Pulmonary arterial hypertension associated with congenital heart disease. Personal perspectives

Area of Science:

• Cardiology
• Pulmonary Hypertension
• Congenital Heart Disease

Background:

• The management of patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) has evolved with targeted therapies.
• There is a growing population of adult Japanese patients diagnosed with PAH associated with CHD.
• Current operability criteria for PAH associated with CHD may be too conservative, especially with advancements in pulmonary vasodilator therapy.

Purpose of the Study:

• To highlight the need for evidence-based guidelines for managing adult Japanese patients with PAH associated with CHD.
• To emphasize the importance of establishing a patient register for PAH associated with CHD.
• To re-evaluate operability criteria in the context of modern pharmacotherapy.

Main Methods:

• Review of current consensus criteria for operability in PAH associated with CHD.
• Discussion of the impact of pulmonary vasodilator testing on treatment decisions.
• Call for data from patient registers and multicenter studies to refine management strategies.

Main Results:

• Existing operability criteria (< 4 Wood Units (WU)·m(2) operable, > 8 WU·m(2) inoperable) are considered conservative.
• Some patients with pulmonary resistance > 8 WU·m(2) may be operable with favorable outcomes if they respond well to vasodilators.
• The long-term effects of pulmonary vasodilators on Fontan circulation outcomes remain unclear.

Conclusions:

• New criteria for determining operability in PAH associated with CHD are needed, informed by patient registers and multicenter studies.
• Optimal management strategies for Eisenmenger syndrome require further investigation using registry data.
• Prospective studies are essential to determine the life expectancy of Eisenmenger syndrome patients under targeted therapy and to clarify the role of vasodilators in Fontan circulation outcomes.