Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024 May 9:11:1360555.
doi: 10.3389/fcvm.2024.1360555. eCollection 2024.

Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management

Paolo Ferrero  1 , Andrew Constantine  2 , Massimo Chessa  1   3 , Konstantinos Dimopoulos  4   5
Affiliations
Review

Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management

Paolo Ferrero et al. Front Cardiovasc Med. .

Abstract

Patients with pulmonary hypertension associated with a left-right shunt include a wide spectrum of pathophysiological substrates, ranging from those characterized by pulmonary over-circulation to those with advanced pulmonary vascular disease. The former group may benefit from shunt repair in carefully selected cases but, when advanced pulmonary vascular disease has developed, defect closure should be avoided, and pulmonary vasodilators may be used to improve effort tolerance and hemodynamics. There is a paucity of evidence, however, to support decision-making in the care of these patients. We discuss the principles of management in patients with pulmonary hypertension and a predominant left-right shunt. The recommendations and statements made in this paper are based on pathophysiological considerations and expert opinion.

Keywords: congenital heart disease; left-right shunt; management; pathophysiology; pulmonary hypertension (PAH).

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Diagram illustrating the pathophysiology of the pulmonary and systemic circulations, which work in parallel to modulate pulmonary blood flow and the severity of the left-right (L-R) shunt. The modulators of pulmonary blood flow and shunt traction can, in principle, be classified as pre- and post-capillary, and should be considered when assessing patients with PAH and left-right shunts. ASD, atrial septal defect; LA, left atrium; LV, left ventricle/ventricular; O2, oxygen; PA, pulmonary artery; Qp, pulmonary blood flow; Qs, systemic blood flow; RA, right atrium; RV, right ventricle; RVOT, right ventricular outflow tract.
Figure 2
Figure 2
Graphical summary of PAH-CHD with a left-right shunt, spanning from the predominantly reversible end of the spectrum, with mildly elevated pulmonary vascular resistance (PVR) and high shunt fraction (Qp/Qs), to the predominantly irreversible pulmonary vascular disease (PVD) or pre-Eisenmenger end. This schematic assumes a large congenital heart defect and no significant left heart disease.
See this image and copyright information in PMC

References

    1. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. (2022) 43:3618–731. 10.1093/eurheartj/ehac237 - DOI - PubMed
    1. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. (2019) 53:1801913. 10.1183/13993003.01913-2018 - DOI - PMC - PubMed
    1. Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galiè N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. (2014) 35:716–24. 10.1093/eurheartj/eht072 - DOI - PubMed
    1. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RMF, Lauer A, et al. Bosentan therapy in patients with eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. (2006) 114:48–54. 10.1161/CIRCULATIONAHA.106.630715 - DOI - PubMed
    1. Dimopoulos K, Inuzuka R, Goletto S, Giannakoulas G, Swan L, Wort SJ, et al. Improved survival among patients with eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. (2010) 121:20–5. 10.1161/CIRCULATIONAHA.109.883876 - DOI - PubMed

LinkOut - more resources