Evolution of the Fontan-Kreutzer procedure.

The surgical techniques used to construct the Fontan-Kreutzer circulation have evolved considerably since the initial reports. In the early years, it was thought that a power source other than the main ventricular chamber was useful or even necessary to serve the pulmonary circulation. Better understanding of the physiology of the Fontan-Kreutzer circulation has led to an understanding that avoidance of flow disturbances and energy losses in the surgically constructed pathways is critically important. In vitro studies and clinical investigations of flow dynamics led to the introduction of the total cavopulmonary connection, or total cavopulmonary connections, designed to minimize flow disturbances and the resultant energy losses. Other important surgical modifications include staging with a superior cavopulmonary connection and creation of a fenestration. These innovations have resulted in extension of the Fontan-Kreutzer procedure to the management of complex univentricular hearts and, in particular, the management of hypoplastic left heart syndrome. There have been significant improvements of early mortality and morbidity following the Fontan-Kreutzer procedure. Yet it is important to recognize that there is continuing late attrition and morbidity for patients with the Fontan-Kreutzer circulation.

Rescue surgery following percutaneous pulmonary valve implantation.

OBJECTIVE: Percutaneous pulmonary valve insertion (PPVI) is an evolving alternative to surgical pulmonary valve insertion. The aim of this study is to review the acute complications of PPVI requiring emergency rescue surgery. PATIENTS AND METHODS: Between 09/2000 and 01/2007, 152 patients (pts), received a PPVI. Patient's charts were reviewed in retrospect. RESULTS: Emergency rescue surgery (ERS) took place in 6 pts (3.9%). Indications for ERS were: homograft rupture two pts, dislodgment of the stented valve in a dilated right ventricular outflow tract two pts, occlusion of the right pulmonary artery one pt and compression of the left main coronary artery one pt. Cardiopulmonary bypass was established through repeat sternotomy incision with femorofemoral cannulation in 2/6 pts. The stented valve was removed in five and replaced with a homograft in three and a valved conduit in two pts. One ruptured homograft was repaired leaving the stented valve in situ. All patients survived, one sustained mild neurological impairment. CONCLUSION: Although some of the acute complications of PPVI were probably related to a learning curve (4 among the first 50 pts and 2 among the last 102 patients) the need for ERS is unlikely to be completely abolished. This experience highlights the importance of close collaboration between cardiologists and surgeons in these evolving technologies. Highly skilled and responsive surgical back up is necessary to support the introduction and to sustain institutional programmes such as PPVI.

Improving patient safety by identifying latent failures in successful operations.

BACKGROUND: The risk of technical failure during operations is recognized, but there is evidence that further improvements in safety depend on systems factors, in particular, effective team skills. The hypotheses that small problems can escalate to more serious situations and that effective teamwork can prevent the development of serious situations, were examined to develop a method to assess these skills and to provide evidence for improvements in training and systems. METHOD(S): Observations were made during 24 pediatric cardiac and 18 orthopedic operations. Operations were classified by accepted indicators of risk and the observations used to generate indicators of performance. Negative events were recorded and organized into 3 levels of clinical importance (minor problems, those negative events that were seemingly innocuous; intraoperative performance, the proportion of key operating tasks that were disrupted; and major problems, events that compromised directly the safety of the patient or the quality of the treatment). The ability of the team to work together safely was classified using a validated scale adapted from research in aviation. Operative duration was also recorded. RESULT(S): Both escalation and teamwork hypotheses were supported. Multiple linear regression suggests that for every 3 minor problems above the 9.9 expected per operation (P <.001), intraoperative performance reduces by 1% (P = .005), and operative duration increases by 10 minutes (P = .032). Effective teams have fewer minor problems per operation (P = .035) and consequently higher intraoperative performance and shorter operating times. Operative risk affected intraoperative performance (P = .004) and duration (P <.001), with the type of operation affecting only duration (P <.001). Eight major problems were observed; these showed a strong association with risk, intraoperative performance, teamwork, and the number of minor problems. CONCLUSION(S): Structured observation of effective teamwork in the operating room can identify substantive deficiencies in the system, even in otherwise successful operations. Decreasing the number of minor problems can lead to a smoother, safer, and shorter operation. Effective teamwork can help decrease the number of small problems and prevent them from escalating to more serious situations. The most effective and sustainable route to improved safety is in capturing these minor problems and identifying related system improvements, combined with training in safe team working. This method is a validated and practical way to improve performance during otherwise successful operations.

Multiscale modelling in biofluidynamics: application to reconstructive paediatric cardiac surgery.

Multiscale computing is a challenging area even in biomechanics. Application of such a methodology to quantitatively compare postoperative hemodynamics in congenital heart diseases is very promising. In the treatment of hypoplastic left heart syndrome, which is a congenital heart disease where the left ventricle is missing or very small, the necessity to feed the pulmonary and systemic circulations is obtained with an interposition shunt. Two main options are available and differ from the sites of anastomoses: (i) the systemic-to-pulmonary conduit (Blalock-Taussig shunt known as the Norwood Operation (NO)) connecting the innominate artery (NO-BT) or the aorta (NO-CS) to the right pulmonary artery and (ii) the right ventricle to pulmonary artery shunt (known as Sano operation (SO)). The proposition that the SO is superior to the NO remains controversial. 3-D computer models of the NO (NO-BT and NO-CS) and SO were developed and investigated using the finite volume method. Conduits of 3, 3.5 and 4 mm were used in the NO models, whereas conduits of 4, 5 and 6 mm were used in the SO model. The hydraulic nets (lumped resistances, compliances, inertances and elastances) which represent the systemic, coronary and pulmonary circulations and the heart were identical in the two models. A multiscale approach was adopted to couple the 3-D models with the circulation net. Computer simulation results were compared with post-operative catheterization data. Results showed that (i) there is a good correlation between predicted and observed data: higher aortic diastolic pressure, decreased pulmonary arterial pressure, lower pulmonary-to-systemic flow ratio and higher coronary perfusion pressure in SO; (ii) there is a minimal regurgitant flow in the SO conduit. The close correlation between predicted and observed clinical data supports the use of mathematical modelling, with a mandatory multiscale approach, in the design and assessment of surgical procedures.

Does the Aristotle Score predict outcome in congenital heart surgery?

OBJECTIVE: The Aristotle Score has been proposed as a measure of 'complexity' in congenital heart surgery, and a tool for comparing performance amongst different centres. To date, however, it remains unvalidated. We examined whether the Basic Aristotle Score was a useful predictor of mortality following open-heart surgery, and compared it to the Risk Adjustment in Congenital Heart Surgery (RACHS-1) system. We also examined the ability of the Aristotle Score to measure performance. METHODS: The Basic Aristotle Score and RACHS-1 risk categories were assigned retrospectively to 1085 operations involving cardiopulmonary bypass in children less than 18 years of age. Multiple logistic regression analysis was used to determine the significance of the Aristotle Score and RACHS-1 category as independent predictors of in-hospital mortality. Operative performance was calculated using the Aristotle equation: performance = complexity x survival. RESULTS: Multiple logistic regression identified RACHS-1 category to be a powerful predictor of mortality (Wald 17.7, p < 0.0001), whereas Aristotle Score was only weakly associated with mortality (Wald 4.8, p = 0.03). Age at operation and bypass time were also highly significant predictors of postoperative death (Wald 13.7 and 33.8, respectively, p < 0.0001 for both). Operative performance was measured at 7.52 units. CONCLUSIONS: The Basic Aristotle Score was only weakly associated with postoperative mortality in this series. Operative performance appeared to be inflated by the fact that the overall complexity of cases was relatively high in this series. An alternative equation (performance = complexity/mortality) is proposed as a fairer and more logical method of risk-adjustment.

Quality assurance in congenital heart surgery.

OBJECTIVE: The aim of this study was to develop a graphical method of risk-stratified outcome analysis in paediatric cardiac surgery to provide a means of continuous, prospective performance monitoring and allow real-time detection of change in outcomes. METHODS: Risk-adjusted survival following open-heart surgery was prospectively measured over a 15-month period (n=460). Outcomes were charted using variable life-adjusted display (VLAD) charts, which indicate the cumulative difference in observed minus expected survival against the cumulative number of cases performed. Risk stratification was based on RACHS-1 (risk adjustment in congenital heart surgery) risk category and age at surgery, using our previously published risk model. The probability of deviation in performance from the expected baseline level was determined using a mathematical model. RESULTS: By the end of the series, observed survival (443/460=96.3%) exceeded that predicted by the risk model (434.5/460=94.5%), equivalent to a one-third reduction in expected mortality. Mathematical modelling indicated a 1-5% likelihood that this difference would have occurred by random variation alone, suggesting the outcomes represented genuine improvement. CONCLUSIONS: VLAD charts provide an effective, easily visualised display of surgical performance and can be applied to paediatric cardiac surgery. Early detection of change, whether improvement or deterioration, is important for ongoing quality assurance within a cardiac surgery programme.

Anatomic variability in coronary arterial distribution with regard to the arterial switch procedure.

BACKGROUND: We investigated the coronary arterial origins and course and the position of the great arteries in hearts with discordant ventriculoarterial connections. At the same time, we sought to evaluate the practicality of alphanumeric classifications in accounting for surgically relevant features of the coronary arteries. METHODS AND RESULTS: We studied 200 postmortem hearts, noting the patterns of coronary arterial branching, the vertical and horizontal location of the arterial orifices within the aortic sinuses, the course of the proximal coronary arteries in relation to the aortic wall, and the relations of the great arteries and their respective commissures. All hearts examined had concordant atrioventricular and discordant ventriculoarterial connections. We found 7 of the 8 predicted patterns for sinusal origin of the 3 major coronary arteries and identified 5 different positions of the arterial trunks relative to each other. A correlation was found between less frequent relationships of the arterial trunks and unusual patterns of coronary arterial branching, as well as with mismatch between the valvar commissures. CONCLUSIONS: The surgically relevant features of the coronary arteries in hearts with discordant ventriculoarterial connections are best described rather than classified. Correlations exist between certain, less frequent relations of the great arteries and unusual patterns of branching of the coronary arteries. The presence of unusual great arterial positions should alert the surgeon to potentially complicated arrangements of the origin and distribution of the coronary arteries.

Extending the boundaries of the primary arterial switch operation in patients with transposition of the great arteries and intact ventricular septum.

BACKGROUND: We have previously suggested that the primary arterial switch operation is a feasible strategy for patients with transposition of the great arteries and intact ventricular septum (TGA-IVS) up to age 2 months. This study reports our current results with this approach and examines whether this policy could be extended beyond age 2 months. METHODS AND RESULTS: 380 patients who underwent arterial switch for TGA-IVS were reviewed. 275 patients were younger than 3 weeks at the time of surgery (early switch group); 105 patients were 3 weeks or older (range, 21 to 185 days) (late switch group). There was no difference in outcome in terms of in-hospital mortality (5.5% versus 3.8%) or need for mechanical circulatory support (3.6% versus 5.7%) between early and late switch groups. However, duration of postoperative ventilation (4.9 versus 7.1 days, P=0.012) and length of postoperative stay (12.5 versus 18.9 days, P<0.001) were significantly prolonged in the late switch group. Primary left ventricular failure resulting in death occurred in 2 patients in the late switch group, with no deaths in 9 patients aged 2 to 6 months. CONCLUSIONS: This experience confirms that in TGA-IVS, the left ventricle maintains the potential for systemic work well beyond the first month of life. Consequently, neonates at high risk or late referrals can benefit from delayed arterial switch, even beyond age 2 months. However, the need for mechanical support in some of the older patients may limit the widespread adoption of such a strategy.

The Fontan circulation: a challenge to William Harvey?

The Fontan operation, which places the systemic and pulmonary circulations in series and is driven by a single ventricular chamber, is the treatment of choice for patients born with one ventricle. Its introduction 35 years ago was the result of a flurry of experimental and clinical research that had started in the 1940s. A large number of children have benefited and continue to benefit from the Fontan operation, but there is a genuine concern that, despite the refinement of the surgical procedures in the past 20 years, continuing attrition might be inevitable. This adverse effect can lead to a decline in functional capacity, and premature late death.

Multiscale modeling of the cardiovascular system: application to the study of pulmonary and coronary perfusions in the univentricular circulation.

The objective of this study is to compare the coronary and pulmonary blood flow dynamics resulting from two configurations of systemic-to-pulmonary artery shunts currently utilized during the Norwood procedure: the central (CS) and modified Blalock Taussig (MBTS) shunts. A lumped parameter model of the neonatal cardiovascular circulation and detailed 3-D models of the shunt based on the finite volume method were constructed. Shunt sizes of 3, 3.5 and 4 mm were considered. A multiscale approach was adopted to prescribe appropriate and realistic boundary conditions for the 3-D models of the Norwood circulation. Results showed that the average shunt flow rate is higher for the CS option than for the MBTS and that pulmonary flow increases with shunt size for both options. Cardiac output is higher for the CS option for all shunt sizes. Flow distribution between the left and the right pulmonary arteries is not completely balanced, although for the CS option the discrepancy is low (50-51% of the pulmonary flow to the right lung) while for the MBTS it is more pronounced with larger shunt sizes (51-54% to the left lung). The CS option favors perfusion to the right lung while the MBTS favors the left. In the CS option, a smaller percentage of aortic flow is distributed to the coronary circulation, while that percentage rises for the MBTS. These findings may have important implications for coronary blood flow and ventricular function.

The potential impact of percutaneous pulmonary valve stent implantation on right ventricular outflow tract re-intervention.

OBJECTIVE: To assess the impact of a percutaneous technique for pulmonary valve implantation on the conventional surgical valve/conduit approach to right ventricular outflow tract re-intervention. METHODS: We have retrospectively reviewed our results following surgical or percutaneous re-intervention to the right ventricular outflow tract in both paediatric and adult groups. Between November 1998 and March 2004, 94 patients underwent surgical re-intervention to the right ventricular outflow tract. Percutaneous pulmonary valve implantation was introduced in October 2002 and 35 procedures were performed to March 2004. The median age was 26 years (6-65 years) in the surgical group and 16 years (9-39 years) in the percutaneous group. Tetralogy of Fallot was the commonest original diagnosis (64.9 and 62.9%, respectively). The predominant indication for re-intervention in the surgical group was pulmonary regurgitation (64.9%) compared to the percutaneous group in which it was homograft/conduit stenosis or a mixed lesion (68.6%). RESULTS: There has been one (1.1%) early death reported in the surgical series and none in the percutaneous group. In the surgical group 9 (9.6%) experienced a procedural complication whilst 3 (8.5%) of those undergoing a percutaneous valve experienced a significant procedural event necessitating urgent surgery. Important early morbidity was 8 (8.5%) in the surgical group and 2 (5.7%) in the percutaneous group. Freedom from re-operation at 1 year was 100% in the surgical group and 86.1% in the percutaneous group due to late restenosis. Median hospital stay in the surgical group was 7 (4-114) days and 2 (2-22) days in the percutaneous group. CONCLUSIONS: Preliminary data suggests that percutaneous pulmonary valve implantation provides a promising additional and complementary approach to a successful surgical programme. Both approaches are safe with acceptable levels of morbidity and low mortality. With current technology the aneurysmal outflow tract remains a problem for the percutaneous approach. Follow-up remains too short, at present, to prove longevity of the percutaneous conduit.

On the use of computational models for the quantitative assessment of surgery in congenital heart disease.

The surgical repair of congenital heart disease often involves significant modifications to the circulatory tree. Resections, reconstructions, graft insertions and the deployment of implants and biomedical devices have an impact on local and systemic haemodynamics, which may be difficult to foresee or to assess quantitatively by clinical investigation alone. Mathematical models can be employed to visualise, estimate or predict events and physical quantities that are difficult to observe or measure, and can be successfully applied to the study of the pre- and post-operative physiology of cardiovascular malformations. This paper analyses the potentialities of computation fluid dynamics in this respect, outlining the method, its requirements and its limitations. Examples are given of lumped parameter models, axi-symmetric models, three-dimensional models, fluid-structure interaction simulations and multiscale computing applied to total cavo-pulmonary connection, aortic coarctation and aortic arch reconstruction.

Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the Norwood procedure for hypoplastic left heart syndrome.

OBJECTIVE: Computational fluid dynamics have been used to study the hemodynamic performance of surgical operations, resulting in improved design. Efficient designs with minimal energy losses are especially important for cavopulmonary connections. The purpose of this study was to compare hydraulic performance between the hemi-Fontan and bidirectional Glenn procedures, as well as the various types of completion Fontan operations. METHODS: Three-dimensional models were constructed of typical hemi-Fontan and bidirectional Glenn operations according to anatomic data derived from magnetic resonance scans, angiocardiograms, and echocardiograms. Boundary conditions were imposed, and fluid dynamics were calculated from a mathematic code. Power losses, flow distribution to each lung, and pressures were measured at three predetermined levels of pulmonary arteriolar resistance. Models of the lateral tunnel, total cavopulmonary connection, and extracardiac conduit completion Fontan operations were constructed, and power losses, total flow distribution, vena caval and pulmonary arterial pressures, and flow distribution of inferior vena caval return were calculated. RESULTS: The hemi-Fontan and bidirectional Glenn procedures performed nearly identically, with similar power losses and nearly equal flow distributions to each lung at all levels of pulmonary arteriolar resistance. However, the lateral tunnel Fontan procedure as performed after the hemi-Fontan operation had lower power losses (6.9 mW, pulmonary arteriolar resistance 3 units) than the total cavopulmonary connection (40.5 mW) or the extracardiac conduit (42.9 mW), although the inclusion of an enlargement patch toward the right in the total cavopulmonary connection was effective in reducing the difference (10.0 mW). Inferior vena caval flow to the right lung was 52% for the lateral tunnel, compared with 19%, 30%, 19%, and 15% for the total cavopulmonary connection, total cavopulmonary connection with right-sided enlargement patch, extracardiac conduit, and extracardiac conduit with a bevel to the left lung, respectively. CONCLUSIONS: According to these methods, the hemi-Fontan and bidirectional Glenn procedures performed equally well, but important differences in energy losses and flow distribution were found after the completion Fontan procedures. The superior hydraulic performance of the lateral tunnel Fontan operation after the hemi-Fontan procedure relative to any other method may be due to closer to optimal caval offset achieved in the surgical reconstruction.

Primary thoracoscopic treatment of empyema in children.

OBJECTIVE: The optimal treatment of pediatric empyema remains controversial. The objective of this study is to compare the use of conventional management versus primary thoracoscopic drainage and decortication in children with empyema. METHODS: Conventional management has consisted of chest drain insertion under general anesthesia plus intravenous antibiotics. Thoracoscopic drainage and decortication has consisted of primary thoracoscopic drainage and decortication plus antibiotics. The clinical course of 54 patients treated conventionally between 1989 and 1997 was compared with that of 21 patients treated by means of thoracoscopic drainage and decortication between September 2000 and September 2001. RESULTS: Results of the study demonstrated that patients in the drainage-decortication group had fewer invasive interventions per patient than those in the conventional management group (1.0 vs 1.26). Patients undergoing thoracoscopic drainage and decortication also had significantly shorter durations of intravenous antibiotic therapy (7.6 +/- 1.2 vs 18.2 +/- 7.5 days), chest tube drainage (4.0 +/- 0.5 vs 10.2 +/- 6.1 days), and hospital stays (7.4 +/- 0.8 vs 15.4 +/- 7.4). Moreover, there were no open thoracotomies and decortications in the thoracoscopic drainage and decortication group, whereas in the conventional management group 39% (21/54) of patients underwent an open procedure. CONCLUSION: Although the 2 groups were not prospectively randomized and they were treated in different time periods, the results of this study support the use of thoracoscopic surgery as the primary therapeutic modality in children presenting with pleural empyema. This strategy appears to offer significant benefits over conventional treatment in terms of duration of treatment and the need for more invasive surgery.

Surgical substrates of postoperative junctional ectopic tachycardia in congenital heart defects.

BACKGROUND: Junctional ectopic tachycardia is a major cause of postoperative morbidity after surgery for congenital cardiac disease. To elucidate the mechanism of junctional ectopic tachycardia, surgical correlations were studied in four types of congenital heart defects involving closure of a ventricular septal defect, relief of right ventricular outflow tract obstruction, or both. METHODS: Between 1997 and 1999, a total of 343 consecutive patients underwent repair of tetralogy of Fallot (n = 114), common truncus arteriosus (n = 10), ventricular septal defect (n = 161), and atrioventricular septal defect (n = 58). Variables studied included demographic and bypass data, surgical approaches toward ventricular septal defect closure and relief of right ventricular outflow tract obstruction, and resection as opposed to division of muscle bundles. RESULTS: Junctional ectopic tachycardia occurred most frequently after repair of tetralogy of Fallot (n = 25; 21.9%), with no cases occurring after repair of common trunk, 6 occurring after repair of ventricular septal defect (3.7%), and 6 occurring after repair of atrioventricular septal defect (10.3%). Stepwise logistic regression revealed that resection of muscle bundles (P <.0001), higher bypass temperatures (P <.03), and relief of right ventricular outflow tract obstruction through the right atrium (P <.05) significantly and independently predicted postoperative junctional ectopic tachycardia. CONCLUSIONS: Relief of right ventricular outflow tract obstruction appears to be more important in the causation of junctional ectopic tachycardia than does ventricular septal defect closure, which may explain the higher incidence of this complication after tetralogy of Fallot repair. Muscular resection seems to be more arrhythmogenic than is simple division. Increased traction through the right atrium for relief of right ventricular outflow tract obstruction would fit the hypothesis that enhanced automaticity of the His bundle, the morphologic substrate for junctional ectopic tachycardia, may result from direct trauma or infiltrative hemorrhage of the conduction system. When feasible, techniques avoiding both extensive muscle resection and excessive traction should be applied during resection of right ventricular outflow tract obstruction.

Improved early outcome for end-stage dilated cardiomyopathy in children.

OBJECTIVE: To review the impact of management changes on the early outcomes of end-stage dilated cardiomyopathy in children. METHODS: We conducted a retrospective study of all consecutive children with end-stage dilated cardiomyopathy who received hospital treatment since 1992. Over the past 3 years the following management changes were made: (1) more aggressive use of mechanical cardiac assistance; (2) high priority listing for transplantation; and (3) ABO incompatible transplants for infants. Outcomes for 46 patients admitted between 1992 and 1999 (group I) were compared with 53 patients between 2000 and March 2003 (group II). RESULTS: In group I, 12 (26%) patients received mechanical support with recovery in 3 and transplantation in 5 (1 died). In group II, 19 (36%) patients received extracorporeal membrane oxygenation, with recovery in 5 and transplantation in 12 (all survived). The use of mechanical assistance was associated with high morbidity related to bleeding, end-organ failure, and long-term mechanical ventilation. Five patients in group II received ABO incompatible transplants and all survived. There have been no episodes of rejection or need for increased immunosuppressive therapy. Hospital mortality has been significantly reduced (group I, 37% vs group II, 11%; P <.05). CONCLUSIONS: Recent refinements in the management of end-stage dilated cardiomyopathy in children have significantly reduced early mortality. Identification of markers of early myocardial recovery and development of mechanical devices for longer term and more physiologic support are essential to achieve further improvements in outcome.

Implications of anomalous right subclavian artery in the repair of neonatal aortic coarctation.

UNLABELLED: Spinal cord perfusion is predominantly from the anterior spinal artery, which arises from the vertebral arteries by way of the subclavian arteries. Anomalous origin of the right subclavian artery and coarctation of the aorta is considered to be an increased risk factor for spinal cord damage, possibly because of the minimal collateral circulation during aortic clamping. The aim of this study is to review 5 consecutive cases of neonatal aortic coarctation with ARSA. METHODS: Five neonates (0.8 to 4.6 kg) underwent operation between July 1999 and December 2000 with resection of the coarctation and end-to-end anastomosis. Both subclavian arteries (n = 5) and left carotid artery (n = 4) were clamped, leaving the right carotid artery as the sole provider of perfusion for the spinal cord. RESULTS: Despite clamping of both subclavian arteries, right radial artery pressure was measurable in 4 of the 5 cases. Aortic cross-clamp times varied from 12 to 26 minutes at a core temperature of 34 degrees to 35 degrees C. There was no operative mortality. None of the neonates developed any major neurologic sequelae. CONCLUSIONS: When clamping the two subclavian arteries during coarctation repair, the spinal artery is left with collateral blood flow that can theoretically originate from the carotid arteries through the circle of Willis and retrogradely down the vertebral arteries. The presence of such collateral circulation was documented as recordable blood pressure in the right radial artery during surgical repair.

Totally anomalous pulmonary venous connection directly to the superior caval vein.

OBJECTIVE: Totally anomalous pulmonary venous connection directly to the superior caval vein is unusual. It is frequently associated with major congenital heart defects such as the syndrome of right isomerism. While improved results have been reported recently for the isolated form, complex cases are still associated with a higher mortality. METHODS: We undertook surgical correction in nine patients with direct pulmonary venous connection to the superior caval vein investigated in our institution from 1991 to 1999. In four of these patients, the venous anomaly was an isolated finding, while five patients with isomeric right atrial appendages had associated cardiac malformations rendering them unsuitable for biventricular repair. In one patient with an isolated form, the venous drainage was obstructed. Two patients with isomerism had previously undergone construction of an aortopulmonary shunt. Other associated surgical procedures in the patients with right isomerism were establishment of bidirectional cavo-pulmonary anastomoses in four cases and banding of the pulmonary trunk in one. RESULTS: There were neither early nor late deaths. Reoperation was needed in one patient because of pulmonary venous stenosis. In the five patients with right isomerism, two later underwent successful creation of the Fontan circulation. CONCLUSION: It is unusual to find direct drainage of all the pulmonary veins to the superior caval vein. When seen, the venous pathway is only rarely obstructed. For this reason, when associated with right isomerism, an aortopulmonary shunt should be constructed as initial palliation, with later repair of the anomalous pulmonary venous drainage at the time of construction of a bidirectional cavo-pulmonary anastomosis. When using this policy, the surgical results can be as good for the complicated variant as for the isolated form.

Closure of recurrent VSD due to dehiscence of calcified patch.

Closure of residual ventricular septal defects may be extremely difficult in the presence of severe calcification of a previous patch. Removal of such calcification carries a risk of damaging the aortic and tricuspid valve as well as the conduction system. We describe a novel technique for closure of such a defect placing a new patch over and around the calcified one in an 18-year-old patient who had undergone initial surgery 12 years ago.

Simplified antegrade cerebral perfusion and myocardial protection during stage I Norwood procedure.

Several important modifications have been introduced in the intraoperative management of neonates with hypoplastic left heart syndrome during first-stage palliation. Among these, utilization of selective antegrade cerebral perfusion and interposition of a conduit between the right ventricle and pulmonary artery are currently favored by many centers. We briefly describe our current approach to the modified stage I Norwood procedure.