Journal of the American College of Cardiology
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Pulmonary arterial hypertension (PAH) is a rare disorder that may be hereditable (HPAH), idiopathic (IPAH), or associated with either drug-toxin exposures or other medical conditions. Familial cases have long been recognized and are usually due to mutations in the bone morphogenetic protein receptor type 2 gene (BMPR2), or, much less commonly, 2 other members of the transforming growth factor-beta superfamily, activin-like kinase-type 1 (ALK1) and endoglin (ENG), which are associated with hereditary hemorrhagic telangiectasia. In addition, approximately 20% of patients with IPAH carry mutations in BMPR2. ⋯ Identification of a familial mutation can be valuable in reproductive planning and identifying family members who are not mutation carriers and thus will not require lifelong surveillance. With advances in genomic technology and with international collaborative efforts, genome-wide association studies will be conducted to identify additional genes for HPAH, genetic modifiers for BMPR2 penetrance and genetic susceptibility to IPAH. In addition, collaborative studies of BMPR2 mutation carriers should enable identification of environmental modifiers, biomarkers for disease development and progression, and surrogate markers for efficacy end points in clinical drug development, thereby providing an invaluable resource for trials of PAH prevention.
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J. Am. Coll. Cardiol. · Jun 2009
ReviewDiagnosis and assessment of pulmonary arterial hypertension.
The diagnosis and assessment of pulmonary arterial hypertension is a rapidly evolving area, with changes occurring in the definition of the disease, screening and diagnostic techniques, and staging and follow-up assessment. The definition of pulmonary hypertension has been simplified, and is now based on currently available evidence. ⋯ As new therapies are developed for pulmonary arterial hypertension, screening, prompt diagnosis, and accurate assessment of disease severity become increasingly important. A clear definition of pulmonary hypertension and the development of a rational approach to diagnostic assessment and follow-up using both conventional and new tools will be essential to deriving maximal benefit from our expanding therapeutic armamentarium.
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J. Am. Coll. Cardiol. · Jun 2009
Clinical TrialIncremental value of copeptin for rapid rule out of acute myocardial infarction.
The purpose of this study was to examine the incremental value of copeptin for rapid rule out of acute myocardial infarction (AMI). ⋯ The additional use of copeptin seems to allow a rapid and reliable rule out of AMI already at presentation and may thereby obviate the need for prolonged monitoring and serial blood sampling in the majority of patients. (Advantageous Predictors of Acute Coronary Syndromes Evaluation [APACE]; NCT00470587).
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J. Am. Coll. Cardiol. · Jun 2009
Lowered B-type natriuretic peptide in response to levosimendan or dobutamine treatment is associated with improved survival in patients with severe acutely decompensated heart failure.
The purpose of this analysis was to examine whether decreases in B-type natriuretic peptide (BNP) levels during the first few days of hospitalization were associated with greater survival in patients with severe acutely decompensated heart failure (ADHF). ⋯ Patients with lowered BNP on treatment for ADHF had reduced mortality risks (31- and 180-day) compared to those with little or no BNP decrease. These results suggest that early lowering of BNP predicts both short- and long-term mortality risks. BNP reduction may therefore serve as a suitable prognostic marker of ACM.
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J. Am. Coll. Cardiol. · Jun 2009
Severely impaired von Willebrand factor-dependent platelet aggregation in patients with a continuous-flow left ventricular assist device (HeartMate II).
This study investigated the influence of the mechanical blood pump HeartMate II (HMII) (Thoratec Corporation, Pleasanton, California) on blood coagulation and platelet function. ⋯ The results imply that HMII treatment is associated with impaired platelet aggregation, which may contribute to an increased tendency to bleed.