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Review
. 2013 Nov 19:1:37.
doi: 10.3389/fped.2013.00037.

Right ventricular failure and pathobiology in patients with congenital heart disease - implications for long-term follow-up

Doreen Köhler  1 , Raoul Arnold  1 , Tsvetomir Loukanov  2 , Matthias Gorenflo  1
Affiliations
Review

Right ventricular failure and pathobiology in patients with congenital heart disease - implications for long-term follow-up

Doreen Köhler et al. Front Pediatr. .

Abstract

Right ventricular dysfunction represents a common problem in patients with congenital heart defects, such as Tetralogy of Fallot or pulmonary arterial hypertension. Patients with congenital heart defects may present with a pressure or volume overloaded right ventricle (RV) in a bi-ventricular heart or in a single ventricular circulation in which the RV serves as systemic ventricle. Both subsets of patients are at risk of developing right ventricular failure. Obtaining functional and morphological imaging data of the right heart is technically more difficult than imaging of the left ventricle. In contrast to findings on mechanisms of left ventricular dysfunction, very little is known about the pathophysiologic alterations of the right heart. The two main causes of right ventricular dysfunction are pressure and/or volume overload of the RV. Until now, there are no appropriate models available analyzing the effects of pressure and/or volume overload on the RV. This review intends to summarize clinical aspects mainly focusing on the current research in this field. In future, there will be increasing attention to individual care of patients with right heart diseases. Hence, further investigations are essential for understanding the right ventricular pathobiology.

Keywords: congenital heart defects; imaging; pathobiology; right ventricle; surgical techniques.

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Figures

Figure 1
Figure 1
(A) Magnetic resonance imaging scan of a patient with ccTGA and dilatation and dysfunction of the systemic right ventricle. (B) Main echocardiographic and MRI parameters to study the function of the right ventricle.
Figure 2
Figure 2
Main mechanisms of right ventricular dysfunction.
See this image and copyright information in PMC

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