Key result
This review highlights the pivotal role of clinical examination, functional capacity assessment, biomarkers, imaging, and hemodynamics in evaluating and managing patients with PAH-CHD.
Why the study?
Patients with congenital heart disease who develop pulmonary arterial hypertension live longer due to PAH-specific therapy and improved tertiary care, requiring optimal evaluation strategies.
This review outlines the epidemiology, pathophysiology, and optimal evaluation strategies for patients with congenital heart disease-related pulmonary arterial hypertension.
PAH-specific therapy may extend survival and QoL in CHD-PAH; leaves open standardized multimodal assessment protocols pending controlled trials.
INTRODUCTION: Patients with congenital heart disease (CHD), who develop pulmonary arterial hypertension (PAH), live longer, and have better quality of life compared to the past due to PAH-specific therapy and improved tertiary care. Areas covered: Clinical examination, objective assessment of functional capacity, natriuretic peptide levels, cardiac imaging, and hemodynamics all play a pivotal role in the evaluation, general care, and management of PAH-specific therapy. This review discusses the epidemiology and pathophysiology of PAH-CHD and provides hints for the optimal evaluation of these patients. Expert commentary: Further research should be performed in the field of PAH-CHD, as there are many of areas lacking evidence that should be addressed in the future. Networking, especially among the tertiary expert centers, could play a key role in this direction.
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Ntiloudi et al. (2018) conducted a review in Congenital heart disease-related pulmonary arterial hypertension. This review highlights the pivotal role of clinical examination, functional capacity assessment, biomarkers, imaging, and hemodynamics in evaluating and managing patients with PAH-CHD.
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