This is the first report from the PVDOMICs study, an NIH funded multicenter project designed to better understand the clinical, biological and genetic features and differences between all forms of pulmonary hypertension. This initial paper describes the clinical features of over 1000 subjects in Groups 1-5 pulmonary hypertension. The notable findings include 1) there is often a mixture of etiologies in each of the groups; 2) DLCO is depressed in Groups 1-3; 3) Right atrial volume is enlarged in Groups 1-4 even when the right ventricle is not; 4) Group 1 patients frequently have ground glass findings on Chest CT scan and nocturnal desaturation; 5) Survival is poorest in Group 3.
The finding that there is right atrial enlargement before it is right ventricular enlargement is particularly relevant to the numerous reports that increased right atrial pressure is a powerful predictor of survival in PH, and that the mechanism appears to be associated with increased stressed blood volume. These data provide support for the potential of levosimendan as a fundamental treatment of pulmonary hypertension.
This is a comprehensive review of the published clinical studies related to pulmonary hypertension in heart failure with preserved ejection fraction (PH-HFpEF). It includes clinical and hemodynamic definitions of the disease, epidemiology, pathophysiology, animal models and treatment approaches, and clinical trials. They importantly stress the urgent unmet need to make progress in this disease so as to reduce the high burden of the disease. This is a wonderful source document for finding the latest research and publications.
This soon-to-be classic review offers a very understandable explanation about the importance of venous return in normal physiology and heart failure. While preload is an old concept, it has never been appreciated to have a major impact in managing heart failure patients until now. The studies referenced in this paper predict novel treatments of heart failure based on the concepts of stressed and unstressed blood volume. Levosimendan is the first drug to ever result in clinical improvement based on these concepts.
This is an in depth authoritative review of the field of pulmonary hypertension due to left heart disease. Written by one of the most renown thought leaders, it covers all facets of basic and clinical science, with insights into potential effective therapies. A must read for anyone who is involved with translational of clinical investigations in this area.
This editorial offers an explanation of the recent science that implicates downregulation of K+ATP channels in various forms of pulmonary hypertension. Given the clinical safety of levosimendan as a K+ATP channel activator, it may hold great promise for many forms of pulmonary hypertension.