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Biomedical subjects

A Serraf

Publications and source records attributed to A Serraf.

At least 19 recordsLinked to original sources
PubMed2000

Congenital mitral stenosis with or without associated defects: An evolving surgical strategy.

BACKGROUND: Congenital mitral stenosis (CMS) remains a surgical challenge, particularly when it is associated with other heart defects. As in other groups of heart defects, there is a trend toward early single-stage complete repair, but the optimal surgical approach remains unanswered. METHODS AND RESULTS: This study was designed to analyze the evolution of surgical strategies in patients with CMS and associated defects through single-stage and staged repair. Between 1980 and 1999, 72 children were operated on for congenital heart defects, including CMS. Preoperative transmitral gradient was 12.6+/-7 mm Hg. Preoperatively, all the patients were NYHA class III to IV. Thirteen patients had an isolated CMS; in 59, it was associated with other heart defects, mainly ventricular septal defect (n=28) or multilevel left ventricular obstruction (n=41). In this group of patients, 33 had a staged approach, and 26 had a single-stage approach. Early mortality was 12.5% (9 patients). There were no deaths in the isolated CMS and single-stage repair groups. Logistic regression revealed that early mortality was influenced by association with left ventricular outflow tract obstruction (P:<0.001) and by use of a staged approach (P:<0.01). There was no late mortality in isolated CMS; there were 2 late deaths in the group of single-stage repair and 6 late deaths in the staged approach group (P:<0.01). Reoperation was required in 24 patients, mainly for residual mitral valve dysfunction or residual left ventricular outflow tract obstruction. Including the reoperations, 10 patients received a prosthetic mitral valve. At 15 years after surgery, survival was 69.6+/-7.5%, freedom from reoperation was 70.8+/-6.3%, and freedom from mitral valve replacement was 69+/-6%. CONCLUSIONS: Surgery for isolated CMS gives excellent early and long-term results. In patients with associated heart defects, a single-stage operation seems superior to a staged approach. Mitral valve replacement in this category of patients should be reserved as a salvage procedure.

Adolescent↗
PubMed2000

Systemic pulmonary shunts in neonates: early clinical outcome and choice of surgical approach.

BACKGROUND: Systemic pulmonary shunt remains a major strategy for the palliation of cyanotic lesions in neonates despite the associated morbidity and mortality. METHODS: Between March 1993 and December 1998, 79 systemic pulmonary shunts were performed in 75 neonates with cyanosis and severely reduced pulmonary blood flow. The mean age was 11.5 days and the mean weight, 3 kg. All neonates were dependent on duct flow and prostaglandin E1 infusion for adequate oxygenation. RESULTS: The systemic pulmonary shunt was performed through a right thoracotomy in 36 patients, left thoracotomy in 6, and median sternotomy in 33 patients. The 30-day mortality was 3 patients (4%). Univariate and logistic regression analyses revealed a weight less than 2 kg (p = 0.039) and preoperative mechanical ventilation (p = 0.008), to be predictors of early mortality, whereas pulmonary hypoplasia (p = 0.55), diagnostic group (p = 0.79), shunt size (p = 0.2), and surgical approach (p = 0.5) were not. There were seven episodes of shunt-related complications that required early intervention. CONCLUSIONS: Systemic pulmonary shunt remains an effective palliative measure in cyanotic neonates despite specific complications. Both low weight and preoperative ventilation represent significant risk factors for early mortality.

Alprostadil↗
PubMed2000

Transaortic closure of residual intramural ventricular septal defect.

BACKGROUND: Residual intramural ventricular septal defect is an unusual cause of left-to-right shunt after biventricular repair of conotruncal anomalies. It results from the insertion of the patch within the trabeculated right ventricular free wall related to the ventriculoinfundibular fold creating a communication through the intertrabeculated spaces to the right ventricular cavity. This complication often leads to unsuccessful reoperations unless the exact mechanism of the shunt has been identified. METHODS AND RESULTS: Five patients presented with residual intramural ventricular septal defects. Three had double outlet right ventricle, one pulmonary atresia with ventricular septal defect, and one tetralogy of Fallot. One patient was unsuccessfully reoperated on for closure of the residual ventricular septal defect through the right ventricular approach. The surgical treatment, which consisted of patch closure of the residual intramural ventricular septal defect through aortotomy, was successful in 3 patients. In the 2 remaining patients the hemodynamically insignificant residual intramural ventricular septal defect remained untouched. No mortality or morbidity occurred. CONCLUSIONS: Residual intramural ventricular septal defect should be suspected in presence of a residual ventricular septal defect after biventricular repair of conotruncal anomalies. It is not accessible through either atriotomy or right ventriculotomy. The transaortic approach allows an easy treatment of this rare complication.

Child↗
PubMed2000

Congenital division of the left atrium: the influence of associated congenital lesions on the timing and mode of presentation.

PURPOSE OF STUDY: Congenital division of an atrium is commonly associated with other cardiac malformations, which may influence presentation. BASIC METHODS: Cases diagnosed at our institution between 1980 and 1997 were reviewed, to determine whether associated lesions influence presentation. PRINCIPLE FINDINGS: 52 patients were diagnosed with atrial division (49 left, 3 right), mean age 4.8 yr. Patients could be divided according to age at diagnosis. Group 1: 32 patients aged < or = 1 yr. 14 patients had associated cardiac malformations other than atrial septal defect (ASD). Presentation was with pulmonary oedema. Group 2: 20 patients > 1 yr. 5 patients had associated major malformations other than ASD. Commonest presentation--isolated shortness of breath. Fifty patients underwent membrane resection and correction of associated anomalies (4% mortality). At follow-up (mean 12 yr) all patients were in NYHA class I or II. CONCLUSIONS: Patients with division of the atrium present early in infancy and with more severe symptoms when associated with other cardiac malformations.

Abnormalities, Multiple↗
PubMed2000

Long-term results after valvotomy for congenital aortic valvar stenosis in children.

As interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10-16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a range from 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15-49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.

Aortic Valve↗
PubMed2000

Left ventricular alterations in a model of fetal left ventricular overload.

Congenital aortic coarctation is well tolerated by the fetus because the foramen ovale and ductus arteriosus equalize intracardiac and great arteries pressures and shunts. The pathologic consequences only emerge after birth with closure of the foramen ovale and ductus arteriosus. There is, however, no documentation of myocardial effects in utero of the left ventricular (LV) pressure overload induced by aortic banding. We investigated whether prenatal aortic banding could be detrimental at the structural and/or functional level. The goal of the present study was to investigate the cardiac effects of LV pressure overload in a fetal lamb model. Nine fetal lambs underwent preductal banding of the aortic arch in utero at midgestation (CoA group), whereas their twins underwent sham surgery. All fetuses were studied between 27 and 37 d after surgery for LV pressure, anatomic and histologic anomalies, and steady state sarcoendoplasmic reticulum calcium ATPase (SERCA 2a) mRNA and protein levels and pump activity. Surgery resulted in severe aortic coarctation in all the animals in the CoA group and was associated with a 65% increase in the LV weight to body weight ratio relative to the sham-operated group (p < 0.001). Hemodynamic and histologic studies showed an evolutionary pattern depending on duration of the experimental coarctation with a shift occurring at 30 d of coarctation. The initial response of cardiomyocytes to ventricular overload was hypertrophy of the myocytes, followed by myocyte hyperplasia. Compared with sham, there was an apparent decrease in the percentage of binucleated cells in the CoA group after 30 d of coarctation. The earliest response to LV pressure overload appears to occur at the molecular level. Indeed, sarcoendoplasmic reticulum calcium ATPase (SERCA 2a) mRNA levels fell significantly to only 28.6% of the sham group value (p = 0.023), independently of the duration of coarctation. In the fetal lamb, the pressure overload-induced hypertrophy resulting from progressive aortic coarctation leads to hemodynamic and lesional abnormalities and slows ontogenic maturation.

Animals↗
PubMed1999

Functional Fas expression in human thymic epithelial cells.

Fas, a cell surface receptor, can induce apoptosis after cross-linking with its ligand. We report that Fas antigen is constitutively expressed in medullary epithelial cells of the human thymus. Expression is decreased in cultured thymic epithelial cells (TEC), similarly to HLA-DR antigen. TEC are resistant to anti-Fas-induced apoptosis after 4 days of primary culture, and this resistance is reversed by concomitant addition of cycloheximide. Cycloheximide also downregulated the expression of Fas-associated phosphatase-1, which has been found to inhibit Fas-induced apoptosis. This phosphatase could be involved in the resistance to Fas-induced apoptosis observed on day 4 of TEC culture. When TEC were subcultured after 10 to 13 days of primary culture, exposure to interleukin-1-beta, tumor necrosis factor-alpha, and interferon-gamma, alone or together, reinduced Fas mRNA and protein expression. In coculture with activated thymocytes, TEC also upregulated Fas protein expression. Cytokine-activated TEC became sensitive to apoptosis induced by an agonistic anti-Fas antibody. This apoptosis was inhibited by Z-VAD-fmk but not by Z-DEVD-fmk and DEVDase activity was slightly increased in Fas-stimulated TEC, suggesting that DEVDase activity is not sufficient to induce TEC apoptosis. Taken together, these data show that the Fas receptor is expressed in medullary epithelial cells of the human thymus and is able to induce apoptosis.

Apoptosis↗
PubMed1999

Surgical treatment of subaortic stenosis: a seventeen-year experience.

OBJECTIVE: The aim of the study was to analyze the long-term results of subaortic stenosis relief and the risk factors associated with recurrence and reoperation. METHODS: One hundred sixty patients with subaortic stenosis underwent biventricular repair. Before the operation the mean left ventricle-aorta gradient was 80 +/- 35 mm Hg, 57 patients had aortic regurgitation, and 34 were in New York Heart Association functional class III or IV. Median age at repair was 10 years. For discrete subaortic stenosis (n = 120), 39 patients underwent isolated membranectomy, 67 underwent membranectomy with associated septal myotomy, and 14 underwent septal myectomy. Tunnel subaortic stenosis (n = 34) was treated by myotomy in 10 cases, myectomy in 12, septoplasty in 7, Konno procedure in 3, and apical conduit in 2. Aortic valve replacement was performed in 6 cases, mitral valve replacement in 2 cases, and mitral valvuloplasty in 4 cases. RESULTS: There were 5 early (3.1%) and 4 late (4.4%) deaths. Within 3.6 +/- 3.3 years a recurrent gradient greater than 30 mm Hg was found in 42 patients (27%), 20 of whom had 26 reoperations. According to multivariable Cox regression analysis survival was influenced by hypoplastic aortic anulus (P =.01) and mitral stenosis (P =.048); recurrence and reoperation were influenced by coarctation and immediate postoperative left ventricular outflow tract gradients. At a median follow-up of 13.3 years, mean left ventricle-aorta gradient was 20 +/- 13 mm Hg. Relief of the subaortic stenosis improved the degree of aortic regurgitation in 86% of patients with preoperative aortic regurgitation. Actuarial survival and freedom from reoperation rates at 15 years were 94% +/- 1.3% and 85% +/- 6%, respectively. CONCLUSION: Although surgical treatment provides good results, recurrence and reoperation are significantly influenced by previous coarctation repair and by the quality of initial relief of subaortic stenosis.

Actuarial Analysis↗
PubMed1999

Pulmonary thromboendarterectomy for chronic thromboembolic obstruction of the pulmonary artery in piglets.

OBJECTIVE: The 2 main causes of death after thromboendarterectomy for chronic pulmonary thromboembolism are incomplete repermeabilization responsible for persistent pulmonary hypertension and acute high-permeability pulmonary edema. We wish to establish an experimental model of chronic pulmonary thromboembolism to replicate the conditions encountered during and after pulmonary thromboendarterectomy. METHODS: Multiple-curled coils and tissue adhesive were embolized in 6 piglets to induce complete obstruction of the left pulmonary artery, documented by angiography. After 5 weeks, the main pulmonary artery was repermeabilized by thromboendarterectomy during circulatory arrest. The left lung was reperfused ex vivo with autologous blood at constant flow, and patency of the pulmonary artery was evaluated on a barium angiogram. The endarterectomy-reperfusion procedure was also done in 6 nonembolized piglets that served as the controls. The severity of lung injury induced by 60 minutes of reperfusion was assessed on the basis of measurements of the lung filtration coefficient and of lung myeloperoxidase activity. RESULTS: Marked hypertrophy of the bronchial circulation was seen in the chronic pulmonary thromboembolism group. Thromboendarterectomy removed the organized obstructing thrombus that was incorporated into the arterial wall and restored patency of the pulmonary artery. Acute lung inflammation and high-permeability edema occurred after reperfusion, as indicated by a 1.5-fold increases in both lung filtration coefficient and lung myeloperoxidase values in the chronic pulmonary thromboembolism group; these 2 variables being correlated. CONCLUSIONS: Our model replicated the perioperative conditions of pulmonary thromboendarterectomy, suggesting that it may prove useful for improving the repermeabilization technique and for investigating the mechanisms and prevention of reperfusion injury.

Animals↗
PubMed1999

Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle.

OBJECTIVES: Increased afterload and multilevel LV obstruction is constant. We assumed that restoration of normal loading conditions by relief of LV obstructions promotes its growth, provided that part of the cardiac output was preoperatively supported by the LV, whatever the echocardiographic indexes. BACKGROUND: Whether to perform uni- or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal LV (hypoplastic left heart syndrome classes II & III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. METHODS: Twenty ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multilevel LV obstruction. Preoperative echocardiographic assessment showed: mean EDLW of 12.4 +/- 3.03 ml/m2 and mean Rhodes score of -1.73 +/-0.8. Surgery consisted in relief of LV outflow tract obstruction by coarctation repair in all associated to aortic commissurotomy in one and ASD closure in 2. RESULTS: There were 3 early and 2 late deaths. Failure of biventricular repair and LV growth was obvious in patients with severe anatomic mitral stenosis. The other demonstrated growth of the left heart. At hospital discharge the EDLVV was 19.4+/-3.12 ml/m2 (p = 0.0001) and the Rhodes score was -0.38+/-1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were 72.5% and 46%, respectively. CONCLUSIONS: Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal LV provided that all anatomical causes of LV obstruction can be relieved. Secondary growth of the left heart then occurs; however, the reoperation rate is high.

Abnormalities, Multiple↗
PubMed1999

Double-outlet right ventricle with non-committed ventricular septal defect.

OBJECTIVE: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. METHODS: Between January 1987 and December 1997, 23 patients having double-outlet right ventricle (DORV) with non-committed VSD underwent biventricular repair. Nine (39%) had undergone previous palliation. The median age was 20 months and the median weight was 8.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 21) and arterial switch operation with VSD to pulmonary artery baffle (n = 2). At repair, 12 (52%) patients required concomitant VSD enlargement. In two other patients presenting with restrictive inlet VSD associated with tricuspid attachments, crossing the subaortic pathway biventricular repair was abandoned at operation. RESULTS: There were two hospital deaths (9%, 70% CL: 3-19%). Eight patients (35%, 70% CL: 23-48%) underwent nine reoperations, six for subaortic stenosis. No late death occurred. At last visit, all patients were asymptomatic and only two had cardiac medication. CONCLUSIONS: The biventricular repair of DORV with non-committed VSD is feasible in the vast majority of cases with comparable results to other subsets of DORV. After repair, the subaortic region is at risk for development of subaortic stenosis.

Cardiac Surgical Procedures↗
PubMed1999

Surgical management of transposition of great arteries associated with multiple ventricular septal defects.

OBJECTIVE: The presence of associated multiple ventricular septal defects (VSDs) increases the risk of the anatomic repair for transposition of the great arteries (TGA). The aim of this study was to define the optimal management of this complex anomaly. METHODS: Between January 1988 and December 1998, 45 patients underwent anatomic repair of TGA associated with multiple VSDs. The median age was 50 days and the median weight 4 kg. Eighteen (40%) had undergone previous palliation including 17 pulmonary artery banding procedure (PAB), seven associated with coarctation repair and one isolated coarctation repair. The perimembraneous septum was involved in 24 patients, the trabecular in 43, the inlet in seven and the infundibular in two. Closure of the VSDs included Dacron or pericardial patchs and matress sutures. The initial approach was through right atriotomy which was sufficient in 15 patients. VSDs were closed through right ventriculotomy in 13 patients, through pulmonary artery in six, through the aorta in one and in the remaining (n = 10) combined approaches were used. Only one patient required left apical ventriculotomy. RESULTS: There were five hospital deaths (11%; 70% CL: 6-18%) including the one early reoperation for residual VSD closure. Five patients had successful early reoperation for secondary PAB for residual VSD. Three late deaths occurred (7%; 70% CL: 3-13%). At the last visit, 95% of survivors were asymptomatic and without any cardiac medication. CONCLUSION: Mid-term survival with good quality of life can be achieved following either one or two-stage repair of this complex anomaly. In the presence of VSD closure failure a secondary PAB may be the procedure of choice.

Cardiac Surgical Procedures↗
PubMed1999

Ascites and weight loss in a child: due to congenital division of the right atrium.

Congenital division of an atrial chamber is a very rare congenital malformation that more commonly affects the left atrium but which may, in rare circumstances, involve the right atrium. Such a divided right atrium may present with symptoms consistent with increased portal venous pressure. Reported is a case with unusual clinical presentation. The patient underwent resection of the dividing shelf with good postoperative results.

Ascites↗
PubMed1999

Vascular endothelium viability and function after total cardiopulmonary bypass in neonatal piglets.

Endothelium dysfunction with severe pulmonary hypertension may occur after total cardiopulmonary bypass (CPB) in infants as a result of a widespread inflammatory response. The aim of this study was to separate out the effects of lung ischemia-reperfusion from membrane oxygenator-induced activation of leukocytes on the function and viability of the pulmonary and systemic endothelia in neonatal piglets submitted to 90-min total CPB followed by 60-min reperfusion or in sham animals. Hemodynamics, gas exchange, endothelial-dependent relaxation in pulmonary and femoral arteries, and lung and skeletal muscle myeloperoxidase activity were assessed before, during, and after CPB, i.e., after reperfusion. Pulmonary and aortic endothelial cells and circulating leukocytes were harvested to assess reperfusion-induced changes in endothelial cells' viability and proliferation, and leukocyte-endothelial cell adhesion and cytotoxicity. Gas exchange worsened after reperfusion with pulmonary hypertension, increase in lung but not skeletal myeloperoxidase, and reduction of endothelial-dependent relaxation in pulmonary but not femoral arteries. After reperfusion, viabilities of pulmonary and aortic endothelial cells were reduced to 50%, endothelial cell growths were faster in pulmonary arteries than aorta, and leukocyte-pulmonary endothelial cell adhesion and cytotoxicity increased. These results suggest that in total CPB lung ischemia-reperfusion aggravates the inflammatory response and predisposes the lung endothelium to leukocyte-mediated injury.

Animals↗
PubMed1999

[Biventricular repair in neonates with hypoplasia of the left ventricle].

BACKGROUND: Whether to perform uni or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal left ventricle and multi level left ventricle obstructions (hypoplastic left heart syndrome class III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. HYPOTHESIS: Increased afterload and multi level left ventricle obstruction is constant. We assumed that restoration of normal loading conditions by relief of left ventricle obstructions promotes its growth, provided that part of the cardiac output was pre operatively supported by the left ventricle, whatever the echocardiographic indices. METHODS: Twenty one ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multi level left ventricle obstruction. Pre operative echocardiographic assessment showed: mean end diastolic left ventricular volume of 13.3 +/- 3.5 mL/m2 and mean Rhodes score of -1.43 +/- 0.9. Surgery consisted in relief of left ventricle outflow tract obstruction by coarctation repair in 21 associated to atrial septal defect closure in 2, aortic commissurotomy in 1 and ascending aorta enlargement in 1. RESULTS: There were 3 early and 3 late deaths. There was no predictive risk factor for failure. Growth of the left heart was demonstrated in most patients. At hospital discharge the end diastolic left ventricular volume was 19.4 +/- 3.12 mL/m2 (p = 0.0001) and the Rhodes score was -0.38 +/- 1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were: 68.5% and 40.75%, respectively. CONCLUSION: Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal left ventricle provided that all anatomical causes of left ventricle obstruction can be relieved. Secondary growth of the left heart then occurs, however the reoperation rate is not low.

Echocardiography↗
PubMed1999

[Right postero-lateral thoracotomy for open heart surgery in infants and children. Indications and results].

In order to avoid the aesthetic prejudice of median sternotomy in young children undergoing open heart surgery for isolated congenital heart disease, a right posterolateral thoracotomy was performed in 146 children aged 5 months to 14 years. The large majority (140/146, 96%) were atrial septal defects: 130 ostium secundum, 5 sinus venosus, 1 low septal defect and 4 ostium primum (partial endocardial cushion defect). Six children had isolated perimembranous ventricular septal defects. One patient died of a probable lesional pulmonary oedema. Minor complications were observed in 15 cases and one had to be reoperated for a residual shunt. With an average follow-up of 2.6 years, all survivors are asymptomatic. The scar is normal, without cheloid or thoracic deformation, invisible to the patient when he looks in a mirror. The use of this approach requires a very accurate anatomical diagnosis, especially with regards to systemic and pulmonary drainage. The presence of a left superior vena cava draining into the coronary sinus is a contra-indication. The right posterolateral thoracotomy is now the approach of choice in its standard indication, the repair of ostium secundum atrial septal defects with large shunts, in young girls under 10 years of age.

Adolescent↗
PubMed1998

Biventricular repair for double-outlet right ventricle. Results and long-term follow-up.

BACKGROUND: The purpose of the present study was to define the optimal management and to identify the risk factors for death and repeat operation in patients with double-outlet right ventricle. METHODS AND RESULTS: From 1985 through 1996, 154 consecutive patients underwent biventricular repair for double-outlet right ventricle. The presence of bilateral infundibular structures was the major inclusion criteria (142 patients). According to the relationship of the ventricular septal defect (VSD) to the great arteries, there were 86 patients with a subaortic VSD (56%), 45 patients with a subpulmonary VSD (29%), 18 patients with a noncommitted VSD (12%), and 5 patients with a doubly committed VSD (3%). Sixty-five patients (42%) had undergone previous palliative procedures. At repair, the median age was 10 months, and the median weight was 6.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 115) and arterial switch operation with VSD-to-pulmonary artery baffle (n = 39). There were 14 hospital deaths (9%; 70% confidence limit [CL], 7% to 12%). The only significant risk factor for early death was the presence of congenital mitral valve anomalies (P = 0.02). Twenty-eight patients (18%) required 39 repeat operations. The repeat operation rate was higher in patients with associated VSD enlargement at baffle construction (n = 29; 19%) (P = 0.01). There were 6 late deaths (4%; 70% CL, 2% to 7%). Patients presenting with pulmonary stenosis constituted a low-risk group for global death (P = 0.008). The median follow-up was 52 months. Ten-year actuarial survival and survival with freedom from repeat operation rates were 86% and 62% (70% CL, 83% to 89% and 54% to 70%), respectively. CONCLUSIONS: Long-term survival with good quality of life can be achieved after either 1- or 2-stage repair of this complex anomaly.

Adolescent↗