Gore-Tex Shunt Research Articles

DOZ047.90: An interesting and extremely rare case report of PCD and HLHS

Abstract An antenatally diagnosed hypoplastic left heart syndrome boy from Kuwait was transferred in utero for postnatal surgical management. He was born to a nonconsanguineously married couple by spontaneous delivery at 33 + 5 weeks of gestation with a birth weight of 2.4 kg. He was on prostaglandin infusion after birth and shortly underwent bilateral pulmonary artery banding with gore-tex shunts (aorta to pulmonary artery shunts) to let him grow and get over his prematurity for his cardiac surgical palliative procedures. He had some right upper lobe collapse, which was treated with physiotherapy and suctioning to proceed for his first stage cardiac surgical repair (Norwood I). Following this, he had a rough course owing to his cardiac condition but was extubated without many problems in due course. He was persistently tachypnoeic, had two episodes of acute decompensations during his stay due to his lung segment collapse needing invasive ventilation, and was very fragile, needing careful management. He needed noninvasive positive pressure ventilation at other times even when stable, to keep his airways from collapsing. He underwent a bronchoscopy and CT scan when stable enough to do it that showed a normal airway but with thick, tenacious, and clear secretions on bronchoscopy while a CT showed some areas of atelectasis and pulmonary interstitial emphysema. He was investigated for cystic fibrosis and primary ciliary dyskinesia as other causes that could lead to persistent and recurrent atelectasis were ruled out and his acute episodes dealt with appropriately. His genetic testing results have confirmed the clinical diagnosis of primary ciliary dyskinesia and he is being discussed for further management from a multidisciplinary point of view.

Hemodynamic volumetry using transpulmonary ultrasound dilution (TPUD) technology in a neonatal animal model.

To analyze changes in cardiac output and hemodynamic volumes using transpulmonary ultrasound dilution (TPUD) in a neonatal animal model under different hemodynamic conditions. 7 lambs (3.5-8.3kg) under general anesthesia received arterial and central venous catheters. A Gore-Tex(®) shunt was surgically inserted between the descending aortaand the left pulmonary artery to mimic a patent ductus arteriosus. After shunt opening and closure, induced hemorrhagic hypotension (by repetitive blood withdrawals) and repetitive volume challenges, the following parameters were assessed using TPUD: cardiac output, active circulating volume index (ACVI), central blood volume index (CBVI) and total end-diastolic volume index (TEDVI). 27 measurement sessions were analyzed. After shunt opening, there was a significant increase in TEDVI and a significant decrease in cardiac output with minimal change in CBVI and ACVI. With shunt closure, these results reversed. After progressive hemorrhage, cardiac output and all volumes decreased significantly, except for ACVI. Following repetitive volume resuscitation, cardiac output increased and all hemodynamic volumes increased significantly. Correlations between changes in COufp and changes in hemodynamic volumes (ACVI 0.83; CBVI 0.84 and TEDVI 0.78 respectively) were (slightly) better than between changes in COufp and changes in heart rate (0.44) and central venous pressure (0.7). Changes in hemodynamic volumes using TPUD were as expected under different conditions. Hemodynamic volumetry using TPUD might be a promising technique that has the potential to improve the assessment and interpretation of the hemodynamic status in critically ill newborns and children.

Determination of TOF Characteristics in a Tertiary Care Centre of Bangladesh

Objective: Tetralogy of Fallot (TOF) is the commonest cause of cyanotic congenital heart disease (CHD) worldwide. The aim of this study was to determine the demography, associated anomalies and status of peripheral pulmonary stenosis (PPS), type of operation, operative results and complications in TOF physiology patient. Materials & Methods: The records of 52 patients were reviewed. These patients were admitted from January 2007 to November 2009 in United Hospital Limited, Dhaka. Results: Male patients were 61.5% and females were 38.5%. The median age of surgery was 5 years and weight was 15 kg with severely undernourished patients were 27% and severely stunted were 11.5% respectively. Among the associated anomalies, percentage of patent ductus arteriosus (PDA), patent foramen ovale (PFO) / atrial septal defect (ASD), right aortic arch (RAA), coronary artery (CA) anomalies and peripheral pulmonary artery stenosis were 57.7%, 26.9%, 21.2%, 11.5% and 13.5% respectively. 13.5% TOF physiology patient had severely hypoplastic pulmonary annulus, 38.4% had severely hypoplastic MPA. TOF was more common in B blood group patients. About 11.5% patients underwent palliative operation (Gore-tex shunt) as the first operation. The youngest case was 7 days and the oldest 7.5 years old. Corrective operation, TC (Total correction) was done in 94%cases. The youngest patient undergoing TC was 1.5 years and the oldest one was 40 years old. Transannular patch (TAP) was used in 24 cases (46%). Post surgical overall mortality rate was 23% in three year which was 17.3% in 1st year, 3.84% in 2nd year and 1.92% 3rd year. Conclusion: In this study total correction (TC) was done in most of the cases (94%) and this should be recommended as the preferred management strategy for TOF physiology patient. The mortality rate decreases significantly as the team get experienced. DOI: http://dx.doi.org/10.3329/bjch.v34i3.10358 BJCH 2010; 34(3): 86-91

Cardiac output measurement with transpulmonary ultrasound dilution is feasible in the presence of a left-to-right shunt: a validation study in lambs

Cardiac output (CO) monitoring remains complex in newborns as most of the current technologies fail to accurately measure systemic blood flow in the presence of shunts. We validated CO measurements using transpulmonary ultrasound dilution (TPUD) in a neonatal lamb model with a left-to-right shunt. Regular arterial and central venous catheters were inserted into seven lambs (3.5-8.3 kg). A surgically constructed left-to-right aorto-pulmonary Gore-Tex(®) shunt was intermittently opened and closed, while CO was manipulated by creating haemorrhagic hypotension. CO measurements with TPUD (COtpud) were compared with those obtained by an ultrasonic transit-time flow probe positioned around the main pulmonary artery (COufp). We performed 72 sessions of three paired CO measurements. The mean COufp was 1.00 litre min(-1) (range 0.47-1.75 litre min(-1)) and mean COtpud 1.05 litre min(-1) (range 0.54-1.87 litre min(-1)). With an open shunt, the mean Qp/Qs ratio was 1.8 (range 1.3-2.6). A comparison between COufp and COtpud showed a mean bias (sd) of 0.03 (0.09) and 0.07 (0.10) litre min(-1), respectively, for measurements with a closed and an open shunt. The percentage error was 18% and 20% for measurements with a closed and an open shunt. Polar plot analysis showed good trending ability for both closed and open shunt groups. TPUD is a reliable technology to measure CO in the presence of a left-to-right shunt.

Stage 1 Palliation for Hypoplastic Left Heart Syndrome Without the Use of Allogeneic Tissue, with Reduced Allogeneic Blood Product Exposure: A Case Report

In the 30 years since Norwood described the palliative procedure for hypoplastic left heart syndrome (HLHS), many modifications have been described which have increased the survival rate of children born with this lesion. We describe further modifications which result in reduced cardiopulmonary bypass time, no cooling or circulatory arrest time, and decreased banked blood exposure. A 16-day-old infant with HLHS undiagnosed during pregnancy presented for stage 1 palliation incorporating the Mee modification, Sano right ventricle to pulmonary artery conduit, dual arterial cannulation of the innominate artery and descending aorta, single venous cannulation of the right atrium, and a bypass prime volume of 130 mL. Anticoagulation and hemostasis were monitored with the Hepcon HMS Plus Hemostasis Management System (Medtronic USA, Minneapolis, MN). Bypass commenced at normothermia. A 5.0 Gore-Tex shunt was placed for the Sano Shunt, and the aortic arch was repaired without use of homologous tissue or synthetic material using a modification of the Mee technique. Bypass time was 92 minutes with a 10 minutes cardiac ischemic time. Modified ultrafiltration (MUF) was performed for 12 minutes and heparinization was reversed with protamine. There was no significant bleeding and no indication to transfuse clotting factors. The patient’s only allogeneic donor exposure was 350 mL of red blood cells during bypass necessary to achieve a post MUF hematocrit of 50% per our current institution policy for cyanotic infants. Using modified surgical and perfusion techniques along with low prime bypass circuits can result in reduced cross clamp and bypass times as well as a decrease in blood donor exposure. Hypothetical benefits include reduced operating room, ventilation, intensive care unit, and hospital times, improved neurodevelopmental outcomes, and an overall reduction in the cost of care for infants with HLHS.

Decompression of the Portal Bed and Twice-Baseline Portal Inflow Are Necessary for the Functional Recovery of a “Small-for-Size” Graft

In partial liver transplant, a reduction in the intrahepatic vascular bed produces a rise in the portal vein flow and the portal venous pressure gradient, leading to endothelial and, thereby, hepatocellular injury and death in a process known as "small-for-size" (SFS) syndrome. To demonstrate that a calibrated portocaval shunt prevents superfluous inflow in a porcine model of SFS transplant. Donor pigs (15-20 kg) underwent 70% hepatectomy. In 2 groups, a 6 mm (S6) (n = 6) or 12 mm (S12) (n = 6) Gore-Tex shunt was placed between the portal vein and infrahepatic inferior vena cava. In a third group, no portocaval shunt was placed (SFS) (n = 17). Grafts were stored for 5 hours at 4°C and then transplanted into recipients (30-35 kg). Five-day survival was 29% in SFS, 100% in S6, and 0 in S12. Postreperfusion portal vein flow was 4-, 2-, and 1-times flow at baseline in SFS, S6, and S12, respectively. With respect to portal venous pressure gradient, both the 6- and 12-mm shunts effectively decompressed the portal bed. Aspartate aminotransferase and bilirubin rose and the Quick prothrombin time fell in all animals after reperfusion but improved significantly by day 5 in S6. Serum levels of endothelin-1 remained elevated in SFS and S12 but returned to baseline by 12 hours in S6: 2.76 (2.05-4.08) and 2.04 (1.97-2.12) versus 0.43 (0.26-0.50) pg/mL, respectively (P < 0.05 for both comparisons). A calibrated portocaval shunt that maintains portal vein flow about twice its baseline value produces a favorable outcome after SFS liver transplantation, avoiding endothelial injury due to portal hyperperfusion or to hypoperfusion because of excess shunting.

Surgical Correction of Tetralogy of Fallot With Unilateral Absence of Pulmonary Artery

Tetralogy of Fallot with unilateral absence of the pulmonary artery is a rare congenital heart defect that still represents a surgical challenge. The purpose of this study is to summarize our experience of surgical treatment of this complex lesion. From 1983 to 2003, 27 patients with tetralogy of Fallot and unilateral absence of the left (n = 25) or right (n = 2) pulmonary artery underwent different surgical interventions. The age of patients ranged from 40 days to 37 years (median, 5.3 years). Pulmonary arterial Nakata index and Nakata index Z-score were used for the quantitative assessment of the contralateral pulmonary artery. Twenty patients underwent various palliative procedures, namely Blalock-Taussig or Gore-Tex shunt, transluminal balloon pulmonary valvuloplasty, and reconstruction of right ventricular outflow tract without ventricular septal defect closure. At a median interval of 3.6 years after palliation, 13 patients underwent complete repair of tetralogy of Fallot. In the other 7 patients, complete repair was performed as a primary intervention. Hospital mortality after palliation and after a complete repair was the same and reached 5%. Sixteen patients with the Nakata index greater than 200 mm2/m2 and Z-score greater than -4 survived after a complete repair. One of 4 patients with Nakata index less than 200 mm2/m2 and Z-score less than -4 died after surgery. Majority of patients with tetralogy of Fallot and unilateral absence of the pulmonary artery require palliative intervention as a first step of surgical treatment. Nakata index greater than 200 mm2/m2 and Nakata index Z-score greater than -4 are criteria for a successful complete repair.

Experimental Orthotopic Heart and Bilateral Lung Transplantation Completed Without Cardiopulmonary Bypass

Most experimental studies of orthotopic heart and lung graft failure are complicated by an inability to eliminate the rejection-specific inflammatory mediator from the cardiopulmonary bypass. The following model was developed in our laboratory to investigate the feasibility of performing an orthotopic heart and bilateral lung transplantation without performing a cardiopulmonary bypass. Nineteen transplants were attempted using 19 pairs of mongrel dogs. The recipient dog (mean weight, 23 kg) was anesthetized, and the ascending aorta, the superior vena cava (SVC), the inferior vena cava (IVC), and the main bronchus were dissected. Then, the donor dog (mean weight, 20 kg) was anesthetized, and the heart and lung block was prepared and explanted from the chest under cardioplegic arrest. A Gore-tex shunt (W. L. Gore; Flagstaff, AZ) was placed side-to-side between the recipient IVC and SVC, and then the donor right atrium was anastomosed to the Gore-tex shunt. The donor ascending aorta was anastomosed to the recipient ascending aorta with a partial clamp. On completion of these anastomoses, the donor heart was reperfused by the recipient heart and allowed to beat. When hemodynamic conditions were stable with double hearts, the recipient SVC and IVC were ligated just proximal to the venous anastomosis and the recipient aorta was ligated proximal to the anastomotic site. The recipient trachea was anastomosed to the donor trachea with an end-to-end anastomosis. Finally, the recipient heart and lungs were removed from the chest and the sternum was closed. Four of the 19 transplants failed. Three died due to left ventricular dysfunction, and one died due to bleeding. Mean (+/- SD) ischemic time was 67 +/- 11 min with a mean (+/- SD) anastomotic time of 54 +/- 12 min. The 15 survivors were hemodynamically stable with or without the minimal use of inotropic support (dopamine, 2 to 3 microg/kg/min) 6 h after grafting, with normal cardiac output, satisfactory oxygenation, and normal wall motion. The sternotomy was repaired without loss of cardiopulmonary function. On the basis of our experiences, the experimental model of orthotopic heart and bilateral lung transplantation completed "off pump" can be technically feasible without the loss of cardiac and pulmonary functions.

Side-to-Side Aorto–Gore-Tex Central Shunt

Background. This report details our experience in 13 patients with a technical modification of the standard central shunt. Methods. The study was performed using a retrospective chart review approach. In our operation, the aorto-Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) anastomosis is created in a side-to-side fashion with the free end of the Gore-Tex shunt being oversewn. Results. All patients had echocardiographic evidence of shunt patency in the immediate postoperative period, and there have been no cases of late shunt occlusion at a mean follow-up period of 10 months. Conclusions. We believe this approach will yield patency rates equivalent to or better than those of the standard central shunt. The technique has the advantage of creating a short, straight-lying shunt that is less likely to kink or be injured on repeated sternotomy and in which flow may be more reliable.

Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified Norwood procedure.

Prospective audit of the first year of implementation of a modified approach to palliation for infants with hypoplastic systemic ventricle and severe systemic outflow obstruction. Tertiary referral centre for neonatal and infant cardiac surgery. 17 of 19 infants (aged < 35 days) presenting to Birmingham Children's Hospital in 1993 with hypoplastic systemic ventricle and severe outflow obstruction underwent surgery. This was performed using a new modification of the Norwood-type arch repair, without the use of exogenous material, and a 3.5 mm Gore-tex shunt between the innominate and right pulmonary arteries. The Gore-tex shunt was replaced by a cavopulmonary shunt between 3 and 5 months later. Clinical, morphological, and functional determinants of outcome were examined. 10 (59%) infants survived initial surgery. All proceeded to cavopulmonary shunt without further loss. Significant atrioventricular valve regurgitation seemed to be the main risk factor for poor outcome. If this was excluded, the morphology of the dominant ventricle seemed to have little effect on the outcome of initial surgery. Early survival was achieved in 59% of patients in the first year of implementation of a protocol for surgery in infants with hypoplastic systemic ventricle and severe outflow obstruction. The construction of a neoaorta without the use of exogenous material may allow improved later growth of the neoaorta. Early cavopulmonary shunt can be performed safely and should reduce mid-term complications from cyanosis and systemic ventricular volume loading.

New Gianturco-Grifka Vascular Occlusion Device

Transcatheter closure of cardiovascular defects remains a challenge. Several occlusion devices are available, but each device has limitations. The purpose of this study was to evaluate the new Gianturco-Grifka vascular occlusion device (GGVOD) in a canine model. A total of 26 GGVODs were implanted as part of short- and long-term studies. In the short-term study, 1 GGVOD was implanted in each of 11 systemic arteries from 3.2 to 9.0 mm in diameter. All 11 arteries were occluded immediately. In the long-term study, an aortopulmonary shunt was placed in 10 dogs (9, Gore-tex graft; 1, subclavian artery) followed by GGVOD implantation; additionally, a GGVOD was implanted in 5 subclavian arteries. The dogs were boarded for 3 to 6 months, then recatheterized and euthanatized. Immediately after implantation, the 5 subclavian arteries and 9 Gore-tex shunts were occluded completely; the 1 subclavian artery shunt had a small residual leak. At recatheterization, all 10 shunts and 5 subclavian arteries were occluded completely. Necropsy revealed all shunts to be occluded, with the aortic and pulmonic orifices covered with a neointimal layer. The mean fluoroscopic time needed for GGVOD implantation was 9 minutes (range, 3 to 22 minutes). (1) In a canine model, the GGVOD is effective for transcatheter occlusion of arteries and aortopulmonary shunts from 3 to 9 mm in diameter. Possible indications in children include aortopulmonary collateral vessels, long patent ductus arteriosus, systemic-pulmonary shunts, AV malformations, and arteries supplying tumors. (2) GGVOD implantation requires a short fluoroscopic time.

Infracardiac total anomalous pulmonary venous connection in tetralogy of Fallot with decreased pulmonary flow and masked pulmonary venous obstruction: report of one case

We describe an 8-month-old male infant, who received a rerouting procedure for infracardiac total anomalous pulmonary venous connection and a 4-mm Gore-Tex central shunt for tetralogy of Fallot with pulmonary atresia. The masking effect of the pulmonary outflow in tetralogy of Fallot on the pulmonary venous obstruction in infracardiac total anomalous pulmonary venous connection is discussed. Preoperative recognition of this rare association is warranted, as palliative surgery alone may be disastrous. In addition, juxtaductal pulmonary artery coarctation in tetralogy of Fallot with pulmonary atresia may play a role in camouflaging the pulmonary venous obstruction in this case. Unmasking pulmonary venous obstruction by oral prostaglandin and augmenting pulmonary blood flow by a Gore-Tex shunt, pari passu, did harm this patient.

Citrate versus heparin anticoagulation in chronic haernodialysis patients

Anticoagulation with citrate at a rate of 0.68 mM/min in combination with a calcium and magnesium-free dialysate and i.v. supplementation of calcium and magnesium at rates of 0.18 mM/min and 0.08 mM/min respectively, was compared with low-dose heparin. The heparin dose was a loading dose of 2500 IU and a sustaining infusion of 750-1250 IU/h; or a loading dose of 1250 IU and a sustaining infusion of 500-750 IU/h until 1 h before the end of the dialysis if the patient was taking concomitantly coumarin anticoagulation for a Goretex shunt. Six chronic haemodialysis patients changed from heparin to citrate anticoagulation because they reported bleeding between dialyses. Heparin, after 2 h dialysis, induced a significant 10% prolongation of each patient's whole-blood activated clotting time (WBACT) as compared to the predialysis value; while the WBACT at the dialyser outlet was less than 3% prolonged as compared to the patient's WBACT. However, after 2 h citrate the patient's WBACT was not prolonged but the WBACT at the dialyser outlet was 20-100% longer, indicating a better anticoagulation of the extracorporeal system without systemic effects. With heparin the shunt pressure time (SPT), i.e. the time needed to stop bleeding from the puncture sites of the Goretex shunts, was 12 of 28 times 20 min or more. Citrate reduced these episodes by 75%. Thus citrate should be considered for chronic haemodialysis patients who are at risk of bleeding because of the concomitant use of anticoagulants.(ABSTRACT TRUNCATED AT 250 WORDS)

Aneurysm of the right ventricular outflow tract: A complication of aorta-main pulmonary (central) shunt

The ascending aorta-main pulmonary artery (central) polytetrafluoroethylene (PTFE) Gore-Tex shunt was introduced in 1975 by Gazzaniga and coworker. It is a widely used palliative procedure. We present a case study with an unusual late complication. An aneurysm was discovered at autopsy in a patient who was diagnosed at birth with hypoplastic right ventricle, pulmonary valve atresia, small main and branched pulmonary arteries. At 4 days of age, the patient underwent an aorta to main pulmonary artery (PTFE) Gore-Tex shunt. Subsequent echocardiogram showed dilatation of the right ventricular outflow tract. To our knowledge, this is the first reported case of right ventricular outflow tract (RVOT) aneurysm following central aortopulmonary shunt.

Surgical treatment of double inlet ventricle ("single ventricle").

The surgical techniques described are the result of an evolution over a number of years in the performance of the septation operation and the modified Fontan-Kreutzer repair for patients with double inlet ventricles. Those with associated pulmonary stenosis are best palliated by a classical Blalock-Taussig or Goretex shunt if an operation is required during the first few years of life and later, between two and four years of age, definitive repair by the modified Fontan-Kreutzer operation is advised. Although controversial, we prefer the use of a large nonvalved right atrial-pulmonary artery connection. Ventricular septation remains the best definitive surgical option when pulmonary stenosis is absent or mild. It is contraindicated by severe pulmonary vascular disease and also by less than moderate ventricular enlargement. The need for concomitant AV valve replacement and the use of an extracardiac conduit are associated with increased hospital mortality in our experience. Infants identified during the first year of life who do not have pulmonary stenosis are a difficult subset to manage. If the VSD and subaortic area is large and unobstructed, pulmonary artery banding early in life will control pulmonary vascular resistance and from this standpoint, permit these patients to become ultimately suited to a modified Fontan-Kreutzer repair. Unfortunately, ventricular hypertrophy usually results from pulmonary artery banding and has been associated with higher hospital mortality at the time of definitive repair. When pulmonary artery banding is undertaken for this subset, debanding and definitive repair seems best advised at about two years of age. Pulmonary artery banding is well known to accelerate the development of subaortic stenosis by spontaneous progressive restriction of the VSD. This results in small ventricular cavity size and increased ventricular hypertrophy, which are incremental risk factors for increased hospital mortality by either definitive procedure. When the VSD or subaortic area is narrow and the patient is identified during the first year of life, isolated pulmonary artery banding is inappropriate. The surgical options for these patients include Ebert's two-stage management program consisting of the initial placement of a loose partial septation patch with concomitant pulmonary artery banding, and later debanding and complete septation. Alternatively, a trial of primary complete septation may be warranted, or the use of a procedure consisting of division of the main pulmonary artery with distal closure and anastomosis of the proximal portion to the side of the ascending aorta, coupled with a systemic-pulmonary artery shunt.

Two-stage correction for tetralogy of Fallot.

The policy for surgical treatment of tetralogy of Fallot in younger patients is still controversial. Our overall 14-year experience has been reviewed with regard to the factors influencing mortality for both shunts and corrective procedures. An attempt has been made to evaluate our current expected cumulative mortality for two-stage correction in patients under 2 years of age. From November, 1966 through April, 1983, 440 shunts and 647 total corrections were performed. Patients under 2 years of age, and those with unfavorable anatomy and/or physiology, generally underwent two-stage correction. Early correction was occasionally performed in this age group on patients with very favorable anatomy, or in case of early shunt failure. Retrospective standard statistical analysis was carried out in order to evaluate the influences of the year of operation, age, and operative technique on mortality. The overall early mortality of shunt procedures was 5.7% (11.4% below and 3.5% over 6 months of age). Since 1978 it has dropped to 2.8% (4.2% below and 2.1% over 6 months). The Waterston shunt had a higher (7%) operative mortality than the Blalock (3%) or Goretex (2.6%) shunts. The overall early mortality of total corrections was 15.1% (25.2% below and 13.5% over 2 years of age). It has dropped to 6.9% since 1978 (29.9% below and 6.2% over 2 years).(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical results and protocols in the spectrum of tetralogy of Fallot.

Between 1967 and July 1982, 1103 operations were performed for the tetralogy of Fallot of all types with 116 (10.5%) hospital deaths. Eighty-eight hospital deaths (10.5%) occurred in the 836 patients undergoing repair. The incremental risk factors for hospital death after repair include pulmonary arterial problems (p = 0.0002), major associated cardiac anomalies (p less than 0.0001), small size (young age) (p less than 0.0001), and more than one previous operation (p = 0.0004). Absent pulmonary valve is a risk factor (p = 0.04). In patients with pulmonary stenosis, the hospital mortality has decreased with time (p = 0.08), but the incremental risk of a high hematocrit (p = 0.0003) and of transannular patching (p = 0.05) has persisted. In the current era, the risk of repair in patients with pulmonary stenosis is estimated to be 1.6% (70% confidence limits [CL] 0.7% to 3.5%) at age 5 years, and at age 12 months to be 4.1% (CL 2.7% to 6.3%) without a transannular patch and 7.7% (CL a5.3% to 11%) with one. When pulmonary atresia is present, the probability of hospital death after repair when a valved extracardiac conduit is used is estimated to be lowest (5%; CL 2% to 8%) between 5 1/2 and 16 years of age. No deaths occurred among 53 patients with pulmonary stenosis receiving a primary palliative Blalock-Taussig or Gore-Tex shunt, and six deaths (12%) occurred in 51 patients with pulmonary atresia. Serious interim complications (sudden death, brain abscess) after these shunts occurred in two (1.9%; CL 0.6% to 4.5%) of the patients operated on. No iatrogenic pulmonary arterial problems have been recognized. Protocols based on these results are presented. Many of these selective recommendations may become unnecessary if the damaging effects of cardiopulmonary bypass are overcome by future research.