Purpose of review Pulmonary hypertension and right heart failure are common findings in patients suffering from heart failure with preserved ejection fraction (HFpEF). In this review, we summarize our current understanding of the pathophysiology of pulmonary hypertension related to heart failure. Recent findings HFpEF is a clinical syndrome with increasing prevalence and a mortality rate similar to heart failure with reduced ejection fraction. Because the pathophysiology and even the definition of this disease are still controversial, we will first outline the current conceptual framework around heart failure with preserved ejection fraction. Next, we will outline our current knowledge on the pathophysiology of pulmonary hypertension related to left ventricular failure and diastolic dysfunction. Diastolic dysfunction induces pulmonary hypertension through passive transmission of elevated end diastolic pressures, reactive pulmonary vasoconstriction, and vascular remodeling. Eventually, right ventricular failure develops that can further potentiate left ventricular failure because of their close mechanical, cellular, and biochemical integration. Summary Exciting new studies have led to an increased understanding of the underlying pathophysiology and indicate that pulmonary hypertension in heart failure may be treatable.