The Aristotle score: a complexity-adjusted method to evaluate surgical results.

OBJECTIVES: Quality control is difficult to achieve in Congenital Heart Surgery (CHS) because of the diversity of the procedures. It is particularly needed, considering the potential adverse outcomes associated with complex cases. The aim of this project was to develop a new method based on the complexity of the procedures. METHODS: The Aristotle project, involving a panel of expert surgeons, started in 1999 and included 50 pediatric surgeons from 23 countries, representing the EACTS, STS, ECHSA and CHSS. The complexity was based on the procedures as defined by the STS/EACTS International Nomenclature and was undertaken in two steps: the first step was establishing the Basic Score, which adjusts only the complexity of the procedures. It is based on three factors: the potential for mortality, the potential for morbidity and the anticipated technical difficulty. A questionnaire was completed by the 50 centers. The second step was the development of the Comprehensive Aristotle Score, which further adjusts the complexity according to the specific patient characteristics. It includes two categories of complexity factors, the procedure dependent and independent factors. After considering the relationship between complexity and performance, the Aristotle Committee is proposing that: Performance = Complexity x Outcome. RESULTS: The Aristotle score, allows precise scoring of the complexity for 145 CHS procedures. One interesting notion coming out of this study is that complexity is a constant value for a given patient regardless of the center where he is operated. The Aristotle complexity score was further applied to 26 centers reporting to the EACTS congenital database. A new display of centers is presented based on the comparison of hospital survival to complexity and to our proposed definition of performance. CONCLUSION: A complexity-adjusted method named the Aristotle Score, based on the complexity of the surgical procedures has been developed by an international group of experts. The Aristotle score, electronically available, was introduced in the EACTS and STS databases. A validation process evaluating its predictive value is being developed.

Functional limitations in young children with congenital heart defects after cardiac surgery.

UNLABELLED: With the recent dramatic decline in mortality rates of infants undergoing open-heart surgery (OHS), there is growing concern regarding neurodevelopmental sequelae. Outcome studies have primarily focused on delineating developmental impairments; however, the impact on function and family burden has not been investigated. The objective of this study was to determine the prevalence of functional limitations and burden of care of young children with congenital heart defects (CHD) after OHS. STUDY DESIGN: One hundred thirty-one eligible infants with CHD undergoing their first OHS were recruited prospectively. Patients were assessed pre- and postoperatively, and again 12 to 18 months after surgery. Functional assessments included the WeeFIM (Functional Independence Measure) and the Vineland Adaptive Behavior Scale. RESULTS: For the WeeFIM, mean quotients were 84.3 +/- 23.8 (self-care), 77.2 +/- 30.0 (mobility), and 92.4 +/- 27.8 (cognition), with an overall quotient of 83.8 +/- 23.4. Only 21% of the cohort was functioning within their expected age range. Moderate disability was noted in 37%, while only 6% demonstrated a severe disability. For the Vineland scale, mean score for daily living skills was 84.4 +/- 17.6, and 80.3 +/- 15.9 for socialization. Functional difficulties in daily living skills were documented in 40%, whereas >1/2 had poor socialization skills. Factors enhancing risk for functional disabilities included perioperative neurodevelopmental status, microcephaly, length of deep hypothermic circulatory arrest, length of stay in the intensive care unit, age at surgery, and maternal education. CONCLUSIONS: The high prevalence of functional limitations and dependence in activities of daily living is currently underappreciated in the clinical setting, and deserves additional attention by pediatricians and developmental specialists.

Systemic steroid pretreatment improves cerebral protection after circulatory arrest.

BACKGROUND: This study evaluates whether systemic steroid pretreatment enhances neuroprotection during deep hypothermic circulatory arrest (DHCA) compared with steroid in cardiopulmonary bypass (CPB) prime. METHODS: Four-week-old piglets randomly placed into two groups (n = 5 per group) were given methylprednisolone (30 mg/kg) into the pump prime (group PP), or pretreated intravenously 4 hours before CPB (group PT). All animals underwent 100 minutes of DHCA (15 degrees C), were weaned off CPB, and were sacrificed 6 hours later. Postoperative changes in body weight, bioimpedance, and colloid oncotic pressure (COP) were measured. Cerebral trypan blue content, immunohistochemical evaluation of transforming growth factor-beta1 (TGF-beta1) expression, and caspase-3 activity were performed. RESULTS: Percentage weight gain (group PP 25.0% +/- 10.4% versus group PT 12.5% +/- 4.0%; p = 0.036), and percentage decrease in bioimpedance (PP 37.2% +/- 14.5% versus PT 15.6% +/- 7.9%; p = 0.019) were significantly lower, whereas postoperative COP was significantly higher in group PT versus group PP (PT 15.3 +/- 1.8 mm Hg versus PP 11.6 +/- 0.8 mm Hg; p = 0.003). Cerebral trypan blue (ng/g dry tissue) was significantly lower in group PT (PT 5.6 x 10(-3) +/- 1.1 x 10(-3) versus PP 9.1 x 10(-3) +/- 5.7 x 10(-4); p = 0.001). Increased TGF-beta1 expression and decreased caspase-3 activity were shown in group PT. CONCLUSIONS: Systemic steroid pretreatment significantly reduced total body edema and cerebral vascular leak and was associated with better immunohistochemical indices of neuroprotection after DHCA.

Neonatal aortic arch reconstruction avoiding circulatory arrest and direct arch vessel cannulation.

BACKGROUND: Aortic arch reconstruction in neonates routinely requires deep hypothermic circulatory arrest. We reviewed our experience with techniques of continuous low-flow cerebral perfusion (LFCP) avoiding direct arch vessel cannulation. METHODS: Eighteen patients, with a median age of 11 days (range 1 to 85 days) and a mean weight of 3.2 +/- 0.8 kg, underwent aortic arch reconstruction with LFCP. Seven had biventricular repairs with arch reconstruction, 9 underwent the Norwood operation and 2 had isolated arch repairs. In 1 Norwood and 7 biventricular repair patients, LFCP was maintained by advancing the cannula from the distal ascending aorta into the innominate artery. In 8 of 9 Norwood patients, LFCP was maintained by directing the arterial cannula into the pulmonary artery confluence and perfusing the innominate artery through the right modified Blalock-Taussig shunt fully constructed before cannulation for cardiopulmonary bypass. In 2 patients requiring isolated arch reconstruction, the ascending aorta was cannulated and the cross-clamp was applied just distal to the innominate artery. RESULTS: LFCP was maintained at 0.6 +/- 0.2 L x min(-1) x m(-2) for 41.0 +/- 13.9 minutes at 18.5 degrees C +/- 1.1 degrees C. In 10 of the 18 patients, blood pressure during LFCP was 15 +/- 8 mm Hg remote from the innominate artery (left radial, umbilical or femoral arteries). In 8 of the 18 patients, right radial pressure during LFCP was 24 +/- 10 mm Hg. The mean mixed-venous saturation was 79.8% +/- 10% during LFCP. Two patients had preoperative seizures, whereas none had seizures postoperatively. One patient died. CONCLUSIONS: Neonatal aortic arch reconstruction is possible without circulatory arrest or direct arch vessel cannulation. These techniques maintained adequate mixed-venous oxygen saturations with no associated adverse neurologic outcomes.

Association between electroencephalographic findings and neurologic status in infants with congenital heart defects.

Neurologic status is of concern in infants with congenital heart defects undergoing open heart surgery. The association between perioperative electroencephalography (EEG) with acute neurologic status and subsequent outcome was examined in a cohort of 60 infants. Preoperative EEG and neurologic examinations were performed within 1 to 2 days prior to surgery (n = 27) and postoperatively (n = 47). Prior to surgery, 15 of 27 infants had normal EEG, whereas 5 had epileptiform activity and 9 had disturbances in background activity that were primarily moderate (8/9) and diffuse (7/9). Postoperatively, only 17 of 47 infants had normal recordings. Newborns (<1 month) were more likely (P< .001) to demonstrate EEG abnormalities than infants. Epileptiform activity was documented in 15, whereas 28 had background abnormalities that were moderate-severe (22/28) and diffuse (20/28) in most. Epileptiform activity prior to surgery was always associated with an abnormal neurologic examination, and this association persisted postoperatively (86%). Moderate to severe background abnormalities in the postoperative EEG was also strongly associated with acute neurologic abnormalities (93%). Severe background abnormalities (n = 5) were 100% predictive of death or severe disability. Long-term follow-up revealed that all children with normal postoperative EEGs had positive neurologic outcomes (P = .04); however, there were many false positives. Perioperative EEG abnormalities increased the likelihood for acute neurologic findings, whereas normal recordings following surgery were reassuring with regard to a favorable outcome.

Transposition complexes with systemic obstruction.

The arterial switch operation is currently the procedure of choice for transposition of the great arteries and double-outlet right ventricle with subpulmonary ventricular septal defect. While the results of surgical repair have improved tremendously in recent years, the presence of associated lesions continues to make this a surgically challenging malformation. The association of these so-called transposition complexes with systemic obstruction has recently received increased attention. Systemic obstruction may occur at the subaortic level, in the aortic arch, or at both levels. Valvar aortic stenosis or atresia is extremely rare. Resection of hypertrophied muscle bundles with or without pericardial patch augmentation is frequently enough to deal with obstruction at the subaortic level, which becomes the subpulmonary area following arterial switch operation. Aortic arch obstruction associated with intracardiac defects has traditionally been addressed with a staged approach, dealing first with the arch obstruction followed later by intracardiac repair. The results with this approach have been disappointing. At the Montréal Children's Hospital, we have obtained superior results using a single-stage approach. Therefore, we have advocated the use of pulmonary homograft patch aortoplasty for aortic arch reconstruction at the time of intracardiac repair to completely remove any anatomic afterload. Since 1989, in 22 consecutive patients undergoing single-stage anatomic repair of transposition complexes associated with aortic arch obstruction, we have had no early deaths, one late death of a noncardiac cause, and one recoarctation requiring balloon dilatation. In the last 2 years, we have been able to perform all our aortic arch reconstructions avoiding the use of circulatory arrest.

Two-Ventricle repair for hypoplastic left heart syndrome.

Hypoplastic left heart syndrome encompasses a spectrum of structural cardiac malformations characterized by severe underdevelopment of the structures in the left heart-aorta complex. The severe end of the spectrum consists of aortic atresia and mitral atresia, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, recently described as hypoplastic left heart complex. These patients may be candidates for biventricular repair. Since November 1988, 12 of 13 patients (92%) with hypoplastic left heart complex have undergone two-ventricle repair and one patient the Norwood operation. Median age at surgery was 7 days (5 to 45 days) and median weight was 3.59 kg (2.6 to 4.3 kg). Biventricular repair consisted of pulmonary homograft patch aortoplasty of the aortic arch and ascending aorta and closure of the interatrial and interventricular communications. The left heart was able to support the systemic circulation in 11 of 12 patients undergoing biventricular repair (92%). The early mortality was two patients (15.4%). The mean follow-up was 67 months (range, 11 months to 133 months). There was one late death at 39 months. Reoperations were necessary in four patients for left ventricular outflow tract obstruction and in two patients for recurrent coarctation. We have successfully achieved biventricular repair in most patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Finally, with increased experience, predictive criteria for the feasibility and optimal outcome of biventricular repair will be more accurately defined.

Single ventricle with systemic obstruction in early life: comparison of initial pulmonary artery banding versus the Norwood operation.

OBJECTIVES: The outcomes of initial pulmonary artery banding (PAB)+/-coarctation repair are compared with the Norwood operation in newborns with single ventricle (SV) and systemic obstruction (SO). METHODS: Between January 1987 and July 2000, 22 patients (median age, 12 days) with SV and aortic arch obstruction (AAO), subaortic stenosis (SAS), or both underwent surgery. Two initial surgical approaches were used: PAB+/-coarctation repair (group I, seven patients); Norwood type operation (group II, 15 patients). RESULTS: The overall mortality was 32% (seven of 22 patients). There was no late mortality. The mortality in group I was 43% versus 27% in group II. Recently, there has been no mortality following the Norwood operation in the last eight patients operated since 1995. Of the survivors, nine patients have undergone the Fontan operation and four patients have had the bidirectional Glenn (BDG) with no deaths. There was one repair of supravalvar aortic stenosis at the time of BDG in group II as opposed to eight reinterventions for SAS and/or AAO in four patients in group I (P=0.01). CONCLUSIONS: PAB+/-coarctation repair for SV and SO is associated with a high mortality and a high reoperation rate for SAS or recurrent AAO. Although the Norwood operation was also associated with a high mortality early on, it can now be performed with excellent outcome. This improvement, combined with a low reintervention rate for SAS or AAO, suggests that the Norwood operation is likely to emerge as the procedure of choice for SV and SO.

Surgical technique to avoid circulatory arrest and direct arch vessel cannulation during neonatal aortic arch reconstruction.

Deep hypothermic circulatory arrest (DHCA) has been used routinely for surgery involving the aortic arch. Recently, techniques have been developed that avoid circulatory arrest and maintain low-flow cerebral perfusion (LFCP) in an attempt to avoid the potential neurological sequelae associated with DHCA. We describe a technique of LFCP that avoids circulatory arrest and direct cannulation of the arch vessels. Five patients underwent reconstruction of the aortic arch with concomitant biventricular intracardiac repair. The distal ascending aorta was cannulated and patients were systemically cooled. The cannula was advanced into the innominate artery and snared in place prior to opening and reconstructing the aorta with continuous LFCP. In all five patients, we completely avoided circulatory arrest and direct cannulation of the arch vessels. All patients survived and there were no adverse neurological outcomes.

Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society.

OBJECTIVES: We sought to determine factors that would predict whether a biventricular repair or Norwood procedure pathway would give the best survival in neonates with critical aortic stenosis. METHODS: Survival and risk factors were determined with parametric time-dependent event analysis for patients undergoing either the Norwood procedure or biventricular repair, and predicted optimal pathway and survival benefit were derived from multivariable linear regression. RESULTS: From 1994 to 2000, 320 neonates with critical left ventricular outflow obstruction were entered into a prospective multi-institutional study. Patients who died without intervention (n = 19) and those with primary cardiac transplantation (n = 6) were excluded. An initial intended biventricular repair pathway was indicated in 116 patients, with survival of 70% at 5 years. An initial Norwood procedure was performed in 179 patients, with survival of 60% at 5 years. Using predictions from separate multivariable hazard models for survival with each of the 2 pathways, we determined predicted optimal pathway and survival benefit for each patient. Significant independent factors associated with greater survival benefit for the Norwood procedure versus biventricular repair included younger age at entry, lower z-score of the aortic valve and left ventricular length, higher grade of endocardial fibroelastosis, absence of important tricuspid regurgitation, and larger ascending aorta. Predicted survival benefit favored the Norwood procedure in 50% of patients who had biventricular repair, and it favored biventricular repair in 20% of patients who had the Norwood procedure. CONCLUSIONS: Morphologic and functional factors can be used to predict optimal pathway and survival benefit in neonates with critical left ventricular outflow obstruction.

Clips versus suture technique: is there a difference?

INTRODUCTION: Coronary artery bypass grafting (CABG) is one of the most common procedures performed today, and wound complications are a major source of morbidity and cost. OBJECTIVE: To determine whether there is any difference in wound outcome (including cost in a Canadian context) between a subcuticular suture technique and skin stapling technique for closure of sternal and leg incisions in CABG patients. PATIENTS AND METHODS: One hundred and sixty-two patients undergoing CABG were prospectively, randomly placed to have their sternal and leg incisions closed with either a subcuticular suture technique or with a skin clip. Data were obtained through chart review, in-hospital assessments and follow-up visits. Nonblinded assessments were made regarding wound leakage, inflammation, infection, necrosis, swelling, dehiscence and cosmesis. Each of the parameters was graded on a scale from 1 to 4. The cost was evaluated in Canadian dollars. RESULTS: There were trends toward increased rates of in-hospital sternal (P=0.09) and leg (P=0.17) incision inflammation when the wounds were closed with skin clips. There was a significantly greater (P=0.05) rate of sternal wound infection with clips, as well as a tendency (P=0.15) toward a greater rate of mediastinitis at follow-up assessment. Cosmetic outcome was similar for both groups. The cost incurred was significantly greater when skin clips were used for closure. There was a greater than threefold difference, which translates to a greater than $10,000 difference over one year. CONCLUSIONS: Closure with a subcuticular technique achieves better outcomes than the use of skin clips. When factoring in the increased cost incurred by using clips, as well as other intangible factors such as surgical skill acquisition, subcuticular suture closure appears to be a favourable method of wound closure in CABG patients compared with the use of skin stapling techniques.

Norwood operation without circulatory arrest: a new surgical technique.

Circulatory arrest (CA) is associated with potential neurologic injury. We have developed a new surgical technique to eliminate CA during the Norwood operation. A modified Blalock-Taussig shunt (BTS) was fully constructed before cannulation for cardiopulmonary bypass. The aortic cannula was inserted in the patent ductus arteriosus to allow systemic cold perfusion. When deep hypothermia was reached, the aortic cannula was redirected into the pulmonary artery (PA) confluence. Both cerebral and systemic perfusion were maintained through the right PA and BTS into the innominate artery.

Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery.

BACKGROUND: Neurodevelopmental disabilities in children with congenital heart defects (CHDs) have been primarily attributed to intraoperative events without consideration of preoperative and postoperative factors. OBJECTIVE: To describe the preoperative and postoperative neurodevelopmental status of newborns and infants with CHDs. STUDY DESIGN: One hundred thirty-one children (56 newborns and 75 infants) were evaluated before and after surgery by using standardized neurobehavioral (newborn) and motor assessments (infant) and neurologic examinations. RESULTS: In newborns, neurobehavioral abnormalities were documented in >50% before surgery, with abnormalities persisting in most after surgery. In infants, neurodevelopmental abnormalities were observed in 38% before surgery. There was a significant association between preoperative and postoperative neurodevelopmental status, with status remaining unchanged in most. Newborns with acyanotic heart lesions were more likely to demonstrate neurologic compromise than those with cyanotic defects. For infants, arterial oxygen saturations <85% were significantly associated with an abnormality. There was a trend for a longer circulatory arrest time to be associated with greater risk for neurologic sequelae in newborns, whereas prolonged cardiopulmonary bypass was an important risk factor for infants. CONCLUSIONS: Neurodevelopmental abnormalities are common in young infants with CHDs and are often present before open heart surgery. These developmental concerns are clinically underappreciated. Early systematic developmental screening may be warranted in this population of interest.

Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia--ventricular septal defect.

Pulmonary atresia (PA) and ventricular septal defect (VSD) is a complex and extremely heterogeneous cardiopulmonary malformation that has not been accurately defined, as evidenced by the synonymous use of the term with tetralogy of Fallot with PA. The anatomy and morphology of the pulmonary circulation to a large extent determines the surgical approach and overall outcome, with the intracardiac anatomy playing a secondary role. Based on the characterization of the pulmonary circulation a new classification of PA-VSD is proposed. In type A, there are only native pulmonary arteries (NPA). In type B, pulmonary blood flow is provided by both NPA and by major aortopulmonary collateral arteries [MAPCA(s)]. In type C, there are only MAPCA(s) and no NPA. This new classification is proposed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) encompasses a spectrum of structural cardiac malformations that are characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity and mass. The severe end of the spectrum consists of aortic atresia and mitral atresia with a nonexistent left ventricle, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, and milder degrees of left ventricular hypoplasia, recently described as hypoplastic left heart complex (HLHC). Although the overwhelming majority of the patients can only have a univentricular repair, a small minority of patients with HLHS, particularly those that are described as having HLHC, may be candidates for biventricular repair. In this paper, the extant nomenclature for HLHS is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Nomenclature and Database Committee and representatives from the European Association for Cardiothoracic Surgery. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented, which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing, and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle.

Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle. Although the presence of aortic-mitral discontinuity and bilateral coni are important descriptors, they should not serve as absolute prerequisites for the diagnosis of DORV. The morphology of DORV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the great artery relationships to each other, the coronary artery anatomy, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), the tricuspid-pulmonary annular distance, and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the left ventricle to the systemic circulation by an intraventricular tunnel repair connecting the VSD to the systemic semilunar valve. This ideal surgical therapy is not always possible due to the presence of confounding anatomical barriers. A multitude of alternative surgical procedures has been devised to accommodate these more complex situations. A framework for the development of the DORV module for a pediatric cardiac surgical database is proposed.

Congenital Heart Surgery Nomenclature and Database Project: double outlet left ventricle.

Double outlet left ventricle (DOLV) is a type of ventriculoarterial connection in which both great arteries arise entirely or predominantly from the left ventricle. Although it was initially believed that bilateral absence of conus is a prerequisite for such diagnosis, all possible conal configurations have been described in this malformation. The morphology of DOLV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the right ventricle to the pulmonary circulation by an intraventricular tunnel repair connecting the VSD to the pulmonary semilunar valve. This ideal surgical therapy is not always possible, because of the presence of confounding anatomical barriers. Several alternative surgical procedures have been devised to accommodate these more complex situations. A framework for the development of the DOLV module of a pediatric cardiac surgical database is proposed.

Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries.

OBJECTIVE: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit. METHODS: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway. RESULTS: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years). CONCLUSIONS: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.

Multimodality evoked potential findings in infants with congenital heart defects.

Evoked potentials are sensitive prognostic tools in young infants at risk for developmental disability. The objective of this prospective study was to determine whether infants with congenital heart defects demonstrate evoked potential abnormalities prior to or following open heart surgery, and to examine the association between these abnormalities and developmental status 1 year following surgery. A consecutive series of newborns (less than 1 month old) and infants (1 month to 2 years old) were recruited. Somatosensory and brain stem auditory evoked potentials were carried out before or after cardiac surgery, or both. One year later, neurologic examination and standardized measures of motor performance and functional independence were carried out. Twenty-seven newborns and 31 infants underwent perioperative somatosensory evoked potential recordings. Results indicate that perioperative somatosensory evoked potential abnormalities were common in newborns (41%) but not in infants (13%) with congenital heart defects. Brainstem conduction times were within normal limits in all subjects; however, 32% presented with mild elevations in hearing thresholds. All newborns with abnormal somatosensory evoked potentials had abnormal neurologic examinations both perioperatively and again 1 year after open heart surgery. Moreover, standardized developmental assessments 1 year following surgery indicate that all newborns with somatosensory evoked potential abnormalities had developmental deficits in one or more domains. Somatosensory evoked potential abnormalities in the perioperative period are common in newborns with congenital heart defects, and are strongly predictive of persistent developmental delay later.

Hemolytic anemia after atrioventricular septal defect repair without synthetic material.

We report a rare case of severe hemolytic anemia following repair of a congenital heart defect without the use of prosthetic material. A review of the literature, diagnosis, and management are described. Although this is an unusual complication following congenital heart surgery, a high index of suspicion must be maintained and a possible mechanical cause should be sought and corrected.