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The literature has few data regarding the use of polyclonal anti-thymocyte globulin in pediatric cardiac transplantation. We describe our single-center, retrospective study of the use of Thymoglobuline in a pediatric population. We included in the study 31 consecutive heart transplant recipients (mean age, 7.8 years; median age, 9 years; range, 4 months-17 years), who all survived surgery. To induce immunosuppression, all patients received Thymoglobuline therapy at age-dependent doses (1-1.5 mg/kg/day between 0 and 1 year; 1.5-2 mg/kg/day from 1 year to 8 years; and 2.5 mg/kg/day >8 years). Duration of treatment was 1 to 7 days. In patients <1 year, the total number of lymphocytes was maintained at >500/mm(3). Thirty of 31 patients are alive at the end of follow-up. During the first 3 months, 3 Grade 3A and 10 Grade 1A (Working Formulation grading system) rejection episodes occurred. All reversed after steroid treatment. Eleven viral infections, 2 bacterial infections, and 1 fungal infection occurred. Not all patients with infection were symptomatic but all responded successfully to treatment. One episode of post-transplantation lymphoproliferative disease regressed after decreasing immunosuppression therapy and after acyclovir therapy. At the end of follow-up, 19 patients are without steroids. Immunosuppression therapy with Thymoglobuline is safe in the pediatric age group if the number of lymphocytes is monitored strictly.

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Late survival after Mustard repair of transposition of the great arteries is generally good but is often characterized by progressive deterioration of ventricular function and by late postsurgical arrhythmias, thus imposing the need for permanent pacing. To evaluate how chronic pacing affects long-term exercise capacity, we compared two groups of these patients: group 1, comprising 12 patients, aged 9.0 +/- 2.6 years, without pacemaker; and group 2, comprising 18 patients, aged 9.3 +/- 2.0 years, with pacemaker. Patient evaluation included history, physical examination electrocardiograph, Holter monitoring, and echocardiography. Pacing modes were as follows: AAI (6 patients), AAIR (9 patients), VVI (2 patients), and VVIR (1 patient). At exercise test we evaluated exercise tolerance, maximum heart rate, blood pressure, oxygen consumption, and cardiac output at rest and at peak exercise. The two groups were comparable for all variables examinated. All pacemakers showed normal function. During the exercise, 11 of 12 patients in group 1 showed sinus rhythm, and in group 2, 11 patients showed sinus rhythm, 5 junctional rhythm, and 2 continuous pacing. There were no significant differences between groups. Chronically paced Mustard patients but with restoration of spontaneous rhythm during the exercise test do not show reduced exercise tolerance in comparison with nonpaced Mustard patients.

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The congenital absence of the pulmonary valve cusps can occur either isolated or in association with other heart lesions. We report a very rare case of a 40-day-old infant with transposition of the great arteries, ventricular septal defect, pulmonary annular stenosis, absent pulmonary valve and aneurysmal dilation of the central pulmonary arteries, who received surgical treatment at our institution.

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OBJECTIVES: The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. BACKGROUND: Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization. METHODS: A retrospective analysis was performed on the patients treated from June 1996 to December 1998. RESULTS: One hundred seventy-one children (median age 5.8 years, median weight 22.1 kg) underwent 52 device implants, 72 minimally invasive surgical operations and 50 conventional sternotomy operations. There were no deaths and no residual left to right shunt in any of the groups. The overall complication rate causing delayed discharge was 12.6% for minimally invasive surgery, 12.0% for midline sternotomy and 3.8% for transcatheter device closure (p < 0.01). The mean hospital stay was 2.8 +/- 1.0 days, 6.5 +/- 2.1 days and 2.1 +/- 0.5 days (p < 0.01); the skin-to-skin time was 196 +/- 43 min, 163 +/- 46 min and 118 +/- 58 min, respectively (p < 0.001). Extracorporeal circulation time was 49.9 +/- 10.1 min in the minithoracotomy group versus 37.2 +/- 13.8 min in the sternotomy group (p < 0.01) but without differences in aortic cross-clamping time. Sternotomy was the most expensive procedure (15,000 EUR +/- 1,050 EUR vs. 12,250 EUR +/- 472 EUR for minithoracotomy and 13,000 EUR +/- 300 EUR for percutaneous devices). CONCLUSIONS: While equally effective compared with sternotomy, the cosmetic and financial appeal of the percutaneous and minimally invasive approaches must be weighed against their greater exposure to technical pitfalls. Adequate training is needed if a strategy of surgical or percutaneous minimally invasive closure of ASD in children is planned in place of conventional surgery.

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The total cavopulmonary connection (TCPC) is a common operation, meant to restore a proper pulmonary blood flow in heart defects with only one functional ventricle. It consists of the direct connection of the venae cavae to the pulmonary arteries in a cross-shaped disposition which entails a peculiar hemodynamics: Side effects can occur, such as recirculation zones and pressure drop across the connection. Our study is aimed at the quantitative investigation of the flow field of a successful Fontan-type operation, in view of the clinical importance of assuring a nearly physiological pulmonary blood flow, especially if one considers that many pediatric patients are eligible for this operation. A glass-blown TCPC phantom, realized according to nuclear magnetic resonance data, was employed in a steady-flow loop. Thus, a realistic model of this Fontan-type operation was realized using materials which enable advanced measurement techniques such as particle image velocimetry (PIV). The mean flow rates at each branch of the cavopulmonary shunt could be independently varied with a vertical shift of the corresponding upstream reservoir. The PIV technique was used successfully in identifying the flow field characteristics. The flow field in this TCPC topology was shown to be well organized and regulated by the presence of a vortex at the confluence of the venae cavae. The effect of different loading conditions, which realistically can be found in vivo, is studied with a high spatial resolution, showing the possibility to use pulmonary resistance as a parameter in designing the surgical geometry.

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OBJECTIVE: The initial experience with the right submammary minithoracotomy incision for correction of intracardiac congenital defects is reported. METHODS: Between March 1997 and March 1999, 100 children underwent repair of congenital heart disease through this approach. Their mean age and weight were 4.6 years and 20 kg, respectively. Diagnosis included: atrial septal defect (78), ventricular septal defect (7), tetralogy of Fallot (6), partial atrioventricular canal (5), double-chambered right ventricle (3) and single ventricle with dextrocardia (1). The standard technique entailed a 5 to 6 cm right submammary incision, entering the chest through the third or fourth intercostal space (depending on the body weight), direct aortic and bicaval cannulation and aortic cross-clamping with cardioplegic protection. RESULTS: There were no hospital deaths. Postoperative morbidity included bleeding in two cases, recurrent atrial septal defect in one, spleen injury in one. The average hospital stay was 3.5 days. All patient are currently free of symptoms and medications. CONCLUSIONS: (1) This approach for repair of selected congenital cardiac malformations is technically feasible, safe and effective; (2) younger age is a facilitating factor; (3) hospital stays are effectively reduced.

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UNLABELLED: Foetal cardiac surgery is the ultimate goal in the treatment of congenital cardiac malformations. The aim of our research is to elucidate some of the features of the necessarily invasive experimental protocol to be used in an animal model of foetal cardiac surgery. In particular, we assessed the foetal placental reactivity to prolonged cardiac bypass in steady-flow conditions. METHODS: Two cases were selected to show the outcome of prolonged (> 30 minutes) extracorporeal circulation (ECC) instituted without oxygenator under steady-flow assistance. Following the instrumentation of the animal (placement of pressure, flow and myocardial fiber length transducers) and the baseline recordings, a 60-minute bypass period was established with an axial turbopump (Hemopump 14 Fr), after systemic heparinisation and artero-venous cannulation. At the end of the circulatory assistance, the cannulae were removed and a 90 minute observation period followed. The cardiac function was assessed by means of indirectly obtained P-V loops. RESULTS: Case A showed a marked reduction in the end-systolic pressure-volume relationship (ESPVR) during ECC, corresponding to a rightward shift of the P-V loop, with a gradual recovery after the assisted circulation. On the contrary, case B was subjected to progressive placental dysfunction, as evidenced by haemogasanalytical data. Consequently, the haemodynamic data also outlined a negative outcome, with high ESPVR values after bypass. CONCLUSIONS: The present study, while confirming the possibility of cardiac intervention in the foetus, underlines the critical role of minimally invasive protocol to limit both foetal stress and placental dysfunction.

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OBJECTIVE: Predicting postrepair right ventricular/left ventricular pressure ratio has prognostic relevance for patients undergoing total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. To this purpose, we currently rely on 2 novel parameters: (1) preoperative total neopulmonary arterial index and (2) mean pulmonary artery pressure changes during an intraoperative flow study. METHODS: Since January 1994, 15 consecutive patients (aged 64 +/- 54 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals were managed according to total neopulmonary arterial index. Seven patients with hypoplastic pulmonary arteries and a total neopulmonary arterial index less than 150 mm(2)/m(2) underwent palliative right ventricular outflow tract reconstruction followed by secondary 1-stage unifocalization and ventricular septal defect closure. The other 8 patients with a preoperative index of more than 150 mm(2)/m(2) underwent primary single-stage unifocalization and repair. The ventricular septal defect was closed in all cases (reopened in 1). In 9, such decision was based on an intraoperative flow study. RESULTS: Patients treated by right ventricular outflow tract reconstruction had a significant increase of pulmonary artery index (P=.006) within 22 +/- 6 months. Repair was successful in 14 cases (postrepair right ventricular/left ventricular pressure ratio = 0.47 +/- 0.1). One hospital death occurred as a result of pulmonary vascular obstructive disease, despite a reassuring intraoperative flow study. Accuracy of this test in predicting the postrepair mean pulmonary artery pressure was 89% (95% CI: 51%-99%). At follow-up (18 +/- 12 months), all patients are free of symptoms, requiring no medications. CONCLUSIONS: The integrated approach to total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals by preoperative calculation of total neopulmonary arterial index, right ventricular outflow tract reconstruction (when required), and intraoperative flow study may lead to optimal intermediate results.

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A 3-day-old neonate with severe central cyanosis was found to have a right to left shunt across a secundum atrial septal defect, due to prominence of the Eustachian valve. Two-dimensional echocardiography with Doppler and color Doppler allowed a precise morphological and pathophysiological diagnosis. Cardiac catheterization and angiocardiography confirmed the diagnosis. The patient is asymptomatic 12 months after surgical excision of the Eustachian valve and closure of the atrial septal defect, performed at two months of age.

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Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex cardiac anomalies with unrestricted pulmonary blood flow and subaortic obstruction. Between 1986 and 1991 we have treated 23 such patients with a new type of palliation combining a main pulmonary artery-ascending aorta anastomosis with a bidirectional cavopulmonary anastomosis. Their ages averaged 57 +/- 36 months (7 to 155 months). Nine patients had (S,L,L) single ventricule with transposition and the other 14 had more complex cardiac anomalies unsuitable for biventricular repair. Subaortic obstruction was defined by a hemodynamic criterion (n = 6) or by a morphologic criterion (n = 17). There were five hospital deaths (21.7%): All three patients older than 7 years of age died (p = not significant). Follow-up averaged 28 +/- 21 months (range 1 to 58 months). Among the 18 hospital survivors, the proportions surviving 1 and 5 years after the operation were 78% and 63%, respectively. Control cardiac catheterization in 11 patients showed no or trivial subaortic gradient, a mean cavopulmonary pressure of 10 +/- 3 mm Hg, and a mean arterial oxygen saturation of 83%. Nine patients underwent secondary Fontan repair in our institution 21 +/- 4 months after palliation, without deaths. Another patient died elsewhere, 3.7 years after palliation, as a result of hemorrhage at sternal reentry during attempted Fontan operation (overall mortality at repair, 10%). The other eight patients are awaiting Fontan operation. This staged approach reduces both pressure and volume ventricular load and provides adequate oxygenation before the Fontan operation. In our experience, it resulted in reduced mortality at definitive repair.

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We report on two patients with congenitally corrected transposition of the great arteries in situs inversus who underwent successful anatomic repair of associated cardiac anomalies--ventricular septal defect and pulmonary outflow tract obstruction. Surgical intervention was influenced by the recently introduced technique of Ilbawi and colleagues in cases of congenitally corrected transposition in situs solitus. Principles of the correction are as follows: (1) patch redirection of venous flows at the atrial level through an incision in the left-sided right atrium; (2) patch closure of the ventricular septal defect through a right ventriculotomy, baffling the left ventricle to the aorta; and (3) valved conduit interposition between the right ventricle and the pulmonary artery. If the ventricular septal defect is restrictive, it can be safely enlarged by extensive resection of the anterosuperior border, because of the posteroinferior location of the bundle of His in this anomaly. With this technique, the left ventricle and the native mitral and aortic valves are recruited for systemic work, with intrinsic long-term advantages. It is, however, a complex procedure involving substantial use of prosthetic material. More experience is necessary to establish whether this approach is superior to currently available physiologic repairs.

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Between December 1986 and December 1990, a bidirectional cavopulmonary anastomosis was performed in 27 patients younger than 2 years of age, including 12 with heterotaxia syndrome. Age and weight of patients averaged 14.2 +/- 6.6 months and 8.1 +/- 2.2 kg, respectively. Eleven had pulmonary atresia and 16 had pulmonary stenosis. The main pulmonary artery was ligated in seven patients in the latter group (subsequently reopened in one) and left open in nine (subsequently ligated in two). There were four hospital deaths (15%). All patients were discharged with anticoagulant/antithrombotic therapy to be continued for 6 months. There were two late deaths before further operations (8.7%). Two patients, one with acquired massive pulmonary arteriovenous fistulas and one with progressive common atrioventricular valve regurgitation, subsequently underwent definitive repair (biventricular in one), and both died. Heterotaxia syndrome (p = 0.087) and preoperative mean pulmonary artery pressure higher than 15 mm Hg (p = 0.09) were the only risk factors for overall mortality.

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Success with the arterial switch operation for D-transposition of the great arteries and the concept of left ventricular suitability for systemic work stimulated this literature review of the age-dependent mechanisms in normal cardiac growth and pressure-induced left ventricular hypertrophy. Normal postnatal myocardial growth is markedly influenced by hemodynamic factors. It consists of an early hyperplastic phase of both myocytes and capillaries that is followed by a myocyte hypertrophic phase. Similarly, imposition of a pressure overload induces both myocyte hyperplasia/hypertrophy and increased angiogenesis in neonates, but only myocyte hypertrophy at a later age. The functional consequences of ventricular hypertrophy are the result of adaptive and nonadaptive changes resulting from the overload stimulus, for example, induction of protooncogene expression, myosin isoenzyme shifts, degree of coronary perfusion, responsiveness to beta-adrenergic stimulation, and myocyte capacity to re-accumulate or sequester cytosolic calcium. Strikingly, both the capacity and the rapidity of left ventricular hypertrophy decrease with increasing age. This experimental information supports the current use of primary arterial switching for neonates with D-transposition of the great arteries and the use of "rapid" two-stage arterial switching in infants more than 3 to 4 weeks of age; it raises some concern about the practice of late retraining of the left ventricle in cases of failed atrial inversion operation.

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Corrected transposition of the great arteries (C-TGA) in situs inversus [I,D,D] is a rare variant of C-TGA (5-8% of the cases). Few anatomic and surgical data on this anomaly have been published. Between 1974 and 1990, 13 such patients (mean age, 12.7 +/- 8.6 yr) underwent repair of associated cardiac anomalies, including ventricular septal defect (VSD) and pulmonary outflow tract obstruction (POTO) in all 13 patients and atrial septal defect (ASD) in 11. Systemic venous anomalies were present in four. Nine patients also had dextrocardia. Patches to close the VSD were placed on the right side of the ventricular septum (through a right [systemic] ventriculotomy) in two patients and on the left side of the ventricular septum in the other 11 via a left ventriculotomy in one or a morphological right (left-sided) atriotomy in 10. The POTO was relieved directly in five patients and bypassed by a left ventricle-pulmonary artery conduit in the other eight. There was one early death due to cerebral hemorrhage. Two patients required a pacemaker for permanent heart block. Other rhythm disturbances included transient heart block in three patients and late atrial fibrillation in one. All the survivors are asymptomatic as late as 15.5 years after the repair. One patient had conduit revision 10.5 years after the initial operation, two had residual POTO of greater than 40 mm Hg, and none had residual VSD. In conclusion, patients with [I,D,D] C-TGA present with a relatively uniform subset of anatomic lesions, including predominantly ASD, VSD, and POTO.(ABSTRACT TRUNCATED AT 250 WORDS)

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Thirteen patients, 12 of whom younger than 2 years, underwent a Damus-Kaye-Stansel procedure for complete transposition of the great arteries, ventricular septal defect, or double-outlet right ventricle and subpulmonary ventricular septal defect. In 6 patients, associated cardiac anomalies caused systemic flow obstruction. There were six hospital deaths (mortality rate, 42%). In a mean follow-up period of 57 months, 5 of 7 survivors required relief of right ventricular hypertension through conduit replacement or enlargement (4 patients) or conduit valve balloon dilation (1 patient). The aortic valve became regurgitant in 2 patients in whom it had been left in potential connection with the right ventricle. One patient has moderate pulmonary valve regurgitation. The main advantage of the Damus-Kaye-Stansel procedure is that it avoids coronary relocation; also, the spatial relationship of the great arteries and the coronary anatomy do not affect its feasibility. One drawback is the need for a conduit in infancy. Our present indication for Damus-Kaye-Stansel procedure is confined to double-outlet right ventricle with subpulmonary ventricular septal defect; 5 of 6 patients survived repair in this series. Possible indications are for patients with associated subaortic obstruction or unusual coronary arrangements. Fresh or cryopreserved homografts as extracardiac conduits and primary closure of the subaortic area may reduce the need for reoperation after Damus-Kaye-Stansel procedure.

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The use of oral propafenone was assessed in 57 children. Mean age was 4.8 +/- 5.2 years (range 1 day to 17 years). Twenty-three patients were less than 1 year of age (40%) and 10 were less than 1 month of age (18%). Arrhythmias included supraventricular tachycardia in 32 patients (57%), atrial flutter in six (10%), and ventricular tachycardia in 19 (33%). Cardiac diagnoses included: an otherwise normal heart in 30 (53%); unoperated congenital heart defect in 11 (19%); cardiomyopathy in nine (16%); and an operated congenital heart defect in seven (12%). Mean dose of propafenone was 13.1 mg/kg/day (range 8 to 15). Success (assessed by relief of symptoms and serial 24-hour ECGs) was achieved in 16 of 32 patients with supraventricular tachycardia (50%), three of six with atrial flutter (50%), and 8 of 19 with ventricular tachycardia (42%). Drug efficacy in patients with and without organic heart disease was 30% (8 of 27) and 63% (19 of 30), respectively (p less than 0.05). Mean duration of therapy in patients in whom the drug was effective was 22.9 +/- 13.2 months (range 6 to 60). Growth was unimpaired in all patients. A neonate with nonsustained monomorphic ventricular tachycardia had sustained ventricular tachycardia during the second day of propafenone therapy at a dose of 13 mg/kg/day. Sustained ventricular tachycardia disappeared when the drug was discontinued. Another patient had an asymptomatic and transient increase in the glutamic pyruvic transaminase level. No adverse hemodynamic effects were observed. In conclusion, propafenone is an effective antiarrhythmic drug in children with normal hearts; it is less effective in patients with heart disease.(ABSTRACT TRUNCATED AT 250 WORDS)

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The patterns of pulmonary circulation were assessed by angiography in a series of 24 patients with right atrial isomerism (asplenia syndrome) and pulmonary atresia. Mean age at catheterization was 21.5 days (range 1 day-11 months). The pulmonary arteries were confluent in 22 cases (91.7%). Eighteen patients (75%) had ductus-dependent pulmonary circulation in presence of confluent pulmonary arteries, similar to cases of pulmonary atresia combined with complex congenital heart disease. The ductus appeared in continuity with the aortic arch, shaping an acute angle with the descending aorta, as in cases of pulmonary atresia with ventricular septal defect. A bilateral ductus was present in 5 cases (20.8%), 3 with confluent and 2 with nonconfluent pulmonary arteries. One patient presented multiple systemic collaterals to the lungs. All the other patients had normal arborization of the pulmonary arteries. Seven patients presented an obstructed total anomalous pulmonary venous connection: 4 in inferior vena cava and 3 in superior vena cava. These anatomical observations provide useful information for the surgical palliation of these anomalies and, probably, warrant pre-operative angiographic evaluation in all the patients with right atrial isomerism.

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