary Hypertension Clinic (Vancouver, British Columbia) between June 1995 and July 2009 were reviewed for cases in which weaning or transition from intravenous epoprostenol was attempted. The Cochrane Collaboration, Cochrane Register of Controlled Trials, Journals@Ovid, MEDLINE, EMBASE and Papers First were searched using predefined key words for publications describing transition of PAH patients from parenteral prostanoids to oral or subcutaneous agents.
Of the six patients who attempted, all transitioned successfully to oral or subcutaneous agents, having been on intravenous epoprostenol for a mean of 3.8 years (range 1.8 to 9.75 years). Five are living, surviving a mean of 5.5 years after transition. The literature search yielded nine studies and, of 127 patients described, 82 transitioned successfully. The length of pretransition prostanoid treatment (range 1.7 to 7.6 years) and the post-transition follow-up period (range two months to 70 months) were shorter than for patients described in the present study.
Given the rarity of PAH, the absolute numbers of patients transitioned from intravenous epoprostenol are still low. With the advent of new therapies, these numbers will hopefully increase; continued study is necessary to identify factors that are predictive of success.
PMCID: PMC3328871  PMID: 21766080
Epoprostenol; Pulmonary hypertension; Transition
7.  Risk factors and outcomes associated with chronic obstructive pulmonary disease exacerbations requiring hospitalization 
Acute respiratory exacerbations are the most frequent cause of medical visits, hospitalization and death for chronic obstructive pulmonary disease (COPD) patients and, thus, exert a significant social and economic burden on society.
To identify the risk factors associated with hospital readmission(s) for acute exacerbation(s) of COPD (AECOPD).
A review of admission records from three large urban hospitals in Vancouver, British Columbia, identified 310 consecutive patients admitted for an AECOPD between April 1, 2001, and December 31, 2002. Logistic regression analysis was performed to identify risk factors for readmissions following an AECOPD.
During the study period, 38% of subjects were readmitted at least once. The mean (± SD) duration from the index admission to the first readmission was 5±4.08 months. Comparative analysis among the three hospitals identified a significant difference in readmission rates (54%, 36% and 18%, respectively). Logistic regression analysis revealed that preadmission home oxygen use (OR 2.55; 95%CI 1.45 to 4.42; P=0.001), history of a lung infection within the previous year (OR 1.73; 95% CI 1.01 to 2.97; P=0.048), other chronic respiratory disease (OR 1.78; 95% CI 1.06 to 2.99; P=0.03) and shorter length of hospital stay (OR 0.97; 95% CI 0.945 to 0.995; P=0.021) were independently associated with frequent readmissions for an AECOPD.
Hospital readmission rates for AECOPD were high. Only four clinical factors were found to be independently associated with COPD readmission. There was significant variability in the readmission rate among hospitals. This variability may be a result of differences in the patient populations that each hospital serves or may reflect variability in health care delivery at different institutions.
PMCID: PMC2734440  PMID: 19707601
Chronic obstructive lung disease; Exacerbations; Hospitalization; Risk factors
8.  The sex factor: Epidemiology and management of chronic obstructive pulmonary disease in British Columbia 
The prevalence and mortality of chronic obstructive pulmonary disease (COPD) in women have been predicted to overtake that of men within the next decade. These predictions are based in part on data from surveys using self-reports of a COPD diagnosis. Whether these predictions have been realized is unknown.
The prevalence and mortality of men and women in British Columbia were compared from fiscal years 1992/1993 to 2003/2004 using administrative health services data. Case definitions for COPD were developed using International Classification of Diseases ninth and 10th revision (ICD-9/10) codes applied to medical and hospital data. Individuals 45 years and older, who had at least two physician visits or one hospitalization for specified COPD ICD-9/10 codes within a 365-day window, were considered to be cases. Cases were ascertained from 1992 to 2004.
In 2003/2004, men had a greater prevalence (4.7% versus 4.0% in women) and a higher all-cause mortality rate (5.4% versus 4.1% in women) than women. Both men and women with COPD had low COPD medication use (45%) and low referral for lung function testing (55%). Including the ICD-9 code for ‘bronchitis, not specified as acute or chronic’ (ICD-9 490) in the case definition resulted in a greater prevalence of COPD in women than in men overall, and in the 45 to 64 year age group.
Prevalence and mortality measured with administrative health data do not show evidence of relative increase in the prevalence of COPD for women in British Columbia. However, further analysis of ICD-9 490 may identify an early ‘at-risk’ group, specifically in women.
PMCID: PMC2682163  PMID: 19107241
Administrative database; Chronic obstructive pulmonary disease; Epidemiology; Gender; Mortality; Prevalence; Sex; Spirometry utilization
23.  Skeletal Muscle Strength and Endurance in Recipients of Lung Transplants 
Purpose: Exercise limitation in recipients of lung transplant may be a result of abnormalities in the skeletal muscle. However, it is not clear whether these abnormalities are merely a reflection of the changes observed in the pretransplant condition. The purpose of this paper was to compare thigh muscle volume and composition, strength, and endurance in lung transplant recipients to people with chronic obstructive pulmonary disease (COPD).
Methods: Single lung transplant recipients (n=6) and people with COPD (n=6), matched for age, sex, and BMI participated in the study. Subjects underwent MRI to determine muscle size and composition, lower extremity strength testing and an isometric endurance test of the quadriceps.
Results: Lung transplant recipients had similar muscle volumes and intramuscular fat infiltration of their thigh muscles and similar strength of the quadriceps and hamstrings to people with COPD who had not undergone transplant. However, quadriceps endurance tended to be lower in transplant recipients compared to people with COPD (15 ± 7 seconds in transplant versus 31 ± 12 seconds in COPD, p = 0.08).
Conclusions: Recipients of lung transplant showed similar changes in muscle size and strength as people with COPD, however muscle endurance tended to be lower in people with lung transplants. Impairments in muscle endurance may reflect the effects of immunosuppressant medications on skeletal muscle in people with lung transplant.
PMCID: PMC2845229  PMID: 20467503
lung transplant; muscle function; MRI
24.  The role of the cytomegalovirus antigenemia assay in the detection and prevention of cytomegalovirus syndrome and disease in solid organ transplant recipients: A review of the British Columbia experience 
The pp65 cytomegalovirus (CMV) antigenemia assay has been used as a means of guiding the pre-emptive therapy of CMV disease in solid organ transplant (SOT) recipients. Recently, concerns have been raised regarding the utility of the test to accurately and precisely detect viral activity early enough to reduce the morbidity and mortality associated with CMV
To determine the performance characteristics of the method of antigenemia testing of SOT recipients used at Vancouver General Hospital, Vancouver, British Columbia.
All SOT recipients between January 1, 1999, and June 30, 2000, were retrospectively reviewed for six months following transplantation. Physical examination results, laboratory parameters, antigenemia results and treatment information were reviewed.
A total of 134 kidney, liver, lung and kidney-pancreas transplant recipients were included in the analysis. The overall performance characteristics of the antigenemia assay in predicting CMV disease included a sensitivity of 64%, a specificity of 81%, a positive predictive value of 76% and a negative predictive value of 71%. A mean of 18 days passed between the onset of signs and symptoms of CMV disease/syndrome and the first recorded positive antigenemia result, and only 26% of patients had a positive test result before the onset of symptoms. It was found that an antigenemia test breakpoint of at least one positive cell for defining a positive test provided the most sensitive and specific prediction, with increased odds of developing CMV disease.
Based on performance characteristics, the Vancouver General Hospital's current method of antigenemia testing to guide pre-emptive ganciclovir therapy in SOT patients is not optimal for the early detection of disease. Further study is needed on new molecular testing methods to determine if our ability to predict CMV disease can be improved.
PMCID: PMC2094995  PMID: 18159516
Antigenemia; Cytomegalovirus; Solid organ transplantation
25.  Diagnostic evaluation and management of chronic thromboembolic pulmonary hypertension: A clinical practice guideline 
Pulmonary embolism is a common condition. Some patients subsequently develop chronic thromboembolic pulmonary hypertension (CTEPH). Many care gaps exist in the diagnosis and management of CTEPH patients including lack of awareness, incomplete diagnostic assessment, and inconsistent use of surgical and medical therapies.
A representative interdisciplinary panel of medical experts undertook a formal clinical practice guideline development process. A total of 20 key clinical issues were defined according to the patient population, intervention, comparator, outcome (PICO) approach. The panel performed an evidence-based, systematic, literature review, assessed and graded the relevant evidence, and made 26 recommendations.
Asymptomatic patients postpulmonary embolism should not be screened for CTEPH. In patients with pulmonary hypertension, the possibility of CTEPH should be routinely evaluated with initial ventilation/ perfusion lung scanning, not computed tomography angiography. Pulmonary endarterectomy surgery is the treatment of choice in patients with surgically accessible CTEPH, and may also be effective in CTEPH patients with disease in more ‘distal’ pulmonary arteries. The anatomical extent of CTEPH for surgical pulmonary endarterectomy is best assessed by contrast pulmonary angiography, although positive computed tomography angiography may be acceptable. Novel medications indicated for the treatment of pulmonary hypertension may be effective for selected CTEPH patients.
The present guideline requires formal dissemination to relevant target user groups, the development of tools for implementation into routine clinical practice and formal evaluation of the impact of the guideline on the quality of care of CTEPH patients. Moreover, the guideline will be updated periodically to reflect new evidence or clinical approaches.
PMCID: PMC3006154  PMID: 21165353
Chronic thromboembolic pulmonary hypertension; Clinical practice guideline; Pulmonary endarterectomy; Pulmonary hypertension; Thromboembolism

Results 1-25 (26)