Anticoagulation in Pulmonary Arterial Hypertension: Do We Know the Answer?

The shear stress and hypoxia in the pulmonary artery in patients with pulmonary arterial hypertension(PAH) causes endothelial dysfunction, smooth muscle proliferation and activation of thrombotic pathways leading to in situ thrombosis. Targeting the thrombotic pathways is a proposed mechanism to slow disease progression and improve survival. Over the years, the survival in patients with PAH has improved due to multiple factors with the increased use of anticoagulation as one of them. Both European Respiratory Society/European Society of Cardiology and American College of Cardiology/American Heart Association guidelines make grade II recommendations for using anticoagulation in PAH. The guidelines are based on weak observational studies with high risk of bias which have only studied warfarin as the choice of anticoagulation. In this article, we review the pathophysiology, rationale and the current literature investigating the role of anticoagulation in PAH.

Incidence and early outcomes associated with pre-transplant antivimentin antibodies in the cardiac transplantation population.

BACKGROUND: In cardiac transplant recipients, the development of antibodies to the endothelial intermediate filament protein vimentin (antivimentin antibodies, AVA) has been associated with rejection and poor outcomes. However, the incidence of these antibodies prior to transplantation and their association with early rejection has not been investigated. METHODS: Pre-transplant serum was analyzed from 50 patients who underwent de novo cardiac transplant at Johns Hopkins Hospital from 2004 to 2012. Demographic, one-yr rejection, and survival data were obtained from the transplant database. RESULTS: The incidence of pre-transplant AVA was 34%. AVA-positive patients were younger (p = 0.03), and there was an a trend toward incidence in females (p = 0.08). Demographic data were similar among both groups. AVA positivity did not predict rejection in the first year post-transplant. There was no difference in rejection-free graft survival (53 vs. 52%, p = 0.85) at one yr. Similarly, there was no difference in graft survival at one yr (82 vs. 88%, p = 0.56) or graft survival at a median follow-up of 23 and 26 months, respectively (76 vs. 85%, p = 0.41). CONCLUSIONS: AVA is common in the cardiac pre-transplant population with a higher incidence in the young. The presence of detectable AVA did not correlate with early post-transplant rejection or graft survival.

Role of non-invasive imaging in the work-up of cardiomyopathies.

Cardiomyopathy is an intrinsic structural disorder of the myocardium that may result in ventricular dysfunction and can progress to clinical heart failure. Non-invasive imaging modalities can determine whether abnormalities are present in the myocardium, valves, pericardium, or vessels. These imaging techniques can also present important prognostic information, as well as direct treatment and further management. Echocardiography is the most common imaging technique used for the initial diagnosis and management of cardiomyopathy; however, other imaging modalities, including nuclear cardiac imaging, cardiac magnetic resonance imaging, and cardiac computed tomography, may play an important role depending on the underlying etiology of the cardiomyopathy. This article reviews the role of non-invasive imaging in the work-up and management of patients with cardiomyopathy.