Key result
PAH-CHD management relies largely on clinical experience due to uncertain long-term effects of novel agents.
Why the study?
The long-term effects of novel pulmonary arterial hypertension medications on patients with PAH-CHD remain somewhat uncertain, leaving treatment plans largely founded on clinical experience.
This review highlights the need for expert management and summarizes current evidence for treating pulmonary arterial hypertension associated with congenital heart disease.
Current management of patients with congenital heart disease has increased their survival into adulthood. This is accompanied by potential cardiac complications, including pulmonary hypertension associated with congenital heart disease (PAH-CHD). PAH-CHD constitutes a challenging subgroup of pulmonary hypertension and requires expert management to improve quality of life and prognosis. Novel agents have shown a significant improvement in morbidity and mortality in patients with pulmonary arterial hypertension. However, the long-term effects of these medications on PAH-CHD patients remain somewhat uncertain, necessitating treatment plans largely founded on the clinical experience of the healthcare providers. The aim of this review is to summarize the current evidence and future perspectives regarding treatment strategies for PAH-CHD to help better guide management of this complex disease.
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Mahmoud et al. (2023) conducted a review in Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). Treatment strategies for PAH-CHD was evaluated. Treatment strategies for pulmonary arterial hypertension associated with congenital heart disease currently rely largely on clinical experience due to uncertain long-term effects of novel agents.
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