Tetralogy of Fallot: in good shape?

Abstract

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease and is associated with a high prevalence of pulmonary regurgitation following repair often requiring later pulmonary valve replacement (PVR) [1–13]. Adults with repaired TOF and significant chronic pulmonary regurgitation are at risk for progressive right ventricular (RV) dilatation and dysfunction [14–23]. Helbing et al. [1] showed that impaired relaxation and restriction to filling affected RV function in patients with repair of TOF and pulmonary regurgitation. Uebing et al. [2] demonstrated that RV endsystolic volume is a useful measure for estimating RV function after TOF repair depicting parameters of systolic and diastolic RV function. Assessment of RV function is important in the management of these patients in particular when it comes to optimal timing of surgery for PVR. Timing of surgery must be carefully considered, weighing the up-front risks of surgery and possible repeat surgery against the risk of ongoing pulmonary regurgitation [24, 25]. Therefore, monitoring RV volume and function is useful for managing patients with TOF. Detailed and precise evaluation of RV function by cardiac imaging has been a long-standing challenge in clinical medicine. Two-dimensional (2D) imaging has been used to this purpose, but the complex shape of the RV has precluded accurate estimation of RV volume by 2D echocardiography using geometric models. Accordingly, all 2D echocardiographic methods show a rather poor performance in comparison with cardiovascular magnetic resonance imaging (CMR) [26]. Consequently, three-dimensional (3D) imaging methods combined with analysis using the multiple slice technique are more accurate because reliance on geometric modeling is eliminated. Over the past years, a vast experience had been built with CMR to investigate LV and RV dynamics [27–41]. As such, CMR has become the gold standard for quantitative analysis of RV anatomy, function, and shape [42–47]. Kayser et al. [48] evaluated the effect of throughplane motion on tricuspid flow measurements performed with CMR velocity mapping in nine normal subjects and 15 patients with RV disease. Eight Editorial comment to the paper entitled ‘‘Correlation of right ventricular ejection fraction and tricuspid annular plane systolic excursion in tetralogy of fallot by magnetic resonance imaging’’ by Sheehan. Doi: 10.1007/s10554-008-9387-0.