Comptes Rendus. Biologies - Review article - On the involvement of the second heart field in congenital heart defects - Volume 347 - Review

Oct 22, 2026

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Congenital heart defects (CHD) affect 1 in 100 live births and arise from disrupted cardiac development. Early heart tube growth depends on progressive addition of second heart field (SHF) progenitor cells to cardiac poles. SHF produces ventricular septal, right ventricular and outflow tract myocardium at the arterial pole, and atrial myocardium at the venous pole. Perturbations in SHF deployment can drive conotruncal and septal CHD, including errors in cardiac looping and outflow tract development programs. This review summarizes SHF cell properties and developmental programs controlling progenitor addition and CHD origins.

LanguageEnglish

CategoryResearch & Report

FormatPDF

Pages11

Reading Time28 min

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Introduction

Early heart development and the second heart field

Comptes Rendus Biologies Clara Guijarro and Robert G. Kelly On the involvement of the second heart field in congenital heart defects Volume 347 (2024), p. 9-18 Online since: 15 March 2024 [https://doi.org/10.5802/crbiol.151](https://doi.org/10.5802/crbiol.151) This article is licensed under the Creative Commons Attribution 4.0 International License. [http://creativecommons.org/licenses/by/4.0/](http://creativecommons.org/licenses/by/4.0/) The Comptes Rendus. Biologies are a member of the Mersenne Center for open scientific publishing [www.centre-mersenne.org](www.centre-mersenne.org)—e-ISSN : 1768-3238 Comptes Rendus. Biologies 2024, Vol. 347, p. 9-18 [https://doi.org/10.5802/crbiol.151](https://doi.org/10.5802/crbiol.151) Review article On the involvement of the second heart field in congenital heart defects Clara Guijarro a and Robert G. Kelly , ∗, a a Aix-Marseille Université, CNRS UMR 7288, IBDM, Marseille, France [E-mail:](E-mail: Robert.Kelly@univ-amu.fr)[ Robert.Kelly@univ-amu.fr](E-mail: Robert.Kelly@univ-amu.fr) (R. G. Kelly) Abstract. Congenital heart defects (CHD) affect 1 in 100 live births and result from defects in cardiac development. Growth of the early heart tube occurs by the progressive addition of second heart field (SHF) progenitor cells to the cardiac poles. The SHF gives rise to ventricular septal, right ventricular and outflow tract myocardium at the arterial pole, and atrial, including atrial septal myocardium, atthe venous pole. SHF deployment creates the template for subsequent cardiac septation and has been implicated in cardiac looping and in orchestrating outflow tract development with neural crest cells. Genetic or environmental perturbation of SHF deployment thus underlies a spectrum of common forms of CHD affecting conotruncal and septal morphogenesis. Here we review the major properties of SHF cells as well as recent insights into the developmental programs that drive normal cardiac progenitor cell addition and the origins ofCHD. Keywords. Heart development, Embryology, Cardiac progenitor cells, Congenital heart defects. Note. Robert G. Kelly is the recipient of the prix Lamonica de Cardiologie - Fondation pour la Recherche Biomédicale PCL 2022 from the Académie des sciences. Funding. Agence National pour la Recherche (PP-heart ANR-20-CE13-0029-01 and Heartbound ANR- 22-CE13 projects), AFM-Telethon, French National Research Agency (ANR-16-CONV-0001), Excellence Initiative of Aix-Marseille University A∗MIDEX (Turing Centre for Living Systems) and the Fondation pour la Recherche Médicale. Published online: 15 March 2024 1. Introduction Defects in the complex process of cardiac morphogenesis underlie congenital heart defects (CHD) that affect 1 in 100 live births [1] . The heart is derived from mesoderm, the central of the three embryonic germ layers, and originates in the anterior region of the early embryo as a tubular structure with venous (inflow) and arterial (outflow) poles. Cardiac progenitor cells located in splanchnic mesoderm, overlying future foregut endoderm, contribute progressively to growth of the heart. These cells, known as the second heart field (SHF), ultimately give rise to ventricular septal, right ventricular and outflow ∗ Corresponding author. tract myocardium at the arterial pole, and atrial, including atrial septal myocardium, at the venous pole. Deployment of SHF cells is coincident with looping morphogenesis, an essential conserved process during which the heart tube rotates to the right to correctly position the future regions of the definitive heart. Subsequent steps of heart development involve cardiomyocyte proliferation driving chamber morphogenesis, development of the coronary arteries and cardiac conduction system coordinating the heartbeat, and cardiac septation. During septation the formation of ventricular and atrial septa generates the four chambered heart, and the embryonic outflow tract is divided into th

FAQs

What is the second heart field (SHF) and what role does it play in heart development?

The SHF comprises cardiac progenitor cells that progressively add to the heart tube. It generates ventricular septal, right ventricular, and outflow tract myocardium at the arterial pole, and atrial myocardium at the venous pole.

How do problems in SHF deployment relate to congenital heart defects (CHD)?

Perturbations in SHF deployment create a spectrum of common CHD forms, including conotruncal and septal morphogenesis defects. These can disrupt separation of systemic and pulmonary circulation.

What does the article focus on besides general heart development?

It reviews how SHF contributes during heart development and examines its involvement in CHD, with emphasis on roles of two T-box transcription factors implicated in human CHD: TBX1 and TBX5.

Oct 22, 2026

Uploaded by Finn