How to evaluate patients with congenital heart disease-related pulmonary arterial hypertension

ABSTRACT

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.

KEYWORDS:

• Congenital heart disease
• diagnosis
• epidemiology
• pathophysiology
• pulmonary arterial hypertension

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.