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Biomedical subjects

W G Williams

Publications and source records attributed to W G Williams.

At least 109 records · Page 6Linked to original sources
PubMed1995

Right to left interatrial communications after the modified Fontan procedure: identification and management with transcatheter occlusion.

OBJECTIVE: To describe unusual venous communications from the right to the left atrium resulting in cyanosis after the modified Fontan procedure, and their management with transcatheter occlusion. METHODS: Between September 1992 and November 1994, eight patients were assessed for persistent cyanosis after a modified Fontan procedure. Desaturation was found to be caused by unusual venous shunts originating at atrial level, and transcatheter occlusion with either a double umbrella or coil was attempted. RESULTS: Three types of venous channels were identified. The first type of communication consisted of thin long tortuous channels originating from the right atrial wall, and draining into the left atrium through a capillary network. The second type of communication was in the superior anterior portion of the atrial baffle, incorporating the pectinate muscles of the right atrium, draining into the neoleft atrium. These channels were shorter and often fanned out into small vessels toward the right atrial appendage. In each instance, the shunts were in the superior suture line of a lateral tunnel modification of the Fontan procedure. The third type of communication originated from the inferior vena cava, connecting inferior phrenic veins to pericardial veins and subsequently to the left atrium, at or close to the ostium of the left pulmonary veins. Before device occlusion, the room air aortic oxygen saturation was 88(SD 4)% (range 84% to 94%), and increased to 95(3)% (range 91% to 100%) following occlusion (PL << 0.001). The mean right atrial pressure was 14(4)mm Hg and remained unchanged after occlusion. In six patients there was complete shunt obliteration, while in two both occluded with umbrella devices, a small residual leak persisted. No complication occurred during or immediately after catheterisation. CONCLUSIONS: Unusual venous communications can evolve after the Fontan procedure, resulting in the development or persistence of cyanosis. Some of these communications may be present preoperatively as normal veins draining into the right atrium, enlarging with the increased atrial pressure after surgery. These observations affect long term function after the Fontan procedure. Transcatheter occlusion of these communications is technically feasible and effective, although recurrence may occur.

Adolescent↗
PubMed1995

Endovascular stents in children under 1 year of age: acute impact and late results.

OBJECTIVES: To review efficacy and safety of endovascular stent implants in children < 1 year of age with congenital heart lesions. DESIGN: Retrospective study of patients in a tertiary care setting. PATIENTS: 26 children (median age of 4.7 months, range 2 days to 1 year) with various vascular obstructive lesions. INTERVENTION: Percutaneous or intraoperative implantation of balloon expandable endovascular stents. RESULTS: Optimal stent placement was obtained in 31 of the 37 deployed implants. Complications resulted primarily from stent malpositioning and one episode of bleeding at a puncture site. Stent implantation in three patients with a restrictive arterial duct allowed for patency and five patients with conduit stenosis had mean (SD) right ventricule to systemic artery pressure ratios falling from 0.99 (0.20) to 0.52 (0.18) (P < 0.05). In 10 patients with pulmonary artery stenosis, the mean vessel diameter increased from 2.8 (0.9) mm to 5.8 (1.4) mm (P << 0.001). No clinical improvement was seen in two patients because of diffuse hypoplasia of the pulmonary vessels. Nine of 10 patients with miscellaneous obstructive lesions improved clinically. Recatheterisation was performed in 19 patients (median 8 months, range 12 days to 28 months) and 11 patients required redilatation (17 stents). CONCLUSIONS: Stent implantation is technically feasible in infants and under specific circumstances may provide an alternative to surgical palliation or avoid reoperation. The long term impact on clinical course, however, involves further interventions directed at stent management.

Arterial Occlusive Diseases↗
PubMed1995

Determinants of success in pediatric cardiac patients undergoing extracorporeal membrane oxygenation.

BACKGROUND: The purpose of this retrospective study is to determine the possible predictors of successful cardiac recovery using extracorporeal membrane oxygenation (ECMO) and the practical limits of ECMO support. METHODS: Information was gathered on 31 consecutive children with myocardial failure who could not be resuscitated with other means and underwent ECMO at the Hospital for Sick Children before January 1994. RESULTS: Of the children who underwent ECMO as a means of cardiac rescue, 14/31 (45%) were weaned successfully. Two distinct groups of children were evident based on their initial indications for ECMO: those who had postcardiotomy myocardial dysfunction (n = 25) and those with cardiomyopathy or myocarditis (n = 6). Children with residual defects after cardiotomy (n = 10) did not survive ECMO. Four of the 6 children with cardiomyopathy or myocarditis were weaned successfully. In either group of patients ECMO support beyond 6 days failed to resuscitate the myocardium; all attempts to violate this "time barrier" in our study inevitably failed. CONCLUSIONS: Postcardiotomy residual defects are a contraindication to ECMO. If children with residual defects are excluded, successful weaning from ECMO can be achieved in almost 70%, with almost all recovery occurring with the first 6 days of ECMO.

Cardiac Catheterization↗
PubMed1995

Trends in pediatric cardiac pacing.

OBJECTIVE: To review retrospectively a 31-year experience with pediatric cardiac pacing at the University of Toronto, with an emphasis on the changing trends in pacemaker implantation in infants and children. DATA SOURCE: Data were obtained from the pediatric pacemaker follow-up clinic at The Hospital for Sick Children, Toronto, Ontario or from the referring pediatric centre. Follow-up was normally continued until the death of the child or referral to an adult hospital at age 18 years. PATIENT SELECTION: The study comprised 397 children (under 18 years of age) who underwent initial pacemaker implantation at The Hospital for Sick Children between January 1962 and December 31, 1992. Follow-up was 99% complete (five children lost) and ranged from one month to 32 years (mean 6.5 years). DATA SYNTHESIS: The use of endocardial versus epicardial leads increased significantly over time (P < 0.001). In addition, significantly more children receiving pacemakers had sick sinus syndrome (P < 0.001). No difference in survival was found between children paced by endocardial versus epicardial leads or between children paced for sick sinus syndrome versus atrioventricular block. The frequency of exit block, by lifetable analysis, did not differ between children who received epicardial versus endocardial leads. CONCLUSIONS: Guidelines for permanent pacemaker implantation in children continue to evolve as developments in lead technology alter trends in pediatric cardiac pacing.

Adolescent↗
PubMed1995

The clinician-information interface.

Technical advances now enable rapid data collection into sophisticated information systems, and the vision of the electronic patient record may soon be a reality. However, despite advances in the human-computer interface, little attention has been given to the structure of the clinical data to be collected in the electronic record. The 'clinician-information interface' embraces the cognitive, analytical, and decision processes that the clinician will need to undergo to capture clinical data in a structured, meaningful, and analyzable form. This paper discusses some of the issues which need to be addressed if the emerging opportunities for a detailed capture of clinical data are to be maximized.

Ambulatory Care↗
PubMed1994

Postoperative follow-up of fibromuscular subaortic stenosis.

OBJECTIVES: This study attempted to determine whether early subaortic resection at lower levels of obstruction reduces the rate of recurrence of subaortic stenosis or reduces secondary damage to the aortic valve, or both. BACKGROUND: Fibromuscular subaortic stenosis is a progressive condition, and at present it is unclear whether early operation reduces the recurrence rate along with decreasing the incidence of aortic insufficiency. METHODS: Thirty-seven patients with fibromuscular subaortic stenosis and no other significant cardiac abnormality who underwent open subaortic resection were evaluated. The preoperative, early and late postoperative catheterization or echocardiographic findings as well as the operative reports were reviewed. The median age at operation was 6.4 years (range 1.1 to 17.3). The entire group has been followed up postoperatively for a median of 5.2 years (range 1.1 to 11). Mean systolic gradients across the left ventricular outflow tract were used for the purpose of this study. RESULTS: There was a significant correlation between the preoperative mean systolic gradient and the incidence of preoperative aortic regurgitation and late postoperative aortic valve thickening as well as the incidence and degree of late postoperative aortic regurgitation. Late postoperative gradient and degree of aortic regurgitation correlated significantly with the follow-up interval. Aortic regurgitation was progressive in some patients despite subaortic resection. A preoperative mean gradient > 30 mm Hg provided a reasonable cutoff for the likelihood postoperatively of needing a reoperation, having a postoperative shelf, a thickened aortic valve, moderate aortic regurgitation or a gradient of > 10 mm Hg. CONCLUSIONS: Our results suggest that although early subaortic resection may not reduce the rate of recurrence of fixed subaortic stenosis, it is likely to reduce acquired damage to the aortic valve.

Adolescent↗
PubMed1994

Small aortic root in childhood: surgical options.

Aortic valve replacement in the pediatric population is complicated by the often complex nature of the left ventricular outflow tract obstruction. Techniques to enlarge the annulus frequently are necessary. From 1977 to 1991, 32 children underwent an annular enlargement procedure at The Hospital for Sick Children, Toronto. During this same era, 110 children underwent a total of 138 aortic valve replacements. Eleven had the annulus enlarged with a posterior patch technique and implantation of a valve (mechanical 8, porcine heterograft 2, homograft 1) ranging from 20 to 25 mm in diameter. Twenty-two children had an anterior annular enlargement (aortoventriculoplasty) and aortic valve replacement with a valve (mechanical 8, porcine 2, homograft 12) 12 to 27 mm in diameter. One child had a posterior patch enlargement performed, followed by a second operation involving anterior annular enlargement. There was one early death in the posterior annuloplasty group and one late death due to failure of a bioprosthetic valve. There were five hospital deaths in the anterior annuloplasty group (22%; 70% confidence interval [CI], 14% to 32%) and two late deaths. Actuarial survival for the 32 children was 78% (70% CI, 70% to 86%) at 5 years and 65% (70% CI, 48% to 82%) at 10 years after repair. Younger children (age less than 1 year) had a significantly worse survival at 5 years (33%; 70% CI, 14% to 52%) than older children (88%; 70% CI, 82% to 95%). The survivors are well, and no reoperations have been necessary because of the children's outgrowing their valve.

Adolescent↗
PubMed1994

Risk factors for atrial tachyarrhythmias after the Fontan operation.

OBJECTIVES: The purpose of this study was to define the incidence and risk factors for atrial tachyarrhythmias after the Fontan operation. BACKGROUND: Atrial tachyarrhythmias cause morbidity after the Fontan operation. Causative factors may be affected by the type of systemic to pulmonary connection. METHODS: The Fontan operation was performed in 270 consecutive patients between 1982 and 1992. The mean age at operation was 7.0 +/- 4.3 years. Direct atriopulmonary connection was used in 138 patients (51%), total cavopulmonary connection in 94 (35%) and right atrial to right ventricular connection in 38 (14%). RESULTS: Atrial tachyarrhythmias were seen early postoperatively in 55 patients (20%), preoperative atrial tachyarrhythmia being the only risk factor. Follow-up was achieved for 228 early survivors (97%) at a mean interval of 4.4 years. There were 20 late deaths. Late atrial tachyarrhythmias were noted in 29% of patients who received an atriopulmonary connection, 14% of those who received a total cavopulmonary connection and 18% of those who received a right ventricular connection (p < 0.02). Significant risk factors as determined by univariate and multiple logistic regression analysis were atriopulmonary connection type (odds ratio 0.40 for total cavopulmonary relative to atriopulmonary connection [p < 0.05] and 0.37 for right ventricular relative to atriopulmonary connection [p = 0.08]), longer follow-up interval (odds ratio 1.32 for each consecutive year [p < 0.002]) and atrial tachyarrhythmia in the operative period (odds ratio 6.31 [p < 0.0001]). CONCLUSIONS: Early postoperative atrial tachyarrhythmias, length of follow-up and atriopulmonary connection are significant independent risk factors for the presence of late atrial tachyarrhythmias.

Analysis of Variance↗
PubMed1994

Oxyradical-induced antioxidant and lipid changes in cultured human cardiomyocytes.

Because the chronically cyanotic myocardium is thought to be more susceptible to oxyradical injury than the noncyanotic myocardium during cardiovascular surgery, we studied the oxyradical susceptibility of human ventricular cardiomyocytes cultured at high and low oxygen tension (PO2) levels. Passage 4 tetralogy of Fallot cardiomyocytes were cultured at PO2 levels of 150 and 40 mmHg for 14 days and then exposed for 10 min to superoxide radicals. The cellular levels of ATP, phospholipid fatty acids, phospholipid conjugated dienes, alpha-tocopherol, and activities of superoxide dismutase, catalase, and glutathione peroxidase were measured. ATP levels decreased more markedly in the low-PO2 group. Although the decrease in alpha-tocopherol levels was similar for both groups, phospholipid conjugated diene formation and phospholipid unsaturated fatty acid loss was greater in the low-PO2 cells. Glutathione peroxidase activity was rapidly inhibited. Superoxide dismutase activity was unaffected, and catalase activity was inhibited by no more than 50%. Although extracellular superoxide dismutase with catalase did not inhibit phospholipid conjugated diene formation and phospholipid breakdown, extracellular glutathione peroxidase with reduced glutathione did limit phospholipid damage. With the occurrence of membrane lipid peroxidation, the decreased glutathione peroxidase activity in the cyanotic tetralogy of Fallot myocardium determines cardiomyocyte membrane susceptibility to oxidant injury.

Cells, Cultured↗
PubMed1994

Thrombin regulation in congenital heart disease after cardiopulmonary bypass operations.

Children with cyanotic congenital heart disease who undergo operation with cardiopulmonary bypass are at increased risk of thromboembolic or hemorrhagic complications, or both. Regulation of thrombin, a key enzyme in coagulation, is essential in preventing these complications. We therefore examined the in vitro capacity of plasma from 15 children with cyanotic congenital heart disease to generate thrombin and to inhibit 125I-thrombin before and after cardiopulmonary bypass. We also assessed whether thrombin had been generated in vivo by assaying levels of fibrinogen, thrombin-antithrombin III complexes, and D-dimer. Plasma levels of the thrombin inhibitors, antithrombin III, alpha-2-macroglobulin, and heparin cofactor II were also measured. Thrombin regulation was normal before operation. After cardiopulmonary bypass, the in vitro capacity to generate thrombin decreased by 50%, and this was primarily a result of hemodilution (31%). Similar postoperative decreases were noted in the levels of antithrombin III, heparin cofactor II, and alpha-2-macroglobulin (26% to 45%). However, the total in vitro plasma thrombin inhibitory capacity decreased by only 13%. Levels of thrombin-antithrombin III and D-dimer increased after operation, indicating that thrombin had been generated and inhibited in vivo. Clinically, there were no thromboembolic complications although six patients required replacement therapy for excessive small-vessel bleeding. In conclusion, thrombin regulation is significantly altered after cardiopulmonary bypass. Although thrombin is generated in vivo, the total residual capacity to do so is impaired because of hemodilution. Despite a concomitant decrease in thrombin inhibitor levels, the total residual in vitro capacity of plasma to inhibit thrombin is relatively spared. This suggests that after cardiopulmonary bypass the risk of hemorrhagic complications after an additional hemostatic challenge is relatively greater than the risk of thrombotic complications. This might be reflected in the predominance of hemorrhagic complications in our patients.

Adolescent↗
PubMed1993

Identification of oxygen responsive elements in the 5'-flanking region of the human glutathione peroxidase gene.

The -1389 to +588 region of the human genomic glutathione peroxidase gene (hgpx1) was amplified using the polymerase chain reaction. This DNA fragment was cloned and sequenced, and various deletion constructs derived from the hgpx1 5'-flanking region were fused to the chloramphenicol acetyltransferase gene. These reporter genes were analyzed in transient transfection assays using primary cultured human ventricular cardiomyocytes obtained from patients with tetralogy of Fallot. Two distinct regions upstream from the transcription start site, which was determined using S1 nuclease analysis, were identified to be responsive to the oxygen tension in culture (pO2 values of 150 or 40 mm Hg). Methylation interference footprinting assays revealed proteins closely apposed to two sequences located at -1232 to -1213 and -282 to -275. We have designated these oxygen responsive elements ORE1 and ORE2, respectively. Gel mobility shift assays using double-stranded oligonucleotides corresponding to each site have demonstrated the formation of specific complexes using both cultured human cardiomyocyte and HeLa nuclear extracts. ORE1 and ORE2 bind disparate proteins with equal precision as bound complexes could be competed away with identical sequences but not with either the other ORE or an unrelated sequence. Insertion of these oxygen responsive elements into a reporter gene governed by a SV40 promoter similarly regulated chloramphenicol acetyltransferase activity according to the oxygen tension in culture.

Base Sequence↗
PubMed1993

Heparin therapy during cardiopulmonary bypass in children requires ongoing quality control.

Heparin therapy for children undergoing cardiopulmonary bypass (CPB) is monitored in the operating room by automated whole blood activated clotting times (ACT). For many years our institution used Hemochron (HC) ACT machines but changed to HemoTec (HT) ACT machines because they required a smaller blood sample and provided results in duplicate. When HemoTec ACT machines were introduced at our institution, the surgical team was concerned that increased amounts of heparin were being administered to our patients during CPB. This study was conducted to investigate the potential mechanisms responsible for these clinical observations. First, we compared ACT values on ex vivo blood samples from 20 consecutive pediatric patients (6 samples each) during CPB. The HC ACT values were significantly and systematically increased over HT ACT values (HC: 750 +/- 40 vs HT: 418 +/- 26, Mean +/- SEM, p < 0.01). 94% of all HC ACT values were above 450 s compared to only 27% of HT ACT values. If HT ACT values had been used for patient monitoring, all patients would have received more heparin to achieve ACT values above 450 s. The two machines reported similar ACT values when heparin was added in vitro to whole blood (0.1-5.0 units/ml), (HC: Y = 98X + 104, r2 = 0.93 HT: Y = 82X + 109, r2 = 0.94). Heparin concentrations in our patients following a bolus of 300 U/kg of heparin, but prior to CPB were 3.2 +/- 0.07 units/ml. Following the initiation of CPB, heparin concentrations decreased to 1.3 +/- 0.05, reflecting, in part hemodilution by the pump prime (1 U of heparin/ml).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Ventriculo-coronary arterial connections in pulmonary atresia with intact ventricular septum, and their influences on ventricular performance and clinical course.

To define the influence of ventriculo-coronary (VC) arterial connections on ventricular performance and late outcome, and associated wall motion abnormalities in patients with pulmonary atresia and intact ventricular septum, 21 patients aged > or = 6 months (mean 4.3 +/- 3.3 years, range 0.5 to 11.8) were reviewed. Ventricular volumes, mass, systolic functional variables and regional wall motion were measured using biplane ventricular cineangiography. Fourteen patients had VC connections, 11 of whom had coronary artery abnormalities. In comparing patients with VC connections and coronary abnormalities (group I) with those without coronary abnormalities (group II), no hemodynamic differences were observed, except the expected increased right-to-left ventricular systolic pressure ratio in group I (1.33 +/- 0.24 vs 0.72 +/- 0.50). Wall motion abnormalities were observed in 8 patients (7 in group I, and only 1 in group II [p < 0.05]). Ejection fraction in patients with abnormal wall motion was significantly lower (50 +/- 6% vs 57 +/- 7%; p < 0.05) and ventricular systolic pressure ratio significantly higher (1.46 +/- 0.16 vs 0.80 +/- 0.45; p < 0.05) than in those with normal wall motion. Five patients died during follow-up, all of whom had wall motion abnormalities. The findings suggest that patients with VC connections and coronary abnormalities have a higher incidence of wall motion abnormalities, which may reflect ongoing ischemia, and are at risk for late death.

Analysis of Variance↗
PubMed1993

Survival analysis of infants under age 18 months presenting with tetralogy of Fallot.

The trend to perform early primary repair of tetralogy of Fallot prompted us to review our experience in the current era with the traditional protocol consisting of palliation during infancy, if required, and repair after infancy. During a 10-year period, 270 infants with tetralogy of Fallot presented aged less than 18 months. Thirteen infants (4.8%) had major noncardiac lesions that precluded definitive care for their congenital heart disease. Twenty infants (7.4%) had major associated cardiac lesions (atrioventricular septal defect or absent pulmonary valve syndrome, or both). Survival in this group was poor, with only 58% +/- 12% reaching the age of 10 years. Four of the seven deaths occurred before intracardiac repair was performed. The remaining 237 infants presented with isolated tetralogy of Fallot. Eight-nine percent +/- 2.3% survived to age 10 years. Sixty percent of these infants required palliation, and survival in these infants did not differ from that in those who never required palliation. However, 19 infants (8%) required palliation in the first month of life. In these children, survival to age 10 years was significantly lower (77%), secondary palliation was frequently required (n = 11), and a transannular patch or conduit at the time of repair (10 of 14 patients) was more likely needed than it was in children who had not undergone a palliative procedure during the neonatal period. The survival in infants with tetralogy of Fallot is unlikely to be different, regardless of whether primary repair or a staged repair is carried out. The quality of survival, including the exercise capability and absence of arrhythmias, must be assessed to determine which protocol is superior.

Abnormalities, Multiple↗
PubMed1993

Regional ventricular wall motion abnormalities in tricuspid atresia after the Fontan procedure.

OBJECTIVES: The purpose of this study was to determine whether wall motion abnormalities are present before or after the Fontan procedure in patients with a univentricular heart of the left ventricular type with an absent right atrioventricular valve connection (tricuspid atresia) and to assess the impact of such abnormalities on ventricular performance and clinical outcome. BACKGROUND: Normal systolic and diastolic ventricular function is critical for a successful Fontan repair. However, there have been no previous studies addressing the relation between regional ventricular function and hemodynamic factors. METHODS: Thirty-seven pediatric patients were studied with biplane ventricular cineangiography. There were 20 male and 17 female patients whose mean age at the time of the Fontan operation was 6.5 +/- 3.5 years (range 2.5 to 15.6). Eighteen patients were studied preoperatively, 25 at > 1 year postoperatively and 6 serially. Wall motion was assessed by a centerline method. Normal ranges for wall motion and other variables were established from 25 normal subjects. RESULTS: Wall motion abnormalities were observed in 2 of 18 patients preoperatively and in 11 of 25 patients postoperatively. Age at operation and ventricular volumes did not differ between postoperative patients who had normal (group I, 14 patients) or abnormal (group II, 11 patients) wall motion. However, ventricular mass and the mass/volume ratio were significantly greater and systolic variables and cardiac index were significantly lower in group II versus group I. Two patients in group I were considered to have a clinically poor outcome (persistent heart failure), and five in group II had heart failure, including one who died late. CONCLUSIONS: These observations suggest that postoperative regional wall motion abnormalities in this setting are not rare, may be related to excessive hypertrophy and may contribute to cardiac dysfunction and a poor clinical outcome.

Adolescent↗
PubMed1993

Influence of ventricular morphology on diastolic filling performance in double-inlet ventricle after the Fontan procedure.

OBJECTIVES: The purpose of this study was to define the influence of dominant chamber morphology on ventricular performance after the Fontan procedure in patients with double-inlet ventricle. BACKGROUND: Previous studies have reported the impact of ventricular morphology on preoperative ventricular performance and surgical outcome. However, the influence on postoperative ventricular performance has not been addressed. METHODS: Twenty-six clinically asymptomatic patients > 1 year after repair (mean age at procedure 6.1 +/- 3.7 years) were evaluated with ventricular cineangiography and radionuclide blood pool studies (18 with a dominant left ventricular morphology [LV group], 8 with a dominant right ventricular morphology [RV group]) and compared with normal control subjects. RESULTS: Ventricular volume, mass and systolic variables were similar between patient groups. In the LV group, however, the mass/volume ratio was significantly elevated compared with values in control subjects (1.11 +/- 0.28, 0.97 +/- 0.19, p < 0.05), whereas this ratio in the RV group (0.90 +/- 0.11) was within the normal range and significantly lower than that in the LV group (p < 0.05). Mean right atrial and pulmonary artery pressures in the RV group were significantly higher than those in the LV group (p < 0.05). Peak filling rates (2.87 +/- 0.70, 2.41 +/- 1.15 and 3.84 +/- 0.51 end-diastolic volume/s [LV and RV groups and control subjects, respectively]) were significantly lower in both groups than in control subjects (p < 0.001), without intergroup difference. CONCLUSIONS: Ventricular filling abnormalities after atrial to pulmonary anastomosis are common regardless of the type of dominant ventricular morphology, and these abnormalities in patients with dominant right ventricular morphology do not coexist with ventricular hypertrophy. Such diastolic abnormalities may be related to either intrinsic myocardial or acquired factors, not to excessive hypertrophy alone. Those differences may become clinically more apparent with longer follow-up and may raise concerns over the long-term course.

Cardiac Catheterization↗
PubMed1993

Experience with the Damus-Kaye-Stansel procedure for children with Taussig-Bing hearts or univentricular hearts with subaortic stenosis.

Anastomosis of the pulmonary artery to the ascending aorta is the essence of a technique for repair of complete transposition proposed by Damus, Kaye, and Stansel. Our Institutional experience with the Damus-Kaye-Stansel procedure (DKS) is limited to 9 children with double-outlet right ventricle and 38 with univentricular heart plus subaortic stenosis. Thirty-eight children (81%) survived the DKS procedure, and there were three late deaths during the mean follow-up of 3.1 years. Five-year survival is 72% (+/- 8%). All six children surviving a DKS and biventricular repair of double-outlet right ventricle have required conduit replacement at a mean interval of 46 months. In the children with univentricular heart, relief of subaortic stenosis with the DKS was successful in all except two. The late function of the semilunar valves is of concern, as 36% of the aortic and 52% of the pulmonary valves have some degree of incompetence.

Aorta↗