Long-term outcomes of domiciliary intravenous iloprost in idiopathic and connective tissue disease-associated pulmonary arterial hypertension.

BACKGROUND AND OBJECTIVE: There are few published data on the efficacy of i.v. iloprost in pulmonary arterial hypertension (PAH). We present long-term outcomes in PAH patients receiving i.v. iloprost in a large UK referral centre. METHODS: Eighty patients with idiopathic PAH (iPAH, n = 46) or PAH associated with connective tissue disease (CTD-PAH, n = 34) were identified as receiving domiciliary i.v. iloprost between January 1999 and April 2015. Baseline characteristics, doses achieved, functional class at follow-up and survival data were retrieved from hospital databases. RESULTS: Median maximum dose achieved was 4.6 ng/kg/min in the iPAH group and 5.0 ng/kg/min in CTD-PAH patients. Exercise capacity significantly improved in the first 6 months of therapy in IPAH patients. Overall 1-, 3- and 5-year survival was 78%, 64% and 52% in iPAH (P = 0.002) and 64%, 26% and 21% in CTD-PAH. Independent predictors of survival were age and exercise capacity. CONCLUSION: We report improved survival to that previously reported in iPAH patients treated with domiciliary i.v. iloprost. This may be in part related to higher administered doses. Patients with CTD-PAH had poorer survival, reinforcing the need for early transplantation referral in suitable patients.

Attitudes and barriers to pulmonary arterial hypertension screening in systemic sclerosis patients: A survey of UK-based rheumatologists.

Objectives: The objectives were to explore rheumatologists' current clinical screening practices of pulmonary arterial hypertension in patients with systemic sclerosis in the United Kingdom and to identify barriers to screening and consider potential solutions. Methods: A survey of 31 questions was developed and included six sections: clinician demographics, the importance of screening, screening practices, barriers to screening, treatment and patient education. The survey was disseminated among rheumatologists working in the United Kingdom. Results: Forty-four rheumatologists working in the United Kingdom participated in the study, and the majority completed all the questions. Around one-third (37.0%) worked in specialised systemic sclerosis units (university or general hospitals (54.5% and 45.4%, respectively)). The majority recognised that systemic sclerosis-pulmonary arterial hypertension is a major cause of morbidity and mortality. Over half (60.0%) reported using the DETECT algorithm to screen for systemic sclerosis-pulmonary arterial hypertension, although other algorithms were also sometimes used. All of the respondents utilised transthoracic echocardiogram, and almost all (95.0%) performed pulmonary function tests for screening purposes. Various challenges and barriers were identified relating to systemic sclerosis-pulmonary arterial hypertension screening, with the difficulty in interpreting results from other hospitals and extended wait times for diagnostic tests being the most reported (76.0% and 74.0%, respectively). Most respondents agreed that access to key investigations (87.0%), ongoing clinician education (82.0%), multidisciplinary meetings (79.5%) and a better understanding of proposed screening algorithms (79.5%) could be potential solutions. Conclusion: Screening patients with systemic sclerosis for pulmonary arterial hypertension is crucial to improve survival, but variable practices exist among UK rheumatologists. Solutions include educating healthcare professionals on guidelines, sharing information between centres and integrating care services.

ERS International Congress 2021: highlights from the Pulmonary Vascular Diseases Assembly.

This article aims to summarise the latest research presented at the virtual 2021 European Respiratory Society (ERS) International Congress in the field of pulmonary vascular disease. In light of the current guidelines and proceedings, knowledge gaps are addressed and the newest findings of the various forms of pulmonary hypertension as well as key points on pulmonary embolism are discussed. Despite the comprehensive coverage of the guidelines for pulmonary embolism at previous conferences, discussions about controversies in the diagnosis and treatment of this condition in specific cases were debated and are addressed in the first section of this article. We then report on an interesting pro-con debate about the current classification of pulmonary hypertension. We further report on presentations on Group 3 pulmonary hypertension, with research exploring pathogenesis, phenotyping, diagnosis and treatment; important contributions on the diagnosis of post-capillary pulmonary hypertension are also included. Finally, we summarise the latest evidence presented on pulmonary vascular disease and COVID-19 and a statement on the new imaging guidelines for pulmonary vascular disease from the Fleischner Society.

Evidence of simian virus 40 infection in multiple organ transplant recipients with renal dysfunction.

Electron microscopy (EM), real-time polymerase chain reaction (PCR) and conventional PCR were used to identify viruses associated with infection in 2 transplantation patients. An autologous haematopoietic stem cell, liver and renal transplant recipient was found to be positive for simian virus 40 (SV40). Dual BK virus and SV40 infection was found in a heart and renal transplantation patient. SV40 infection can occur in immunocompromised patients.

Update in the diagnosis and management of acute pulmonary embolism for the non-respiratory physician.

An increasingly common scenario on the acute medical take is that of 'possible pulmonary embolism'. The aim of this article is to update the reader about the available clinical decision tools that can help to avoid the over investigation of such patients, as well as other tools that can support an outpatient management strategy in appropriate patient groups. The importance of risk stratification methodologies in acute pulmonary embolism management is emphasised. We address the evidence on the long-term risk of venous thromboembolism recurrence and show how this can be used to make decisions about duration of anticoagulation. Finally, we discuss a number of special scenarios, including the implications of incidentally discovered isolated subsegmental pulmonary embolus and the management of pulmonary embolus in malignancy and pregnancy.

ERS International Congress, Madrid, 2019: highlights from the Pulmonary Vascular Diseases Assembly.

The 2019 European Respiratory Society (ERS) International Congress, held in Madrid, Spain, had exciting sessions regarding the field of pulmonary vascular disease. The symposia related to the new ERS/European Society of Cardiology (ESC) Guidelines for the diagnosis and management of acute pulmonary embolism were well received, as were sessions on pulmonary hypertension related to lung disease, demonstrating the concept of pulmonary hypertension not being the rarity that it was previously thought to be. The use of risk stratification in relation to pulmonary arterial hypertension (PAH) was heavily featured and the scientific sessions informing the respiratory community of potential biomarkers and targets for future therapies were thought-provoking. This article discusses highlights of the 2019 pulmonary vascular disease sessions as a summary of current knowledge and practice. We have summarised the key points from the sessions pertaining to the new ERS/ESC Guidelines for the management of acute pulmonary embolism. We have also focused on prognostic factors and potential therapies in pulmonary hypertension related to interstitial lung disease. Relating to PAH, we have reviewed the symposia on risk stratification, along with the use of noninvasive measures and the sessions relating to biomarkers in PAH.

Updates in pulmonary hypertension and other pulmonary vascular diseases.

Update on studies related to pulmonary vascular disease published during 2018, addressing different topics in pulmonary hypertension, pulmonary embolism and chronic thromboembolic disease http://bit.ly/2JJUnUP.

Highlights from the ERS International Congress 2018: Assembly 13 - Pulmonary Vascular Diseases.

The 2018 European Respiratory Society (ERS) International Congress in Paris, France, highlighted the subject of pulmonary vascular disease (PVD). 2018 was an exciting year for the PVD community as it was the first ERS International Congress since the formation of Assembly 13, which is dedicated to PVD, pulmonary embolism and the right ventricle. This article aims to summarise the high-quality studies presented at the 2018 Congress into four subject areas: the use of risk stratification in pulmonary arterial hypertension, the molecular mechanisms and treatment of pulmonary hypertension (PH), understanding and improving the right ventricle in PH, and finally, advances in the field of acute pulmonary embolus.

Isolated anterior cruciate ligament deficiency, knee scores and function.

The aim of this study was to ascertain whether there was a correlation between commonly used scoring scales used for follow-up study of management of knee problems and whether age, gender, and time interval between injury and management and type of management affect the assessment of outcome in the management of ACL insufficiency. One hundred and twelve patients who had received ACL reconstruction were identified from hospital records. Each of them received a package containing three questionnaires, namely the Lysholm knee score, Mothadi-QoL, and the Modified Cincinnati Rating Scale. The overall response rate was 55%. There was a relatively strong relationship between all three scores, with a very strong correlation between Cincinnati and Mohtadi questionnaires. The Lysholm score showed significant correlation between the method of treatment and outcome. Our study shows a good relationship between the three scoring systems, and helps to understand what questions should be asked to the patient with an ACL injury to elicit a true representation of their knee function.

New members of the ECMC and lessons from the ATS Conference.

Meet the new members of the @EarlyCareerERS committee http://ow.ly/DfHA30kE7sk.

Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Comparison of Demographic, Hemodynamic, and MRI Characteristics and Outcomes.

BACKGROUND: Previous studies have identified survival in systemic sclerosis (SSc)-associated pulmonary arterial hypertension (SSc-PAH) as being worse than in idiopathic pulmonary arterial hypertension (IPAH). We investigated differences between these conditions by comparing demographic, hemodynamic, and radiological characteristics and outcomes in a large cohort of incident patients. METHODS: Six hundred fifty-one patients diagnosed with IPAH or SSc-associated precapillary pulmonary hypertension were included. Patients with pulmonary disease or two or more risk factors for left heart disease were identified, leaving a primary analysis set of 375 subjects. Subgroup analysis using cardiac magnetic resonance (CMR) imaging was performed. RESULTS: Median survival was 7.8 years in IPAH and 3 years in SSc-PAH (P < .001). Patients with SSc-PAH were older with less severe hemodynamics but lower gas transfer (diffusing capacity for carbon monoxide [Dlco]). Independent prognostic factors were age, SSc, Dlco, pulmonary artery saturation, and stroke volume. After excluding patients with normal or only mildly elevated resistance, there was no difference in the relationship between pulmonary vascular resistance (PVR) and compliance in IPAH and SSc-PAH. The relationship between mean pulmonary arterial pressure (mPAP) and systolic pulmonary arterial pressure (sPAP) in IPAH was identical to that previously reported (mPAP = 0.61 sPAP + 2 mm Hg). The relationship in SSc-PAH was similar: mPAP = 0.58 sPAP + 2 mm Hg (P value for difference with IPAH = 0.095). The correlation between ventricular mass index assessed at CMR imaging and PVR was stronger in SSc-PAH. CONCLUSIONS: The reasons for poorer outcomes in SSc-PAH are likely to be multifactorial, including but not limited to older age and reduced gas transfer.

Pulmonary hypertension in patients with heart failure and preserved ejection fraction: differential diagnosis and management.

The most common cause of pulmonary hypertension (PH) due to left heart disease (LHD) was previously rheumatic mitral valve disease. However, with the disappearance of rheumatic fever and an aging population, nonvalvular LHD is now the most common cause of group 2 PH in the developed world. In this review, we examine the challenge of investigating patients who have PH and heart failure with preserved ejection fraction (HF-pEF), where differentiating between pulmonary arterial hypertension (PAH) and PH-LHD can be difficult, and also discuss the entity of combined precapillary and postcapillary PH. Given the proven efficacy of targeted therapy for the treatment of PAH, there is increasing interest in whether these treatments may benefit selected patients with PH associated with HF-pEF, and we review current trial data.

Pulmonary arterial hypertension associated with congenital heart disease: Comparison of clinical and anatomic-pathophysiologic classification.

BACKGROUND: Between 5% and 10% of patients with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH-CHD). Patients can be classified using either a clinical or anatomic-pathophysiologic system. No study has previously utilized both systems in a large cohort of patients. METHODS: Two hundred forty consecutive PAH-CHD patients diagnosed at a pulmonary hypertension referral center during 1995 to 2014 were identified from our unit database. Baseline characteristics, treatment and survival data were retrieved and survival analyses was performed. RESULTS: Both systems identified clear differences in baseline characteristics between subgroups. The anatomic-pathophysiologic system identified patients with post-tricuspid defects as having superior survival from point of referral to those with pre-tricuspid or complex defects (p < 0.05). Survival from point of referral was, however, not significantly different when patients were grouped using the clinical classification, although survival in all 4 groups was superior to that of 175 patients with idiopathic pulmonary arterial hypertension. Older age and higher creatinine, lower transfer factor of the lung for DLCO percent predicted and FEV1 percent predicted were independent adverse prognostic factors. CONCLUSION: Both PAH-CHD classification systems identified groups of patients distinct in terms of baseline characteristics. In our cohort, however, only the anatomic-pathophysiologic classification identified significantly different survival from point of referral. The presence of adverse prognostic markers may be useful in identifying patients requiring more aggressive pulmonary vascular therapy.

Prolonged time between donor brain death and organ retrieval results in an increased risk of mortality in cardiac transplant recipients.

In cardiac transplantation longer ischemic times relate to poorer outcomes. However, brain death also promotes donor organ injury. The aim of this study was to ascertain if there was an association between longer time periods between donor brain death and organ retrieval with recipient mortality. This retrospective single centre study included 157 cardiac transplants performed between February 1999 and 2009. The time between the second brain stem death test and the cross-clamp time at organ retrieval was noted in hours. This was compared with survival time in years. Cox regression analysis was performed. The following variables were included: donor and recipient sex, age and cytomegalovirus status; donor smoking history; ischemic time and number of rejection episodes. Of the 157 transplants, 37 recipients have died. The mean follow-up was 4.1 years. The mean time between brain stem death test and cross-clamp time was 13.2±3.96 hours. Considering the above variables, the most significant finding is: increased time between brain stem death test and organ retrieval cross-clamp time, predicted a greater recipient mortality [hazard ratio (HR)=1.15; 95% confidence interval (CI)=1.06-1.24; P<0.001]. Longer delays between donor brain death and cross-clamp time are associated with a higher-risk of mortality in cardiac transplant recipients.