Biomedical subjects
Publications and source records attributed to M Ebaid.
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.
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.
Infections due to coagulase-negative Staphylococcus (CNS) are an ever-increasing nosocomial problem, particularly in the pediatric population. The authors describe a cluster of three primary bloodstream infections due to CNS in a newborn intensive care unit that occurred between November 23 and December 2, 1992. Two children died as a direct consequence of the bacteremia; at autopsy, one had a large bacteria-containing thrombus extending from the insertion site of a central catheter to the superior vena cava. The children were placed in isolation, and the nursing and medical staff were given topical nasal mupirocin. Plasmid analysis performed later disclosed three different blood isolates that also were different from any of the staff's nasal isolates. The authors concluded that molecular methods such as plasmid analysis are important tools in identifying true outbreaks and can prevent needless interventions, such as those during this cluster.
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.
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.
The authors investigated the existence of circulating cellular aggregates in 12 patients with moderate to severe pulmonary hypertension, using scanning electron microscopy. Peripheral venous blood was collected in the presence of 11.5 mM buffered ethylenediaminetetraacetic acid, in order to disperse freshly formed disaggregable aggregates. Irreversible aggregates represented by platelet clusters and/or platelet attachment to either leukocytes or red cells were identified in 7 patients with pulmonary hypertension. Endogenous platelet activation was further confirmed by a significant increase in plasma levels of beta-thromboglobulin in comparison with controls (33.8 +/- 14.1 vs 22.7 +/- 11.5 ng/mL respectively, p < 0.025). The presence of irreversible aggregates in the blood stream strongly suggests that cell-cell interactions actually occur in vivo in these patients. If so, therapeutic measures aimed at preventing in situ thrombosis and its consequences may be beneficial in this disorder.
Abnormalities in endothelial von Willebrand factor (vWF) structure have been reported in pulmonary hypertension. These include loss of high molecular weight plasma multimers, resulting in decreased biologic activity. If endothelial processing of vWF is altered in this disorder, abnormalities in oligomeric composition may also be expected. We examined this possibility in ten adult patients with primary pulmonary hypertension. Enhanced endothelial vWF expression in these patients was indicated by increased plasma levels of vWF antigen (vWF:Ag) (214 +/- 91 vs 99 +/- 51 percent activity in controls, p < 0.001) and intense immunoperoxidase stain of pulmonary arterial endothelium for vWF (autopsy, 1 patient). Plasma from these patients also had a decreased capacity of inducing platelet aggregation in the presence of ristocetin, relative to vWF:Ag levels (57 +/- 20 percent activity). In addition to mild loss of the largest multimers, changes in oligomeric composition of plasma vWF were observed in most patients using both agarose and polyacrylamide gel electrophoresis. These included decreased concentration of dimeric (470 kDa) vWF in most patients, variable concentration of the 860-kDa fraction, and a relative decrease in subunit (223 kDa) density in subjects with elevated vWF:Ag. These findings provide additional information on the mechanisms responsible for endothelial production of dysfunctional vWF in patients with pulmonary hypertension.
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Ischemic cardiomyopathy in childhood is related with congenital and acquired pathologies which could lead to serious cardiac sequelae as myocardial infarction and sudden death. Unfortunately, when coronary artery malformations are excluded, it is difficult in some cases to be completely certain on the etiology of the arterial coronary disease, due the lack of pathognomonic diagnostic tests. Case report of 6 year old child, with severe coronary artery disease: aneurysm of main branch of left coronary artery; left anterior descending branch and right coronary artery occlusions with accentuated myocardial dysfunction. We believe that the diagnosis of Kawasaki disease is presumptive in this patient. The principal aspects to elucidate the etiology of coronary artery disease are analysed, as well as the importance of identifying patients without known previous illness, but with severe coronary artery lesions, as in this reported case.
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Interruption of the aortic arch is an uncommon cardiovascular anomaly. It is usually associated with a large ductus arteriosus (DA) and interventricular septal defects. Two cases of one and two month old girls with restrictive DA are reported. The clinical, anatomical and surgical findings are discussed.