Scientific article
Open access

Pulmonary vascular disease as a complication of pediatric congenital heart diseases

Published inTranslational pediatrics, vol. 12, no. 5, p. 1041-1052
Publication date2023-05-30
First online date2023-05-24

Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile. Cardiac catheterization remains the gold standard exam for PH diagnosis. Treatment of pulmonary arterial hypertension (PAH) associated with CHD (PAH-CHD) can then be started according to the recent guidelines recommendations, although most of the evidence is extrapolated from studies on other causes of PAH. PH in pediatric heart disease is often multifactorial, and sometimes unclassifiable, making the management of these patients complicated. The operability of patients with a prevalent left-to-right shunt and increase of PVR, the management of children with PH associated with left-sided heart disease, the challenges of pulmonary vascular disorders in children with univentricular heart physiology and the role of vasodilator therapy in failing Fontan patients are some of the hot topics discussed in this review.

  • Eisenmenger syndrome
  • Fontan
  • Pulmonary hypertension (PH)
  • Congenital heart disease (CHD)
  • Pulmonary vascular disease (PVD)
Citation (ISO format)
WACKER BOU PUIGDEFABREGAS, Julie Caroline et al. Pulmonary vascular disease as a complication of pediatric congenital heart diseases. In: Translational pediatrics, 2023, vol. 12, n° 5, p. 1041–1052. doi: 10.21037/tp-23-64
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Article (Published version)
ISSN of the journal2224-4336

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