Human urotensin-II (hU-II) is the most potent endogenous cardiostimulant identified to date. We therefore determined whether hU-II has a possible pathological role by investigating its levels in patients with congestive heart failure (CHF). Blood samples were obtained from the aortic root, femoral artery, femoral vein, and pulmonary artery from CHF patients undergoing cardiac catheterization and the aortic root from patients undergoing investigative angiography for chest pain who were not in heart failure. Immunoreactive hU-II (hU-II-ir) levels were determined with radioimmunoassay. hU-II-ir was elevated in the aortic root of CHF patients (230.9 +/- 68.7 pg/ml, n = 21; P < 0.001) vs. patients with nonfailing hearts (22.7 +/- 6.1 pg/ml, n = 18). This increase was attributed to cardiopulmonary production of hU-II-ir because levels were lower in the pulmonary artery (38.2 +/- 6.1 pg/ml, n = 21; P < 0.001) than in the aortic root. hU-II-ir was elevated in the aortic root of CHF patients with nonischemic cardiomyopathy (142.1 +/- 51.5 pg/ml, n = 10; P < 0.05) vs. patients with nonfailing hearts without coronary artery disease (27.3 +/- 12.4 pg/ml, n = 7) and CHF patients with ischemic cardiomyopathy (311.6 +/- 120.4 pg/ml, n = 11; P < 0.001) vs. patients with nonfailing hearts and coronary artery disease (19.8 +/- 6.6 pg/ml, n = 11). hU-II-ir was significantly higher in the aortic root than in the pulmonary artery and femoral vein, with a nonsignificant trend for higher levels in the aortic root than in the femoral artery. The findings indicated that hU-II-ir is elevated in the aortic root of CHF patients and that hU-II-ir is cleared at least in part from the microcirculation.

No abstract

In patients with nonischemic dilated cardiomyopathy, carvedilol leads to a significantly greater improvement in LVEF in patients with the Arg389Arg-beta1 adrenergic receptor phenotype.

Supervised center-based ET was a safe, feasible addition to disease management programs with supported home exercise in patients recently hospitalized with acute HF, but did not reduce combined end-point of death or readmission. (A supervised exercise programme following hospitalisation for heart failure: does it add to disease management?; ACTRN12608000263392).

No abstract

Mutations in FBN1 cause a range of overlapping but distinct conditions including Marfan syndrome (MFS), Weill-Marchesani syndrome (WMS), familial thoracic aortic aneurysms/dissections (FTAAD), acromicric dysplasia (AD), and geleophysic dysplasia (GD). Two forms of acromelic dysplasia, AD and GD, characterized by short stature, brachydactyly, reduced joint mobility, and characteristic facies, result from heterozygous missense mutations occurring in exons 41 and 42 of FBN1; missense mutations in these exons have not been reported to cause MFS or other syndromes. Here we report on probands with MFS and WMS who have heterozygous FBN1 missense mutations in exons 41 and 42, respectively. The proband with WMS has ectopia lentis, short stature, thickened pinnae, tight skin, striae atrophicae, reduced extension of the elbows, contractures of the fingers and toes, and brachydactyly and has a missense mutation in exon 42 of FBN1 (c.5242T>C ;p.C1748R). He also experienced a previously unreported complication of WMS, an acute thoracic aortic dissection. The second proband displays classic characteristics of MFS, including ectopia lentis, skeletal features and aortic root dilatation, and has a missense mutation in exon 41 of FBN1 (c.5084G>A; p.C1695Y). These phenotypes provide evidence that missense mutations in exons 41 and 42 of FBN1 lead to MFS and WMS in addition to AD and GD and also suggest that all individuals with pathogenic FBN1 mutations in these exons should be assessed for thoracic aortic disease and ectopia lentis. Further studies are necessary to elucidate the factors responsible for the different phenotypes associated with missense mutations in these exons of FBN1.

A Large Single-Center StudyLeft ventricular assist device (LVAD) C irculatory support with left ventricular assist devices (LVADs) has emerged as a powerful therapy that can improve outcomes in patients who have advanced heart failure (HF) refractory to medical therapy.1-3 The scarcity of donor organs severely limits transplantation as an option for patients with advanced HF; moreover, transplant patients need lifelong immunosuppression, the medications for which can have their own serious side effects. The newest generation of LVADs comprises continuous-flow (CF) pumps, which use axial or centrifugal technology, deliver flows of up to 10 L/min, 2,4 and are smaller and more durable than previous models. Currently, these LVADs are implanted either as a bridge to transplantation (BTT) or as destination therapy (DT), which offers a permanent alternative to transplantation.Recently, patients with end-stage HF were given new options when the U.S. Food and Drug Administration (FDA) approved 2 continuous-flow LVADs: the HeartMate ® II (Thoratec Corporation; Pleasanton, Calif ) and the HeartWare ® Ventricular Assist System (HeartWare Inc.; Framingham, Mass). The HeartMate II was approved for BTT in 2008 and for DT in 2010, and the HeartWare was approved for BTT in 2012. These milestones initiated modern LVAD therapy, enabling this treatment to become available to a larger population of patients. [5][6][7][8][9] Consequently, LVAD use has dramatically increased throughout the world, particularly for DT, and growing numbers of medical centers are offering device therapy. [9][10][11] This trend has been bolstered by reports that LVAD use, in HF patients 70 years of age or older, is associated with

The efficacy and safety data from a clinical trial of the VentrAssist were favourable and resulted in gaining European regulatory approval for this indication. Notably, the survival success rate for the VentrAssist was higher than that reported for other left ventricular assist devices. The overall number of implants with the VentrAssist has now surpassed that of any other third-generation centrifugal device.