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Biomedical subjects

W G Williams

Publications and source records attributed to W G Williams.

At least 73 records · Page 4Linked to original sources
PubMed1998

Mortality in potential arterial switch candidates with transposition of the great arteries.

OBJECTIVES: We reviewed the factors contributing to or causing death before surgery in neonates with d-transposition of the great arteries (TGA) despite anatomy suitable for the arterial switch operation (ASO) to develop strategies to minimize preoperative attrition. BACKGROUND: Currently the ASO for neonates with TGA carries a low operative mortality. However, there is a paucity of information regarding the patients who die before the ASO. Strategies to ensure survival to operation are of importance to improve overall outcome. METHODS: We reviewed all neonates with TGA and patent forearm ovale (PFO) < or = 2 mm, a birthweight <2 kg, or who died before surgery, between 1988 and 1996. RESULTS: We identified 12 out of 295 neonates with TGA (4.1%) with anatomy suitable for the ASO who died prior to surgery. All had TGA/intact ventricular septum (IVS) and presented with a severely restrictive PFO. In 11 of 12 cases the cause of death was attributed to the sequelae of profound hypoxemia from inadequate mixing. Contributing factors were prematurity, 41.7%; severe respiratory distress syndrome, 25%; and persistent pulmonary hypertension of the newborn (PPHN), 16.7%. All patients received prostaglandin E1 (PGE1) infusion. Urgent balloon atrial sepstostomy (BAS) was performed in 66.7% with improved oxygenation. No cases were diagnosed prenatally. In contrast, all patients with a PFO < or = 2 mm who survived to ASO had a significantly better response to PGE1 infusion (p=0.03) than nonsurvivors. The ASO was accomplished without mortality in four of nine with a weight <2 kg. CONCLUSIONS: Of those neonates admitted with TGA, 4.1% died before surgery. Eleven of 12 (3.7%) died due to consequences of inadequate interatrial mixing despite PGE1 infusion. Earlier diagnosis and BAS are critically important in determining survival. Early ASO may improve survival in patients weighing <2 kg. Prenatal diagnosis with delivery in a high-risk obstetrical unit with facilities for immediate BAS and supportive therapy for pulmonary hypertension and ventricular failure may be necessary to salvage this group of patients.

Cause of Death↗
PubMed1998

Anomalous origin of one pulmonary artery from the ascending aorta: 36 years' experience from one centre.

UNLABELLED: Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16). CONCLUSION: While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.

Aorta↗
PubMed1998

Transzygomatic coronoidectomy through an extended coronal incision for treatment of trismus due to an osteochondroma of the coronoid process of the mandible.

Osteochondroma is the most common benign tumor of the skeletal system. In the craniofacial skeleton, however, osteochondroma is uncommon. Even more rare is osteochondroma of the coronoid process. A review of the literature reveals only 31 reported cases of osteochondroma of the coronoid process of the mandible. The preponderance of patients were young men (67.7%) and most presented with a facial deformity. As a benign tumor, the problems with surgical treatment have dealt with the inaccessibility of the lesion and hence the surgical approach of choice. The previous surgical approaches have either been intraoral, external, or a combination of both. We present a transzygomatic approach via a coronal incision that gives excellent access and a good cosmetic result, and protects the facial nerve from damage.

Bone Plates↗
PubMed1998

The Le Fort I-palatal split approach for skull base tumors: efficacy, complications, and outcome.

The Le Fort I, split-palate approach provides intraoral surgical access to a region of the midline skull base ranging from the upper clivus to the second cervical vertebra. Although this approach provides perhaps the largest exposure of all the intraoral techniques, there is little concerning it in the literature. Furthermore, there are no detailed descriptions of case histories, complications, and outcome. The purpose of this study was to evaluate this procedure's effectiveness and identify associated complications as well as outcome. Seven cases of patients who underwent eight skull base surgeries using the Le Fort I, split-palate approach were evaluated retrospectively. Particular attention was paid to postoperative occlusion, speech, mouth opening, infection, tumor recurrence, postoperative recovery period, and viability of maxillary bone and teeth. Follow-up ranged from 4 months to 7 years with a mean of 3.9 years. Pathologic diagnoses included three chordomas (two recurrent), one recurrent meningioma, one liposarcoma, one chondrosarcoma, and one inflammatory mass. One patient with chordoma underwent a second operation using the same approach. No deaths or major neurologic problems related to the procedure occurred. One patient who had known local metastases at the time of operation died several months after surgery. All other patients are still living. Duration of hospital stay ranged from 5 to 53 days with a mean of 25.4 days. Postoperative complications included one case of meningitis with an associated cerebrospinal fluid leak, three cases of malocclusion, one case of velopharyngeal insufficiency, and one extracranial soft-tissue infection. The case of meningitis was successfully treated by antibiotics. The malocclusions were corrected by conservative treatment. No problems with mouth opening or bone or tooth viability occurred. Tumor recurred in both cases in which malignancy was involved, whereas only one recurrence was noted among the benign cases. It is concluded that the Le Fort I-palatal split technique is a relatively safe and effective means for approaching midline skull base tumors. Several modifications to the surgical protocol and surgical technique are detailed herein.

Adult↗
PubMed1998

Myocardial aerobic metabolism is impaired in a cell culture model of cyanotic heart disease.

A human pediatric cardiomyocyte cell culture model of chronic cyanosis was used to assess the effects of low oxygen tension on mitochondrial enzyme activity to address the postoperative increase in lactate and decreased ATP in the myocardium and the high incidence of low-output failure with restoration of normal oxygen tension, after technically successful corrective cardiac surgery. Chronically hypoxic cells (PO2 = 40 mmHg for 7 days) exhibited significantly reduced activities for pyruvate dehydrogenase, cytochrome-c oxidase, succinate cytochrome c reductase, succinate dehydrogenase, and citrate synthase. The activity of NADH-cytochrome c reductase was unaffected. Lactate production and the lactate-to-pyruvate ratio were significantly greater in hypoxic cardiomyocytes. Western and Northern analysis demonstrated a decrease in the levels of various mRNA and corresponding polypeptides in hypoxic cells. Thus hypoxia influences mitochondrial metabolism through acute and chronic adaptive mechanisms, reflecting allosteric (posttranscriptional) and transcriptional modulation. Transcriptional downregulation of key mitochondrial enzyme systems can explain the insufficient myocardial aerobic metabolism and low-output failure in children with cyanotic heart disease after cardiac surgery.

Adenosine Triphosphate↗
PubMed1997

Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation.

BACKGROUND: Several perioperative risk factors influence outcomes after repair of complete atrioventricular septal defects. This study was conducted to determine their association with perioperative mortality and need for reoperation. METHOD AND RESULTS: Between July 1982 and February 1995, 363 children underwent defect repair (median age, 8.3 months; mean, 17.4+/-1.3). Tetralogy of Fallot was present in 21 patients, double-outlet right ventricle in 4, subaortic stenosis in 8, ventricular hypoplasia in 8, coarctation in 5, atrial isomerism in 2, and other congenital anomalies in 9. Down's syndrome was present in 235 (65%). One-patch technique was applied in 99, two-patch in 243. During repair, the anterior bridging leaflet was divided in 12, the posterior bridging leaflet in 31, both leaflets in 71, and neither in 249. Left atrioventricular valve (LAVV) cleft was closed partially in 181 and completely in 112. Early mortality was 10.5%; 10-year survival, 83% (95% confidence interval, 0.79 to 0.87). At 10 years, freedom from reoperation for LAVV repair was 86%; LAVV replacement, 90%; subaortic stenosis, 95%; residual ventricular septal defect, 97%; permanent pacemaker insertion, 98%; and other types of reoperation, 95%. At the time of operation, greater age, shorter ischemic time, absence of a double-orifice LAVV, and cleft closure were found to be significant independent predictors of survival. CONCLUSIONS: Repair of atrioventricular septal defects has acceptable early and late mortality and a low incidence of reoperation. Double-orifice LAVV remains a risk factor. Repairs that include complete cleft closure may confer better survival.

Follow-Up Studies↗
PubMed1997

Sustained ventricular tachycardia in adult patients late after repair of tetralogy of Fallot.

OBJECTIVES: We sought to determine the features associated with sustained monoform ventricular tachycardia (VT) in adult patients late after repair of tetralogy of Fallot (TOF) and to review their management. BACKGROUND: Patients with repair of TOF are at risk for sudden death. Risk factors for ventricular arrhythmia have been identified from patients with ventricular ectopic beats because of the low prevalence of sustained VT. METHODS: From a retrospective chart review of patients assessed between January 1990 and December 1994, 18 adult patients with VT were identified and compared with 192 with repaired TOF free of sustained arrhythmia. RESULTS: There was no significant difference in age at repair, age at follow-up or operative history. Patients with VT had frequent ventricular ectopic beats (6 of 9 vs. 21 of 101), low cardiac index ([mean +/- SD] 2.4 +/- 0.4 vs. 3.0 +/- 0.8) and more structural abnormalities of the right ventricle (outflow tract aneurysms and pulmonary or tricuspid regurgitation) than control patients. Electrophysiologic map-guided operation was performed in 10 of 14 patients who required reoperation. VT has reoccurred in three of these patients. Four patients did not undergo operation (three received amiodarone; one underwent defibrillator implantation). Two patients with VT also had severe heart failure and died. CONCLUSIONS: Most patients with VT late after repair of TOF have outflow tract aneurysms or pulmonary regurgitation, or both. These patients have a greater frequency of ventricular ectopic beats than arrhythmia-free patients after repair of TOF. A combined approach of correcting significant structural abnormalities (pulmonary valve replacement or right ventricular aneurysmectomy, or both) with intraoperative electrophysiologic-guided ablation may reduce the potential risk of deterioration in ventricular function and enable arrhythmia management to be optimized.

Adult↗
PubMed1997

Overexpression of transforming growth factor-beta1 and insulin-like growth factor-I in patients with idiopathic hypertrophic cardiomyopathy.

BACKGROUND: Idiopathic hypertrophic cardiomyopathy (HCM) is characterized by regional myocardial hypertrophy. To investigate involvement of growth factors on myocardial hypertrophy in HCM patients, we evaluated gene expression and cellular localization of transforming growth factor-beta1 (TGF-beta1), insulin-like growth factors (IGF-I and IGF-II), and platelet-derived growth factor-B (PDGF-B) in ventricular biopsies obtained from patients with HCM (n=8), aortic stenosis (AS) (n=8), or stable angina (SA) (n=8) and from explanted hearts with ischemic cardiomyopathy (TM) (n=7). METHODS AND RESULTS: Levels of TGF-beta1, IGF-I, IGF-II, and PDGF-B transcripts were quantified with the use of multiplex RT-PCR. Glyceraldehyde 3-phosphate dehydrogenase was used as an internal standard. Antibodies against TGF-beta and IGF-I were used to localize their peptides within the myocardium. Antisense and sense (control) cRNA probes of TGF-beta1 and IGF-I, labeled with digoxigenin, were used to localize the growth factor transcripts by in situ hybridization. mRNA levels (densitometric ratio of growth factor/glyceraldehyde-3-phosphate dehydrogenase) of TGF-beta1 and IGF-I in HCM (0.75+/-0.05 and 0.85+/-0.15, respectively; mean+/-1 SEM) were significantly (P<.01 for all groups) elevated in comparison with non-HCM myocardium (AS: 0.38+/-0.07, 0.29+/-0.06; SA: 0.32+/-0.04, 0.18+/-0.05; TM: 0.25+/-0.03, 0.15+/-0.03). mRNA levels of TGF-beta1 and IGF-I in the hypertrophic AS myocardium were greater (P=.02, P=.05) than those in the explanted myocardium (TM). Immunohistochemical and in situ hybridization studies showed increased expression of TGF-beta1 and IGF-I in the HCM cardiomyocytes. CONCLUSIONS: Gene expression of TGF-beta1 and IGF-I was enhanced in idiopathic hypertrophic cardiomyopathy and may be associated with its development.

Aged↗
PubMed1997

Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn.

The clinical impact of transcatheter perforation and dilatation of the right ventricular outflow tract in neonates with pulmonary atresia and intact ventricular septum was reviewed. Between April 1992 and December 1994, 8 neonates underwent transcatheter perforation of the right ventricular outflow tract. Radiofrequency energy was employed in 6 patients and wire perforation in 2 patients. Mean patient age at intervention was 1.9 +/- 0.6 days and weight 3.4 +/- 0.5 kg. Median tricuspid valve annulus was 10.9 mm (range: 4.0-13.0 mm) and Z-value -0.85 (range: -4.5-1.0). The mean right ventricular systolic pressure fell from 117 +/- 16 to 55 +/- 15 mm Hg (P < 0.0001), and the right ventricular to aortic pressure ratio decreased from 1.81 +/- 0.33 to 0.82 +/- 0.28 (P < 0.0001). The arterial duct was patent in all. No acute complications occurred. Aortopulmonary shunts were performed in 7 patients at a median 6 days (range: 3-23 days) following catheterization. One patient developed sepsis and died after surgical resection of infected tissue, while a second patient died of a blocked aortopulmonary shunt 17 months following discharge. Median follow-up for the 6 surviving patients was 8 months (range: 4-32 months). One patient has achieved and a second is awaiting biventricular repair. Transcatheter perforation appears to be a promising form of therapy in selected patients with pulmonary atresia, and potentially facilitates algorithms leading to a biventricular repair.

Angioplasty, Balloon↗
PubMed1997

Pediatric primary benign cardiac tumors: a 15-year review.

An increase in the incidence of primary cardiac tumors has been reported since the development or enhancement of noninvasive imaging modalities. We identified 56 children with primary cardiac tumors. Forty-four (78%) children had rhabdomyomas, 6 (11%) fibromas, 1 (2%) pericardial teratoma, 1 (2%) epicardial lipoma, 1 (2%) multicystic hamartoma, and 3 (5%) unspecified tumors. The mean age at diagnosis was 19 +/- 35 months (median 4.7 months, range 0.03 to 204 months), excluding 12 patients who were given the diagnosis before birth. Among 27,640 patients assessed for cardiac disease, the incidence of tumors was 0.06% (1980 to 1984), 0.22% (1985 to 1989), and 0.32% (1990 to 1995). Diagnosis was made in 55 of 56 patients by echocardiography. Catheterization was performed in five patients and magnetic resonance imaging in nine. No tumor-related deaths occurred. Nine patients had surgery because of hemodynamically significant obstruction or arrhythmias. Partial or complete regression occurred in 24 (54%) of 44 patients with rhabdomyomas. Overall, the prognosis was excellent. Individualized surgery allowed early safe treatment of symptomatic tumors.

Child↗
PubMed1997

Effect of heparin loading during congenital heart operation on thrombin generation and blood loss.

BACKGROUND: The heparin protocols used during cardiopulmonary bypass (CPB) in children undergoing surgical repair for congenital heart disease are extrapolated from adult data. Studies are needed that assess the optimal heparin dosing in these children, whose heparin clearance is increased compared with that in adults. METHODS: We assessed the effects of two commonly used doses of heparin in the prime solution at the start of CPB operation on plasma heparin levels, on thrombin production (thrombin-antithrombin III complexes, prothrombin fragment 1 + 2, D-dimer, and antithrombin III), and on the risk of hemorrhage. Before CPB, 48 children with congenital heart disease received heparin intravenously in a loading dose of 300 U/kg, followed by either 1 U/mL of heparin in the prime (low-dose group: 22 patients-acyanotic, 9; cyanotic, 13) or 3 U/mL of heparin in the prime (group: high-dose, 26 patients-acyanotic, 15; cyanotic, 11). RESULTS: In all patients, CPB resulted in the generation of thrombin. The duration of CPB was a significant covariate factor for heparin levels (p = 0.002), thrombin production (p < 0.001), and postoperative blood loss (p < 0.001). In the patients in the high-dose group, the total heparin dose and the plasma heparin levels were higher (p = 0.0005 and 0.005, respectively) and the D-dimer levels tended to be lower (p = 0.06). The postoperative blood loss was higher in the cyanotic patients (p = 0.02; both high-dose and low-dose groups), with 2 cyanotic patients (1 in low-dose group, 1 in high-dose group) requiring reoperation, one of whom subsequently died. The increased heparin dose had no significant effect on the rate or volume of postoperative blood loss. CONCLUSIONS: Increasing the heparin dose in the prime solution from 1 to 3 U/mL increased the plasma heparin levels and showed a trend toward reducing the postoperative laboratory values indicative of fibrinolysis. Thrombin generation during CPB and the incidence of postoperative hemorrhage were not significantly altered. Larger randomized trials are needed to determine the optimal heparin-dosing regimen in patients with congenital heart disease.

Adolescent↗
PubMed1997

Pulmonary atresia with intact ventricular septum: results of the Fontan procedure.

BACKGROUND: Children with pulmonary atresia and an intact ventricular septum show a heterogeneous spectrum of cardiac anomalies. A biventricular repair is attainable in some; a Fontan procedure or a one-and-a-half ventricle is the only possible repair for others. Children with right ventricle-to-coronary artery connections, with or without right ventricle-dependent coronary artery blood flow, are a high-risk group. METHODS: Between May 1980 and December 1994, 22 children underwent a Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum at The Hospital for Sick Children, Toronto. The mean age was 5.8 years (median, 4.9 years). All children had had at least one pre-Fontan palliative procedure; 19 had two, and 7 of these had three or more. Right ventricle-to-coronary artery connections were present in 15 children, including 5 with right ventricle-dependent coronary artery blood flow. Thromboexclusion of the right ventricle was done in 10 children, with 7 undergoing it before and 3 at the time of the Fontan procedure. RESULTS: There were three early deaths (13.6%) and one late death. The actuarial survival at 10 years after the Fontan operation was 80%. Early postoperative complications occurred in 4 children. Follow-up was completed in all children at a mean of 4 years (range, 1 to 12.5 years) after the Fontan operation. Atrial arrhythmia occurred in 3 children, and permanent pacemakers were required in 4. CONCLUSIONS: Results of the Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum are satisfactory. Thromboexclusion of the right ventricle is indicated in the presence of right ventricle-to-coronary artery connections without right ventricle-dependent coronary artery blood flow. The right ventricle should not be decompressed or thromboexcluded in children with right ventricle-dependent coronary artery blood flow, and at the Fontan operation, saturated blood must enter the right ventricle.

Child, Preschool↗
PubMed1997

Pulmonary valve replacement late after repair of tetralogy of Fallot.

BACKGROUND: Pulmonary valve incompetence is usually well tolerated after tetralogy of Fallot repair but may result in late progressive right heart failure as manifested by increasing fatigue, dyspnea, and frequently arrhythmias. METHODS: All patients who underwent pulmonary valve replacement in our center late after repair of tetralogy of Fallot were reviewed. RESULTS: Eighty-five patients had elective pulmonary valve replacement late (median, 9.3 years) after repair. Operative risk was low (1.1%). Ninety percent of survivors are in New York Heart Association class I. Survival 10 years after pulmonary valve replacement is 95%, with 86% of the patients free of reoperation for valve failure. CONCLUSIONS: Pulmonary valve replacement is infrequently required after repair of tetralogy of Fallot. Pulmonary valve replacement may be performed electively with little risk; it improves symptoms of right heart failure and provides satisfactory long-term survival with low risk of early valve failure. As the population of patients who have had repair of tetralogy of Fallot ages, pulmonary valve replacement will become a more frequent consideration.

Adolescent↗
PubMed1997

Outflow obstruction after the arterial switch operation: a multiinstitutional study. Congenital Heart Surgeons Society.

OBJECTIVE: Our objectives were to discover whether outflow obstruction immutably accompanies the arterial switch operation and to identify factors that may decrease its prevalence. METHODS: Percutaneous or surgical reintervention for obstruction after an arterial switch was selected as an end point for obstruction. Its risk factors were identified by time-related multivariable analyses of yearly follow-up data from 514 neonates with simple transposition or transposition with ventricular septal defect entering 23 institutions before 15 days of age between January 1, 1985, and March 1, 1989. RESULTS: Sixty-two patients underwent 86 reinterventions for right-sided obstruction (83% free at 10 years) and six for left-sided obstruction (98% free at 10 years). After 2 years, right-sided obstruction occurred at a rate of about 1% per year and left-sided at a rate of about 0.1% per year. Right ventricular infundibular or valvular obstruction was associated with the aorta and pulmonary trunk positioned side-by-side, coexisting coarctation, use of prosthetic material in sinus reconstruction, one institution, and earlier institutional experience. Pulmonary trunk or pulmonary artery obstruction was associated with lower birth weight, left coronary artery arising from sinus 2, coronary explantation away from the transection site, three institutions, and earlier institutional experience. CONCLUSIONS: A risk-adjusted base incidence (0.5% per year) of reintervention for right-sided obstruction continues late after operation. It is due in part to congenital variability or abnormality of right ventricular outflow structures and to experience and surgeon variability resulting in suboptimal pulmonary trunk reconstruction. The same sources of variability probably affect the aortic root, but its native characteristics plus higher distending pressure make the base incidence considerably less (0.1% per year).

Cross-Sectional Studies↗
PubMed1997

The modified Blalock-Taussig shunt: clinical impact and morbidity in Fallot's tetralogy in the current era.

BACKGROUND: The Blalock-Taussig shunt is considered a low-risk management option for palliation in tetralogy of Fallot, but the morbidity associated with a Blalock-Taussig shunt can have a significant impact on patient care. We reviewed the outcome for this operation in the current era. METHODS: Between 1990 and 1994, 65 children with tetralogy of Fallot received a modified Blalock-Taussig shunt. Sixty patients who had follow-up angiography were assessed for clinical outcomes and shunt-related morbidity and mortality. From the same study period, 68 of 247 pediatric patients who underwent angiography and tetralogy repair, but did not receive palliation, were randomly selected to comprise a comparison group. RESULTS: Palliation was more likely in the presence of a complicated tetralogy malformation or if there was an associated medical condition. Median age at palliation was 58 days (range: 1 to 535 days). Ninety-five percent of shunts were right-sided. Self-limited morbidity complicated 11% of shunt operations. Significantly smaller distal right pulmonary arteries were observed in the palliated group before total repair compared with findings in the group without palliation and 33% of patients who underwent palliation had angiographic evidence of pulmonary artery distortion. Shunt stenosis was common and correlated with younger age at palliation. Shunt occlusion resulted in one death. Excluding noncardiac causes of death, overall survival was 90% in the palliated group versus 97% in the nonpalliated group (p = 0.09). CONCLUSIONS: Pulmonary artery hypoplasia and angiographic evidence of pulmonary artery distortion are common after initial palliation by a modified Blalock-Taussig shunt. Neonatal palliation was associated with significantly smaller pulmonary arteries before repair, which necessitated additional interventions.

Anastomosis, Surgical↗
PubMed1997

Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience.

OBJECTIVES: Our purpose was to assess the risk factors for late mortality, loss of sinus rhythm and atrial flutter after the Mustard operation. BACKGROUND: The Mustard operation provides correction of cyanosis with low surgical risk in transposition of the great vessels. However, right ventricular failure, loss of sinus rhythm, atrial flutter and death are frequent long-term complications. METHODS: Records of 534 children who underwent the Mustard operation at a single center since 1962 were reviewed for demographic, anatomic, electrocardiographic and physiologic predictors and outcomes. RESULTS: There were 52 early deaths (9.7%). Survival analysis was undertaken for 478 early survivors with a mean follow-up interval of 11.6 +/- 7.2 years. There were 77 late deaths (16.1%), with sudden death (n = 31) the most frequent cause. Survival estimates were 89% at 5 years and 76% at 20 years of age. Risk factors were an earlier date of operation, operative period arrhythmia and an associated ventricular septal defect. Risk (hazard) of late death declined in the first decade, with further peaks in the second decade. Sinus rhythm was present in 77% at 5 years and 40% at 20 years. Loss of sinus rhythm was associated with previous septectomy, postoperative bradycardia and late atrial flutter. Freedom from atrial flutter was 92% at 5 years and 73% at 20 years of age. Risk factors for atrial flutter were the occurrence of perioperative bradyarrhythmia, reoperation and loss of sinus rhythm during follow-up. Risk of atrial flutter demonstrates a late increase. CONCLUSIONS: Ongoing loss of sinus rhythm and late peaks in the risk of atrial flutter and death necessitate continued follow-up.

Arrhythmias, Cardiac↗