Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico

Aortic dissection is a life-threatening disease that requires immediate surgical intervention. Marfan syndrome is a hereditary disease with an autosomal dominant transmission, which affects the connective tissue, with skeletal, cardiovascular and ocular involvement. It is one of the most prevalent c...

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Autores principales: Espinoza S,Christian, Selman A,Rafael, Pauchard T,Felipe, Rivera F,Juan, Iturra U,Sebastián, Montecinos R,Fernando, Eliash D,Héctor, Undurraga H,Francisco
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2009
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000100015
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spelling oai:scielo:S0034-988720090001000152009-04-13Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínicoEspinoza S,ChristianSelman A,RafaelPauchard T,FelipeRivera F,JuanIturra U,SebastiánMontecinos R,FernandoEliash D,HéctorUndurraga H,Francisco Aortic aneurysm Marfan syndrome Pregnancy complications cardiovascular Aortic dissection is a life-threatening disease that requires immediate surgical intervention. Marfan syndrome is a hereditary disease with an autosomal dominant transmission, which affects the connective tissue, with skeletal, cardiovascular and ocular involvement. It is one of the most prevalent connective tissue disorders, presenting a rísk of aortic dissection of approximately 1% even without dilatation of the aorta. When dissection occurs during pregnancy and requires surgical intervention (type A dissection), maternal mortality is high (2096-30%). We report a 38 year-old woman with Marfan syndrome that had an acute type A aortic dissection and severe aortic regurgitation at 37 weeks of gestation. The patient underwent a cesarean section and delivered a healthy baby. Afterwards, aortic valve repair and ascending aortic replacement was successfully performed under circulatory arrest with deep hypothermia. Additionally mitral valve repair for degenerative disease with posterior, autologous pericardium mitral valve ring was performed.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.137 n.1 20092009-01-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000100015es10.4067/S0034-98872009000100015
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Aortic aneurysm
Marfan syndrome
Pregnancy complications
cardiovascular
spellingShingle Aortic aneurysm
Marfan syndrome
Pregnancy complications
cardiovascular
Espinoza S,Christian
Selman A,Rafael
Pauchard T,Felipe
Rivera F,Juan
Iturra U,Sebastián
Montecinos R,Fernando
Eliash D,Héctor
Undurraga H,Francisco
Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico
description Aortic dissection is a life-threatening disease that requires immediate surgical intervention. Marfan syndrome is a hereditary disease with an autosomal dominant transmission, which affects the connective tissue, with skeletal, cardiovascular and ocular involvement. It is one of the most prevalent connective tissue disorders, presenting a rísk of aortic dissection of approximately 1% even without dilatation of the aorta. When dissection occurs during pregnancy and requires surgical intervention (type A dissection), maternal mortality is high (2096-30%). We report a 38 year-old woman with Marfan syndrome that had an acute type A aortic dissection and severe aortic regurgitation at 37 weeks of gestation. The patient underwent a cesarean section and delivered a healthy baby. Afterwards, aortic valve repair and ascending aortic replacement was successfully performed under circulatory arrest with deep hypothermia. Additionally mitral valve repair for degenerative disease with posterior, autologous pericardium mitral valve ring was performed.
author Espinoza S,Christian
Selman A,Rafael
Pauchard T,Felipe
Rivera F,Juan
Iturra U,Sebastián
Montecinos R,Fernando
Eliash D,Héctor
Undurraga H,Francisco
author_facet Espinoza S,Christian
Selman A,Rafael
Pauchard T,Felipe
Rivera F,Juan
Iturra U,Sebastián
Montecinos R,Fernando
Eliash D,Héctor
Undurraga H,Francisco
author_sort Espinoza S,Christian
title Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico
title_short Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico
title_full Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico
title_fullStr Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico
title_full_unstemmed Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico
title_sort disección aórtica y síndrome de marfán en el embarazo: a propósito de un caso clínico
publisher Sociedad Médica de Santiago
publishDate 2009
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000100015
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AT pauchardtfelipe diseccionaorticaysindromedemarfanenelembarazoapropositodeuncasoclinico
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AT eliashdhector diseccionaorticaysindromedemarfanenelembarazoapropositodeuncasoclinico
AT undurragahfrancisco diseccionaorticaysindromedemarfanenelembarazoapropositodeuncasoclinico
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