The clinical profile of Ebstein's malformation as seen from the fetus to the adult in 52 patients

Cardiology in the Young
Alejandro Flores ArizmendiManuel Quero Jiménez

Abstract

Ebstein's malformation of the tricuspid valve is a rare but complex congenital cardiac lesion characterised by a variable degree of dysplasia and displacement of the proximal attachments of its inferior and septal leaflets from the true atrioventricular junction. The aim of our retrospective study is to report the risk factors for mortality, and to determine the clinical profile as seen in 52 cases diagnosed in our service between 1978 and 2002, concentrating in particular on the outcome for the neonatal patient. There were 26 females and 26 males, and the age at presentation ranged from 30 weeks gestational age to 46 years. We found 23 associated cardiac anomalies in 20 cases. Of the patients, 11 patients (21%) died. Actuarial survival at 30 years was 65%. Predictors of death included fetal or neonatal presentation, presence of associated defects, a grade within the Celermajer index of 3 or 4, and a cardiothoracic ratio equal to or greater than 65%. The diagnosis was made in the neonatal period in 24 patients, nine of whom died. Of the 15 survivors, only 4 are free of symptoms. Surgical treatment was undertaken in 9 patients, with a mortality rate of 33% without late deaths, with all the survivors being in good condition. The ...Continue Reading

References

Apr 1, 1978·The American Journal of Cardiology·K R Anderson, J T Lie
Apr 1, 1992·Journal of the American College of Cardiology·D S CelermajerJ E Deanfield
Apr 1, 1992·Journal of the American College of Cardiology·D D Mair
Apr 1, 1992·Journal of the American College of Cardiology·D S CelermajerJ E Deanfield
Jan 1, 1991·British Heart Journal·L D AllanK Priestley
Jan 1, 1991·Journal of the American College of Cardiology·L K HornbergerK L Reed
Mar 15, 1991·Journal of the American College of Cardiology·G K SharlandL D Allan
Aug 1, 1990·International Journal of Cardiology·G K SharlandL D Allan
Nov 1, 1989·Journal of the American College of Cardiology·D A Roberson, N H Silverman
Oct 1, 1971·The American Journal of Cardiology·M LevR A Arcilla
Feb 1, 1984·Journal of the American College of Cardiology·A ShiinaA J Tajik
Jan 12, 1994·JAMA : the Journal of the American Medical Association·L S CohenM L Weiner
Jan 1, 1994·Journal of the American College of Cardiology·D S CelermajerJ E Deanfield
Jun 1, 1997·The Annals of Thoracic Surgery·N AugustinF Sebening
Nov 5, 1997·American Heart Journal·N M AmmashJ B Seward
Apr 4, 1998·The American Journal of Cardiology·A T YetmanB W McCrindle
Jun 18, 1998·American Heart Journal·M PavlovaM Robertson
Jul 15, 1998·European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery·S ChauvaudA Carpentier
Nov 7, 1998·American Journal of Medical Genetics·P de Lonlay-DebeneyV Cormier-Daire
Mar 30, 1999·The Journal of Thoracic and Cardiovascular Surgery·C KreutzerE A Kreutzer
May 8, 2000·The Annals of Thoracic Surgery·J A Dearani, G K Danielson
Aug 17, 2000·Revista española de cardiología·A Garrido MartínJ A Sobrino Daza
Sep 16, 2000·Pediatric Dermatology·S KrengelR Ruiz-Maldonado
Sep 27, 2000·The Thoracic and Cardiovascular Surgeon·S Chauvaud
Oct 12, 2000·European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery·V V LazorishinetsG V Knyshov
Jun 25, 2002·The Annals of Thoracic Surgery·Christopher J Knott-CraigJerry D Razook
Jan 1, 1956·British Heart Journal·J C ADAMS, R HUDSON

❮ Previous
Next ❯

Citations

Jul 3, 2007·Current Cardiology Reports·Michelle Gurvitz, Karen Stout
Oct 31, 2013·Cardiology in the Young·Lianne M Geerdink, Livia Kapusta
May 25, 2007·Neonatal Network : NN·Sarah E Pashia
May 14, 2008·Journal of Cardiovascular Medicine·Massimiliano CantinottiReza Razavi
Mar 24, 2018·Archives of Disease in Childhood·Lianne M GeerdinkLivia Kapusta
Jan 27, 2011·Prenatal Diagnosis·Jimmy E JadaonMoshe Ben-Ami
Oct 17, 2015·Seminars in Cardiothoracic and Vascular Anesthesia·Faith J RossDenise Joffe
Sep 26, 2009·Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine·Nobuhiro HidakaNorio Wake
Oct 16, 2016·Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine·Greggory R DeVoreMark Sklansky
Apr 6, 2011·American Journal of Medical Genetics. Part a·Philip J LupoLaura E Mitchell
Nov 3, 2021·Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine·Juanjuan LiuMingxing Xie

❮ Previous
Next ❯

Related Concepts

Related Feeds

Atrial Fibrillation

Atrial fibrillation is a common arrhythmia that is associated with substantial morbidity and mortality, particularly due to stroke and thromboembolism. Here is the latest research.

Arrhythmia

Arrhythmias are abnormalities in heart rhythms, which can be either too fast or too slow. They can result from abnormalities of the initiation of an impulse or impulse conduction or a combination of both. Here is the latest research on arrhythmias.

Birth Defects

Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.