Biventricular repair of Ebstein's anomaly with pulmonary atresia in a low birth weight neonate

Cyanotic neonates with Ebstein's anomaly associated with pulmonary atresia are a subgroup with the high mortality rate in pediatric cardiac surgery. We report a case of a severely symptomatic low birth weight neonate with Ebstein's anomaly and pulmonary atresia. A biventricular repair with Danielson-type tricuspid valve repair was performed. The baby tolerated the procedure with adequate hemodynamics, but died of peritoneal hemorrhage on the second postoperative day. This report could be an addition to the recent literature regarding the feasibility of biventricular repair for symptomatic neonates with Ebstein's anomaly even in low birth weight neonates.     

Surgical management of Ebstein's anomaly in the adult

Ebstein's anomaly is a rare cardiac malformation that affects the tricuspid valve, right ventricle, and right atrioventricular junction. These anatomical and functional abnormalities cause important tricuspid regurgitation that results in right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Diagnosis is made by echocardiography. Operation includes tricuspid valve repair or replacement, closure of any interatrial communications, and appropriate antiarrhythmia procedures. Repair of Ebstein's anomaly eliminates right-to-left intracardiac shunting, improves exercise tolerance and functional class, and reduces supraventricular arrhythmias. In addition, quality of life and longevity are improved.     

新生儿围手术期处理

新生儿期的外科疾病绝大多数为先天性畸形.新生儿的各个系统发育尚不完善,机体处于不稳定状态,调节功能和外界环境的适应能力差,但有些畸形直接影响患儿的生命,必须在此期内施行手术治疗,因此新生儿围手术期的处理是否正确与及时直接影响患儿的手术成败及预后.     

Right ventricular outflow tract reconstruction in two neonates with pulmonary atresia and intact ventricular septum

Two neonates, aged 8 and 18 days, with pulmonary atresia and intact ventricular septum underwent right ventricular outflow tract reconstruction with an autologous pericardial transannular patch. Preoperative cardiac catheterization revealed a tripartite right ventricular morphology with suprasystemic right ventricular morphology with suprasystemic right ventricular systolic pressure (right ventricular to left ventricular peak pressure ratio was 1.2 in case 1 and 2.0 in case 2). Right ventricular volume was 73.5% of normal and 115% of normal respectively. Generous resection of the infundibular myocardium was done and a patch was inserted with the heart kept beating as the perfusate temperature of 32 degrees C. In case 1 a modified Blalock-Taussig shunt was added on the postoperative day 21, because of the insufficient growth of the right ventricle, but in case 2 prostaglandin E1 could be discontinued on the postoperative day 9.     

A successful case of tricuspid valvulectomy for Ebstein's anomaly with severe tricuspid stenosis]

A 44-year-old male was admitted to our hospital complaining of general fatigue and dyspnea on exertion with congestive heart failure (NYHA IV). Cardiac catheterization and angiogram revealed Ebstein's anomaly with severe tricuspid stenosis and functioning small right ventricle. Because of tiny right ventricular chamber, the usual surgical treatment for Ebstein's anomaly i.e. tricuspid valve replacement or valvuloplasty were deemed to be inappropriate. Tricuspid valvulectomy was performed to relieve the inflow stenosis of the right ventricle and excellent symptomatic relief was achieved. It is very rare to seen an adult case of Ebstein's anomaly with severe tricuspid stenosis and functioning small right ventricle. Tricuspid valvulectomy may be justified to use in this rare condition.     

Perioperative management of living-donor liver transplantation in two patients with severe portopulmonary hypertension

Liver transplantation for patients with severe portopulmonary hypertension (PPHTN) has been associated with high mortality. We conducted perioperative management of two patients with severe PPHTN for living-donor liver transplantation. The first case was a 17-year-old male with biliary atresia. He developed dyspnea at the age of 14, for which he was treated with intravenous epoprostenol for 8 months. As a result, the mean pulmonary artery pressure (MPAP) was reduced from 61 to 40 mmHg. Intraoperatively, he was treated with intravenous epoprostenol and nitric oxide (NO) inhalation. His intraoperative course was uneventful but he died from right heart failure on postoperative day (POD) 11. The second case was a 6-year-old girl with biliary atresia. When she was 5 years old, examination for a persistent cough revealed MPAP of 49 mmHg. Neither intravenous epoprostenol nor NO inhalation was effective, and she twice showed transient pulmonary hypertension during the operation. She was extubated 14 hours after the surgery, transferred out of ICU on POD 3 and discharged from the hospital on POD 99. When we compare the two cases, the factors responsible for the success of the management of the second case appear to be early extubation and the short duration of PPHTN.     

The management of oesophageal atresia in neonates with right-sided aortic arch

BACKGROUND/PURPOSE: The management of oesophageal atresia (OA) in association with a right-sided aortic arch (RAA) is challenging. It is controversial whether right or left thoracotomy is the most appropriate surgical approach. The aim of this study was to determine the prevalence of RAA in patients with OA and the most appropriate surgical approach. METHODS: The case records of all the neonates with OA treated over an 18-year period (1980 through 1997) were reviewed. Patients with RAA were analysed with particular reference to the preoperative investigations, operative approach and findings, and postoperative complications. RESULTS: Of the 476 case notes reviewed, 12 (2.5%) had a RAA, and 5 of these infants had major cardiac anomalies. The diagnosis was not suspected on preoperative chest x-ray in any of these infants. In only 1 of the 5 neonates who had a preoperative echocardiogram was RAA suspected. A right thoracic approach was performed in the 11 infants in whom a RAA was not suspected. Immediate conversion to a left thoracotomy with primary anastomosis was carried out in 2 infants. In 2 further infants, fistula ligation alone was performed because of a long gap atresia. Anastomosis via the right thoracotomy was performed in 7 infants. There were 3 anastomotic leaks, 1 intractable stricture, and 1 recurrent fistula. Three patients (25%) had a double aortic arch, and, as a result, a left thoracotomy had to be abandoned in 2. There were 3 deaths, 2 as a result of major cardiac anomalies and 1 related to prematurity and sepsis. CONCLUSIONS: RAA occurs in 2.5% of infants with OA. Preoperative chest x-ray and echocardiogram are unreliable as diagnostic modalities. Anastomosis via a right thoracotomy is associated with a 42% leak rate. The presence of a double aortic arch can make the oesophageal anastomosis via a left thoracotomy as difficult as via a right thoracotomy.     

Reconstruction of the right heart in an elderly woman with Ebstein's anomaly and severe heart failure

We report the case of a 53-year-old woman with Ebstein's anomaly and intractable heart failure who had undergone only tricuspid valve replacement 30 years earlier. She was treated conservatively for 1 month; however, she was placed in the New York Heart Association functional class IV. Therefore, we operated on her with the objective of improving her quality of life and cardiac function. One-and-a-half repair and a second tricuspid valve replacement with right ventriculoplasty were quite effective in ameliorating her critical condition.     

Staged surgical management of pulmonary atresia with diminutive pulmonary arteries.

A particular problem in treatment is presented by patients who have atresia of the pulmonary valve and ventricular septal defect but whose pulmonary arterial tree is extremely diminutive. The pulmonary blood flow results from naturally occurring large bronchial collateral arteries. In the experience reported, satisfactory palliation was provided for all 3 patients in whom right ventricle--to--pulmonary artery continuity was constructed by placing a pericardial patch graft during open-heart operation. The ventricular septal defect was left unrepaired. Early evidence supports the hypothesis that, besides providing palliation of cyanosiis, the procedure may allow progressive enlargment of the hypoplastic pulmonary arteries. This offers hope that later second-stage completion of total repair, involving closure of the ventricular septal defect and ligation of any remaining large bronchial collateral arteries, may become feasible.     

Anesthetic management for surgery of the ankle joint fracture in a patient with Ebstein's anomaly

The authors describe the successful anesthetic management of a patient with Ebstein's anomaly and Wolff-Parkinson-White syndrome who underwent repair for foot joint fracture. Anesthesia was provided by combined spinal-epidural block to minimize hemodynamic changes which cause an increase in right-to-left shunt and hypoxemia. We estimated hemodynamic status using radial arterial pressure, central venous pressure and ECG from the start of anesthesia. During anesthesia, his vital signs were unchanged to any significant degree. The recovery was rapid and the postoperative course was uneventful.     

Surgical repair of Ebstein's anomaly.

Ebstein's anomaly is a complex malformation that has been treated by various surgical techniques, with variable results, since 1958. In 1972, the authors developed a repair that consisted of plication of the free wall of the atrialized portion of the right ventricle, posterior tricuspid anuloplasty, and right atrial reduction. The repair is based on the construction of a monocusp valve by the use of the anterior leaflet of the tricuspid valve, which is usually enlarged in this anomaly. Not all patients are candidates for this procedure, however, because of significant abnormalities of the anterior leaflet such as inadequate size or attachment of the free edge of the leaflet to the ventricular wall. This report describes a ten-year experience with surgical repair of Ebstein's anomaly.     

Operative treatment of Ebstein's anomaly.

From April 1972 to February 12, 1991, 189 patients with Ebstein's anomaly underwent repair. Ages ranged from 11 months to 64 years (median 16 years, mean 19.1 years). In 58.2%, tricuspid valve reconstruction was possible, and in 36.5%, a prosthetic valve, usually a bioprosthesis, was inserted. In 5.3%, a modified Fontan or other procedure was performed. There were 12 hospital deaths (6.3%). All 28 patients who had accessory conduction pathways (Wolff-Parkinson-White syndrome) underwent successful ablation of the pathways as part of the operative treatment. Follow-up was obtained in 151 (85.3%) patients. Of those patients followed up more than 1 year after operation, 92.9% were in New York Heart Association class I or II. There were 10 late deaths: seven cardiac (four sudden), two noncardiac, and one of an unknown cause. Postoperative Doppler echocardiographic assessment showed the atrial septum was intact in all patients and tricuspid valve function was good to excellent in most patients. Four of the 110 patients (3.6%) who underwent valve reconstruction required reoperation 1.4 to 14.1 years later. Postoperative reduction in heart size was usual, atrial arrhythmias were reduced, and late postoperative exercise testing showed a significant improvement in performance: Maximal oxygen consumption increased from a mean of 47% of predicted value before the operation to a mean of 72% after the operation. Nine patients had a total of 12 successful pregnancies with deliveries of normal children.