Revista Española de Cardiología (English Edition) Revista Española de Cardiología (English Edition)
Rev Esp Cardiol. 2017;70:1007 - Vol. 70 Num.11 DOI: 10.1016/j.rec.2017.04.023

TGA+VSD and Subpulmonary Conus: From Fetus to a 3-dimensional Model

Begoña Manso a,, Lutgardo García-Díaz b, Israel Valverde a,c

a Sección de Cardiología y Hemodinámica Pediátrica, Servicio de Pediatría, Hospital Virgen del Rocío, Sevilla, Spain
b Sección de Medicina Fetal, Servicio de Ginecología, Hospital Virgen del Rocío, Sevilla, Spain
c Grupo de Fisiopatología Cardiovascular, Instituto de Biomedicina de Sevilla-IBIS, Hospital Virgen del Rocío/CSIC/Universidad de Sevilla, Sevilla, Spain

Article

We present an echocardiogram recorded in a 21-week old fetus. The image was indicative of transposition of the great arteries (TGA), with atrioventricular concordance and ventriculoarterial discordance. Additional findings included ventricular shunt (VS) and subpulmonary stenosis. An atypical characteristic was the presence of subpulmonary conus (arrows) and absence of subaortic conus (stars). Only 29 such cases have been published, 22 of which corresponded to postmortem reports. There have been no reports of cases in a living fetus. Subpulmonary conus arises from a mitropulmonary discontinuity (not seen in typical TGA) and the absence of subaortic conus, aortic-tricuspid continuity (Figure 1, the mark on the image before the selected one is a measurement in 2-dimensional mode; Ao, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle). The subpulmonary conus appears to be stenotic from its proximal portion. The great vessels are located side by side, with the aorta in a slightly posterior position to the pulmonary artery. The VS is below the subpulmonary conus and closer to the aortic valve.

Figure 1.

The findings were confirmed by postnatal echocardiography (Figure 2), which ruled out the patient for arterial switch surgery in the neonatal period. Magnetic resonance imaging was performed at 2 months of life to provide more detailed anatomical information and facilitate planning of the surgical strategy (Figure 3). A 3-dimensional model was printed from these imaging data (Figure 3).

Figure 2.

Figure 3.

To the best of our knowledge, this is the first report in the literature of fetal diagnosis of this uncommon type of TGA and VS. Although the pathophysiological behavior is like any other case of TGA + VS with subpulmonary stenosis, the anatomical characteristics described for this malformation make treatment a challenge. Complementary images and 3-dimensional reconstructions were used to design an appropriate therapeutic strategy.

FUNDING

Part of this investigation was funded by the Instituto de Salud Carlos III, of the Ministry for Science and Innovation, Health Research Project, Health Research Grant no. PI14/00180.

Corresponding author: bemangar@hotmail.com

1885-5857/© 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved

Cookies
x
To improve our services and products, we use cookies (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here.
Cookies policy
x
To improve our services and products, we use cookies (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here.