Hemolysis following correction of double-outlet right ventricle.

A 41/2-year-old child developed a severe degree of intravascular hemolysis within 24 hours after intraventricular correction of double-outlet right ventricle with a Dacron patch. The child developed jaundice, with a serum bilirubin of 4.3 mg. per cent. The hematocrit value fell from 47 to 25 per cent and the reticulocyte count rose to 22 per cent. The hemolysis diminished after 3 weeks and completely disappeared after 6 weeks. We presume that the hemolysis was due to turbulence caused by a long, curved patch and that its disappearance coincided with the endothelialization of the patch. The child is well 3 years after the operation.     

改良Rastelli手术治疗右心室双出口合并左前位主动脉

目的 探讨右心室双出口合并左前位主动脉患者采用改良Rastelli的手术效果。方法 对10例右心室双出口患者采用改良Rastelli手术,9例为右心室双出口合并右前位主动脉,1位为镜面右位心型右心室双出口。除1例外,9例患者均有肺动脉瓣及瓣下狭窄。室间隙缺损位于肺动脉下或远离两大动脉。体外循环下行改良Rastelli手术,经右心室切口用涤纶片或人工血管片修补空间隔缺损,形态左心室至主动脉的内隧道。切断主肺动脉,闭合近端。用带瓣同种肺动脉或主动脉及人造血管,建立右心室流入道三关瓣环下方至主肺动脉的外管道。结果 10例患者术后恢复良好,超声心去科显示所有内隧道及外管道功能正常,无血流梗阻征象。结论 改良Rastelli手术是治疗右心室双出口合并左前位主动脉的满意术式,它可以彻底矫治肺动脉瓣及瓣下狭窄和右向左分流。     

Modified Rastelli procedure for double outlet right ventricle with left-malposition of the great arteries: report of 9 cases

BACKGROUND: Double outlet right ventricle with L-malposition of the great arteries is a rare type of double outlet right ventricle. This article reviews our experience in treating this disease surgically. METHODS: Between September 1995 and May 1999, 9 patients with double outlet right ventricle [S, D, L] underwent modified Rastelli procedure at Fu Wai Hospital. In all patients, the two great arteries originated completely from the right ventricle. The malformation was associated with subaortic conus and L-transposition of the great arteries. The ventricular septal defect (VSD) was subpulmonary in 3 patients, and non-committed in 6. All but one patient had either pulmonary valvular stenosis or subpulmonary stenosis. No patient had any type of palliative operation before. A right ventriculotomy was made to repair the VSD with a Dacron or vascular prosthesitic patch, and an intraventricular tunnel was made between the left ventricle and the aorta. The main pulmonary artery was divided and the proximal end was closed. A homograft conduit was implanted between the inlet of the right ventricle and the main pulmonary artery. In the Rastelli procedure, the conduit is usually positioned between the right ventricular outflow tract and the pulmonary artery. RESULTS: All patients survived and recovered uneventfully. Echocardiography demonstrated that all intraventricular tunnels and valved conduits were functioning well. The results were satisfactory. CONCLUSIONS: Modified Rastelli procedure is an optimal method for surgically treating double outlet right ventricle with left-malposition of the great arteries. It can completely correct the right ventricular outflow tract stenosis, and right to left shunt, and avoid injuring the right coronary artery.     

A case report: Rastelli operation for double outlet right ventricle associated with a chordae insertion to the infundibular septum]

A successful Rastelli operation for double outlet right ventricle (DORV) with a chordae insertion of the tricuspid valve to the infundibular septum was reported. A patient was a 6-year-old boy and the diagnosis was DORV, d-malposition of the aorta and pulmonary stenosis. The infundibular septum was not resected but mobilized with two incision, one anterior and vertical, and other one subaortic. This procedure allowed the construction of the tunnel similar to the closure of a large, subarterial VSD and of the straight unobstractive left outflow tract. The postoperative echocardiographic and angiographic examination revealed neither the pressure gradient in the left ventricular outflow tract nor the tricuspid regurgitation.     

Modified extended septoplasty for left ventricular outflow tract obstruction after repair of double-outlet right ventricle report of a case

A three-year-old boy with residual VSD and LVOTO after repair of double outlet right ventricle underwent modified extended septoplasty successfully. Preoperative left ventriculography showed a residual VSD and LVOTO. The peak systolic pressure gradient between the left ventricle and aorta was 38 mmHg. Operation aimd not only at relief of the obstruction, but also at streamlining of the LVOT by removal of protrusions. We modified the extended septoplasty reported by Belli et al in 1996. The postoperative course was uneventful and patient was discharged at 15 postoperative day. The most recent echocargiogram revealed no residual LVOTO.     

Superoinferior ventricular heart with situs inversus, levo-loop and dextro-malposition (I,L,D), and double-outlet right ventricle: a case report

A case of a 20-year-old man with superoinferior ventricular heart complicating situs inversus, levo-loop and dextro-malposition (I,L,D), and double-outlet right ventricle is described. The associated anomalies were hypoplastic right ventricle, ventricular septal defect, atrial septal defect secundum, infundibular and valvular pulmonary stenosis, and mitral regurgitation. Intraventricular conduit repair was successfully performed.     

Successful repair of double-outlet right ventricle by a modified Fontan operation

Patients having a double-outlet right ventricle with an unfavorable anatomy for reparative conduit procedure present a formidable surgical challenge. Three such patients, aged 10, 13, and 14 years, with double-outlet right ventricle associated with atrioventricular discordance in 2 and atrioventricular concordance in 1, levotransposition of the great vessels and dextrocardia. A large-diameter direct anastomosis of the right atrium to the pulmonary artery was performed after the closure of the atrioventricular and pulmonary valves. All patients were operated on successfully with good clinical and hemodynamic results. Postoperatively, all 3 were acyanotic and had an improved physical activity compared with their preoperative status. Mean cardiac index at rest was 3.23 L/min/m2 and was increased to 5.04 L/min/m2 during exercise. These results indicate that this procedure can be of value for patients with double-outlet right ventricle in whom reparative surgery is technically difficult or infeasible.     

Results of the Damus-Stansel-Kaye procedure for transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect

From 1975 through 1982, the Damus-Stansel-Kaye procedure was performed on 20 patients with complete transposition of the great arteries (TGA) and on 4 with double-outlet right ventricle (DORV) and subpulmonary ventricular septal defect (VSD). The patients ranged from 6 days to 20 years old (median age, 13 months). Associated anomalies included atrial septal defect (24 patients), VSD (14), and others (25). Thirteen patients had had palliative operations previously. Of the 14 hospital deaths (58%), 13 occurred among the 17 patients with one or more risk factors: age less than 18 months, weight less than 10 kg, and left ventricular peak systolic pressure less than 75% of systemic pressure. Follow-up ranged from 12 to 87 months (mean, 51 months). One patient died of cardiac failure two years postoperatively, and 2 required conduit replacement at 40 and 50 months because of stenosis. All 9 survivors are free from major symptoms. The Damus-Stansel-Kaye repair is most suitable for patients with TGA or DORV with subpulmonary VSD who are older than 18 months, weight more than 10 kg, and have a "prepared left ventricle," and whose coronary artery anatomy precludes transplantation.     

Myxoma of the free wall of the right ventricle: a case report

We present a 30-year-old female patient with a myxoma of the right ventricle, which was attached to the free wall of the right ventricle and was moving in and out the pulmonary valve causing right ventricular outflow tract obstruction. Myxomas of the right ventricle are rare and their surgical excision can be challenging especially if they infiltrate into important structures of the myocardium. They can be part of a broader category of diseases known as Carney complex with a familial predisposition.     

The role of the Fontan procedure in the surgical treatment of congenital heart malformations with double-outlet right ventricle

The Fontan procedure in one of its many modifications is applicable to the surgical treatment of those patients with double-outlet right ventricle who are not amenable to bi-ventricular correction. Included in this group are patients with significant hypoplasia of one of the ventricles, patients with distant (non-committed) ventricular septal defect, patients with significant straddling of the atrioventricular valves, patients with abnormal atrioventricular connections (such as absence of one of the valves), or common atrioventricular valve connection. The presence of complex associated lesions such as those seen in the heterotaxia syndromes may, by virtue of the surgical risk of attempts at bi-ventricular repair, make the Fontan approach a suitable option for these patients. The presence of a subaortic conus and the potential for subaortic obstruction, a frequent findings in patients with double outlet right ventricle, plays an important role in the preparation and conduct of the Fontan operation. Copyright © 2000 by W.B. Saunders Company     

A valved conduit replacement for a calcified homograft 12 years following a Rastelli operation: a case report

A 25 year-old male with transposition of great arteries, ventricular septal defect and pulmonary stenosis successfully underwent Rastelli procedure with aortic homograft in 1969. After the procedure he grew up and became an engineer without any complaints. However, gradually his homograft degenerated with calcification and the pressure gradient between pulmonary artery and right ventricle increased to 77 mmHg in systolic phase. Twelve years after the conduit repair, we operated upon and enlarged the ventricular septal defect, repaired the residual interventricular shunt, removed his homograft and, replaced it with the Hancock valved conduit from the right ventricular outflow tract through cardiopulmonary bypass. On postoperative cardiac catheterization, the pressure gradient decreased, and the patient returned to his work.     

Sequential combined spinal-epidural anesthesia for cesarean section in a woman with a double-outlet right ventricle

The number of women with complex cyanotic heart disease reaching childbearing age is continuously increasing. For anesthesiologists, management of this 'new' obstetric population is particularly challenging. We report the case of a parturient with a palliated double-outlet right ventricle, who underwent a cesarean section at 34 weeks with low-dose sequential combined spinal-epidural anesthesia with patient-controlled epidural postoperative analgesia. Anesthetic considerations and specific limitations of invasive monitoring are discussed, along with a review of recent literature on maternal and neonatal complications associated with pregnancies in women with cyanotic congenital heart disease.