OBJECTIVE:Choices of pharmacologic therapies for pulmonary arterial hypertension (PAH) are ideally guided by high-level evidence. Th e objective of this guideline is to provide clinicians advice regarding pharmacologic therapy for adult patients with PAH as informed by available evidence. METHODS:Th is guideline was based on systematic reviews of English language evidence published between 1990 and November 2013, identified using the MEDLINE and Cochrane Library databases. Th e strength of available evidence was graded using the Grades of Recommendations, Assessment, Development, and Evaluation methodology. Guideline recommendations, or consensus statements when available evidence was insufficient to support recommendations, were developed using a modifi ed Delphi technique to achieve consensus. RESULTS:Available evidence is limited in its ability to support high-level recommendations. Th erefore, we draft ed consensus statements to address many clinical questions regarding pharmacotherapy for patients with PAH. A total of 79 recommendations or consensus statements were adopted and graded.CONCLUSIONS: Clinical decisions regarding pharmacotherapy for PAH should be guided by high-level recommendations when suffi cient evidence is available. Absent higher level evidence, consensus statements based upon available information must be used. Further studies are needed to address the gaps in available knowledge regarding optimal pharmacotherapy for PAH. ABBREVIATIONS : 6MWD 5 6-min walk distance ; AHRQ 5 Agency for Healthcare Research and Quality ; ARIES 5 Ambrisentan in Pulmonary Arterial Hypertension, Randomized Double-Blind, PlaceboControlled, Multicenter, Efficacy Study ; BNP 5 brain natriuretic peptide ; CB 5 consensus-based ; CCB 5 calcium channel blocker ; CO 5 cardiac output ; COI 5 conflict of interest ; CTEPH 5 chronic thromboembolic pulmonary hypertension ; EPC 5 Evidence-Based Practice Center ; ETRA 5 endothelin receptor antagonist ; FC 5 functional class ; FDA 5 US Food and Drug Administration ; GOC 5 Guidelines Oversight Committee ; GRADE 5 Grades of Recommendations, Assessment , Development , and Evaluation ; HR 5 hazard ratio ; IOM 5 Institute of Medicine ; IPAH 5 idiopathic pulmonary arterial hypertension ; mPAP 5 mean pulmonary artery pressure ; PAH 5 pulmonary arterial hypertension ; PDE5 5 phosphodiesterase-5 ; PH 5 pulmonary hypertension ; PVR 5 pulmonary vascular resistance ; RCT 5 randomized controlled trial ; WHO 5 World Health Organization For treatment naive PAH patients with WHOFC I symptoms, we suggest continued monitoring for the development of symptoms that would signal disease progression and warrant the initiation of pharmacotherapy (Grade CB) . 5. We suggest that patients at risk for the development of PAH (eg, patients with systemic sclerosis or the presence of a known mutation placing the patient at risk for PAH) be monitored for the development of symptoms of PAH (Grade CB) . 6. We suggest also that contributing causes of PH (eg, sleep apnea and systemic hypertension) in patients wi...

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KTP laser combined with bevacizumab in HHT epistaxis is superior to KTP laser treatment alone. It significantly decreases frequency and severity of nosebleeds and blood transfusion requirements, and significantly improves work ability and quality of life.

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Despite improved understanding of the pathobiology of pulmonary arterial hypertension (PAH), it remains a severe and progressive disease, usually culminating in right heart failure, significant morbidity and early mortality. Over the last decade, some major advances have led to substantial improvements in the management of PAH. Much of this progress was pioneered by work in animal models. Although none of the current animal models of pulmonary hypertension (PH) completely recapitulate the human disease, they do provide insight into the cellular pathways contributing to its development and progression. There is hope that future work in model organisms will help to define its underlying cause(s), identify risk factors and lead to better treatment of the currently irreversible damage that results in the lungs of afflicted patients. However, the difficulty in defining the etiology of idiopathic PAH (IPAH, previously known as primary pulmonary hypertension) makes this subset of the disease particularly difficult to model. Although there are some valuable existing models that are relevant for IPAH research, the area would value from the development of new models that more closely mimic the clinical pathophysiology of IPAH.

Schistosomiasis, a parasite-borne disease, is highly prevalent in Africa and Asia; it is estimated that close to 20 million people worldwide have a severe form of the disease. The chronic form can affect the gastrointestinal system and lead to hepatosplenic disease, and it may cause cardiopulmonary complications, including pulmonary hypertension. The exact pathogenesis of schistosomiasis-associated pulmonary hypertension (Sch-PH) remains unclear, although several mechanisms, including parasitic arterial embolization, pulmonary arteriopathy, and portopulmonary hypertension-like pathophysiology, have been suggested. The immunopathology of the disease is also unclear, although there are similarities with the immunology of idiopathic pulmonary arterial hypertension (PAH). Finally, the treatment of Sch-PH has not been well studied. There is some evidence on treating the underlying infection, with unclear effect on Sch-PH, and advanced PAH therapies are now being suggested, but more studies are needed to confirm their efficacy.

Background: In March 2020, many elective medical services were canceled in response to the coronavirus disease 2019 (COVID-19) pandemic. The daily case rate is now declining in many states and there is a need for guidance about the resumption of elective clinical services for patients with lung disease or sleep conditions. Methods: Volunteers were solicited from the Association of Pulmonary, Critical Care, and Sleep Division Directors and American Thoracic Society. Working groups developed plans by discussion and consensus for resuming elective services in pulmonary and sleep-medicine clinics, pulmonary function testing laboratories, bronchoscopy and procedure suites, polysomnography laboratories, and pulmonary rehabilitation facilities. Results: The community new case rate should be consistently low or have a downward trajectory for at least 14 days before resuming elective clinical services. In addition, institutions should have an operational strategy that consists of patient prioritization, screening, diagnostic testing, physical distancing, infection control, and follow-up surveillance. The goals are to protect patients and staff from exposure to the virus, account for limitations in staff, equipment, and space that are essential for the care of patients with COVID-19, and provide access to care for patients with acute and chronic conditions. Conclusions: Transmission of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a dynamic process and, therefore, it is likely that the prevalence of COVID-19 in the community will wax and wane. This will impact an institution’s mitigation needs. Operating procedures should be frequently reassessed and modified as needed. The suggestions provided are those of the authors and do not represent official positions of the Association of Pulmonary, Critical Care, and Sleep Division Directors or the American Thoracic Society.