Among all congenital heart disease,Ebstein anomaly is the lesion with the widest spectrum of presentation.It can present as early as the neonatal period where immediate intervention is often needed to the adult with n...Among all congenital heart disease,Ebstein anomaly is the lesion with the widest spectrum of presentation.It can present as early as the neonatal period where immediate intervention is often needed to the adult with no discernable symptoms.It is also one where overall outcome greatly relates to age at presentation.Generally,presentation in the neonatal period is associated with poor outcomes while presentation beyond the neonatal period has excellent outcomes with low operative mortality.It is thus apparent that understanding every aspect of the anatomy and physiology of Ebstein anomaly and how it manifests clinically is paramount to successful treatment.The purpose of this review is to outline the current concepts and strategies in the surgical management of Ebstein anomaly,and to discuss the current expert consensus on how best to tackle this congenital heart disease at different ages of diagnosis.展开更多
Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestati...Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestations of cyanosis, right-sided heart failure and arrhythmias. The leaflet tethering and dysplasia, together with dilatation of the tricuspid valve ring, constitute the anatomic cause of tricuspid regurgitation observed in this condition. Case Reports: The spectrum of leaflet tethering from mild to extreme with varying degrees of tricuspid regurgitation were documented by echocardiography in a 16-year-old cyanotic male with Ebstein’s anomaly associated with an atrial septal defect and mild low tricuspid regurgitation (TR), 22-year-old acyanotic male with right-sided heart failure due to severe tricuspid regurgitation and an intact atrial septum, 55-year-old asymptomatic female with moderate high tricuspid regurgitation. The extreme form was described as an “atretic” mem-brane in a 28-year-old cyanotic male and as a “blanket” of leaflet tissue in a 30-year-old cyanotic male. Conclusion: Ebstein’s anomaly has to be sus-pected clinically in presence of cyanosis with a WPW (Wolf-Parkinson-White) or atrioventricular (AV) block pattern of ECG and its management is complex and must be individualized. RV (right ventricular) exclusion procedures are preferred in advanced cases.展开更多
Background Ebstein’s anomaly is a rare and complex congenital malformation of the tricuspid valve and the right ventricle with variable pathological anatomy. The surgical treatment of this anomaly has long been contr...Background Ebstein’s anomaly is a rare and complex congenital malformation of the tricuspid valve and the right ventricle with variable pathological anatomy. The surgical treatment of this anomaly has long been controversial. We reviewed cases corrected with Hetzer technique to analyze the uni/biventricular strategy,timing of surgery and results. Methods Between October 2009 and December 2013, 21 patients with Ebstein’s anomaly were admitted to Guangdong General Hospital and underwent surgical correction. There were 11 males and 10 females. Type A, 3 cases; Type B, 16 cases; Type C, 1 case, Type D, 1 case. The 2 patients of Type C and Type D undergoing Starnes procedure were excluded, all other 19 patients who underwent surgical repair with Hetzer technique in this time frame were included in the analysis. In this “Hetzer group”, the age at the time of the repair ranged from 13 months to 42 years（median： 8 years） and weight ranged from 11.3 to 55kg（median： 19.5 kg）. Combined anomalies include： 2 of 3 Type A combined with secondum atrial septal defect（ASD）, 14 of 16 cases of Type B combined with secondum ASD, both of 2 cases of Type C and D combined with secondum ASD. 2 of 21（9.5%） of these patients have Wolff-Parkinson-White（WPW）syndrome. Results There was no early, or late mortality. One 13-month infant boy, after valvuloplasty, ASD closure and bidirectional Glenn（BD-Glenn） procedure, presented with low cardiac output, depression of the left ventricle, severe tricuspid regurgitation（TR） with massively dilated right ventricle, the patient underwent emergent redo procedure, however, even after re-valvuloplasty, Transesophageal echocardiography（TEE）showed moderate to severe TR, we decided to reopen and enlarge the ASD, sewed a 5 mm fenestrated pericardial patch to close the tricuspid orifice partially, after 7 days, the patient weaned successfully from the ventilator. Another two 1-year old infants after valvuloplasty, BD Glenn and partial ASD closure showed no improvement of TR, but postoperative oxygen saturation increased, symptoms diminished and exercise tolerance improved significantly. Other 16 patients, whose age ranged from 5-42 years, had satisfactory results.Echocardiography showed： 14 of the 16 patients’ TR improved from severe to mild, 2 of them improved from moderate to trace or mild. Except the redo case, the pulse oxygen saturation at rest goes up to over 95%without cyanosis; 15 of 18 patients shows 85%-90% O2 saturation after exercise in room air. Conclusions Hetzer technique is a duplicable surgical approach to Ebstein’s anomaly with satisfactory results for older patients, especially for adults. However, we notice a trend that this technique may be not suitable for small infants, and further evaluation will be needed.展开更多
文摘Among all congenital heart disease,Ebstein anomaly is the lesion with the widest spectrum of presentation.It can present as early as the neonatal period where immediate intervention is often needed to the adult with no discernable symptoms.It is also one where overall outcome greatly relates to age at presentation.Generally,presentation in the neonatal period is associated with poor outcomes while presentation beyond the neonatal period has excellent outcomes with low operative mortality.It is thus apparent that understanding every aspect of the anatomy and physiology of Ebstein anomaly and how it manifests clinically is paramount to successful treatment.The purpose of this review is to outline the current concepts and strategies in the surgical management of Ebstein anomaly,and to discuss the current expert consensus on how best to tackle this congenital heart disease at different ages of diagnosis.
文摘Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestations of cyanosis, right-sided heart failure and arrhythmias. The leaflet tethering and dysplasia, together with dilatation of the tricuspid valve ring, constitute the anatomic cause of tricuspid regurgitation observed in this condition. Case Reports: The spectrum of leaflet tethering from mild to extreme with varying degrees of tricuspid regurgitation were documented by echocardiography in a 16-year-old cyanotic male with Ebstein’s anomaly associated with an atrial septal defect and mild low tricuspid regurgitation (TR), 22-year-old acyanotic male with right-sided heart failure due to severe tricuspid regurgitation and an intact atrial septum, 55-year-old asymptomatic female with moderate high tricuspid regurgitation. The extreme form was described as an “atretic” mem-brane in a 28-year-old cyanotic male and as a “blanket” of leaflet tissue in a 30-year-old cyanotic male. Conclusion: Ebstein’s anomaly has to be sus-pected clinically in presence of cyanosis with a WPW (Wolf-Parkinson-White) or atrioventricular (AV) block pattern of ECG and its management is complex and must be individualized. RV (right ventricular) exclusion procedures are preferred in advanced cases.
基金supported by Medical Scientific Research Foundation of Guangdong Province(No.B2012014)
文摘Background Ebstein’s anomaly is a rare and complex congenital malformation of the tricuspid valve and the right ventricle with variable pathological anatomy. The surgical treatment of this anomaly has long been controversial. We reviewed cases corrected with Hetzer technique to analyze the uni/biventricular strategy,timing of surgery and results. Methods Between October 2009 and December 2013, 21 patients with Ebstein’s anomaly were admitted to Guangdong General Hospital and underwent surgical correction. There were 11 males and 10 females. Type A, 3 cases; Type B, 16 cases; Type C, 1 case, Type D, 1 case. The 2 patients of Type C and Type D undergoing Starnes procedure were excluded, all other 19 patients who underwent surgical repair with Hetzer technique in this time frame were included in the analysis. In this “Hetzer group”, the age at the time of the repair ranged from 13 months to 42 years（median： 8 years） and weight ranged from 11.3 to 55kg（median： 19.5 kg）. Combined anomalies include： 2 of 3 Type A combined with secondum atrial septal defect（ASD）, 14 of 16 cases of Type B combined with secondum ASD, both of 2 cases of Type C and D combined with secondum ASD. 2 of 21（9.5%） of these patients have Wolff-Parkinson-White（WPW）syndrome. Results There was no early, or late mortality. One 13-month infant boy, after valvuloplasty, ASD closure and bidirectional Glenn（BD-Glenn） procedure, presented with low cardiac output, depression of the left ventricle, severe tricuspid regurgitation（TR） with massively dilated right ventricle, the patient underwent emergent redo procedure, however, even after re-valvuloplasty, Transesophageal echocardiography（TEE）showed moderate to severe TR, we decided to reopen and enlarge the ASD, sewed a 5 mm fenestrated pericardial patch to close the tricuspid orifice partially, after 7 days, the patient weaned successfully from the ventilator. Another two 1-year old infants after valvuloplasty, BD Glenn and partial ASD closure showed no improvement of TR, but postoperative oxygen saturation increased, symptoms diminished and exercise tolerance improved significantly. Other 16 patients, whose age ranged from 5-42 years, had satisfactory results.Echocardiography showed： 14 of the 16 patients’ TR improved from severe to mild, 2 of them improved from moderate to trace or mild. Except the redo case, the pulse oxygen saturation at rest goes up to over 95%without cyanosis; 15 of 18 patients shows 85%-90% O2 saturation after exercise in room air. Conclusions Hetzer technique is a duplicable surgical approach to Ebstein’s anomaly with satisfactory results for older patients, especially for adults. However, we notice a trend that this technique may be not suitable for small infants, and further evaluation will be needed.
