In this trial, 261 patients with chronic thromboembolic pulmonary hypertension were assigned to placebo or to the soluble guanylate cyclase stimulator riociguat. At 16 weeks, riociguat had significantly improved the 6-minute walk distance and pulmonary vascular resistance. Chronic thromboembolic pulmonary hypertension is characterized by obstruction of the pulmonary vasculature by residual organized thrombi, 1 leading to increased pulmonary vascular resistance, progressive pulmonary hypertension, and right ventricular failure. 2 , 3 Patients with chronic thromboembolic pulmonary hypertension have a poor prognosis unless they receive treatment early. 4 Pulmonary endarterectomy is the standard treatment for chronic thromboembolic pulmonary hypertension and is the only potentially curative treatment. 5 However, surgery is not an option for all patients; some patients are ineligible for surgery owing to the occlusion of distal vessels or coexisting conditions, some decline surgery, and some do not have access to expert surgical . . .
SUMMARY POINTS Pulmonary hypertension has many causes so prognoses and treatments vary The condition is diagnosed by systematically evaluating the breathless patient and screening patients at high risk Patients at high risk of severe and treatable pulmonary hypertension include those with systemic sclerosis, portal hypertension, congenital heart disease, and previous pulmonary embolism Specialist centres provide access to tailored investigative and treatment pathways and support networks for patients Patients with severe pulmonary hypertension can deteriorate rapidly—do not delay referral to perform specialist investigations Only selected patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension benefit from interventions directed at pulmonary vasculature; for most patients treatment is aimed at the underlying condition
Electrocardiographic and echocardiographic recordings, coupled with epicardial mapping in Langendorff-perfused hearts, histology analysis, patch clamping, gene expression, and protein measurements of the LV in rats with pressure-induced RV failure were compared with controls. [...]intraoperative epicardial LV mapping was also performed in patients with CTEPH who underwent pulmonary endarterectomy. Future investigations should focus on innovative treatments to minimize such secondary adaptation to abnormal LV unloading and mechanisms of "reverse remodeling." Because interventricular delay in systolic contraction and diastolic relaxation occur in patients with CTEPH and other forms of RV pressure overload, pre-exciting the RV with RV pacing may provide resynchronization, minimize diastolic interventricular delay, improve LV filling and stroke volume (34).