Venous congestion in a d-TGA patient after Mustard procedure

A 45-year-old patient with dextro-transposition of the great arteries (d-TGA) repaired via Mustard procedure at the age of 5 years presented with lower limb venous congestion. Transthoracic echocardiography revealed turbulent flow at the systemic-venous-return baffle (video 1 of the supplementary da...

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Main Authors: Francisco Javier Ruperti-Repilado, Félix Coserria-Sánchez, Amadeo Wals-Rodríguez, Agustín Guisado, José Díaz-Fernández, Pastora Gallego
Format: Article
Language:English
Published: Permanyer 2025-08-01
Series:REC: Interventional Cardiology (English Ed.)
Online Access:https://recintervcardiol.org/en/index.php?option=com_content&view=article&id=2645
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author Francisco Javier Ruperti-Repilado
Félix Coserria-Sánchez
Amadeo Wals-Rodríguez
Agustín Guisado
José Díaz-Fernández
Pastora Gallego
author_facet Francisco Javier Ruperti-Repilado
Félix Coserria-Sánchez
Amadeo Wals-Rodríguez
Agustín Guisado
José Díaz-Fernández
Pastora Gallego
author_sort Francisco Javier Ruperti-Repilado
collection DOAJ
description A 45-year-old patient with dextro-transposition of the great arteries (d-TGA) repaired via Mustard procedure at the age of 5 years presented with lower limb venous congestion. Transthoracic echocardiography revealed turbulent flow at the systemic-venous-return baffle (video 1 of the supplementary data). Spiroergometry showed reduced peak VO2 and an O2-pulse curve with an early plateau and decline towards the end of exertion (figure 1A), both signs of reduced cardiac output at exertion. Cardiac magnetic resonance and fluoroscopy revealed heavily calcified and stenotic baffles (figure 1B; video 2 of the supplementary data). A 3 mmHg gradient between the superior (SVC) and inferior (IVC) vena cavae and the baffle chamber was present. The patient underwent baffle dilation with a covered stent at both SVC and IVC levels (figure 1C; videos 3 and 4 of the supplementary data). Follow-up echocardiography demonstrated restored laminar flow through the systemic-venous-return baffle with peak velocity of 0.8 cm/s (video 5 of the supplementary data). Self-limiting atrial arrhythmias occurred shortly after the procedure, highlighting the importance of follow-up and a low threshold for aggressive management of atrial arrhythmias given their association with sudden cardiac death in these patients. Mustard procedure—introduced with the Senning procedure for the management of d-TGA—uses...
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spelling doaj-art-e4f8408116ac421386fa95970ee0079b2025-08-20T03:41:54ZengPermanyerREC: Interventional Cardiology (English Ed.)2604-73222025-08-017319319410.24875/RECICE.M25000500Venous congestion in a d-TGA patient after Mustard procedureFrancisco Javier Ruperti-Repilado0Félix Coserria-Sánchez1Amadeo Wals-Rodríguez2Agustín Guisado3José Díaz-Fernández4Pastora Gallego5Unidad de Cardiopatías Congénitas del Adulto, Servicio de Cardiología, Hospital Universitario Virgen del Rocío, Instituto de BioMedicina de Sevilla (IBIS), Seville, Spain. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, Madrid, Spain. European Reference Network for Rare, Low-Prevalence, or Complex Diseases of The Heart (ERN GUARD-Heart), Amsterdam, The Netherlands. Division of Cardiology, University Hospital Basel, University of Basel, Basel, Switzerland. Cardiovascular Research Institute Basel, Division of Cardiology, University Hospital Basel, Basel, SwitzerlandServicio de Cardiología Pediátrica, Hospital Universitario Virgen del Rocío, Seville, SpainUnidad de Cardiopatías Congénitas del Adulto, Servicio de Cardiología, Hospital Universitario Virgen del Rocío, Instituto de BioMedicina de Sevilla (IBIS), Seville, Spain. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, Madrid, Spain. European Reference Network for Rare, Low-Prevalence, or Complex Diseases of The Heart (ERN GUARD-Heart), Amsterdam, The NetherlandsServicio de Cardiología, Hospital Universitario Virgen del Rocío, Seville, SpainServicio de Cardiología, Hospital Universitario Virgen del Rocío, Seville, SpainUnidad de Cardiopatías Congénitas del Adulto, Servicio de Cardiología, Hospital Universitario Virgen del Rocío, Instituto de BioMedicina de Sevilla (IBIS), Seville, Spain. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, Madrid, Spain. European Reference Network for Rare, Low-Prevalence, or Complex Diseases of The Heart (ERN GUARD-Heart), Amsterdam, The NetherlandsA 45-year-old patient with dextro-transposition of the great arteries (d-TGA) repaired via Mustard procedure at the age of 5 years presented with lower limb venous congestion. Transthoracic echocardiography revealed turbulent flow at the systemic-venous-return baffle (video 1 of the supplementary data). Spiroergometry showed reduced peak VO2 and an O2-pulse curve with an early plateau and decline towards the end of exertion (figure 1A), both signs of reduced cardiac output at exertion. Cardiac magnetic resonance and fluoroscopy revealed heavily calcified and stenotic baffles (figure 1B; video 2 of the supplementary data). A 3 mmHg gradient between the superior (SVC) and inferior (IVC) vena cavae and the baffle chamber was present. The patient underwent baffle dilation with a covered stent at both SVC and IVC levels (figure 1C; videos 3 and 4 of the supplementary data). Follow-up echocardiography demonstrated restored laminar flow through the systemic-venous-return baffle with peak velocity of 0.8 cm/s (video 5 of the supplementary data). Self-limiting atrial arrhythmias occurred shortly after the procedure, highlighting the importance of follow-up and a low threshold for aggressive management of atrial arrhythmias given their association with sudden cardiac death in these patients. Mustard procedure—introduced with the Senning procedure for the management of d-TGA—uses...https://recintervcardiol.org/en/index.php?option=com_content&view=article&id=2645
spellingShingle Francisco Javier Ruperti-Repilado
Félix Coserria-Sánchez
Amadeo Wals-Rodríguez
Agustín Guisado
José Díaz-Fernández
Pastora Gallego
Venous congestion in a d-TGA patient after Mustard procedure
REC: Interventional Cardiology (English Ed.)
title Venous congestion in a d-TGA patient after Mustard procedure
title_full Venous congestion in a d-TGA patient after Mustard procedure
title_fullStr Venous congestion in a d-TGA patient after Mustard procedure
title_full_unstemmed Venous congestion in a d-TGA patient after Mustard procedure
title_short Venous congestion in a d-TGA patient after Mustard procedure
title_sort venous congestion in a d tga patient after mustard procedure
url https://recintervcardiol.org/en/index.php?option=com_content&view=article&id=2645
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AT amadeowalsrodriguez venouscongestioninadtgapatientaftermustardprocedure
AT agustinguisado venouscongestioninadtgapatientaftermustardprocedure
AT josediazfernandez venouscongestioninadtgapatientaftermustardprocedure
AT pastoragallego venouscongestioninadtgapatientaftermustardprocedure