Stage I bilateral pulmonary artery banding maintains systemic flow by prostaglandin E1 infusion or a main pulmonary artery to the descending aorta shunt for hypoplastic left heart syndrome

Since 2002, we have performed bilateral pulmonary artery banding for stage I palliation and maintained systemic flow by prostaglandin E1 infusion or a main pulmonary artery to the descending aorta shunt, and here report our experience. Three of the 4 patients were diagnosed with aortic atresia/mitral atresia and 1 with aortic stenosis/mitral stenosis. Balloon atrial septostomy was performed in 2 before stage I. Bilateral pulmonary artery banding (right circumference: 10 or 14, left circumference: 10.5 to 14 mm) was performed from 7 to 19 days after birth. Systemic flow was maintained by prostaglandin E1 infusion in 2 patients and a Van Praagh procedure was performed in the other 2. Balloon atrial septostomy was required in 2 patients, and an atrial septal defect enlargement was in one during the interstage before stage II palliation, which was performed at ages 3 to 9 months. Bidirectional cavopulmonary shunt with aortic arch and coronary flow reconstruction was also performed. For patients younger than 4 months, we do not require pulmonary arterioplasty in stage II. All patients are alive and well and waiting for Fontan completion. Excellent early results were obtained for this surgical strategy that avoids the stage I Norwood palliation.     

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

BACKGROUND: Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. METHODS: A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. RESULTS: Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end-diastolic volume significantly increased but tricuspid valve diameter did not change. CONCLUSIONS: The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.     

Functional total anomalous pulmonary venous drainage by left atrium-to-superior vena cava shunt

Total anomalous pulmonary venous connection is occasionally associated with a functional single ventricle and is thought to be a risk for the staged Fontan approach. We report a patient with a very rare presentation of mitral atresia and intact atrial septum with physiologically total anomalous pulmonary venous drainage by a connecting vein between the left atrium and the superior vena cava. A surgical atrioseptectomy as a functional repair of total anomalous pulmonary venous connection and pulmonary artery banding was successfully performed as the first palliation for Fontan completion.     

Definitive repair in patients with pulmonary atresia and intact ventricular septum

Between 1977 and 1988, 22 patients underwent definitive repair for pulmonary atresia with intact ventricular septum. Fifteen underwent biventricular repair (mean age 24 months). All had mild to moderate right ventricular hypoplasia at the time of definitive repair. Repair consisted of closure of the atrial septal defect with enlargement of the right ventricular cavity and outflow tract with a patch in eight, insertion of a valved homograft in three, and superior vena cava-pulmonary artery connection in four. There was one operative death (7%). Seven patients had severe right ventricular hypoplasia at the time of definitive repair and underwent a Fontan procedure (mean age 46 months). In two a valved connection was made to the right ventricle and in five a right atrial-pulmonary artery nonvalved connection. There were two operative deaths (29%). Three patients had right ventricular-coronary sinusoids: Two of them underwent a Fontan procedure and one a biventricular repair. We conclude that with adequate early palliation a biventricular repair may be successfully performed for patients with mild or moderate right ventricular hypoplasia, and the Fontan procedure may be used for those with severe right ventricular hypoplasia.     

Tricuspid Atresia with Dextrotransposition,Dextrocardia, and Mitral Insufficiency: Successful Circulatory Correction

A 13-year-old patient with visceral and atrial situs solitus, dextrocardia, dextro (d) transposition of the great vessels with subaortic conus, tricuspid atresia, massive mitral valve insufficiency, and previously performed pulmonary artery banding was seen at our hospital. A right atrial to pulmonary artery conduit procedure and mitral valve replacement successfully repaired this child's circulation.     

分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的 总结分期全腔静脉.肺动脉连接术(TCPC)治疗复杂先天性心脏病的经验.方法 1998年6月至2008年3月,22例先天性心脏病复杂畸形患者接受分期TCPC.本组中单心室合并肺动脉狄窄9例,合并肺动脉闭锁3例;完全性大动脉转位,十字交叉心,肺动脉瓣狭窄1例;完全性房室通道,左心窒发育不良,肺动脉闭锁,房室瓣少-中量反流1例;完全性房室通道,动脉导管未闭,肺动脉狭窄,双向Glenn术后伴吻合口狭窄房室瓣大量反流1例;镜面右位心,功能性单心室,肺动脉闭锁,动脉导管未闭,体肺侧支,双侧双向Glenn术后2年,右下肺动-静脉瘘1例;三尖瓣闭锁并肺动脉狭窄4例;三尖瓣闭锁伴肺动脉闭锁,动脉导管未闭1例;镜面右位心,右心室双出口,窒间隔缺损,左心室发育小良,功能性单心房,肺动脉瓣狭窄,三尖瓣少量反流,粗大体肺侧支1例.第一期体肺分流术后,二期双向Glenn术5例,第一期单侧或双侧双向Glenn手术17例.行双向Glenn手术的年龄为(5.9±4.4)岁,Glenn术前肺动脉压为17～20 mm Hg(1 mm Hg=0.133 kPa);房室瓣膜关闭不全3例;一期手术前Nakata指数小于200 mm2/m2者4例.接受TCPC术平均年龄为(9.6±4.9)岁,与双向Glenn术间隔时间(3.7 ±1.2)年,术前经上腔静脉置管测得肺动脉压力均小于15 mm Hg.全组22例均采用心外管道TCPC,房窜瓣成形1例.结果 全组住院死亡1例,病死率为4.5%.死亡病例为单心室,肺动脉闭锁,第一期体肺分流术后,二期左肺动脉成形双向Glenn术后5年第三期行TCPC,术后左肺反复多次大出血死亡.余患者均顺利出院,术后中心静脉压12～18 mm Hg.出院前经皮测血氧饱和度为90%～96%,活动量明显增加,发绀及气促症状消失,心功能均为NYHA分级Ⅰ～Ⅱ级.结论 对于高危患者根据病情选择分期TCPC,可以使更多有高危因素不能行Fontan手术的患者得到救治的机会,扩大手术适应证,并取得满意疗效.     

A successful two-staged Fontan procedure for tricuspid atresia with bilateral superior venae cavae and left pulmonary arterial stenosis]

A 10-year-old boy with tricuspid atresia (type I-b), bilateral superior venae cavae and left pulmonary arterial branch stenosis was treated successfully by a two-staged Fontan procedure. At 7 years of age, left Glenn shunt was performed after creation of a communicating vessel between the right and left superior vena cava, using a GORE-TEX graft of 10 mm of diameter. Three years later, after having confirmed graft patency, Fontan procedure was achieved without any problems. We think the innominate vein creation by an artificial material was a key design for the following successful two-staged Fontan procedure in this case.     

Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow

Objective: The infant with a functional single ventricle (SV) and unobstructed pulmonary blood flow (UPBF) requires early protection of the pulmonary vascular bed to ensure suitability for a subsequent Fontan procedure. Systemic obstruction by aortic arch obstruction, subaortic stenosis, or combination of both, has been widely recognized as an important risk factor for poor outcome in children with SV–UPBF who are palliated with pulmonary artery banding (PAB). We reviewed our experience with primary PAB in the subset of patients with SV–UPBF to identify risk factors for subsequent palliative procedures and Fontan completion. Methods: Between January 1990 and May 2004, 80 patients (median age, 14 days) with functional SV and UPBF underwent PAB as their primary palliative procedure. Thirty-five neonates had concomitant aortic coarctation or interrupted aortic arch repair (44%). A Damus–Kaye–Stansel procedure was subsequently performed in 19 patients, and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in five. Results: There were 4 operative deaths, and 15 late deaths. The actuarial overall survival is 84% at 1 year, 76% at 5 and 15 years. Follow-up is complete in all but six children at a mean interval of 4.9±3.7 years (range, 2 months–15 years). Thirty-seven patients (49%; 37 of 76) have undergone the hemi-Fontan procedure (with three hospital deaths) and 40 patients (53%; 40 of 76; 12 children without previous hemi-Fontan) have undergone the completion Fontan procedure without mortality or Fontan takedown. Conclusion: In infants with single ventricle physiology with or without systemic outflow obstruction and unobstructed pulmonary blood flow, a strategy of pulmonary artery banding carries acceptable operative and mid-term mortality in a high-risk group of patients. Pulmonary artery banding does not compromise performance of subsequent Damus–Kaye–Stansel procedure or completion Fontan palliation.     

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.     

Outcomes of tricuspid atresia in the Fontan era

BACKGROUND: Whereas indications expand and results improve with increasing refinements to the Fontan procedure the overall impact on outcomes related to tricuspid atresia remains suboptimally defined. METHODS: We reviewed 225 consecutive patients presenting between 1971 and 1999. All patients had classic tricuspid atresia with absent right atrioventricular connection and with D-transposition of the great arteries in 21%, pulmonary outflow obstruction in 75%, and aortic outflow obstruction in 11%. RESULTS: Ten patients died before any intervention and 3 patients were lost to follow-up. Palliative procedures (includes 151 with systemic shunt, 27 pulmonary artery banding, 60 venous shunt) were performed in 203 patients, with 44 deaths, 8 patients awaiting Fontan, 12 patients Fontan contraindicated, and 11 patients lost-to-follow-up. A total of 137 patients had the Fontan procedure (9 patients without previous procedures) with 7 early deaths, 11 late deaths, and 3 patients progressing to heart transplantation. Total survival for the cohort was 90% at the age of 1 month, 81% at 1 year, 70% at 10 years, and 60% at 20 years with no significant change over the time period. Independent factors associated with ineligibility or death without Fontan (n = 68, 30%) included earlier birth date, lower birth weight, presence of aortic arch anomaly and greater right ventricular hypoplasia, and no palliative procedure. There were no significant changes in mortality with Fontan over the study time period with survival of 95% at 1 month, 93% at 1year, and 82% at 10 years. CONCLUSIONS: Improvements in outcomes with tricuspid atresia will require attention to management and risk factors before Fontan.     

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.     

Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum.

Twelve children with pulmonary atresia and intact ventricular septum underwent closure of the tricuspid valve as a part of a new surgical procedure. In two cases a concomitant Fontan operation was performed. In each patient the right ventricle was very small and right ventricular pressure was higher than systemic pressure. Ventricle-coronary connections provided flow of desaturated blood from the right ventricle into the coronary arteries in 11 of 12 cases. Five of the 12 children did not survive operation and postmortem examination of each revealed severe acute and chronic myocardial ischemic damage and high-grade obstruction or interruption of the proximal left anterior descending coronary artery. Preoperative angiography demonstrated occlusive changes in the coronary arteries, resulting in right ventricular dependent circulation, in all five children who died and in one child who survived operation. Seven children who survived operation are well 4 months to 3.5 years later. Two have undergone subsequent successful Fontan operation and two others are considered suitable candidates for this operation. Tricuspid valve closure is recommended for a carefully selected group of infants with pulmonary atresia and intact ventricular septum provided a right ventricular-dependent coronary circulation can be excluded on the basis of preoperative coronary cineangiography.     

Absorbable pulmonary artery banding in tricuspid atresia

A child with tricuspid atresia, concordant ventriculoarterial connections, large ventricular septal defect, and elevated pulmonary artery pressure underwent pulmonary artery banding with a polydioxanone ribbon. This procedure was successful in this patient as the ventricular septal defect became restrictive while the banding was fully absorbed after 5 months. This technique could be included in the panel of surgical strategies for patients with single ventricle physiology and potential but insufficient subpulmonary stenosis in early infancy.     

Definitive operation for pulmonary atresia with intact ventricular septum. Results in twenty patients

Definitive operation was performed on 20 patients (aged 25 to 178 months) with pulmonary atresia and intact ventricular septum. All patients had one or more prior preliminary palliative procedures. Right ventricular outflow tract reconstruction with atrial septal defect closure and shunt removal was done on 10 patients. Tricuspid annular circumference was at least 70% of normal in seven patients and between 55% and 70% in three patients. Two patients died during hospitalization. The eight surviving patients were asymptomatic 3 to 145 months after operation. The modified Fontan operation was performed on 10 patients. None of these patients had a tricuspid annular circumference greater than 70% of normal; the circumference was less than 55% in nine patients and between 55% and 70% in one patient. One patient died during hospitalization and one died later. Follow-up 6 to 48 months after operation showed that six patients were in the New York Heart Association Class I and two were in Class II. After effective preliminary palliation of pulmonary atresia with intact ventricular septum, definitive operation can be done with an operative risk of 15% (three of 20 patients) and excellent late results. Right ventricular outflow tract reconstruction can be done as a complete repair for patients who have adequate tricuspid annular size. The modified Fontan operation is the only option for definitive repair when the tricuspid anulus is severely hypoplastic.     

Surgical treatment of tricuspid atresia

Despite increasing experience with the surgical treatment of tricuspid atresia, controversy exists regarding the early and late results of the various surgical options. Between January 1, 1967, and September 1, 1982, 92 patients underwent 147 operations for tricuspid atresia. Eighty-five patients underwent 1 or more palliative operations (108 procedures), with 8 hospital deaths (9%; confidence limits [CL], 6 to 14%). Thirty-eight patients underwent 44 classic (Blalock-Taussig or Gore-Tex) shunts, with 3 early (7%; CL, 3 to 13%) and 3 late deaths. Actuarial survival at 5 years was 78%, but only 56% were alive and free from having to undergo further operation at 5 years. Thirty-seven patients underwent a Fontan procedure, with 5 hospital deaths (14%; CL, 8 to 22%). Of these 37 patients, 34 (92%) had a nonvalved connection between the right atrium and the right ventricular infundibulum or the pulmonary artery. Incremental risk factors for hospital mortality after the Fontan procedure in patients with tricuspid atresia as well as other cardiac anomalies include young age (p = 0.0003), diagnosis other than tricuspid atresia (p = 0.03), and complex associated procedures (p = 0.02). During the year 1983, hospital mortality was 7% (1 out of 14; CL, 1 to 22%) for the Fontan procedure without complex additional procedures. Actuarial survival after a Fontan procedure was 71% at 5 years, with 3 patients requiring late reoperation. Of the survivors, 96% are in New York Heart Association Functional Class I or II. The Fontan procedure without a valve offers good intermediate survival, good functional results, and few reoperations. In patients with diminished pulmonary blood flow, a classic shunt also provides good palliation and good intermediate survival, but there is a necessity for additional operations in many patients within 5 years.     

Single ventricle (single- or double-inlet) complicated by subaortic stenosis: surgical options in infancy

Intracardiac obstruction to the systemic circulation can develop in patients with many forms of congenital heart disease. When transposition of the great arteries accompanies tricuspid atresia, narrowing of the ventricular septal defect (VSD) leads to subaortic stenosis. In a similar fashion, a restrictive bulboventricular foramen compromises systemic arterial outflow in patients who have single left ventricle with subaortic outflow chamber. The same effect can be seen in VSD closure in mitral atresia with normally related great vessels. Although some forms of single ventricle can be treated by ventricular septation, the modified Fontan procedure can be more generally applied to this entity. Pulmonary vascular resistance must be low, however, which excludes the application of the Fontan principle in infants. While pulmonary artery banding may protect the lungs from the development of excessive pulmonary vascular resistance, it may also accelerate the development of subaortic obstruction. We have successfully applied the Norwood operation, as described for hypoplastic left heart syndrome, to palliate subaortic stenosis due to restrictive VSD in 3 infants with single ventricle or atrioventricular valve atresia. Consideration should be given to a primary Norwood procedure in infants with single ventricle in whom subaortic stenosis is likely to develop. Patients who do receive pulmonary artery bands should be followed closely for the development of subaortic stenosis and should undergo an early, physiologically corrective operation if it occurs.     

Results of systemic-to-pulmonary artery anastomosis for tricuspid atresia with reduced pulmonary blood flow.

Fifty-six patients with tricuspid atresia and decreased pulmonary blood flow received a systemic-to-pulmonary artery anastomosis as a preliminary operation. Thirty-five had a Waterston shunt, 12 a Blalock-Taussig anastomosis, and nine various other procedures. The age at operation ranged from 2 days to 10 years (median 4.5 months). Pulmonary atresia was present in eight newborn infants. Four patients (7%), all less than 3 months old, died in hospital from persistent servere hypoxia despite the palliative procedure. By actuarial methods, 93% of the survivors were alive at and after one year (longest follow-up:9.8 years), while 83% had not required further palliation at and after four years from the first operation. None of the patients subsequently restudied had raised pulmonary pressure or resistances, and in most of them the pulmonary arterial tree was deemed adequate for the Fontan operation.     

Repair of pulmonary valve insufficiency using an autologous monocusp

A 3 1/2-year-old boy experienced right ventricular failure approximately two years after an operation for critical pulmonic stenosis. Severe pulmonary and tricuspid valvular insufficiency was documented echocardiographically and at cardiac catheterization. Treatment consisted of a tricuspid valve annuloplasty and creation of an autologous monocusp valve using the anterior wall of the pulmonary artery. The procedure was well tolerated, and early competence of the pulmonary valve was shown intraoperatively by pressure recordings and postoperatively by Doppler echocardiography. However, at recatheterization one year later, there was no evidence that the monocusp valve was functioning. This experience does not support the prior successful application of this technique in animals.     

Fontan operation for tricuspid atresia with dysplasia of the right ventricular myocardium and absence of pulmonary valve

We report a case of successful staged repair of tricuspid atresia with dysplasia of the right ventricular myocardium and absence of the pulmonary valve. The patient underwent the modified Blalock-Taussig shunt operation at 1 month the bidirectional Glenn procedure at 11 months, and the total cavopulmonary connection procedure using extracardiac graft at 2 years. It is important to prevent the left ventricular outflow tract obstruction (LVOTO) caused by the non-functioning right ventricle when the Fontan completion. We were safely able to disconnect the pulmonary trunk from the right ventricle using the transesophageal echocardiography to evaluate the LVOTO during operation.     

体外循环心脏不停跳下心外管道Fontan手术

目的评估体外循环心脏不停跳下心外管道Fontan手术的方法及其效果。方法本组42例中,男性31例、女性11例,年龄3～19岁,其中心室双人口19例,三尖瓣闭锁10例,二尖瓣闭锁3例,其他复杂先天性心脏病10例。均在常温体外循环心脏不停跳下施行心外管道Fontan手术,其中8例加行开窗术;1例先做了双向腔肺动脉分流术,术后2年行心外管道Fontan手术。结果术后早期和晚期各死亡1例,分别死于急性肝功能衰竭和反复肺部感染,随访1．0～4．5年,40例心功能为Ⅰ级和Ⅱ级,动脉血氧饱合度92％～96％。结论体外循环心脏不停跳下心外管道Fontan手术的近、中期效果满意．可在单一心室修复中选用。