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. 2012 Jun 15;8(10):1-11.
doi: 10.4172/2155-9880.S8-010.

The Right Heart in Congenital Heart Disease, Mechanisms and Recent Advances

Julien Guihaire  1 , François Haddad, Olaf Mercier, Daniel J Murphy, Joseph C Wu, Elie Fadel
Affiliations

The Right Heart in Congenital Heart Disease, Mechanisms and Recent Advances

Julien Guihaire et al. J Clin Exp Cardiolog. .

Abstract

In patients with congenital heart disease, the right heart may support the pulmonary or the systemic circulation. Several congenital heart diseases primarily affect the right heart including Tetralogy of Fallot, transposition of great arteries, septal defects leading to pulmonary vascular disease, Ebstein anomaly and arrhythmogenic right ventricular cardiomyopathy. In these patients, right ventricular dysfunction leads to considerable morbidity and mortality. In this paper, our objective is to review the mechanisms and management of right heart failure associated with congenital heart disease. We will outline pearls and pitfalls in the management of congenital heart disease affecting the right heart and highlight recent advances in the field.

Keywords: Congenital heart disease; Right heart.

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Figures

Figure 1
Figure 1
General mechanisms contributing to RV failure in patients with CHD. Several mechanisms contribute to maladaptative RV remodeling in patients with CHD. The schematic does not take into account associated congenital heart defects that can contribute to the ventricular dysfunction. Please refer to text for further explanation.
Figure 2
Figure 2
Selected echocardiographic signals and indices used to measure right ventricular function and pulmonary flow. Section A shows tricuspid annular plane systolic excursion (TAPSE) in a patient with preserved RV systolic function. Section B shows a tissue-Doppler signal of the proximal right ventricular wall; isovolumic acceleration is measured as the maximal isovolumic velocity divided by the time to peak isovolumic velocity; e is the maximal early diastolic velocity and a represents the maximal late or atrial associated velocity. Section C shows a pulmonary flow signal; a notch is present and usually indicates decreased pulmonary compliance and altered reflection waves. Section D shows a tricuspid regurgitation signal. Both the pulmonary ejection time and the tricuspid regurgitation time are used to measure the right ventricular myocardial performance index (RVMPI).
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References

    1. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. First of two parts. N Engl J Med. 2000;342:256–263. - PubMed
    1. Pierpont ME, Basson CT, Benson DW, Jr, Gelb BD, Giglia TM, et al. Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation. 2007;115:3015–3038. - PubMed
    1. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010) Eur Heart J. 2010;31:2915–2957. - PubMed
    1. Haddad F, Doyle R, Murphy DJ, Hunt SA. Right ventricular function in cardiovascular disease, part II: pathophysiology, clinical importance, and management of right ventricular failure. Circulation. 2008;117:1717–1731. - PubMed
    1. Haddad F, Hunt SA, Rosenthal DN, Murphy DJ. Right ventricular function in cardiovascular disease, part I: Anatomy, physiology, aging, and functional assessment of the right ventricle. Circulation. 2008;117:1436–1448. - PubMed

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