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.
Landmark paper on the extensive pulmonary vascular pathology found in all forms of left heart failure. The authors report venous and small vessel intimal thickening that was more severe than arterial intimal thickening in HF, with a pattern similar to patients with pulmonary veno-occlusive disease.