Functional status after operation for Ebstein anomaly: the Mayo Clinic experience.

Published on Aug 5, 2008in Journal of the American College of Cardiology20.589
· DOI :10.1016/J.JACC.2008.03.064
Morgan L. Brown24
Estimated H-index: 24
(Mayo Clinic),
Joseph A. Dearani95
Estimated H-index: 95
(Mayo Clinic)
+ 6 AuthorsDavid J. Driscoll71
Estimated H-index: 71
(Mayo Clinic)
Sources
Abstract
Objectives The objective of this study was to review the long-term functional outcome of patients with Ebstein anomaly who had cardiac operation at our institution. Background Ebstein anomaly is a spectrum of tricuspid valvular and right ventricular dysplasia. Many patients will require operation in an attempt to improve quality of life. Methods From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly underwent 604 cardiac operations at the Mayo Clinic in Rochester, Minnesota. Patient records were reviewed, and all patients known to still be alive were mailed a medical questionnaire or contacted by telephone. Results At the initial operation at our institution, the mean age of the patients was 24 years (range 8 days to 79 years) and 53% were female patients. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%, respectively. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years, respectively. Surveys were returned by 285 of 448 (64%) patients known to be alive at the time of this study. Two hundred thirty-seven (83%) patients were in New York Heart Association functional class I or II, and 34% were taking no cardiac medication. One hundred three patients (36%) reported an incident of atrial fibrillation or flutter, 5 patients (2%) reported having had endocarditis, and 1 patient ( Conclusions Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein anomaly. Atrial arrhythmias are common both before and after surgery. Many patients have had one or more successful pregnancies with a low-recurrence risk of congenital heart disease.
📖 Papers frequently viewed together
141 Citations
348 Citations
191 Citations
References11
Newest
#1Morgan L. Brown (Mayo Clinic)H-Index: 24
#2Joseph A. Dearani (Mayo Clinic)H-Index: 95
Last. David J. Driscoll (Mayo Clinic)H-Index: 71
view all 9 authors...
Objective Our objective was to review the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery at our institution. Methods Patient records were reviewed, and all patients were mailed a medical questionnaire or contacted by means of telephone. Patients who had pulmonary atresia with an intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance were excluded. Results From April 1, 1972, to January ...
141 CitationsSource
#1Willem Drenthen (UMCG: University Medical Center Groningen)H-Index: 15
#2Petronella G. Pieper (UMCG: University Medical Center Groningen)H-Index: 41
Last. Zahara investigatorsH-Index: 6
view all 12 authors...
A search of peer-reviewed literature was conducted to identify reports that provide data on complications associated with pregnancy in women with structural congenital heart disease (CHD). This review describes the outcome of 2,491 pregnancies, including 377 miscarriages (15%) and 114 elective abortions (5%). Important cardiac complications were seen in 11% of the pregnancies. Obstetric complications do not appear to be more prevalent. In complex CHD, premature delivery rates are high, and more ...
437 CitationsSource
#1Joseph A. Dearani (Mayo Clinic)H-Index: 95
#2Patrick W. O'Leary (Mayo Clinic)H-Index: 40
Last. Gordon K. Danielson (Mayo Clinic)H-Index: 104
view all 3 authors...
Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet ( Fig. 1 ). The second feature is anterior and apical rotational displa...
49 CitationsSource
#1Anant Khositseth (Mayo Clinic)H-Index: 8
#2Gordon K. Danielson (Mayo Clinic)H-Index: 104
Last. Co-Burn J. Porter (Mayo Clinic)H-Index: 13
view all 5 authors...
Abstract Objective This study was undertaken to review the role of electrophysiology testing and to determine the early and late results of medical and surgical management of supraventricular tachyarrhythmias in Ebstein anomaly. Methods We reviewed 130 patients between 1990 and 2001 with Ebstein anomaly and history of tachyarrhythmia with a median age of 25 years (mean age 27.5 years); 106 underwent electrophysiologic testing and 24 had documented atrial flutter or fibrillation. We excluded 21 p...
119 CitationsSource
Abstract Ebstein's anomaly is a rare congenital heart defect that is characterized by a spectrum of anatomical abnormalities of the tricuspid valve that also involve the right atrium and right ventricle. The extant nomenclature for Ebstein's anomaly and our approach to the description of the severity of Ebstein's anomaly are reviewed with the objective of establishing a unified reporting system. Although there are common features in Ebstein's anomaly, there is a wide spectrum of pathology with a...
107 CitationsSource
Abstract Objectives. The purpose of this study was to identify the determinants of exercise tolerance in patients with Ebstein’s anomaly. Background. Patients with Ebstein’s anomaly of the tricuspid valve may have exercise limitation that improves after surgical repair. Methods. One hundred seventeen patients performed cycle ergometry for a total of 124 tests (preoperative test in 76 patients, postoperative test in 23, test but no operation in 18, preoperative and postoperative test in 7). Multi...
54 CitationsSource
Objectives. The outcome of pregnancy in Ebstein's anomaly was studied in 72 such patients (44 women, 28 men) who had had pregnancies or fathered children. Background. Patients with Ebstein's anomaly often reach childbearing age. Reports of the outcome of pregnancy in Ebstein's anomaly are available; however, the number of patients is small. Methods. The medical and surgical data bases at the Mayo Clinic were reviewed, and 145 patients (62 men, 83 women) of childbearing age with Ebstein's anomaly...
115 CitationsSource
#1David J. Driscoll (Mayo Clinic)H-Index: 71
#2Virginia V. MichelsH-Index: 57
Last. William H. WeidmanH-Index: 31
view all 10 authors...
Background. Knowledge of the risk of occurrence of congenital heart defects in offspring of individuals with a congenital heart defect is important for genetic counseling and understanding the etiology of congenital heart diseases. Methods and Results. A portion of the questionnaire mailed to all patients in the Second Natural History Study of Congenital Heart Defects (NHS-2) addressed marital status, pregnancy, miscarriage, and presence or absence of congenital heart defects or other congenital...
44 Citations
#1David J. Driscoll (Mayo Clinic)H-Index: 71
#2Carl D. Mottram (Mayo Clinic)H-Index: 12
Last. Gordon K. Danielson (Mayo Clinic)H-Index: 104
view all 3 authors...
Abstract To determine the effects of definitive operation for Ebstein's anomaly on rest and exercise cardiorespiratory function, cycle exercise studies were performed on 38 patients with Ebstein's anomaly before definitive operation and on 11 patients after operation. An atrial septal defect was present in 29 of the 38 preoperative patients and in none of the postoperative patients. Seven of the postoperative patients had tricuspid valvuloplasty and four had valve replacement. Exercise tolerance...
54 CitationsSource
#1Anderson KrH-Index: 1
#2Lie JtH-Index: 1
: Ebstein's anomaly of the tricuspid valve is associated with right ventricular dilatation in approximately two-thirds of cases. Dilatation may be massive. It involves not only the right ventricular wall proximal to the tricuspid valve (atrialized ventricle) but also the right ventricle distal to the valve (funtional right ventricle), including the right ventricular infundibulum. For further definition of the pathogenesis of dilatation of the functional right ventricle (distal chamber), a morpho...
65 Citations
Cited By78
Newest
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2William R. Miranda (Mayo Clinic)H-Index: 14
Last. Joseph A. Dearani (Mayo Clinic)H-Index: 95
view all 4 authors...
Background Although tricuspid valve surgery improves functional capacity in patients with Ebstein anomaly, it is not always associated with improvement in aerobic capacity. The purpose of this study was to identify the determinants of improved aerobic capacity after tricuspid valve surgery in adults with Ebstein anomaly with severe tricuspid regurgitation. Methods Retrospective study of patients with severe tricuspid regurgitation due to Ebstein anomaly that had tricuspid valve surgery at Mayo C...
1 CitationsSource
2 CitationsSource
#1Jimmy Windsor (Roy J. and Lucille A. Carver College of Medicine)H-Index: 10
#2Chaitra Mukundan (Roy J. and Lucille A. Carver College of Medicine)
Last. Harish Ramakrishna (Mayo Clinic)H-Index: 20
view all 4 authors...
Abstract Adult congenital heart disease (ACHD) continues to rapidly increase worldwide. With an estimated 1.5 million adults with ACHD alone in the USA, there is a growing need for better education in the management of these complex patients and multiple knowledge gaps exist. This manuscript will comprehensively review the recent (2020) updated ESC Guidelines for the management of adult congenital heart disease created by the Task Force for the management of adult congenital heart disease of the...
Source
#1Helmut BaumgartnerH-Index: 63
#2Julie De BackerH-Index: 44
Last. Bernard IungH-Index: 83
view all 53 authors...
126 CitationsSource
#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
#2William R. Miranda (Mayo Clinic)H-Index: 14
Last. Heidi M. Connolly (Mayo Clinic)H-Index: 75
view all 4 authors...
Background: Left ventricular global longitudinal strain (LVGLS) can detect early phases of LV systolic dysfunction, but its application has not been studied in Ebstein anomaly. We hypothesized that...
Source
#1Melchior Burri (TUM: Technische Universität München)H-Index: 8
#2Karim Mrad Agua (TUM: Technische Universität München)H-Index: 1
Last. Rüdiger Lange (TUM: Technische Universität München)H-Index: 56
view all 10 authors...
Abstract Objectives We aimed to investigate tricuspid valve (TV) function and adverse events following conventional repair and valve replacement for Ebstein's anomaly and compare them with the Cone-repair. Methods The medical records of 151 patients (mean age 25 years, 62% female) operated in a single centre from 1985 to 2018 were retrospectively analysed. To determine tricuspid valve regurgitation (TR) during follow-up, serial echocardiographic examination was used (n=2397, TR-grades were graph...
9 CitationsSource
#1Mathias Possner (UW: University of Washington)H-Index: 4
#2Francisco J. Gensini (UW: University of Washington)H-Index: 1
Last. Zachary L. Steinberg (UW: University of Washington)H-Index: 4
view all 5 authors...
Purpose of review Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population. Recent findings Increased understanding of anatomic accessory atrioventricular pathways in EA has res...
2 CitationsSource
#1Margaret M. Fuchs (Mayo Clinic)H-Index: 2
#2Heidi M. Connolly (Mayo Clinic)H-Index: 75
Ebstein anomaly is a congenital malformation involving primarily the tricuspid valve, with failure of delamination from the underlying myocardium and right ventricular myopathy. Echocardiography is diagnostic in most patients and demonstrates apical displacement of the septal leaflet and variable tethering of leaflet tissue to the right ventricular myocardium. Operative intervention is considered for exertional symptoms, progressive right ventricular enlargement, or right ventricular dysfunction...
Source
Purpose: Different surgical procedures were defined due to degree of Ebstein anomaly. In this study, we are reporting our surgical experience of adult patients with Ebstein anomaly. Methods: We analyzed the patients, who had operated with the diagnosis of Ebstein anomaly from March 2011 through February 2018, retrospectively. We evaluated patients in two groups: patients with cone type surgical repair were in Group 1 and patients with other surgical (Danielson, Carpentier) procedures were in Gro...
2 CitationsSource
OBJECTIVE: To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly. METHODS: We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning. RESULTS: Of 116 eligible patients, 72 (62%) responded. About 96% r...
3 CitationsSource