The bidirectional cavopulmonary shunt.

The bidirectional cavopulmonary shunt improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance. Since 1983, 17 patients have undergone a cavopulmonary shunt procedure (five primary operations, 12 secondary operations). Diagnoses were single ventricle complex (n = 4), hypoplastic right heart syndrome (n = 10), and hypoplastic left ventricle (n = 3). Age at primary operation ranged from 3 1/2 to 30 months (median 6 months). Weight ranged from 3.5 to 9.7 kg. Age at secondary operation ranged from 10 months to 14 years (median 15 months). Seven cavopulmonary shunt operations were performed without cardiopulmonary bypass (six via thoracotomy and one via sternotomy) and 10, with cardiopulmonary bypass. All patients in the bypass group had additional procedures: takedown of modified Blalock-Taussig shunt, seven patients; revision of right ventricular outflow tract, four patients; reconstruction of pulmonary arteries, four patients; tricuspid valvuloplasty, one patient; and Damus procedure, one patient. There was one (1/17) operative death (Damus procedure). One patient required early revision. Follow-up ranges from 1 to 53 months (median 23 months). Twelve of 16 had a good to excellent late result, with a rise in mean arterial oxygen saturation from 69% to 83%. Three patients died late (4 to 53 months) (pulmonary vascular disease, pulmonary arteriovenous malformations, and pneumonia, one patient each). There was one late failure (converted to Glenn shunt). The cavopulmonary shunt is an excellent palliative procedure when right atrium-pulmonary artery connection (modified Fontan) must be deferred because of age, weight, or anatomic considerations. Five patients have undergone right atrium-pulmonary artery connection later. In addition, at the time of the modified Fontan operation, the cavopulmonary shunt approach may optimize the anatomic connection (eight additional patients).     

姑息手术在肺动脉闭锁合并室间隔缺损分期矫治中的应用

目的 总结改良Blalock-Taussig shunt(B-T分流术)、Waterston分流术和右心室肺动脉连接重建术3种不同姑息手术在肺动脉闭锁合并室间隔缺损((pulmonary atresia with ventricular septal defect,PAVSD)中的应用效果.方法 回顾性分析北京阜外心血...     

Anatomic correction for complete transposition and double-outlet right ventricle

Between February, 1981, and December, 1984, 30 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 8), transposition with ventricular septal defect (n = 15), and double-outlet right ventricle with subpulmonary ventricular septal defect, the Taussig-Bing anomaly (n = 7). At operation, ages ranged from 18 hours to 6 years (mean 11.3 months) and weights ranged from 2.6 to 16.4 kg (mean 6.1 kg). The group with transposition and intact ventricular septum on average was younger (mean 1.2 months) and smaller (mean 3.5 kg) than the other two groups. Associated congenital heart defects were seen in 12 patients, including five with coarctation, three with multiple ventricular septal defects, two with right ventricular hypoplasia, two with juxtaposed atrial appendages, and one each with interrupted aortic arch, Wolff-Parkinson-White syndrome, and left ventricular outflow tract obstruction. All 10 patients who had undergone prior palliative operations had pulmonary artery banding. In addition, four of these patients had coarctation repairs, four had atrial septectomy, and one had systemic/pulmonary shunting. All recognized patterns of coronary anatomy were encountered. The aorta and pulmonary artery were side by side in 14 patients and anteroposterior in 16 patients. The Lecompte maneuver to establish right ventricular-pulmonary arterial continuity was successfully used in 12 of 13 patients with anteroposterior great vessels but in none of those with side-by-side arteries. Seven patients had subvalvular right ventricular outflow tract obstruction, recognized either at operation (five) or postoperatively (two). This was responsible for death in three patients. The 30 day hospital mortalities were as follows: one death (12.5%) in the group with transposition and intact ventricular septum, six deaths (40%) in the group with transposition plus ventricular septal defect, and one death (14.3%) among patients with double-outlet right ventricle and subpulmonary ventricular septal defect; the overall mortality was eight deaths (26.7%). There have been no late deaths (mean follow-up 17.2 months). Ninety-five percent of the survivors are in New York Heart Association Functional Class I. Postoperative catheterization in 13 patients has shown normal left ventricular function, no coronary stenosis, and no aortic incompetence. Sixty-nine percent of these patients had clinically unsuspected gradients across the right ventricular outflow tract, which may be prevented by avoiding the Lecompte maneuver or the use of conduits.     

The use of internal and external vascular conduits for correction of D-transposition of the great arteries and double-outlet right ventricle with pulmonary atresia.

Between 1967 and 1978, an 11-year-old boy had undergone 3 separate systemic-pulmonary artery shunt operations. Two of the 3 shunt operations failed. The patient was restudied because of progressive cyanosis and was diagnosed as having D-transposition of the great arteries and double-outlet right ventricle with pulmonary atresia. Repair was successfully accomplished with the use of an internal vascular conduit and an external vascular conduit with a glutaraldehyde-treated porcine valve. Four months after the corrective procedure, the patient's exercise tolerance had improved dramatically.     

Long-term results after Fontan procedure and its modifications

The Fontan procedure is an effective method of treatment for patients with tricuspid atresia, univentricular heart, and other complex lesions. Modifications of the Fontan procedure have been developed to treat various anatomic malformations. From 1975 to 1984, 31 patients (17 male and 14 female) have undergone 35 Fontan operations. The youngest patient was 4 and the oldest 26 years of age (average 12.2 years). Sixteen patients had tricuspid atresia, and 15 univentricular heart. Twenty-three patients had undergone 37 palliative operations before the Fontan procedure. The original Fontan procedure was performed in 10 patients until 1977, and there were three early deaths (30%). After that time, the operative technique was modified and the early mortality dropped to 12% (3/25 patients). A valved prosthetic conduit was used in 11 patients (right atrium to pulmonary artery in five; right atrium to right ventricle in six), a valveless conduit in 14 patients (right atrium to pulmonary artery in nine; right atrium to right ventricle in five), and direct anastomosis (right atrium to pulmonary artery) in 10 patients. Postoperative cardiac catheterization was performed in 20 patients and revealed excellent results in 15. The remaining five had reduced oxygen saturation; three of them had had a Glenn anastomosis 8 and 10 years before the Fontan procedure, and one had an anomaly of the coronary sinus with a shunt to the left atrium. This patient required reoperation. Three other patients underwent reoperation because of calcified stenotic and/or outgrown conduits. Postoperative mean right atrial pressure varied from 10 to 20 mm Hg. The hemodynamic ventricular parameters were within the normal range. We conclude that in patients with tricuspid atresia, univentricular heart, and other complex cardiac lesions, inactivating the right ventricular pump function by means of the Fontan procedure does not adversely affect the systemic ventricle. Although the response to exercise was abnormal, the clinical condition of these patients was good to excellent.     

Pulmonary artery configuration after palliative operations for hypoplastic left heart syndrome

Pulmonary artery architecture and symmetry after palliative operations for hypoplastic left heart syndrome may affect subsequent suitability for a modified Fontan operation. Two-dimensional echocardiography was used to measure pulmonary artery diameter and assess symmetry after two types of systemic-pulmonary artery shunts: modified right Blalock-Taussig shunt (14 patients) and central shunt (from underside of aortic arch gusset to pulmonary artery confluence) (14 patients). Age, weight, preoperative diameter of right and left pulmonary arteries (proximal, middle, and distal segments), and mean interval between preoperative and postoperative echocardiographic studies (20.2 +/- 4.4 days in the Blalock shunt group; 19.1 +/- 6.8 days in the central shunt group) were similar. Early postoperatively, patients with a Blalock shunt showed a significant decrease in the diameter of all pulmonary artery segments except the distal right pulmonary artery. The diameters tapered from distal right to distal left pulmonary artery in this group. Patients with the central shunt had a significant decrease in the diameter of all pulmonary artery segments. There were no significant differences when cross comparisons were made of the various pulmonary arterial segments in patients after a central shunt. Similar findings persisted in 19 patients from both groups who had a late postoperative echocardiogram (mean interval between studies = 271 days in the group of 10 patients with Blalock shunt and 167 days in the group of nine patients with a central shunt). In conclusion, the central shunt preserves pulmonary artery symmetry, which may be important in candidates for the Fontan operation in infancy.     

Palliative right ventricle outflow reconstruction in tetralogy of Fallot with pulmonary atresia and hypoplastic pulmonary artery

The management of small pulmonary artery in tetralogy of Fallot (TOF) with pulmonary atresia (PA) is complicated. Our strategy is palliative right ventricle outflow tract reconstruction (RVOTR). The aim of this study is to determine whether palliative RVOTR is useful as the first stage operation. Since 1994, 7 patients with TOF, pulmonary atresia and hypoplastic pulmonary arteries underwent palliative RVOTR. All patients survived operation without complication except for 1. He had pseudoaneurysm at right ventricular outflow patch requiring aneurysmectomy and re-RVOTR 1.5 months after the initial RVOTR. Nine months after RVOTR in the average, pulmonary artery index (PAI) increased from 139 +/- 87 to 306 +/- 156 (p < 0.05). No patient had pulmonary high flow or pulmonary hypertension. Two of them had pulmonary coractation due to ductal tissue in the pulmonary artery, necessitating additional modified Blalock-Taussig shunts at 8 and 10 months old. One patient with TOF, PA, major aorto-pulmonary collateral artery (MAPCA) had corrective surgery after 2 additional operations and interventional catheterization. Five patients of this series already have undergone corrective surgery, whereas 1 is waiting for it. RVOTR is a useful method for pulmonary artery growth in patients with TOF, PA and diminutive pulmonary artery.     

Staged Approach for Congenitally Corrected Transposition of the Great Arteries Associated with Dextrocardia and Nonconfluent Pulmonary Artery

Abstract   We completed double switch operation (Senning plus Rastelli procedure; SLL) after staged palliative operations for SLL, dextrocardia, congenitally corrected transposition of the great arteries ventricular septal defect, atrial septal defect, and nonconfluent pulmonary artery. The patient had undergone three preparatory operations including central pulmonary artery reconstruction with autologous pericardium and morphologic right ventricle-pulmonary artery shunt and then double switch operation was performed. During the double switch operation, the previous conduit which was established as right ventricle-pulmonary artery shunt in the third palliation was replaced by a larger conduit. This conduit was used as Rastelli external conduit to avoid coronary artery injury. When systemic venous chamber was reconstructed, suture line pouch technique was used, in which dog ears were made at the sites of superior and inferior vena cava orifices, respectively, to prevent systemic venous return obstruction. Pulmonary venous chamber was reconstructed without any augmentation, which reduced the possibility of postoperative arrhythmia. Systemic and pulmonary venous pathway obstructions were not observed after the operation. Postoperative arrhythmia was not detected.     

Lessons learned in pediatric heart transplantation

Between February 1, 1982, and June 30, 1988, 32 children underwent cardiac transplantation for treatment of congenital heart disease (10) and other cardiomyopathies (22). The 6-year actuarial survival was a disappointing 36% because of a high perioperative mortality (12 of 32, 37.5%) and because of five late deaths due to uncontrolable rejection. The perioperative mortality was a staggering 60% (6 of 10) for the patients with congenital heart disease. Four of the 6 recipients with congenital heart disease died because of acute failure of the donor right ventricle. This included 2 patients who required reconstruction of the pulmonary arteries for stenosis secondary to previous systemic-to-pulmonary shunts and 2 others in whom the pulmonary vascular resistances were underestimated because of undetected recent pulmonary emboli (1) and complicated pulmonary vascular anatomy (1). Five of the six late deaths were due to rejection-related events, and all were patients with acquired cardiomyopathy.     

Rudimentary right ventricle to pulmonary artery shunt in the Norwood procedure

We describe here successful palliative repair of tricuspid atresia, hypoplastic right ventricle, transposition of the great arteries, and hypoplastic aortic arch in a neonate. The repair consisted of the Norwood procedure with a rudimentary right ventricle to pulmonary artery shunt, which was located on the right side of a neo-aorta. This procedure could be a useful adjunct to avoid left ventriculotomy and its subsequent dysfunction.     

Modified Fontan operation for a single ventricle with a nonconfluent pulmonary artery]

A 8-year-old boy with a double inlet right ventricle with a non-confluent pulmonary artery was operated on with a modified Fontan operation. He had right isomerism, right aortic arch, bilateral superior caval veins, and left-sided inferior caval vein. Hepatic veins were separately drained to the right-side atrium. Left Blalock-Taussig shunt and right central shunt operations had been previously performed. Firstly, we had reconstructed the central pulmonary artery with a 16 mm porcine pericardial roll to unify the nonconfluent pulmonary arteries. Secondly, about 2 months after the first operation, we performed a modified Fontan operation. Systemic venous return from the inferior caval vein and the hepatic veins were drained to a reconstructed pericardial roll with an intraatrial Gore-Tex graft, and bilateral superior caval veins were also anastomosed to the roll. The structure of the pulmonary arterial system is one of the most important factors to determine the outcome of a modified Fontan operation. Even if the central pulmonary artery is absent, however, a modified Fontan operation is applicable for the patient whose peripheral pulmonary arteries have enough growth.     

法洛四联症合并肺动脉闭锁的手术治疗

目的 探讨法洛四联症合并肺动脉闭锁(TOF-PA)的手术适应证、手术方法和效果.方法 1984年6月至2009年6月对66例TOF-PA患者进行了外科手术69次.其中男34例,女32例;年龄6个月～29岁.包括Ⅰ型31例,Ⅱ型14例,Ⅲ型12例,Ⅳ型9例.11例行姑息性分流术;2例行一期单源化和室间隔缺损开放;40例行一期修复术,13例行一期单源化和心内修复术.3例分流术后再次心内修复术.结果术后早期死亡6例,其中修复手术后死亡5例,分别为严重低心排血量综合征3例,灌注肺1例,多脏器功能衰竭1例;分流术后死亡1例,为切口感染、心内膜炎.47例随访3个月～15.5年,NYHA心功能Ⅰ-Ⅱ级44例,Ⅲ-Ⅳ级3例.结论 TOF-PA肺血管的解剖学和形态学特点是决定手术适应证和方法的重要指标,对于存在自体肺动脉并且周围肺动脉发育较好的TOF-PA,尽可能早期进行一期矫治手术.     

Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

OBJECTIVE: To assess the results of a staged surgical approach for tetralogy of Fallot with pulmonary atresia, hypoplastic or absent pulmonary arteries, and major aortopulmonary collateral arteries. METHODS: We retrospectively reviewed a consecutive series of these patients from a single institution. RESULTS: From July 1993 to April 2001, 46 consecutive patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries were treated with staged surgical repair. The operative sequence usually began with a central aortopulmonary shunt followed by unifocalization of aortopulmonary collateral arteries depending on the source and distribution of pulmonary blood flow. Twenty-eight patients (61%) subsequently underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery connection. Those patients who underwent complete repair had a median of 3 total operations (range 1-6). The ratio of the mean pulmonary artery pressure to the mean systemic blood pressure at the time of complete repair was 0.36 (range 0.19-0.58). Two of the 28 repaired patients (7.1%) required subsequent fenestration of the ventricular septal defect closure due to later development of supersystemic right ventricular pressure and right ventricular failure. Eighteen patients (39%) have undergone 1 or more staging operations and are considered good candidates for eventual complete repair. There were no hospital deaths. There was 1 late death (2.2%; 95% CI 0.4-11.3%) in a patient born prematurely who developed severe bronchopulmonary dysplasia precluding complete repair. CONCLUSIONS: For tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, a staged surgical approach yields low overall mortality and acceptable hemodynamics after complete repair.     

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.     

Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum includes 26 patients with pulmonary atresia and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates managed initially by transventricular valvotomy; 6 later required a secondary procedure, with 100% survival. Group 2 had 11 neonates managed by aorta-pulmonary artery shunting without operative death. However, only 3 have survived over the long term and 1 has required an additional shunt procedure. Group 3 had 9 infants who underwent concomitant valvotomy and shunting. There were 4 operative deaths and 1 late death. Finally, Group 4 included 2 infants managed by primary repair at 3 days and 6 days old with prosthetic enlargement of the right ventricle; 1 required the addition of a shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who survived neonatal palliative procedures have undergone reparative operations. Two had no growth of the right ventricle and underwent repair after conversion to tricuspid atresia, by a Fontan procedure. Five had prosthetic enlargement of the right ventricle in childhood with 1 late death. Findings of this review were as follows: (1) effective palliation of pulmonary atresia and intact ventricular septum or critical pulmonary stenosis with cavitary hypoplasia of the right ventricle is rare unless transventricular flow can be established; (2) establishment of transventricular flow produces a high incidence of cavitary "growth," which permits later repair; (3) the Fontan operation is available for repair in patients who have no cavitary growth; and (4) when all three portions of the right ventricular cavity can be identified by angiography, a primary repair can be performed in the neonatal period with a good long-term prognosis.     

57例婴幼儿复杂心脏畸形的外科治疗

报告１９９１年９月至１９９４年８月期间收治的５７例婴幼儿复杂先天性心脏病，包括右室双出口１６例、完全性大动脉转位８例、完全性心内膜垫缺损７例、完全性肺静脉畸形引流６例、肺动脉闭锁４例、单心房４例、三尖瓣闭锁和主动脉弓中断各３例、左冠状动脉右室瘘２例、单心室和镰刀综合征各１例及其他复杂畸形２例。６例行姑息性手术，手术早期死亡１例（１６．７％）；５１例行一期矫治术，手术早期死亡９例（１７．６％）。总手术早期死亡为１７．５％，最近２年早期死亡率为１２．５％。主要死亡原因为肾功能衰竭和低心输出量综合征。     

The long-term clinical outcome of patients with tricuspid atresia. II. Influence of surgical procedures.

One-hundred and eleven patients with tricuspid atresia were seen during 1972-1982 and thirteen of these were not operated upon. The remaining 98 patients have undergone at least one operation. Those with low pulmonary blood flow had 1-4 shunt procedures; in 15 patients with high pulmonary blood flow the pulmonary artery was banded. The mortality rate after the first shunt was 10% (8/82), 9% after the second shunt (3/32) and 12% after the third and fourth shunts. Three out of 15 (20%) patients died after pulmonary artery banding. Twenty three patients underwent a Fontan operation and five died (22%); eighteen survivors are clinically well (after 1-7 years). One patient required a pacemaker implantation and revision of his Fontan procedure. The survivors of the palliative operations and the unoperated patients were more restricted, the older ones having developed pulmonary vascular obstructive disease, chronic heart failure or arrhythmias.     

External compression of bronchus by aneurysm from divided major aortopulmonary collateral artery after unifocalization.

The right ventricle to pulmonary artery connection with an extracardiac conduit, left pulmonary artery reconstruction, ligation of patent ductus arteriosus, and take-down of right Blalock--Taussig shunt were performed on a 1-year-8-month-old boy who had pulmonary atresia, ventricular septal defect, patent ductus arteriosus, and major aortopulmonary collateral arteries. He previously underwent the unifocalization and right modified Blalock--Taussig shunt at 9 months of age. He repeatedly had a difficulty in weaning from the mechanical ventilator. After removing the aneurysm from the divided major aortopulmonary collateral artery that compressed the left main bronchus externally, it was possible to wean him from the mechanical ventilator.     

Pulmonary atresia with intact ventricular septum

Neonates with pulmonary atresia with intact ventricular septum are ductus dependent from birth. The initial approach in these patients is to ensure continued adequate pulmonary blood flow in anticipation of imminent spontaneous closure of the ductus. Our experience and evolving approach in the management of 16 consecutive neonates with pulmonary atresia with intact ventricular septum from 1978 to 1984 is presented. On the basis of the revised classification by Bull and associates, we divided the patients into four different groups according to right ventricular morphology. Basically, three types of palliative surgical procedures were performed: systemic-pulmonary artery shunt; systemic-pulmonary artery shunt with pulmonary valvotomy; and pulmonary valvotomy alone. Systemic-pulmonary artery shunt is the most important part of neonatal palliation. Our present routine is to perform left subclavian-main pulmonary artery shunt with a polytetrafluoroethylene tube in all patients and, in addition, to perform an open transpulmonary valvotomy without cardiopulmonary bypass in patients with a patent infundibular portion of the right ventricle. Our technique and the advantages of this type of shunt procedure are discussed. Patients with right ventricular sinusoidal-coronary artery communications are a subgroup with an additional problem. In our series, patients with this anomaly are categorized into groups with major and minor connections and their surgical significance is discussed. Overall, there was one death among 16 patients after neonatal palliative procedures and one death among five patients after hemodynamic repair.     

Comparison of hemodynamics between Norwood procedure and systemic-to-pulmonary artery shunt for single right ventricle patients

Objective: Despite that surgical outcomes of patients with hypoplastic left heart syndrome have improved, one of the problems remaining is the high interstage mortality after a stage I Norwood procedure. The purpose of this study was to determine the hemodynamic characteristics of hypoplastic left heart syndrome after a Norwood procedure. We examined the perioperative hemodynamic differences of the staged operation between the first stage of the Norwood procedure and systemic pulmonary shunt for single right ventricle patients. Methods: Data from 39 patients who underwent a Norwood procedure (right ventricle to pulmonary artery conduit: 19, Blalock–Taussig shunt, 20) were analyzed. There were nine early and seven interstage deaths. Bidirectional cavopulmonary shunt was performed in 15 patients and the Fontan procedure in 9 (group H). We defined the control group as 26 patients who underwent the first stage of a systemic pulmonary shunt for a single ventricle. Bidirectional cavopulmonary shunt was performed in 14 patients and the Fontan procedure in 8 (group C). We compared the perioperative hemodynamics of the staged operation between the two groups. Results: Cardiothoracic ratio and single ventricular diastolic dimension before bidirectional cavopulmonary shunt were acutely increased in group H (P=0.02, <0.001). There was no significant difference between the two different types of Norwood procedures. The pulmonary artery index for the right heart bypass operation was lower in group H than in group C (P<0.001). Oxygen saturation before bidirectional cavopulmonary shunt in group H decreased (P<0.001) and thus was lower than that in group C (P=0.003). Mortality and the postoperative clinical parameters of the right heart bypass operation were not different between the two groups. Conclusions: Patients with hypoplastic left heart syndrome showed hemodynamic instability of acutely increased cardiothoracic ratio, and single ventricular diastolic dimension despite decreased oxygen saturation interstage after stage I of a Norwood procedure. This suggests that this hemodynamic characteristics in hypoplastic left heart syndrome correlates with the higher mortality before second stage palliation than in found with single right ventricle patients.