Subclavian-pulmonary artery shunts with polytetrafluorethylene interposition grafts

Systemic-pulmonary artery shunts remain an important treatment in cyanotic patients. Central shunts continue to pose early and late problems when standard Blalock-Taussig shunts are not possible. Twenty patients underwent subclavian-pulmonary artery shunt procedures with polytetrafluoroethylene (PTFE) prostheses between October, 1980, and August, 1982. Their ages ranged from 1 day to 15 years; 11 patients were less than 14 days old. The arterial oxygen tension rose from 30.7 +/- 11.9 mm Hg to 51.3 +/- 9.1 mm Hg (standard deviation; p less than 0.001) and from 26.4 +/- 7.5 mm Hg to 50.5 +/- 9.3 mm Hg (p less than 0.001) among the 11 neonates. There were no hospital deaths and only 2 late deaths (not shunt related). All patients have patent shunts and excellent relief of cyanosis. The 18 survivors have been followed for an average of 19 months (range, 7 to 29 months). No patient has required reoperation for shunt inadequacy or thrombosis. Recatheterization in 11 patients has demonstrated normal pulmonary pressures and good pulmonary artery growth without vessel distortion. Subclavian-pulmonary shunts using PTFE provide long-term palliation in cyanotic patients. This type of shunt appears to offer important advantages over other shunt procedures, including the classic Blalock-Taussig operation, in newborns.     

Modified Blalock-Taussig shunts: results in infants less than 3 months of age.

The optimal procedure for shunting palliation in cyanotic infants remains to be determined. Sixty-two infants less than 3 months of age underwent 63 modified Blalock-Taussig shunts. Their age range at operation was 1 to 84 days (mean, 16 +/- 20 days). Shunts were constructed using 5-mm polytetrafluorethylene tubes in 20 patients and 4-mm polytetrafluoroethylene grafts in 43 patients. There were 13 early deaths (21%; CL, 15% to 27%) of which three deaths (5%; confidence limits, 2% to 9%) were shunt related. The survivors were followed up from 6 to 53 months (mean, 29 +/- 12.5 months). Shunt failure (occlusion, inadequate palliation) occurred in 27 patients. The overall probability rate of adequate shunt function was 58% +/- 8% at 2 years. Univariate and multivariate analyses showed that the size of the graft was a risk factor of shunt failure. Severe distortion of the pulmonary arterial branch was noted in 12 patients. The inferences are: (1) modified Blalock-Taussig shunts provide satisfactory early palliation but late shunt failure is frequent; (2) similar results should be obtained with other shunting procedures; and (3) the optimal procedure should be selected for each cyanotic infant on an individual basis.     

Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology

OBJECTIVES: Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle-tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure. METHODS: Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10. RESULTS: There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years. CONCLUSIONS: We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.     

Modified Blalock-Taussig shunt following long-term administration of prostaglandin E1 for ductus-dependent neonates with cyanotic congenital heart disease

In the treatment of cyanotic heart diseases in neonates who are ductus dependent, the small size of the pulmonary artery and/or subclavian artery often precludes construction of a sufficient Blalock-Taussig shunt. To avoid an emergency operation and to obtain pulmonary and subclavian arteries of adequate size (3 mm), a low dose of prostaglandin E1 was given to 16 neonates for a mean of 47 days (range 26 to 65 days), and a modified Blalock-Taussig shunt operation was performed in neonates whose mean age was 50 days. The pulmonary artery grew significantly (mean 4.5 mm) and the mean diameter of the subclavian artery was 3 mm, which was large enough for a good shunt at the time of operation. There were two late deaths and one operative death. All surviving patients shunts at the mean follow-up period of 22 months. The shunt was constructed on the same side as the ductus arteriosus so that the ductus could be ligated at the same operation in selected cases; the ductus may not close after discontinuation of prostaglandin E1 infusion and may cause uncontrollable congestive heart failure because of excessive pulmonary blood flow, particularly in patients with a univentricular heart. The ductus, dissected in nine patients, was enlarged and elongated in all and was ligated in four patients without complication.     

A Five-Year Clinical Experience with 112 Blalock-Taussig Shunts

In spite of recent advances in neonatal open repair for complex cyanotic heart disease, some patients require palliation with a systemic-to-pulmonary artery shunt. We report a 5-year experience (1985–1990) with 112 Blalock-Taussig shunts. Forty-six of the 92 patients had some variant of tetralogy of Fallot, with a wide spectrum of diagnoses in the remainder. The median age at surgery was 3 months. A classic Blalock-Taussig shunt was done in 26% (group I), and a 4- or 5-mm PTFE graft was utilized in the remainder (group II). The technical aspects of each of the procedures are reviewed. There were three early deaths in the entire group, none of them related to Blalock-Taussig shunt function. There was no incidence of early shunt insufficiency, bleeding, infection, limb ischemia, or pulmonary artery distortion. There was a 21% incidence of clinical congestive heart failure, seen somewhat more commonly in group I. The overall need for reshunting/open repair was similar in both groups, but there was a statistically longer interval between the initial Blalock-Taussig shunt and the second procedure in group I (21.6 vs 12.4 months). The Blalock-Taussig shunt remains a safe, reliable, and effective means of increasing pulmonary flow. (J Card Surg 1993; 8:9–17)     

Glenn Shunt: Long-Term Results and Current Role in Congenital Heart Operations

Fifty cyanotic patients (aged 2 days to 22 years) underwent Glenn shunts for tricuspid atresia and other cyanotic heart defects. Thirteen of 15 operative deaths occurred in infants less than 4 months old, and only 1 death has occurred in the last 9 years. Results were poor in patients with Ebstein's anomaly, truncus arteriosus, transposition of the great vessels, and complex defects other than tricuspid atresia and univentricular heart.Of the 35 patients followed from 0.9 to 14.8 years, 12 were followed for more than 10 years. None of the 11 late deaths could be attributed to complications of the shunt. Minimal evidence of intrapulmonary shunting was found by angiography, pulmonary venous oximetry, or radioisotopic studies. Late deterioration due to venous collaterals and decreased flow to the opposite lung necessitated Blalock-Taussig shunts in 6 and Fontan procedures in 10. All survived the Fontan procedures with minimal morbidity.These data support the concept that Glenn shunts do not necessarily result in pulmonary abnormalities and may be indicated as a staged procedure in a few selected patients prior to a Fontan procedure.     

Systemic-pulmonary shunts in infants and children. Early and late results

From September, 1978, to January, 1983, 44 cyanotic infants and children underwent a systemic-pulmonary artery shunt for the treatment of reduced pulmonary blood flow. Age ranged from 18 hours to 4 years (mean age = 0.49 years). Weight ranged from 1.7 kg to 13.2 kg (mean weight = 4.9 kg). There were 12 classic Blalock-Taussig shunts, five central polytetrafluoroethylene shunts, six interposition modified Blalock-Taussig shunts, and 21 Great Ormond Street type of modified Blalock-Taussig shunts. No direct aorta-pulmonary artery anastomoses were performed. There was one postoperative death (1/44 = 2.3%) in a 1,700 gm neonate born with pulmonary atresia and intact ventricular septum (shunt patent). Four shunts required early revision: one thrombosed central shunt, a kinked patent interposition Blalock-Taussig shunt, a small but patent Blalock-Taussig shunt, and one excessive Great Ormond Street type of Blalock-Taussig shunt. Two late deaths were probably shunt-related: one Blalock-Taussig and one central. All four types of shunts provided good palliation, but the Great Ormond Street type of Blalock-Taussig shunt is our preferred shunt because of (1) low operative risk, (2) predictable patency (100% in our series), (3) lack of distortion of pulmonary arterial anatomy, and (4) technical ease of insertion as well as takedown.     

Pseudoaneurysm following modified Blalock-Taussig shunt mimicking suprasternal abscess: A rare presentation

The modified Blalock-Taussig shunt is a well-recognized palliative procedure for cyanotic congenital heart disease. Post procedure pseudoaneurysm mimicking a suprasternal abscess is of very rare occurrence. We present a child of L-Transposition of great arteries (L-TGA), pulmonary atresia and ventricular septal defect (VSD) with small pulmonary artery size that underwent modified Blalock Taussig shunt for severe cyanosis at 1 1/2 years. Six months after the procedure the child presented with a suprasternal swelling and fever. Angiography showed pseudoaneurysm from the proximal anastomotic site. The pseudoaneurysm was suitably tackled by interventional techniques and surgery for the underlying cardiac condition is being planned.     

Blalock-Taussig shunts in the infant

Eighteen consecutive patients 3 to 30 months of age underwent Blalock-Taussig shunt for palliation of cyanotic congenital heart disease. Nine patients weighed less than 8 kg. Follow-up has ranged from 12 to 62 months. All shunts have remained patent, and in none of the patients has a second shunt been required. The Blalock-Taussig shunt provides useful palliation for cyanotic congenital heart disease during the first 2 years of life.     

Technique and experience with azygos patch modified Blalock-Taussig anastomosis for congenital cyanotic heart disease

Seventeen patients 1 day to 28 months old with congenital cyanotic heart disease underwent a modification of the Blalock-Taussig shunt. Eight were newborn infants weighing 2.6 to 4.8 kg. All infants had complex congenital heart defects that were not considered amenable to early correction. There were no early deaths and no shunt failures. Postoperative complications were restricted to prolonged intubation in 2 patients and a subcutaneous wound infection in a 14-day-old infant. Follow-up from 1 to 31 months revealed minimal cyanosis, stable hemoglobin levels, and good shunt murmurs, and there have been no upper extremity complications. There were 2 late deaths; 1 (the oldest patient) was related to medication, and the second resulted from small bowel necrosis. The concept of the azygos vein patch modified Blalock-Taussig shunt involves two factors: (1) mobilizing as much length as possible of the subclavian artery in spite of its distal small size to allow for a tension-free shunt to prevent tension on the anastomosis as growth occurs, and (2) enlarging the subclavian artery distal to the vertebral artery origin with an autologous azygos patch. During performance of a standard Blalock-Taussig shunt, a longitudinal incision is made through the pulmonary artery across the anastomosis into the upper subclavian artery. The appropriate length of harvested azygos vein is used as a patch angioplasty across the shunt. A tension-free shunt with a patulous distal portion is achieved. Pulmonary overcirculation is avoided by the limiting size of the proximal subclavian artery. Temporary occlusion of the shunt at operation resulted in an increased mean blood pressure from 6 to 18% in all infants.(ABSTRACT TRUNCATED AT 250 WORDS)     

Progress of patients with pulmonary atresia after systemic to pulmonary arterial shunts.

Between February 1980 and June 1987, 42 shunts were placed in 39 infants with pulmonary atresia: 33 were modified Blalock-Taussig shunts with polytetrafluoroethylene (PTFE) and 9 were classic Blalock-Taussig shunts. There were four hospital deaths not related to the shunts. The remaining 35 patients were followed up for 1.6 months to 6.3 years (mean, 24.7 +/- 18 months). Repeat cineangiocardiographic studies revealed stenosis or distortion of the pulmonary arteries related to the site of the shunt in 11/22 patients (50%) with PTFE shunts and in 1/6 (17%) with classic Blalock-Taussig shunts; the stenosis was severe in only 1 patient. Mean increase in the pulmonary arterial index in the group with classic Blalock-Taussig shunts was 117 +/- 52 mm2/m2 (not significant) and in the group with PTFE shunts, 158 +/- 21 mm2/m2 (p less than 0.001). Late shunt occlusion occurred in 1 patient 23 months postoperatively. Thereafter, shunt patency rate remained at 94% +/- 6%. At the end of 1 year 81% +/- 7% of patients were judged to have adequate palliation, but between 2 and 3 years, only 60% +/- 10%. Univariate analysis showed that after 2 years the ranking order for successful palliation was classic Blalock-Taussig, 5-mm PTFE, and 4-mm PTFE shunts, but differences did not achieve statistical significance.     

A current appraisal of the Waterston shunt

Recent reports have suggested that the classical Blalock-Taussig shunt and its prosthetic modification have a low patency rate in neonates. This prompted a review of 58 neonates undergoing a Waterston shunt for cyanotic congenital heart disease. The 4 mm anastomosis was constructed using a calibrated clamp. There were 20 (35%) hospital deaths, of which 3 were directly related to shunt failure. Operative mortality was associated with increasing complexity of the cardiac lesion, emergency operation, weight less than 3 kg and age less than 48 hours. There were 5 late deaths resulting in an actuarial survival of 52% at ten years. There was one case of late occlusion corresponding to a patency of 92% at 2 years. Anastomotic kinking with preferential flow to one lung occurred in 2 (7%) of 28 patients undergoing repeat angiography at 3 and 5 years respectively. Nineteen patients underwent corrective surgery a mean of 27 months after shunt construction. Six (32%) required an angioplastic repair of the pulmonary artery. Six (16%) of the 38 operative survivors required diuretic therapy for excessive pulmonary flow. The high patency ensures that the Waterston shunt continues to play an important role in the management of low weight neonates who require an emergency systemic-pulmonary shunt. The use of a calibrated clamp reduces the incidence of excessive pulmonary flow. Early corrective surgery and shunt dismantling may reduce the necessity for angioplastic repair.     

The Blalock-Taussig shunt. Low risk, effective palliation, and pulmonary artery growth

The Blalock-Taussig shunt has been used at our institution in 64 infants with cyanotic congenital heart disease who required palliation. Thirty-one of these children were less than 2 months of age at the time of operation. There were no early shunt closures. There were two hospital deaths (3%). There was only one hospital death in the 31 patients less than 60 days old. According to a life-table analysis, 87% +/- 9% (+/- 95% confidence limits) of the shunts were functioning at 1 year and 78% +/- 12% at 2 years. Results are similar in the subgroup of children less than 60 days of age at the time of operation or in the subgroup of children weighing less than 3,999 gm at operation. In infants with small pulmonary arteries, pulmonary arterial growth was excellent (ipsilateral pulmonary artery/descending aorta ratio 0.70 leads to 0.95 in 550 days, p less than 0.001; contralateral ratio 0.73 leads to 0.99 in 550 days, p less than 0.001). The Blalock-Taussig shunt can be performed with low risk, provides excellent palliation, and is associated with excellent pulmonary artery growth.     

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.     

Modified Blalock-Taussig shunt in infants and young children. Clinical and catheterization assessment

The effectiveness of 19 modified Blalock-Taussig shunts performed with expanded polytetrafluoroethylene was evaluated clinically and by cardiac catheterization with angiography 4 to 24 months after operation. Fifteen patients underwent operation in infancy. Conduit diameters included 4 mm (nine cases), 5 mm (eight cases), and 6 mm (two cases) sizes. Two of the 4 mm conduits failed after 1 year following implantation. The remaining 17 shunts (89%) remained widely patent. In patients with patent shunts, the oxygen saturation values were significantly improved from the preoperative values. Two children demonstrated associated subclavian artery occlusion distal to the graft anastomosis. There were no deaths. Thirteen children underwent more complete elective cardiac repair 5 to 24 months later. Although the modified Blalock-Taussig procedure is an effective short-term alternative to the classic Blalock-Taussig shunt, the effectiveness of the 4 mm diameter conduit may be limited without postoperative anticoagulant therapy.     

Classic shunting operations as part of two-stage repair for tetralogy of Fallot.

One hundred forty-nine consecutive patients with tetralogy of Fallot, with or without pulmonary atresia, underwent Blalock-Taussig or Waterston operation for initial palliation. Of these patients, 45 were less than 6 months old, and 63 were less than 1 year old. The type of shunt, and the presence or absence of pulmonary atresia did not have a significant effect (p greater than 0.2) on hospital mortality. Parametric analysis showed a significant effect of age (p = 0.03), the risk of hospital death being 6% at 1 month of age, 4% at 3 months, 3% at 6 months, and 2.5% at 12 months. No late deaths occurred before the age of 3 years. Six patients (4.2% of the hospital survivors) required another operation before they were 3 years old. Severe arm ischemia occurred after a Blalock-Taussig shunt in 1 infant with Down's syndrome.     

Long-term results of the Blalock-Taussig shunts]

One-hundred and thirty-six patients received the classical Blalock-Taussig shunts between 1980 and 1990. Their age range at operation was 4 days to 12.8 years and their median age was 13 months. The operative mortality rate was 0.7% (1/136). The survivors were followed up from 1 month to 11.5 years, 5.4 years in average. Twenty-five patients required another shunt and the mean interval to that procedure was 2.4 years (modified Blalock-Taussig 23, classical Blalock-Taussig 2, Glenn 1, internal mammary artery-pulmonary artery shunt 1). Forty-five patients received corrective operations, there were four operative deaths (Fontan 2, Rastelli 2). There were 15 late deaths of which three deaths were not cardiogenic. One year after operation, 91.0% of patients remained in well-palliated condition. At three years after operation, 76.4% of patients continued to be in well-palliated condition. There were twelve neonates in this series. Their age range was 4 to 26 days and their median age was 13 days. There was no operative death. Five patients required second shunt. There were two late deaths. At present, six patients continue to be in well-palliated condition 8 months to 9 years after first operation. Angiographic findings showed the stenotic change of the vascular anastomoses in 49.3% (35/71) of patients. This study suggests that polydioxanone suture (PDS) will be useful for the growth of the anastomoses in Blalock-Taussig operation.     

Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt

BACKGROUND: Multiple systemic-to-pulmonary artery shunts often represent a deviation from the initial management strategy for cyanotic congenital heart disease. This study analysed the outcome in patients undergoing a second shunt. METHODS: Between 1965 and 1998, 80 patients required a second shunt. Patient age ranged from 11 days to 11.2 years at the initial shunt, and 4 months to 25.6 years at the second shunt. The interval between shunts ranged from 11 days to 19.6 years. RESULTS: The patient survival rates at 1, 6, 12, 60 and 120 months following the second shunt were 95.0 +/- 2.4%, 92.5+/-3.0%, 87.4+/-3.7%. 82.4+/-4.5% and 74.8+/-5.8%, respectively. Although excess pulmonary flow was not encountered after the second shunt, mode and incidence of morbidity were otherwise similar to the first one. Between 9 months and 7.5 years after the second shunt, 24 patients underwent successful definitive repair. Multivariable analysis identified pulmonary atresia (p=0.027) and a short (<1 year) interval between the two shunts (p=0.034) as the independent risk factors for long-term shunt failure. Single ventricle physiology (p=0.002) and a central approach for the second shunt (p=0.016) were independent risk factors for lack of application, or failure of intracardiac definitive repair. CONCLUSIONS: A significant limitation in longevity and quality of life is common in patients requiring a second shunt, especially those associated with pulmonary atresia, the single ventricle physiology heart, or ungraftably hypoplastic contralateral branch pulmonary artery to the first shunt. Since excess pulmonary blood flow leading to congenital heart failure and/or ventricular diastolic dysfunction is unlikely, all patients who preclude definitive repair due to decreased pulmonary blood flow even after the first shunt should be shunted again.     

Acute pulmonary edema after Blalock-Taussig anastomosis.

Acute pulmonary edema developed in 2 patients after a Blalock-Taussig shunt procedure. Both patients had a univentricular heart complex with pulmonary stenosis. One patient underwent a modified Blalock-Taussig shunt using a polytetrafluoroethylene tube and the other underwent a classic Blalock-Taussig anastomosis. Acute pulmonary edema after the Blalock-Taussig shunt is rare, but once it has occurred, immediate treatment is obligatory.