Pulmonary Hypertension with Valvular Heart Disease: When to Treat the Valve Disease and When to Treat the Pulmonary Hypertension

This article will review the current guidelines for therapeutic intervention in (pulmonary hypertension) PH related to left heart disease (PH-LHD). The 6th World Symposium on Pulmonary Hypertension (WSPH) recommended that the mean pulmonary artery pressure (mPAP) should be lowered to 20 mmHg. In several randomized controlled trials performed in patients with PH-LHD, pulmonary arterial hypertension (PAH)–specific drug therapy demonstrated no evidence of beneficial effects. Furthermore, in the sildenafil for improving outcomes after valvular correction (SIOVAC) trial, the use of sildenafil in the context of PH post-valvular heart disease (VHD) intervention is associated with an increased risk of clinical deterioration and death. Therefore, medical therapy such as PAH-specific drugs is still not recommended in PH-LHD. The principle of PH-LHD therapy is the treatment of underlying VHD. It is crucial to undergo surgical intervention at an appropriate time prior the development of potentially irreversible PH. Stress echocardiography (SE) is helpful to define symptoms and can be useful to assess the systolic pulmonary artery pressure (SPAP) and stratify severity of VHD. This comprehensive review of the literature highlights the role of SE imaging to assess VHD and is needed for the asymptomatic patients with severe VHD or symptomatic patients with non-severe VHD in the context of PH-LHD. The focus of patient evaluation should be on identifying patients with significant underlying valvular heart disease and referring in a timely manner for VHD treatment per society guidelines as pharmacologic pulmonary vasodilator therapy for PH-LHD has not shown efficacy as seen in other forms of PH.