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Introduction: There is a high incidence of scoliosis in patients who have undergone cardiothoracic surgery for correction of congenital cardiac disease, this risk being 10 times higher than in the general population., Materials and Methods: So as to analyse the surgical and postoperative complications, we designed a retrospective study to include every child who underwent spinal orthopaedic surgery, and who had previously undergone cardiothoracic surgery because of a congenital cardiac malformation. We excluded those patients who had syndromes associated with the development of scoliosis., Results: We identified 18 patients with surgically treated congenital cardiac disease who had undergone surgery for scoliosis over a period of 7 years. This group came from a total number of 87 patients undergoing spinal fusion over the same period. Of those with congenitally malformed hearts, 61% had acyanotic lesions, with ventricular septal defect being the most frequent single lesion, present in 40%. All the patients needed blood transfusions during the surgery, with aprotinin used in 73% to reduce the bleeding, and inotropes needed for 4 children. During the immediate postoperative period, 1 patient died in the first 24 hours, while 7 (39%) had different complications, pneumonia in 4, pleural effusions in 2, and rhabdomyolysis in the other, as opposed to a rate of complications of 27% in patients without heart disease., Conclusion: The surgical and postoperative complications in these patients depend on the specific cardiac lesion. A multidisciplinary team with experience in the treatment of congenitally malformed hearts is essential for appropriate management of these patients.

Objective: To study the efficacy of high doses of aprotinin during cardiopulmonary bypass (CPBP) in pediatric patients with a tendency to intra- and postoperative bleeding: children undergoing repeated operations and those with cyanotic cardiopathy., Patients and Methods: A simple retrospective, random sample of 71 pediatric patients with cyanogenic cardiopathy was studied. Group I (n = 36) received a test dose of 50,000 KIU in 2 min. The initial dose was 1,700,000 KIU (170 ml/m2 of body surface) i.v., at an infusion rate of 150 ml/h. CPBP circuit prime dose was 1,700,000 (170 ml/m2 of body surface), with replacement at a volume equivalent to the circuit prime. The maintenance dose was 400,000 KIU (40 ml/m2/h) i.v. Infusion was suspended 2 hours after a patient's arrival at the intensive care unit (ICU). We recorded red blood cell loss and blood product requirements in the first 24 hours in the ICU, duration of surgery, intra- and postoperative diuresis, and complications., Results: Group I required 13.96 +/- 12.09 ml/kg/h of packed red cells whereas Group II required 22 +/- 16.22 ml/kg/h (p < 0.05). The volume lost was less in Group I than in Group II (0.77 +/- 0.48 ml/kg/h vs. 2.12 +/- 3.9 ml/kg/h; p < 0.05). The means for intra- and postoperative diuresis in the first 24 hours were not significantly different., Conclusions: Aprotinin significantly reduces red blood cell loss and homologous blood transfusion requirements. No complications attributable to aprotinin were observed.

Introduction and Objectives: A divided left atrium because of cor triatriatum is a relatively rare cardiac anomaly requiring corrective surgery. We describe here our clinical and surgical experience with this congenital heart defect as well as the different medium and long term diagnostic, surgical and evolution aspects., Patients and Methods: From 1981 to 1999, 15 children with cor triatriatum without complex associated cardiovascular defects underwent surgery at a mean age of 13 months (excision of the obstructive membrane). The surgical reports were reviewed and the clinical and echocardiographic data were analyzed before and after the intervention; six of these patients (40%) were referred to operating room only with the 2-D echo Doppler technique and color flow mapping information. The follow-up period ranged from 8 months to 19.3 years., Results: Diagnosis was confirmed during the surgical procedure. One 9 month old patient died 60 days after a successful corrective surgery because of sepsis (7%). No late deaths or reoperations were found in the follow-up period. All 14 patients who survived the operation have a functional class I (NYHA), and they are asymptomatic in the follow-up. The overall survival rate was 93% (70% CI: 87-90)., Conclusions: Corrective surgery with excision of the obstructive membrane dividing the left atrium restores normal anatomic, hemodynamic and clinical status in children with cor triatriatum without complex associated defects.

Postoperative thrombosis after the Fontan procedure has been well noted in the literature, and its risk factors are also well known. In contrast, thrombosis after the bilateral cavo-pulmonary shunt (Glenn) has been rarely reported and almost always occurs around the anastomosis itself or near it, mainly causing pulmonary embolism. We present 2 cases with cerebral embolism 2-7 months after pulmonary artery closure and Glenn procedure, due to dislodgement of a thrombus in the proximal pulmonary artery stump. Based on these two cases and a few others reported in the literature, we want to call the attention to this new cause of thromboembolism after Glenn and stimulate discussion about its incidence, risk factors and preventive measures.

In spite of progressive improvement not only in myocardial protection but also in anesthetic and surgical techniques, the repair of complex congenital heart defects can still lead to cardiopulmonary compromise refractory to conventional treatment. We describe two patients in whom successful surgical repair of congenital heart defects was followed by severe heart failure refractory to medical treatment. Both patients were managed by extracorporeal membrane oxygenation (ECMO). We discuss the indications, contraindications and use of ECMO in the postoperative intensive care unit.

We describe a patient with angiographic findings of a left superior persistent vena cava draining in the left atrium with absence of the coronary sinus' roof and coexisting with Holmes' heart. The diagnosis can be made by selective angiography in the left atrium, pulmonary vein or at the left superior vena cava. We want to emphasize the importance of knowing this sort of anomaly before surgical treatment.

Nine patients with complete atrioventricular canal and tetralogy of Fallot underwent intracardiac repair of both anomalies between 1982 and 1989. The ages of the patients ranged from 6 months to 7 years. Six of the 9 had one or more previous systemic-pulmonary artery shunts. Two-dimensional echocardiography showed diagnostic characteristics of both malformations in all patients. The diagnosis was confirmed by cardiac catheterization and cineangiography. The ventricular septal defect was repaired by a combined right atrial and ventricular approach in every patient. Outflow tract reconstruction was performed with the use of a transannular patch (4), infundibular patch (4), and a valved conduit (1). There were no hospital deaths. Meningitis was responsible for the death of a patient 2 years after repair. We recommend early palliation, complete repair in those older than 4 or 5 years, surgical technique depending on the anatomical findings, combined atrial and ventricular approach, 2 separate patches to close the ventricular septal defect in required cases, adjusted correction of the right ventricular outflow tract, and careful postoperative care.

A 19 month-old girl diagnosed of truncus arteriosus with absence of the left pulmonary artery and severe truncal valve insufficiency underwent total correction. A non previously diagnosed severe mitral insufficiency required a new operation to implant a Bjork-Shiley supraanular mitral prosthesis. The postoperative period was hemodynamically normal, except for the presence of persistent fever of unexplained origin. The clinical picture was thought to be an infectious endocarditis due to the late appearance of an aortic prosthetic leak. Because of failure to medical treatment a reoperation was performed to replace aortic prosthesis. Patient died eight hours postoperatively. Autopsy revealed a correct surgical repair, absence of endocarditis and severe miliary disseminated tuberculosis. Authors consider of interest to present this case-report due to the rarity of: a) correction of truncus with a single pulmonary artery under two years of age; b) associated mitral insufficiency; c) the presence of tuberculosis, difficult differential diagnosis with infectious endocarditis in a patient with valvular prosthesis.

We present a case of tetralogy of Fallot associated with total anomalous pulmonary venous return, with successful surgical correction. This association presents problems as regards surgical indications and technique.

Eighteen children sustained unilateral phrenic nerve paralysis (PNP) after cardiac surgical procedures. Ten (Group I), under 7 months (mean: 2.9 +/- 2.2), required long-term ventilatory assistance (mean: 23.9 +/- 13.0 days); they failed to be weaned from the ventilator. All underwent diaphragmatic plication (DP). DP was performed late in 7 cases (Group Ia) with a mean time of 30.8 days between surgery and DP, and early in 3 others (Group Ib) with a mean time of 10.2 days. Eight children (Group II), older than 1 year, tolerated PNP better and could be extubated early without diaphragmatic plication. In Group Ia severe lung infections were recorded in 5 before or/and after DP, and two died at 3 and 30 days after plication. Five children from Group Ia and all 3 from Group Ib were late survivors. They could be weaned from ventilatory support in a mean time of 3 days after DP, although those with severe lung infection (Group Ia) took the longest time. All from Group II were late survivors. We conclude: PNP is well tolerated without plication in children older than 1 year. However early DP offers excellent and immediate results in infants with PNP. Early DP in these children avoids or reduces severe lung infections and death.

A very unusual observation of survival after surgical treatment of neonatal maceration of liver and spleen is reported. The patient had a normal birthweight and the only possible etiologic factor was a oxitocin-induced rapid delivery. The literature on this topic is reviewed.

In this work the clinical features, diagnostic methods, surgical procedures and postoperative follow-up are reviewed in 19 cases of atrioventricular septal defect with associated tetralogy of Fallot. The clinical situation was always related to the degree of pulmonary ischemia, that pointed out the urgency and need for a palliative surgical procedure before corrective surgery. There was no data in the ECG for the differential diagnosis of isolated forms of atrioventricular septal defect. In 50% of the cases the cardio-thoracic index was increased by right atrial enlargement. The diagnosis of the malformation was made in every case by means of 2D echocardiography. In 16 cases palliative surgery was done (aortic-pulmonary shunts), with one death (6.2%) due to facts unrelated to the technique. Of the 6 cases with corrective surgery (5 cases with previous shunts), one needed a mitral valve replacement (St Jude prosthesis). There was no mortality in this group and the functional status in the long-term follow-up was: 3 cases were in the functional class I and 3 cases in class II. The overall mortality for all surgical procedures carried out was 4.3%. In conclusion, we consider the use of the corrective surgery adequate to the clinical stability of the patients with atrioventricular septal defect with associated Fallot's tetralogy.