The outcomes of operations for 539 patients with Ebstein anomaly

Brown, Morgan L.; Dearani, Joseph A.; Danielson, Gordon K.; Cetta, Frank; Connolly, Heidi M.; Warnes, Carole A.; Li, Zhuo; Hodge, David O.; Driscoll, David J.

doi:10.1016/j.jtcvs.2008.02.034

Cited by 192 publications

(144 citation statements)

References 33 publications

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“…The authors demonstrated comparable late results after TV plasty procedures and implantation of artificial TV valves both with low -few percent mortality rate (39). Nevertheless, after artificial valve implantation, often bothersome anticoagulation treatment is necessary and with passing time, a reoperation is almost always required, hence the continuous search for new possibilities of performing a successful TV plasty in patients with various forms of EA.…”

Section: Surgical Treatmentmentioning

confidence: 81%

Ebstein Anomaly

Pajak

2013

Journal of Rare Cardiovascular Diseases

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Ebstein anomaly (EA) is a rare congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, with atrialization of the right ventricle and variable degree of malformation and displacement of the anterior leaflet. Patients can have a variety of symptoms related to the anatomic abnormalities of EA and their hemodynamic effects or associated structural and conduction system disease. Most frequently cyanosis, palpitations, fatigue and dyspnea can be observed. In severe forms edema and ascites. Treatment of EA is complex and depends of the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Options of treatment include medical therapy, radiofrequency ablation, and surgical therapy. Main aim of the surgical therapy is to correct the underlying tricuspid valve, right ventricular abnormalities and any associated intracardiac defects if exist. Palliative procedures and cardiac transplantation can be considered in most sever EA patients. JRCD 2013; 1 (4): 139-143

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Section: Surgical Treatmentmentioning

confidence: 81%

Ebstein Anomaly

Pajak

2013

Journal of Rare Cardiovascular Diseases

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“…In contrast, other authors consider that surgery should be delayed until symptoms appear, cyanosis becomes evident or paradoxical embolism occurs. 9 Currently, there are insufficient data on the evolution of EA after surgical treatment, and, even though many studies report good outcome after surgery, general consensus on the timing of surgical management is lacking. The aim of this study was therefore to investigate the long-term prognosis of EA with and without surgical treatment, and analyze the factors associated with mortality.…”

Section: Methodsmentioning

confidence: 99%

“…Interestingly the surgical group had higher early mortality (18.36%) than in other series: 5.9% at 30 days. 9 Chauvaud, in his series of 142 operated patients, noted an early mortality of 10%; and 5 patients with akinetic RV and a very thin ventricular wall had very early mortality (24 h). 13 Early mortality in the present series was explained by RV failure in 77% of patients.…”

Section: Rv Dysfunction and Survivalmentioning

confidence: 97%

“…11, 12 Brown et al noted a late survival of 84.7% and 71.2% at 10 and 20 years, respectively, in a big surgical group. 9 Attenhofer Jost et al, in a study of 81 patients aged spectrum of disease. Due to its retrospective nature we did not have follow up on RV function; and last, novel echocardiographic techniques were not used to assess RV function due to their unavailability.…”

Section: Rv Dysfunction and Survivalmentioning

confidence: 99%

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Impact of Surgery in Ebstein’s Anomaly Using Current Surgical Criteria

Ávila-Vanzzini

Fritche-Salazar

Herrera-Bello

et al. 2017

Circ J

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terns depend on anatomical severity, degree of right-to-left atrial shunt, and age at presentation. 4, 5 Long-term outcome is usually good for patients who survive the neonatal period and for those with no symptoms following the first years of life. 3 Therefore, many patients will develop symptoms and surgical treatment may be necessary. Current surgical criteria according to European or US guidelines include: (1) presence of symptoms or worsening functional class; (2) cyanosis (oxygen saturation <90%); (3) paradoxical embolism; (4) progressive cardiomegaly on chest X-ray; and (5) progressive RV dilation or RV systolic dysfunction. 6 Other authors recommend surgical repair in patients with more than moderate TR in the presence of symptoms (New York Heart Association [NYHA] class >II or arrhythmias) or worsening exercise capacity. 7 Optimal timing for surgical management in this patient subset remains controversial. Badiu et al found that NYHA functional class >II and cardiothoracic index >0.6 are important prognostic factors associated with long-term mortal-E bstein's anomaly (EA) accounts for <1% of all congenital heart disease. There is general consensus that EA affects not only the tricuspid valve but also the whole right ventricle (RV). This results in a particular type of right-sided cardiomyopathy. 1 The characteristic features of this anomaly are: (1) predominant septal and inferior tricuspid leaflet attachment to the underlying myocardium due to failure of delamination; (2) anterior and apical rotational displacement of the functional annulus; (3) dilation of the "atrialized" portion of the RV with variable degrees of hypertrophy and wall thinning; (4) redundancy, fenestration and tethering of the anterior leaflet; (5) dilation of the right atrioventricular junction (the true tricuspid annulus); and (6) variable degree of ventricular myocardial dysplasia and dysfunction. 1-3These anatomical and functional abnormalities cause important tricuspid regurgitation (TR) in many patients that contributes to right chamber dilation and atrial or ventricular arrhythmias. Hemodynamics and clinical pat-

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“…7 Surgical repair of Ebstein anomaly, with either tricuspid valve repair or replacement, is associated with very good outcomes when performed before significant right and left ventricular dysfunction. 9 However, similar to other congenital heart defects, the risk of recurrent atrial arrhythmias increases as the patient ages despite surgical repair. 10 Therefore, lifelong follow-up by a congenital heart disease specialist is required even after surgical repair is performed.…”

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confidence: 99%