The presence of PH in patients who suffer from CHF is common and predicts a poor outcome. However, precise definitions for PH associated with left heart disease, or 'out-of-proportion' PH as well as standardised vasodilator testing protocols are lacking. Moreover, apart from single-centre observations no large-scale trial to date has demonstrated a long-term benefit from pulmonary vasoactive drugs. As a result, there are currently no consensus recommendations for the treatment of PH in the presence of CHF. Off-label use of specific vasodilators in this patient population is discouraged. In a majority of cases, treatment of the underlying left heart disease leads to a decrease in pulmonary pressures. In light of novel agents to treat PH, trials that specifically address 'out-of-proportion' PH in CHF patients are warranted.