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Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis

Cor triatriatum dexter (CTD) is an extremely uncommon and underreported congenital cardiac anomaly in which the persistence of the embryonic right venous valve separates the right atrium into two chambers with varying degrees of obstruction to antegrade flow and variable degree of right to left shun...

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Autores principales: Picciolli, Irene, Francescato, Gaia, Colli, Anna Maria, Cappelleri, Alessia, Mayer, Alessandra, Raschetti, Roberto, Di Cosola, Roberta, Pisaniello, Marco, Annoni, Giuseppe Alberto, Papa, Marco, Maldi, Mimoza, Olivieri, Guido, Mosca, Fabio, Marianeschi, Stefano
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9139359/
https://www.ncbi.nlm.nih.gov/pubmed/35626853
http://dx.doi.org/10.3390/children9050676
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author Picciolli, Irene
Francescato, Gaia
Colli, Anna Maria
Cappelleri, Alessia
Mayer, Alessandra
Raschetti, Roberto
Di Cosola, Roberta
Pisaniello, Marco
Annoni, Giuseppe Alberto
Papa, Marco
Maldi, Mimoza
Olivieri, Guido
Mosca, Fabio
Marianeschi, Stefano
author_facet Picciolli, Irene
Francescato, Gaia
Colli, Anna Maria
Cappelleri, Alessia
Mayer, Alessandra
Raschetti, Roberto
Di Cosola, Roberta
Pisaniello, Marco
Annoni, Giuseppe Alberto
Papa, Marco
Maldi, Mimoza
Olivieri, Guido
Mosca, Fabio
Marianeschi, Stefano
author_sort Picciolli, Irene
collection PubMed
description Cor triatriatum dexter (CTD) is an extremely uncommon and underreported congenital cardiac anomaly in which the persistence of the embryonic right venous valve separates the right atrium into two chambers with varying degrees of obstruction to antegrade flow and variable degree of right to left shunt at atrial level. Depending on the size of the valves, clinical manifestations vary from absence of symptoms to severe hypoxia, requiring urgent surgical correction. We herein describe the diagnostic difficulties in a case of neonatal CTD, who developed increasingly severe and unresponsive cyanosis, first interpreted as postnatal maladjustment with pulmonary hypertension. The failure to respond to oxygen and pulmonary vasodilators led us to reconsider a different diagnosis. The use of contrast echocardiography improved the diagnostic performance of transthoracic echocardiogram (TTE) and revealed a massive right-to-left shunt secondary to the presence of an atrial membrane that required urgent surgery.
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spelling pubmed-91393592022-05-28 Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis Picciolli, Irene Francescato, Gaia Colli, Anna Maria Cappelleri, Alessia Mayer, Alessandra Raschetti, Roberto Di Cosola, Roberta Pisaniello, Marco Annoni, Giuseppe Alberto Papa, Marco Maldi, Mimoza Olivieri, Guido Mosca, Fabio Marianeschi, Stefano Children (Basel) Case Report Cor triatriatum dexter (CTD) is an extremely uncommon and underreported congenital cardiac anomaly in which the persistence of the embryonic right venous valve separates the right atrium into two chambers with varying degrees of obstruction to antegrade flow and variable degree of right to left shunt at atrial level. Depending on the size of the valves, clinical manifestations vary from absence of symptoms to severe hypoxia, requiring urgent surgical correction. We herein describe the diagnostic difficulties in a case of neonatal CTD, who developed increasingly severe and unresponsive cyanosis, first interpreted as postnatal maladjustment with pulmonary hypertension. The failure to respond to oxygen and pulmonary vasodilators led us to reconsider a different diagnosis. The use of contrast echocardiography improved the diagnostic performance of transthoracic echocardiogram (TTE) and revealed a massive right-to-left shunt secondary to the presence of an atrial membrane that required urgent surgery. MDPI 2022-05-06 /pmc/articles/PMC9139359/ /pubmed/35626853 http://dx.doi.org/10.3390/children9050676 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Picciolli, Irene
Francescato, Gaia
Colli, Anna Maria
Cappelleri, Alessia
Mayer, Alessandra
Raschetti, Roberto
Di Cosola, Roberta
Pisaniello, Marco
Annoni, Giuseppe Alberto
Papa, Marco
Maldi, Mimoza
Olivieri, Guido
Mosca, Fabio
Marianeschi, Stefano
Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis
title Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis
title_full Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis
title_fullStr Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis
title_full_unstemmed Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis
title_short Cor Triatriatum Dexter: Contrast Echocardiography Is Key to the Diagnosis of a Rare but Treatable Cause of Neonatal Persistent Cyanosis
title_sort cor triatriatum dexter: contrast echocardiography is key to the diagnosis of a rare but treatable cause of neonatal persistent cyanosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9139359/
https://www.ncbi.nlm.nih.gov/pubmed/35626853
http://dx.doi.org/10.3390/children9050676
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