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
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.
