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Review
. 2018 Jul;14(4):343-353.
doi: 10.2217/fca-2017-0065. Epub 2018 May 24.

Pulmonary hypertension in congenital heart disease

Emma Pascall  1 , Robert Mr Tulloh  1
Affiliations
Review

Pulmonary hypertension in congenital heart disease

Emma Pascall et al. Future Cardiol. 2018 Jul.

Abstract

Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. A multifactorial cause is recognized, relating to the size and nature of cardiac defect as well as environmental and genetic factors. More complex disease is increasingly recognized rather than pure Eisenmenger complex. Remodeling of the pulmonary vascular bed causes increased pulmonary vascular resistance diagnosed by a collection of investigations including echocardiography, exercise testing, cardiac catheterization, MRI and CT scanning. Management employs disease-modifying medications which are now used with increasing benefit.

Keywords: Eisenmenger; congenital heart disease; pulmonary hypertension; pulmonary vascular resistance.

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Conflict of interest statement

Financial & competing interests disclosure

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.

No writing assistance was utilized in the production of this manuscript.

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