No abstract

No abstract

No abstract

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...

chronic cough are presented in algorithmic form (Fig 1-3). Guidelines with algorithms for evaluating chronic cough in pediatric patients < 15 years of age are presented in the section entitled "Guidelines for Evaluating Chronic Cough in Pediatrics" 2,4 [Fig 4, 5]. For a full discussion on how to use the algorithms, please refer to these sections. Summary and Recommendations Recommendations for each section of these guidelines are listed under their respective section titles. For an in-depth discussion or clarification of each recommendation, readers are encouraged to read the specific section in question in its entirety. Methodology and Grading of the Evidence for the Diagnosis and Management of Cough 5 • The recommendations were graded, by consensus by the panel, using the American College of Chest Physicians Health and Science Policy Grading System, which is based on the following two components: quality of evidence; and the net benefit of the diagnostic and therapeutic procedure. • The quality of evidence is rated according to the study design and strength of other methodologies used in the included studies.