Cardiology Letters 2022, 31(2):100-106

Combination treatment - a tool to improve the prognosis of patients with pulmonary arterial hypertension

Luknár M, Lesný P, Gonçalvesová E

Centrum pre pľúcnu artériovú hypertenziu, Kardiologická klinika Lekárská fakulta Univerzita Komenského a Národný ústav srdcových a cievnych chorôb, a. s., Bratislava, Slovenská republika

Despite significant recent therapeutic advances, pulmonary arterial hypertension remains an incurable disease with an unfavourable prognosis. Treatment decisions are based on risk assessment at diagnosis as well as at regular structured follow-up assessments. Risk stratification according to the European Society of Cardiology is comprehensive. Interpretation of the mosaic of risk status can be subjective and is associated with several issues. This is why risk stratification schemes able to simplify and quantify the risk assessment are needed. This could provide for the objective and optimal choice of treatment strategy. Risk stratification of French and German researchers is based on the European guidelines. An American risk scheme utilizes parameters from the REVEAL Registry. Drug combination becomes the standard of care in all risk groups. Recently, several retrospective analyses have been published documenting haemodynamic and clinical benefits of triple combination including parenteral prostanoids. This initial triple combination seems to have a beneficial effect on long-term survival, especially in high-risk patients. However, evidence from prospective and randomized trials is lacking. In patients with high and high-intermediate risks, initial triple combination including parenteral prostanoids could be optimal. In patients with low-intermediate and low risks, double combination or triple oral combination is warranted. Monotherapy is suitable for a marginal group of patients with unambiguously low risk or few other particular subgroups. The risk of mortality should be regularly assessed using structured follow-up. The aim of treatment is to achieve the low-risk status according to an appropriate risk scheme. In case the patient does not achieve the low risk status, treatment escalation and initiation of parenteral prostanoid should be considered.

Keywords: pulmonary arterial hypertension; prognosis; combination treatment; risk stratification

Published: February 1, 2022  Show citation