Success and limitations of right ventricular sinus myectomy for pulmonary atresia with intact ventricular septum.

OBJECTIVES: Right ventricular sinus myectomy has been proposed for pulmonary atresia with intact ventricular septum for morphology falling within the uncertain area for eventual biventricular repair. Our objective was to evaluate right ventricular sinus myectomy by characterizing the morphologic spectrum of these patients, determining whether biventricular repair was achieved, ascertaining growth of right-sided structures, and assessing survival. METHODS: We evaluated medical records, all imaging studies, and follow-up data (complete in all but 1 patient) from 43 patients with pulmonary atresia with intact ventricular septum treated from October 1993 to July 2005, 16 of whom underwent right ventricular sinus myectomy. Serial echocardiographic measurements of right-sided cardiac structures were converted to Z values to estimate their growth relative to somatic growth. RESULTS: Patients undergoing right ventricular sinus myectomy had mild-to-moderate right ventricular size diminution (grade -1.2 +/- 3.2) and a tricuspid valve Z value of -4.9 +/- 1.9. Thirteen (87%) of the 16 patients achieved biventricular repair. After right ventricular sinus myectomy, mean right ventricular cavity size grade increased to 1.4 +/- 0.66, but the tricuspid valve Z value did not change appreciably over time. Five-year survival after sinus myectomy was 85%; late deaths were in patients with the smallest tricuspid valves at presentation (Z value < -7). CONCLUSIONS: Right ventricular sinus myectomy in the uncertain area for biventricular repair of pulmonary atresia with intact ventricular septum leads to immediate increase in right ventricular volume. It, in combination with establishing right ventricle-pulmonary trunk continuity, allowed early biventricular repair in 87% of patients. However, tricuspid valve growth in relation to somatic growth was minimal. Thus, small tricuspid valve size might limit the long-term success of biventricular repair achieved by means of right ventricular sinus myectomy.

Perioperative serum interleukins in neonates with hypoplastic left-heart syndrome and transposition of the great arteries.

OBJECTIVE: The primary study objective was to examine the impact of diagnosis on the inflammatory response in neonates with congenital heart disease undergoing cardiac surgery. The secondary objective was to study the impact of the inflammatory response on postoperative outcome in these neonates. DESIGN: Observational study. SETTING: Tertiary care children's hospital heart center. PATIENTS: Neonates with hypoplastic left-heart syndrome (HLHS) undergoing stage I repair and patients with transposition of the great arteries (TGA) undergoing arterial switch operation. MEASUREMENTS AND MAIN RESULTS: There were 24 neonates with HLHS and 21 neonates with TGA. Serum samples to measure interleukin (IL)-6 and -10 were obtained before and after CPB at 1, 3, 6, and 24 hours postoperatively. Time to extubation, intensive care unit (ICU) length of stay, and peritoneal fluid drainage were compared between the groups. Serum IL-6 and IL-10 concentrations increased after CPB when compared to the preoperative concentration. Preoperative concentrations of IL-6 were significantly elevated in the HLHS group (HLHS: 32 [21.1, 69.6] pg/mL v TGA: 7.2 [3.6, 22.5] pg/mL [median, 25th, and 75th percentile], p = 0.003) and remained elevated immediately after CPB, and at 3 and 6 hours postoperatively. The IL-10 to IL-6 ratio was lower in the HLHS group preoperatively and immediately after CPB. ICU length of stay was significantly longer in the HLHS group (TGA 4 [3-6] days v HLHS 6 [5-8] days, p = 0.031). Mortality in the HLHS group (4/24) was associated with significantly higher IL-6 postoperatively (IL-6 immediately postoperatively: HLHS survivors 59.9 [34.3, 65.7] pg/mL v nonsurvivors 98.7 [94.4, 104.5] pg/mL, p < 0.011). CONCLUSIONS: All neonates with TGA or HLHS have a significant inflammatory response after CPB. Neonates with HLHS have evidence of an activated inflammatory response before CPB, which remains significant in the postoperative period. Accelerated interleukin expression and an abnormal cytokine balance correlate with longer time to extubation, longer ICU length of stay, and increased peritoneal fluid volume.

Aortic valve repair for congenital abnormalities of the aortic valve.

BACKGROUND: Due to shortcomings of any valve replacement, repairing and retaining the native valve may be beneficial for congenital aortic valve disease. METHODS: Retrospective review of data and follow-up of aortic valve repair from a single institution. RESULTS: From 1993 to 2001, 56 patients underwent aortic valve repair [median age 13.4 years (range 1 day to 45 years)]. The predominant aortic valve lesion was mixed aortic stenosis/aortic insufficiency 25 (45%), aortic insufficiency 24 (43%) and aortic stenosis 7 (13%). Repair techniques included sub-commissural plication 36 (64%), commissurotomy 24 (43%), cusp plication 15 (27%), pericardial patch cusp extension 8 (14%) and resuspension of commissures 4 (7%). Most patients (88%) required a combination of techniques; 61% required additional procedures. Hospital survival was 55/56 [98%; (95% CI 91-100%)] no patient was discharged on anticoagulation for aortic valve pathology. Fifty-three patients [95%; (95% CI 85-98%)] remain alive after a median follow-up of 37 months; four survivors required aortic valve replacement and two required repeat aortic valve repair [84%; (95% CI 72-91%) reintervention-free survival]. CONCLUSIONS: (1) In this study, aortic valve repair for congenital abnormalities avoided reoperation in the majority of patients, avoided anticoagulation and retained growth potential of the valve. (2) Repeat aortic valve repair or replacement was used to treat subsequent valve deterioration.

Quantitative analysis of procalcitonin after pediatric cardiothoracic surgery.

Procalcitonin appears to be an early and sensitive marker of bacterial infection in a variety of clinical settings. The use of levels of procalcitonin to predict infection in children undergoing cardiac surgery, however, may be complicated by the systemic inflammatory response that normally accompanies cardiopulmonary bypass. The aim of our study was to estimate peri-operative concentrations of procalcitonin in non-infected children undergoing cardiac surgery. Samples of serum for assay of procalcitonin were obtained in 53 patients at baseline, 24, 48, and 72 hours following cardiac surgery. Concentrations were assessed using an immunoluminetric technique. Median concentrations were lowest at baseline at less than 0.5 nanograms per millilitre, increased at 24 hours to 1.8 nanograms per millilitre, maximized at 48 hours at 2.1 nanograms per millilitre, and decreased at 72 hours to 1.3 nanograms per millilitre, but did not return to baseline levels. Ratios of concentrations between 24, 48 and 72 hours after surgery as compared to baseline were 6.15, with 95 percent confidence intervals between 4.60 and 8.23, 6.49, with 95 percent confidence intervals from 4.55 to 9.27, and 4.26, with 95 percent confidence intervals between 2.78 and 6.51, respectively, with a p value less than 0.001. In 8 patients, who had no evidence of infection, concentrations during the period from 24 to 72 hours were well above the median for the group. We conclude that concentrations of procalcitonin in the serum increase significantly in children following cardiac surgery, with a peak at 48 hours, and do not return to baseline within 72 hours of surgery. A proportion of patients, in the absence of infection, had exaggerated elevations post-operatively.

Management of the failing systemic right ventricle.

Conditions in which the right ventricle serves as the systemic pumping chamber are frequently complicated by the development of right ventricular failure and tricuspid valve regurgitation. The right ventricle is the systemic ventricle in conditions of ventriculoarterial discordance with atrioventricular concordance (transposition of the great arteries) or with atrioventricular discordance (congenitally corrected transposition of the great arteries). Concerns regarding actual or potential systemic right ventricular failure in these cases may lead to surgical evaluation and treatment designed to reestablish the left ventricle as the systemic pump. In cases where the left ventricle has prolonged exposure to low pressures in the pulmonary circulation, the left ventricle must be "retrained" to assume a systemic pressure load. Anatomic repair, with or without a preparatory period of left ventricular retraining, is a consideration for three clinically relevant scenarios: (1) patients with transposition of the great arteries after an atrial level switch (Senning or Mustard procedure), (2) patients with congenitally corrected transposition who are unoperated or who have undergone physiologic ("classic") repair, and (3) unoperated patients with transposition who present after the neonatal period.

The double switch operation with accent on the Senning component.

In congenitally corrected transposition (ccTGA) the most common configuration is atrial situs solitus with left ventricular loop and left transposition of the great arteries (SLL). Less common is ccTGA with atrial siti inversus (IDD). In both configurations there is a high incidence of ventricular septal defect, pulmonary stenosis, or atresia and some anatomic abnormality of the morphologic tricuspid valve (mTV). The morphologic right ventricle (mRV) is the systemic ventricle and prone to premature failure, particularly in the presence of early TV regurgitation, atrial arrhythmias conduction defects, and prior surgical ventricular septal defect closure. With a long experience with the Senning operation and then the arterial switch, it seemed feasible that these could be combined in ccTGA to restore the mLV to the systemic circuit. This was first attempted in 1989 by the author and was successful. Many of the more recently graduated congenital heart surgeons have little or no experience with the inflow switch. For this reason, the author was asked to write this article, accenting the technical details of the inflow switch. The author uses the Senning operation, with those modifications needed to accommodate the differences between the morphologic right atrium, conduction system, and quite frequent discordance between the atrial situs and the position of the apex of the heart, in ccTGA as compared with TGA.

Increased transcription factor expression and permeability of the blood brain barrier associated with cardiopulmonary bypass in lambs.

BACKGROUND: The pathophysiology of neurocognitive dysfunction and developmental delay after cardiopulmonary bypass (CPB) in infants is not known. It is known that head trauma, stroke, and seizures cause dysfunction of the blood brain barrier (BBB) that is associated with increased inducible transcription factor gene expression in the cells of the barrier. The purpose of this study was to determine the effects of CPB and hypothermic circulatory arrest on expression of the transcription factor FOS and the function of the BBB in an infant animal model. METHODS: Infant lambs (n = 36; 10-12 days) were exposed to 0, 15, 30, 60, or 120 minutes of normothermic (38 degrees C) CPB or 2 hours of hypothermic circulatory arrest at 16 degrees C. After terminating bypass 15 animals had their brains perfusion-fixed and removed for immunohistochemical analysis of expression of the transcription factor FOS. The other animals were perfused with fluorescent albumin to visualize the brain microvasculature. Brain sections were analyzed with a laser scanning confocal microscope. RESULTS: Control animals (n = 6, sham operated and cannulated) exhibited normal vasculature with negligible leakage and no FOS protein expression in neurons or endothelial cells anywhere in the brain. Significant FOS expression in barrier-associated structures including the blood vessels, choroid plexus, and ependyma but not neurons occurred at all times on bypass. CPB caused leakage of fluorescent albumin from blood vessels in all animals. Two hours of normothermic CPB (n = 4) caused significant (p < 0.01) leakage in the cerebellum, cortex, hippocampus, and corpus callosum. Animals exposed to circulatory arrest experienced severe leakage throughout the brain (p < 0.001) and FOS expression in all cells. CONCLUSIONS: These experiments indicate that the BBB is dysfunctional after all time points on normothermic CPB, BBB dysfunction is worsened by hypothermic circulatory arrest, and BBB dysfunction is associated with intense molecular activity within the barrier-forming cells. Dysfunction of the BBB may contribute to neurologic complications after heart surgery.

HLHS with severe aortic insufficiency in a patient with 45,X/46,XY mosaicism.

Aortic insufficiency is not a part of the hypoplastic left heart syndrome. This report describes a rare case of congenital aortic insufficiency from a detached leaflet in a patient with hypoplastic left heart syndrome and 45,X/46XY mosaicism. The patient was subsequently treated with the modified Norwood procedure along with suture closure of aortic valve.

Apical right ventriculotomy for closure of apical ventricular septal defects.

BACKGROUND: Apical ventricular septal defects (VSDs) are difficult to visualize through a transatrial approach, while the use of a left ventriculotomy may result in progressive ventricular dysfunction. Transcatheter closure has not been reliable, especially in small infants. Apical right ventriculotomy provides good exposure, preserves left ventricular function, and allows secure closure of apical VSDs. METHODS: From November 1994 through April 2002, nine children, median age 8 months (range, 74 days to 2.5 years) underwent 10 operations for VSD closure via apical right ventriculotomy (one patient with a residual defect underwent successful VSD closure via a second apical right ventriculotomy). Two patients were status post pulmonary artery banding and two patients had previous unsuccessful attempts at closure via a transatrial approach. RESULTS: There was no hospital mortality; there were 2 late deaths (78% survival; 95% confidence interval [CI], 45% to 94%), 3 months and 4 years postoperatively. Postoperative echocardiography demonstrated no residual VSDs in 3 and insignificant residual VSDs in 4 of the survivors. All survivors currently exhibit normal biventricular function during a median follow-up of 25 months (range, 11 to 104 months). CONCLUSIONS: Apical right ventriculotomy provides excellent exposure allowing safe and effective closure of apical VSDs. The observed late morbidity and mortality reflects the complexity that often exists in these cases due to additional irreparable lesions.

Selective timing for the arterial switch operation.

BACKGROUND: To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status. METHODS: A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. RESULTS: From July 1993-April 2001, 117 infants underwent an arterial switch operation before 90 days of age. Seventy-five patients (64%) had transposition of the great arteries with intact ventricular septum with the aim of operation before 14 days of age; however, 8 of these patients had delayed presentation (range 15-46 days). Thirty-five patients (30%) had transposition with a ventricular septal defect (30 patients) or double outlet right ventricle (5 patients) and normal arch anatomy and were repaired within the first 90 days of life depending on the severity of heart failure at a median of 12 days of age (range 3-83 days). Seven patients (6%) had associated aortic coarctation (5 patients) or interrupted aortic arch (2 patients). One patient died during hospitalization (0.85% hospital mortality) and one patient died from noncardiac causes during a median follow-up of 35 months (1.7% total mortality). Four patients required intervention during follow-up (3.4%) for new aortic coarctation (2 patients), supravalvar pulmonic stenosis (1 patient), or right hemi-diaphragm paralysis (1 patient). CONCLUSIONS: Individualized timing for the arterial switch operation within the first ninety days of life produces excellent survival rates for all types of transposition physiology with the expectation of a satisfactory course during follow-up.

Optimizing cardioplegia strategy for donor hearts.

Variability in organ preservation strategy has thus far prevented meaningful analysis of clinical donor heart cardioplegia strategies. This paper describes our donor heart procurement protocol, techniques, and recovery team responsibilities. We present 21 patients receiving cardiac transplantation at our institution with an adopted cardioplegia protocol. The procurement team perfusionist ensures consistent attention to myocardial protection with standards that are similar to those applied to native hearts for all congenital heart surgeries.

Long-term results of left ventricular reconditioning and anatomic correction for systemic right ventricular dysfunction after atrial switch procedures.

OBJECTIVES: Systemic right ventricular failure after atrial switch procedures for transposition of the great arteries has been addressed at Melbourne's Royal Children's Hospital (1981-1993) and the Cleveland Clinic Foundation (1993-2001) with reconditioning of the morphologically left ventricle by means of pulmonary artery banding followed by an arterial switch operation and an atrial reseptation. METHODS: Thirty-nine patients (Royal Children's Hospital, 19; Cleveland Clinic Foundation, 20) with a median age of 10.8 years (range, 13 months-24 years) entered this protocol a median of 10.3 years (range, 0.5-24 years) after an atrial switch procedure. RESULTS: The median duration of pulmonary artery banding was 13 months (range, 0.5-5.4 years). Ten (28%) patients responded unfavorably to morphologically left ventricular reconditioning (5 mortalities: 4 transplantations and 1 PAB still in place). Twenty-four (83%) of the 29 patients who underwent an atrial switch operation and atrial reseptation survived. During a median follow-up period of 8.2 years (range, 1-16 years), 3 patients had cardiac-related deaths. All 18 long-term survivors are asymptomatic. At last echocardiographic evaluation, the morphologically left ventricular function was normal or mildly decreased in 16 (89%) patients, and all had normal or mildly decreased systemic right ventricular function with no or mild tricuspid regurgitation. Age greater than 12 years was associated with a greater probability of morphologically left ventricular failure and not completing the protocol (P =.02) and a higher operative mortality at anatomic correction (P =.02). CONCLUSIONS: Morphologically left ventricular reconditioning and an anatomic correction protocol should be integrated into a cardiac transplantation program when treating patients with morphologically right ventricular failure after Mustard and Senning procedures. It is an alternative to cardiac transplantation in selected patients, with good long-term results. The response to morphologically left ventricular reconditioning past adolescence is inconsistent.

Arterial switch with full-flow cardiopulmonary bypass and limited circulatory arrest: neurodevelopmental outcome.

OBJECTIVES: Neonatal cardiac surgery has been associated with unfavorable neurodevelopmental events. We investigated a patient cohort operated on predominantly with full-flow cardiopulmonary bypass (150 mL x kg(-1) x min(-1), alpha-stat, alpha-blockade, median arrest = 6 minutes, temperature of 22 degrees C) as the major support strategy for neonatal arterial switch operations (transposition of the great arteries and intact ventricular septum). METHODS: Seventy-four patients and "best-friend" control subjects were assessed 109 months (range, 48-166 months) postoperatively with general medical and neurologic evaluation, IQ testing, formal movement scores, and detailed parent-teacher behavioral-social reports. Fetal, neonatal, and perioperative data were collated. RESULTS: The prevalence of perioperative seizures was 6.8% (4/5 cases occurring preoperatively). The incidence of all perioperative neurologic abnormalities was 20%. Patients who had a neurologic event were (as a group) older at the time of operation and had a lower arterial blood pH before the operation. Selected perioperative factors (not related directly to cardiopulmonary bypass variables) predicted early (before discharge) neurologic outcome in a multivariate model. At late assessment, patients were more likely than control subjects to have a mild neurologic abnormality (P = 0.002). Full-scale IQ scores (Wechsler Preschool and Primary Scale of Intelligence and Wechsler Intelligence Scale for Children-Third Edition) were higher in control subjects (101.9 [SD = 13] vs 108.6 [SD = 12], P =.0007), with both groups having scores greater than the population-based test means. Full-scale IQ scores related most significantly to years of paternal education (beta = 1.51, P =.0078) but were also influenced by perioperative neurologic abnormalities, birth weight, and circulatory arrest time. Patients had higher motor impairment scores (Movement Assessment Battery) than control subjects (P =.0004). Parents (Achenbach Child Development Checklist) assigned higher total social-behavioral competence scores to control subjects (P =.05). Teachers (Achenbach Teacher Report Form) suggested that patients were more likely to be perceived as having various speech and expressive language problems, as well as minor behavioral problems. CONCLUSION: With the perioperative strategies used, not all survivors can be considered (neurodevelopmentally) normal at late follow-up, although the risk of important impairment is low. Perioperative events might have long-term prognostic value. On the basis of this study and published data regarding other strategies, continued application of full-flow cardiopulmonary bypass is justified, with the proviso that further investigation is required.

Factors associated with early extubation after cardiac surgery in young children.

OBJECTIVE: Children undergoing congenital heart surgery require mechanical ventilation. We sought to identify pre- and intraoperative factors (PrO, IO) associated with successful early extubation <24 hrs. DESIGN AND PATIENTS: We performed a retrospective chart review of children <36 months old who underwent congenital heart surgery from January 1998 to July 1999. SETTING: Pediatric intensive care unit in a children's hospital. MEASUREMENTS: Generalized Estimating Equation models were fit to assess the association between PrO and IO and early extubation while accounting for the correlation between surgeries performed on the same patient. Estimated odds ratios (EOR) and 95% confidence intervals were calculated. Multivariable models were constructed using a forward selection process with inclusion criteria of p<.05. Multivariable models, which included PrO and IO variables, were adjusted for procedure group. The area under the receiver operating characteristic curve was computed for each model. RESULTS: A total of 203 children underwent 219 surgeries; 103 (47%) children were extubated in <24 hrs, with only one (1%) failed extubation. PrO variables associated with successful early extubation included age >6 months (EOR, 6.1), absence of pulmonary hypertension (EOR, 9.1), gestational age >36 wks (EOR, 4.6), and absence of congestive heart failure (EOR, 2.4). IO variables were less likely to be associated with successful early extubation. Our model of PrO variables with multiple factors showed that presence of two factors was associated with an EOR of 4.2 for successful early extubation compared with children with zero or one factor. Presence of three and four factors was associated with an EOR of 18.0 and 76.5, respectively. The area under the receiver operating characteristic curve for this model is 0.816. Incision type, complex vs. simple procedure, and palliative vs. complete repair were not associated with success of early extubation. CONCLUSIONS: Early extubation is possible in many very young children undergoing congenital heart surgery, with a low rate of failed extubation. The model would be improved by prospective validation with larger numbers at multiple institutions.

Surgical treatment of congenital mitral stenosis: medium-term results

OBJETIVO: Analisar a evolução de pacientes portadores de estenose mitral congênita (EmiC) submetidos a tratamento cirúrgico, buscando estudar os fatores desfavoráveis da plastia valvar e os seus marcadores de durabilidade. MÉTODO: De 1989 a 2002, 23 pacientes foram submetidos a tratamento cirúrgico da EmiC, excluindo aqueles com defeito do septo atrioventricular, e formas univentriculares. A idade mediana foi de 15,5 meses (variação de 2 a 204), e o peso mediano foi de 11 kg (variação 4,5 a 51,6 kg). Anomalias associadas estiveram presentes em 17 (73,9 por cento), complexo de Shone em nove (39,1 por cento) e hipertensão pulmonar em 14 (60,9 por cento). A estenose mitral era grave em 14 (60,9 por cento) pacientes, e moderada no restante (gradiente transmitral mediano de 16 mmHg, variação 8,5 a 32). A plastia valvar foi realizada em 18 (78,3 por cento) pacientes e a troca em cinco (21,7 por cento). As técnicas de plastia incluiram a papilarotomia (n=10), ressecção de membrana supravalvar (n=9) e comissurotomia (n=8). Doze (52,2 por cento) pacientes requereram procedimentos associados. RESULTADOS: Não houve óbitos precoces ou tardios num seguimento médio foi de 58,5 ñ 46,7 meses (variação 1 a 156 meses). O tempo médio de hospitalização foi de 12,7 ñ 8,2 dias. Não foram encontrados fatores de risco associados a insucesso da plastia valvar. A curva atuarial livre de reoperaçäes em cinco anos foi de 67,1 por cento (IC 95 por cento: 56,8 por cento a 77,4 por cento). O grupo submetido a plastia valvar necessitou de reoperação em oito (44,4 por cento) pacientes, sendo dois precoces e seis tardios, comparados com uma (20 por cento) reoperação no grupo submetido à troca valvar. A presença de hipertensão pulmonar no pré-operatório esteve relacionada significativamente (p<0,005) a maiores taxas de reoperação. Todos os pacientes, exceto dois, encontram-se em classe funcional I da NYHA e o ecocardiograma mostra no máximo insuficiência e/ou estenose mitral leve. CONCLUSåES: As reoperaçäes foram o maior fator de morbidade no seguimento a médio prazo e a hipertensão pulmonar pôde ser considerada o fator adverso de durabilidade da plastia mitral, a qual foi o tratamento de escolha para a estenose mitral congênita.

Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries.

OBJECTIVE: To assess the results of a staged surgical approach for tetralogy of Fallot with pulmonary atresia, hypoplastic or absent pulmonary arteries, and major aortopulmonary collateral arteries. METHODS: We retrospectively reviewed a consecutive series of these patients from a single institution. RESULTS: From July 1993 to April 2001, 46 consecutive patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries were treated with staged surgical repair. The operative sequence usually began with a central aortopulmonary shunt followed by unifocalization of aortopulmonary collateral arteries depending on the source and distribution of pulmonary blood flow. Twenty-eight patients (61%) subsequently underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery connection. Those patients who underwent complete repair had a median of 3 total operations (range 1-6). The ratio of the mean pulmonary artery pressure to the mean systemic blood pressure at the time of complete repair was 0.36 (range 0.19-0.58). Two of the 28 repaired patients (7.1%) required subsequent fenestration of the ventricular septal defect closure due to later development of supersystemic right ventricular pressure and right ventricular failure. Eighteen patients (39%) have undergone 1 or more staging operations and are considered good candidates for eventual complete repair. There were no hospital deaths. There was 1 late death (2.2%; 95% CI 0.4-11.3%) in a patient born prematurely who developed severe bronchopulmonary dysplasia precluding complete repair. CONCLUSIONS: For tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, a staged surgical approach yields low overall mortality and acceptable hemodynamics after complete repair.

A novel operative approach to scimitar syndrome.

Scimitar syndrome is a rare congenital cardiac anomaly defined by an anomalous right pulmonary vein draining the right lung to the inferior vena cava. We describe a unique operative approach performed on 2 patients with infantile scimitar syndrome.

Results of the double switch operation for congenitally corrected transposition of the great arteries.

OBJECTIVE: To determine the outcomes for a program that utilizes the double switch operation as the primary approach for congenitally corrected transposition. METHODS: The records of 46 consecutive patients from a single institution who had undergone a double switch operation from October 1993 to March 2002 were reviewed. The records of 24 patients who were evaluated during the same period and felt not to be candidates for the double switch operation or who are awaiting double switch after pulmonary artery banding were also reviewed. RESULTS: The median age at operation was 28 months (range 2 months to 16.3 years). Associated defects included ventricular septal defect 40, pulmonic stenosis 13 and pulmonary atresia 16. Twenty-six patients underwent an arterial switch operation combined with a Senning procedure while 20 patients underwent combined Rastelli and Senning procedures. Before the double switch, 12 patients had required pulmonary artery banding and 21 patients had systemic to pulmonary artery shunts. The median duration of stay in the intensive care unit was 3.5 days (range 2-60 days) and the median duration of total hospital stay was 8 days (range 5-60 days). There were no hospital deaths; one patient died 5 months after discharge due to an arrhythmogenic cardiac arrest during a median follow-up of 24 months [long-term survival 98% (95% CI 89-100%)]. CONCLUSIONS: The double switch operation may be performed with excellent hospital and long-term survival. The theoretical advantages of this procedure which enables the morphologic left ventricle and mitral valve to support a systemic pressure load must be established by careful follow-up of these patients.