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Immunosuppressive therapy for transplanted patients exposes them to a high risk of developing posttransplantation lymphoproliferative disorders (PTLD). We report the case of a child undergoing heart transplantation at seven months of age who developed PTLD at nine years of age, diagnosed by resection of a pulmonary nodule.

We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed.

A possible relationship between C.pneumoniae (CP) infection, atherosclerosis and acute myocardial infarction is a debated matter. Now we performed the search of CP in histological segments of fatal ruptured plaques and of stable plaques by histochemistry (Macchiavello stain), immunohistochemistry and in situ hybridization techniques. Electron microscopy and confocal laser microscopy techniques were used in two additional cases. The semi-quantification of CP + cells (0-4+) and quantification of lymphocytes demonstrated greater amount of CP + cells and more inflammation in the adventitia of vulnerable plaque vessel segments than of stable ones, larger amount of CP + cells in adventitia than in the plaque and high frequency of CP + cells in all groups studied. This preliminary study strongly suggests a direct pathogenetic involvement of adventitial CP in the rupture of the atheromatous plaque, development of acute myocardial infarction and also in the development of atherosclerosis.

Absent right atrioventricular connection, with the left atrium connected to a dominant left-sided morphologically right ventricle is a rare situation. We report five cases with these anatomical features, with emphasis on the morphological and clinical aspects. Although this combination of lesions is very uncommon, the sequential segmental analysis can easily provide an accurate description of the anomaly.

Purpose: To determine the value of the quantitative analysis of lung biopsies from patients with congenital cardiac defects., Methods: Fourty nine biopsies were examined, from patients: 43 patients increased pulmonary blood flow, 3 with pulmonary atresia and large systemic-to-pulmonary collateral vessels, and 3 with decreased pulmonary flow. The degree of lesion was determined as in Heath-Edwards and of Rabinovitch and col., Results: The Heath and Edwards grade was determined in 41 cases; I in nine; II in 23; III in eight; IV in one; 3 biopsies showed evidence of reduced pulmonary flow and 5 had no signs of vascular disease. The Down patients (7) presented a greater proportion of severe lesions. Quantitative evaluation was obtained in 35 biopsies: 11 had grade B and 24 had grade C. Wall atrophy and dilatation of intraacinar arteries were detected in 7 cases, what suggested the existence of obstructive lesions in proximal vessels, even if not sampled. Medical thickness greater than 2 times the normal were observed in pre-acinar arteries from 14 biopsies., Conclusion: The morphometric approach allowed us to detect severe lesions which the qualitative analysis alone would not indicate adequately. In the patients presenting decreased pulmonary flow, morphometry made possible to assess if the degree of arterial wall hypertrophy was compatible with a surgery of atrio-pulmonary anastomosis.

The association of tetralogy of Fallot with atrial isomerism has been rarely reported. Eight cases (five with left isomerism and three with right isomerism) are known. This paper reports two other cases of tetralogy of Fallot with left atrial isomerism. The syndrome's defects were disguised and without clinical expression because of the presence of the right ventricular outlet obstruction of tetralogy of Fallot. These diagnostic elements, not recognized in one of the patients previous to surgical correction of tetralogy of Fallot, were present: junctional rhythm, bronchial isomerism, partial anomalous pulmonary vein connection, agenesy of inferior vena cava and abdominal heterotaxy; their identification previous to surgical correction of tetralogy of Fallot, is necessary for an adequate surgical management.

Two patients with huge aortopulmonary window, both ten months-old, that showed curiously up at a clinical viewpoint, including echocardiography, as a mitral valve regurgitation with a dilated mitral ring secondary to the accentuated blood volume through it, were reported. Early signs of heart failure, accentuated systolic murmur at mitral area and overload of left heart cavities, characterized clinical impression of a congenital mitral insufficiency, in both cases. The correct diagnosis was established at operation and at necropsy study, bringing it to us the need to a more accurate analysis of the different diagnostic elements, especially in anomalies which clinical manifestations show great similarities.

Purpose: The aim of this study was to analyse the effectiveness of patent ductus arteriosus (PDA) closure by the video-assisted thoracic surgery (VATS)., Methods: The technique was utilized in 6 newborn lambs firstly. Three to four small incisions (3 to 10mm) were used in each animal to permit the introduction of lung retractors, video equipment, dissectors and clip appliers. The procedure was accompanied by video monitoring and after the dissection, the PDA was closed by 2 titanium clips. Seven days after, the animals were sacrificed and submitted to pathological study. Based on this initial experience seven patients (ages between 17 and 108 months) were operated on with this technique., Results: In lambs, we have some difficulty to retract the lung. Despite this fact the closure of PDA was successful and proved by pathologic study. In children the dissection of PDA was easier due to manual ventilation. The interruption of PDA using the proposed method was obtained in 4 patients, those with good relation between ductus diameter and clip size and defined by echocardiography and angiographic studies., Conclusion: The use of VATS for interruption of PDA in both, experimental and initial clinical experiences, has showed to be an effective method.

Purpose: To analyze some morphological aspects of the tetralogy of Fallot which have been raising controversies in the literature, due to the different approach to the nomenclature of congenital heart defects by different authors regarding the definition of double outlet right ventricle and its concomitance with tetralogy., Methods: We reviewed the original description of the anomaly, and also analyzed the morphology of 22 anatomical specimens, describing the degree of aortic overriding, the type of ventricular septal defect, the degree of sub-pulmonary stenosis, etc., Results: Regarding the degree of aortic overriding, there was only one case where that vessel connected predominantly to the left ventricle. In the remaining hearts, the degree of overriding was between 50% and 75% in 10 cases and greater than 75% in 11. The intensity of infundibular stenosis was evaluated as mild in half the available hearts, and the pulmonary valve was bicuspid in 11 cases. Right aortic arch was present in 40% of the available hearts and the ventricular septal defect had muscular borders in just one case (5%)., Conclusion: We could conclude that if double outlet is only a type of ventricle-arterial connexion, and so, depending on the degree of overriding of the aorta over the trabecular septum, we may have the malformation coexisting with the tetralogy.

Two cases of transposition of the great arteries (TGA) with posterior aorta are reported. The first was submitted to the Senning procedure with good outcome; the other had the diagnosis of double outlet right ventricle with severe pulmonary stenosis, and a Blalock-Taussig shunt was accomplished for hypoxic crisis. The patient died 11 days after surgery and the necropsy demonstrated TGA with posterior aorta. In both cases there was a sub-pulmonary infundibulum. The presence of ventricular septal defects allowed aortic-mitral fibrous continuity, a finding previously described in anatomical reports. Clinical and angiographic aspects are discussed.

Purpose: To study the pathogenesis, evolution and prognosis of the complete heart block of the fetus., Methods: Bidimensional echocardiography associated to M-mode and doppler was performed in 600 patients. All cases of congenital heart block were referred because the fetuses presented hydrops, bradycardia and/or cardiac malformation suspected by routine ultrasound., Results: Isolated heart block was found in 6 fetuses (5 cases of complete type and 1 case of 2nd degree type 2:1). Heart block associated with complex cardiac disease and left atrial isomerism was found in 6 fetuses with no survivors (5 cases of complete type and 1 case of 2nd degree type 2:1). Heart block associated with atrioventricular discordante was found in 1 case., Conclusion: The findings of this study agree the literature about the relation between maternal anti-RO antibodies and isolated complete heart block. We also found a poor prognosis in the group with heart block and complex cardiac malformations.

Three newborn children with cyanosis due to Ebstein's anomaly of tricuspid valve with an important hemodynamic repercussion presented features of imperforation of pulmonary valve. On the follow-up, clinical and laboratory findings verified that pulmonary atresia was functional resulting from a combination of low right ventricular pressure and high pulmonary vascular resistance.

Purpose: The authors intended to verify the most common anatomical presentations of hearts with double inlet ventricle (DIV) and the associated lesions, in order to provide information regarding surgical palliation/correction., Material and Methods: Twenty hearts presenting DIV, from pathological collections. The sequential segmental analysis was applied., Results: Fourteen cases showed usual atrial arrangement, and six isomeric atria (4 with right isomerism and 2 with left isomerism). "Situs solitus" showed two atrioventricular (AV) valves, and the others a single valve. The main ventricle was morphologically left in 13 cases, right in one and of indeterminate morphology in six. The ventriculoarterial connexion was discordant in 10 cases, concordant in two, of double outlet type in eight (6 from an indeterminate ventricle, one from the main chamber and one from the rudimentary ventricle). Pulmonary obstructive lesions were common in the group with atrial isomerism (67%). AV valve stenosis was present in 4 cases, two of the left valve and two of the right one. Straddling of one AV valve was observed in 5 cases and aortic coarctation in three. The ventricular septal defect was restrictive in two cases, both with double inlet to a dominant left ventricle and a concordant AV connexion (Holmes'heart)., Conclusion: In those with atrial isomerism, the obstructive pulmonary lesions are the dominant feature and require early attention. For the other group, the atriopulmonary anastomosis procedures should take into account peculiarities such as AV valve dysfunctions and aortic coarctation.

Purpose: To analyse the histological evolution of endomyocardial biopsies from children with active myocarditis, submitted or not to immunosuppressive therapy., Patients and Methods: Four groups of patients were compared, clinically treated as follows: group I--anticongestive drugs (4 patients); group II--prednisone (5 patients); group III--prednisone plus azathioprine (9 patients); group IV--prednisone and cyclosporine (5 patients)., Results: No patient from group I presented any histological improvement during a mean period of 9 months, while evident histological improvement occurred in 25% of patients from group I, 67% from group III and 80% from group IV. The microscopical aspect of resolving myocarditis was only observed in patients from groups III and IV, after treatment., Conclusion: The immunosuppressive therapy with azathioprine or cyclosporine plus prednisone leads to decrease of active myocarditis intensity in a higher proportion of cases than the treatment with only prednisone or no immunosuppressive drugs.

Purpose: To detect in prenatal life fetal arrhythmia and congenital heart disease. We performed fetal echocardiography following classical indications according to the literature., Patients and Methods: Two-dimensional echocardiography associated with M-mode and Doppler was performed in 200 patients according to the indications: maternal diabetes, fetal arrhythmia, maternal congenital heart disease, previous fetus with cardiac defect, intrauterine growth retardation, nonimmune fetal hydrops, rubeola, isoimmunization, fetal malformation by ultrasound, polyhydramnios, gemelarity, oligohydramnios, maternal ingestion of drugs, lupus and others., Results: Structural abnormalities of the heart were found in 6 cases and associated with bradiarrhythmia in 5 cases (total of 5.5%). Fetal arrhythmias without cardiac malformation were found in 35 cases (17.5%) and premature atrial contractions were the most frequent., Conclusion: The findings in this study indicate that it is possible the accurate definition of the cardiac anatomy and function. We have emphasized the method trying to spread it, objecting to improve assistance to the fetus and newborn with arrhythmias and/or congenital heart disease.

The first case of a hyperacute rejection of a human cardiac allograft in Brazil is reported. The histopathological aspects of hyperacute cardiac rejection in its earlier moments are described when degenerative and necrotic features of the cardiac fibers are not totally developed. The authors believe that neutrophilic exudation and lesion of the vessel wall are good signs for a correct diagnosis of hyperacute rejection. Furthermore, they observed that the process is diffuse enough to justify the indication of endomyocardial biopsy when there are reasons to suspect this diagnosis. Presence of IgM and complement (C3) are also useful.