Clinical profile and natural history of Ebstein's anomaly of tricuspid valve

Jaiswal, Praveen Kumar; Balakrishnan, K.G.; Saha, Anup Kumar; Venkitachalam, C.G.; Tharakan, Jaganmohan; Titus, Thomas

doi:10.1016/0167-5273(94)90031-0

Cited by 25 publications

(9 citation statements)

References 10 publications

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“…This prevalence is not different from the ones reported in populations of EA patients of all ages, ranging from 10% to 21% [2, 3, 6, 7, 9, 11, 13, 14, 17, 18, 24, 25, 27]. As EA is a malformation of the right side of the heart, it is not surprising that in our study, as in others [2, 3, 6, 7, 9, 11, 13, 14, 17, 18, 24, 25, 27], only right-sided accessory pathways are found. The pediatric algorithm to predict the location of the accessory pathway based on QRS polarity in all ECG leads as devised by Boersma et al [4] appeared to be more congruent with the results during catheter ablation than the adult algorithm as developed by d’Avila [8].…”

Section: Discussioncontrasting

confidence: 57%

“…Thus, a potential substrate for accessory atrioventricular connections and ventricular preexcitation is created [10]. This is reflected in the fact that preexcitation and Wolff–Parkinson–White (WPW) syndrome are more frequently associated with EA than with any other congenital heart defect [16], with a reported prevalence varying between 0 and 44% [2, 3, 6, 7, 9, 11, 13, 14, 17, 18, 24, 25, 27]. However, agreement on the prevalence in the young EA population is lacking.…”

Section: Introductionmentioning

confidence: 99%

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A Multicenter, Long-Term Study on Arrhythmias in Children with Ebstein Anomaly

et al. 2009

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To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff–Parkinson–White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and followed by pediatric cardiologists between 1980 and 2005 in The Netherlands. During a follow-up after EA diagnosis of 13 years 3 months (range: 6 days to 28 years 2 months), 16 (17%) of the 93 pediatric EA patients exhibited rhythm disturbances. Nine patients showed arrhythmic events starting as of the neonatal period. Supraventricular tachycardia was noted in 11 patients. One patient died in the neonatal period due to intractable supraventricular tachycardia resulting in heart failure and one patient died at 5 weeks of age most probably due to an arrhythmic event. The 14 surviving patients all show preexcitation, albeit 4 of them intermittently, and all have a right-sided accessory pathway location. Nine patients underwent catheter ablation of an accessory pathway. Only four patients are currently on antiarrhythmic drugs. The 17% prevalence of rhythm disturbances in pediatric EA patients, most commonly supraventricular arrhythmias, is significantly lower than in adult EA patients. Life-threatening rhythm disturbances are not frequent early in life. Symptomatic patients are well treated with radiofrequency catheter ablation.

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Section: Discussioncontrasting

confidence: 57%

Section: Introductionmentioning

confidence: 99%

A Multicenter, Long-Term Study on Arrhythmias in Children with Ebstein Anomaly

et al. 2009

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“…Several articles focused on the mortality and morbidity in adult patients with CCHD such as Eisenmenger syndrome [3][4][5][6][7][8][9][10][11][12][13], tricuspid atresia [14], pulmonary atresia with ventricular septal defect [15], Glenn shunts [16], palliation with cavopulmonary or aortopulmonary shunts [17], Ebstein's anomaly [18] or univentricular heart [19]. However, most of these studies were conducted by a single center and the number of patients was relatively small.…”

Section: Introductionmentioning

confidence: 99%

Predictive factors for long-term prognosis in adults with cyanotic congenital heart disease — Japanese multi-center study

Sakazaki¹,

Niwa

Echigo

et al. 2007

International Journal of Cardiology

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“…The mean age of death is approximately 20 years, with 15% alive at 60 years. 158 VSD, pulmonary stenosis or atresia, and mitral valve abnormalities may accompany Ebstein's anomaly. Eighty percent of patients with Ebstein's anomaly will have an ASD or PFO.…”

Section: Ebstein's Anomalymentioning

confidence: 99%

“…160 There are also fewer cells in the Ebstein's right ventricle than are found in a normal right ventricle, which contributes to ventricular dilation in addition to the significant tricuspid regurgitation. 158 If a child survives the neonatal period, cyanosis may resolve as the infant ages and PVR is lowered. 160 Functional class is an excellent gauge of the severity of Ebstein's anomaly before administering an anesthetic for a noncardiac procedure.…”

Section: Ebstein's Anomalymentioning

confidence: 99%