[Current view of the indications for surgical treatment of congenital heart diseases].
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Biomedical subjects
Publications and source records attributed to E Atik.
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PURPOSE: To evaluate hemodynamic parameters and clinical outcome of children with severe cardiomyopathy who are candidates for heart transplantation. METHODS: Twenty four children aged from 4 months to 10 years and 8 months (mean 3.7 +/- 2.5 years) from February 1992 to May 1996, were submitted to hemodynamic study and the following parameters were measured: cardiac output, mean pulmonary artery pressure (MPAP) and capillary wedge pressure. The pulmonary vascular resistance index (PVRI) and transpulmonary pressure gradient (TGP) were calculated. RESULTS: Ten (41.6%) children were transplanted (group A), 5 (20.8%) are still waiting heart transplantation (group B) and 9 (37.6%) died (group C). The mean age of the B and C group were significantly different. The PVRI, MPAP and TPG were significant lower in group A than group C (p = 0.01; p = 0.044 and p = 0.023 respectively). CONCLUSION: The profile of the hemodynamic parameters of children with severe dilated cardiomyopathy was compatible with the clinical findings. The age was the only difference comparing groups B and C. PVRI, MPAP and TPG were significant factors between group A and C. The older the patient, the worse was the prognosis.
In order to determine the positivity rate and genotype of Epstein-Barr virus (EBV) in cases with Hodgkin's disease (HD) in Turkey, 40 tissue specimens from HD patients were analysed. Ten non-lymphoid tissue samples from individuals without any evidence for lymphoma were used as controls. The cases with HD included 33 males and 7 females with a mean age of 28 years. Nodular sclerosis was the most prevalent histological subtype (16/40) followed by mixed cellularity (10/40), lymphocyte predominance (9/40), and lymphocyte depletion (5/40). After histopathological evaluation, deparafinisation and lysis of the specimens, one-stage polymerase chain reaction (PCR) and two-stage (nested) PCR assays were performed with the primers common for both EBV genotypes and the primers specific for EBV types 1 and 2, respectively. EBV DNA was detected in 22 of 40 (55%) cases with HD and in 1 of 10 (10%) control specimens. The distribution of EBV DNA positivity according to the histological subtypes was as follows: 10 of 16 (62.5%) for nodular sclerosis, 3 of 5 (60%) for lymphocyte depletion, 5 of 9 (55.6%) for lymphocyte predominance, and 4 of 10 (40%) for mixed cellularity. Although most of the HD patients were males of 15-34 years of age, there were no significant differences between EBV positivities obtained from different sex and age groups. The rates of EBV genotypes were 82% for type 1, 9% for type 2, and 9% for both types, respectively.
OBJECTIVE: Introduce a new surgical technique for biventricular correction of double-outlet right ventricle with noncommitted ventricular septal defect. METHODS: From April 1987 to February 1996, 15 patients with double-outlet right ventricle with noncommitted ventricular septal defect were operated on using a new technique for biventricular repair with multiple bovine pericardial patches to create a tunnel between the left ventricle and the aorta. Ages ranged from two months to 13 years (mean age 4.8 years). Thirteen patients had situs solitus and levocardia, one patient had situs inversus and dextrocardia, and one patient had situs solitus and dextrocardia. Construction of the tunnel began at the right atrium. The ventricular septal defect (VSD) was enlarged anteriorly, if restrictive or small, and the first patch was sutured in the infero-posterior edge of the VSD. The second, third and sometimes the fourth patches were sutured in sequence, through the right ventriculotomy, directing the tunnel to the aortic annulus. RESULTS: Overall mortality was 20%, with two early and one late death. The surviving patients were followed-up for a period ranging from ten months to nine years (mean 33 months), and all were in functional class I (NYHA). Minimal residual ventricular septal defect was observed in one patient, stenosis in two patients and moderate pulmonary insufficiency in one. There was no obstruction of the intraventricular tunnel between the LV and the aorta. CONCLUSION: Based on these data, we conclude that this technical modification for the biventricular repair of the double-outlet right ventricle with noncommitted VSD allows for the construction of a tunnel with adequate internal diameter, respecting the spatial changes between the VSD and aorta. In addition, the intraventricular bovine pericardial tunnel takes up less space, thus reducing the incidence of right ventricle outlet obstruction.
The authors present a case of a two-year-old boy with stridor since birth, and also recurrent episodes of pulmonary infections, due to mechanical compression of distal trachea by a left pulmonary artery sling. They discussed diagnostic approach, surgical management and review of the literature. To our knowledge, it is the first case of sling of the pulmonary artery reported in Brazil and the fifth in a latin language country.
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PURPOSE: To compare clinical course, causes and symptoms beginning enset time in children with complete atrioventricular canal with and without Down's syndrome. METHODS: Records of 80 patients < 2 years of age, were reviewed. There were 55 (69%) with Down's syndrome-group I (GI) and 25 (31%) without-group II (GII). Age at synpton enset intensity, functional class, clinical repercussion and anatomic variations in patients undergoing corrective surgery were evaluated. RESULTS: Mean age at symptoms onset was similar for the two groups (50 +/- 75 days). Class II (NYHA) was more frequent in GI (31 patients-56.5%) and class III-IV (NYHA) in GII (19 patients-76%) p < 0.005. Clinical repercussion evaluation showed that congestive heart failure was present in 34 (62%) patients of GI and 21 (84%) of GII; and, pulmonary hypertension was in 21 (38%) patients of GI and 4 (16%) patients of GII p < 0.04. Mean pulmonary arterial pressure of 50 mmHg or more was present in 68% of children with Down's syndrome and in 35% of GII. Clinical course until surgical correction was down hill in 33 (60%) from GI and 21 (84%) from GII p < 0.03. Seventy seven patients underwent surgical correction. CAVC type A of the Rastelli classification was predominant in both groups, GI 37 (67%)-GII 25 (100%). There or more severe valvar morphologic lesions in group II (38%) than in group I (8%). CONCLUSION: There seems to be a pulmonary vascular hyperreactivity predominance in Down's children and cardiac insufficiency signs in the normal genetic group.
PURPOSE: To evaluate the clinical and surgical experience with consecutive patients with isolated total anomalous pulmonary venous connection admitted to the Heart Institute, São Paulo, Brasil. METHODS: Hospital records of 70 consecutive patients (January 1985 to December 1993) were retrospectively reviewed. There were 28 girls and 42 boys whose ages ranged from two days to four years (mean 20.4 +/- 29 weeks). The following variables were selected for analysis: anatomic type, age at admission, clinical status, the presence of infection, restrictive atrial septal defect, and the presence of pulmonary venous obstruction. Univariate and multivariate analysis were used to identify possible risk factors for death. RESULTS: The abnormal anatomical connection was supracardiac in 63%, cardiac in 17%, infracardiac in 8.5% and mixed in 11.5% of the cases. Eighteen (25.7%) patients were admitted in poor clinical condition. Infection, mainly pulmonar, was present in 31 (44%) patients. Pulmonary venous obstruction was present in 21% of the patients. Atrial septostomy was performed in 38 patients, 13 of them admitted in a poor clinical status. The preoperative mortality was 15.7% and the operative, 17%. Statistical analysis demonstrated that infection, poor clinical status and age were risk factors for death. CONCLUSION: Surgical correction of total anomalous pulmonary venous drainage in infancy can be performed at low risk with good results. However early referral to a cardiac care center, agressive preoperative stabilization and postoperative care are important.
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PURPOSE: Heart transplantation has offered children with complex congenital heart diseases and severe cardiomyopathies a chance for survival. The present article was written to show the three year experience of this procedure at the Instituto do Coração-HCFMUSP. METHODS: The methodology used was based on heart transplant indication criteria, inclusion criteria for donors, postoperative management, immunosuppression and prophylaxis as well as treatment of potential complications. RESULTS: From November 1992 to November 1995, 11 children, aged 12 days old to six years (mean 2.5 years) underwent transplantation. Sixty percent of recipients were male; weight ranged from 3.5 to 17.8 kg (mean 10.3 kg). The mean age of donors was 4.4 years (a range of three weeks to ten years), 80% male, weight ranging from 3.8 to 20 kg (median 14.3 kg). The survival rate was 91% and the remaining 10 children are doing well. The most important complications were systemic hypertension, acute rejection and infection. The number of rejections and infections per patient were 3.5 and 4.7 episodes, respectively. The follow-up was between one month to three years (average 16 months). CONCLUSION: In this experience, heart transplantation has given an additional opportunity for children with complex congenital heart diseases and cardiomyopathies, with a survival rate of 91% in three years.
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.
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A 22 months old infant with agenesis of right pulmonary artery, without other associated congenital heart defects, is reported. Reconstruction of pulmonary arteries by connecting them through a 7mm Goretex tube was performed, with clear clinical improvement in view of disappearance of signs of cardiac insufficiency and of hypoxemia. Contralateral pulmonary hypertension, at systemic level, decreased to 77% in the immediate postoperative period. So, this technique becomes the first option, before considering these cases for cardiopulmonary transplantation.
A case of pulmonary atresia with intact ventricular septum and a hypoplastic right ventricle, with three wall formed portions, that became naturally well developed until 2 years of age, is related. A Blalock-Taussig shunt had been performed early in life. Z value of tricuspid valve of 0.3 and left ventricular in ECG has been changed to a well developed tricuspid annulus of 22 mm diameter and to a right ventricle overload after 2 years of follow-up, being possible surgical correction at that time. This observation, until today unknown, the natural increase of a congenitally hypoplastic cardiac chamber, favors the possibility to postpone the corrective operation to a better stage in this anomaly, when the risk becomes lower.
PURPOSE: To study the surgical and clinical evolution of 32 cases with absent pulmonary valve to propose the ideal period of time for surgical correction. METHODS: Clinical and laboratorial analysis were performed in 32 infants, under 12 months of age, between 1980 an 1993, in an evolutive character. From the clinical viewpoint, hypoxic and/or congestive features were considered in previous and late periods related to surgical repair. Laboratorial studies as ECG (cavities overload), chest X-ray (cardiac size and pulmonary vascular markings) and echocardiogram (associated defects, pressure gradients and anatomical aspects of pulmonary arteries) were also analyzed. Cardiac catheterization was performed in 15 patients. RESULTS: Early cyanosis in 84% of cases and "to and for "murmur in 90% of them facilitate clinical diagnosis in whom tetralogy of Fallot was associated in 30 patients. Refractory respiratory and cardiac insufficiency were responsible for operative indication in 12 patients, half of them, operated on under 12 months of age, died. Survival patients were repaired between two to 11 years old. Four deaths occurred early in life, before any surgical consideration and the 16 remaining patients will electively be considered for an opportune repair. CONCLUSION: Conservative clinical treatment is indicated, waiting for a more rigid bronchial wall can support the pressure of the dilated pulmonary arteries. This way, surgical repair is postponed for at least two years of age.
A four year-old boy with an anomalous origin of the left coronary artery (LCA) directly from the right pulmonary artery was reported. He had been underwent to mitral valve replacement by a St Judes no. 29, due to severe mitral insufficiency, secondary to an extensive myocardial infarction including the anterior and posterior papilary muscles, beside the implantation of the LCA in the aorta. Instead of this favorable outcome, it's emphasized the need for earlier recognition to avoid risk sequelae.