Abstract

Atrial septal defect (ASD) and pulmonary valve stenosis (PVS) are common congenital heart diseases predominantly affecting right ventricular (RV) function, leading to the development of symptoms in patients. In the past, clinical studies focusing onquantitative RV functional assessment were scanty since RV has undoubtedly been the forgotten chamber for quite a long time. Recent published data consistently confirmed the clinical importance of studying RV function. Among different parameters evaluating RV function, tricuspid annulus plane systolic excursion (TAPSE)which is a simple and reproducible surrogate to assess segmental RV long-axis motion, has been recommended by guidelines for clinical use [1].We recently reported that chronic volumeoverloading accentuates while pressure-overloading attenuates RV long-axis excursion and its impairment was associated with the presence of symptoms in adult patients with either ASD or PVS [2]. We appreciate the complements fromMartin Koestenberger et al., in their letter. Koestenberger has raised an interesting observation that RV longaxis function remained normal in ASD with mild degree of volumeoverloading but decreased in tetralogy of Fallot or other congenital heart diseases complicated with secondary pulmonary arterial hypertension even in childhood [3]. Currently, most of available data are acquired in adult patients; therefore, the results from pediatric patients consistently demonstrating the association between different loading condition and RV long-axis excursion may also impact the clinical practice of pediatricians caring for these patients. We concur with the suggestion from Koestenberger et al., that longterm, prospective follow-up studies should be carried out in either pediatric patients or young adolescents to characterize the dynamic changes of the RV long axis-function and identify the clinical and imaging predictors responsible for the development of RV dysfunction and symptoms. These findings will provide crucial objective information to help doctors make appropriate clinical decisions. We also agree that routine comprehensive assessmentof RV function is necessary in patients with congenital heart diseases whichmay affect RV regardless of their age or disease severity. Currently such practice is not common in contrast to the attention that has been paid in assessing left ventricular function [1]. A simple surrogate for RV function like TAPSE should definitely be included in routine RV assessment protocol [1,4]. While emerging new technologies such as tissue Doppler, two-dimensional speckle tracking imaging and real-time three-dimensional imaging may provide novel insight into the RV functional adaptation especially in congenital heart diseases predominantly affecting RV, further studies are needed before recommending them for routine clinical use.

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