[Transposition of great arteries. The reality of its cure today].
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Biomedical subjects
Publications and source records attributed to E Atik.
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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.
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.
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.
From June 1987 to October 1991, 12 patients with congenital mitral valve stenosis underwent surgical correction. Their ages ranged from 2 to 74 months. Nine patients were less than 22 months of age. Five patients were in New York Heart Association functional class IV, and seven patients were in class III. In nine, parachute-type mitral stenosis was clearly definite. In three, a complex congenital valvular and subvalvular stenosis was found. Associated anomalies were present in every patient; five had undergone previous operations. Correction of the mitral stenosis was done through an apical left ventriculotomy. The removal of the mitral obstruction starts from below; the papillary muscle was split and the chordae were divided or fenestrated. The commissurotomies were performed from the ventricular aspect of the mitral valve. Associated anomalies were corrected simultaneously. The operative mortality rate was zero. There was one late death, which was unrelated to cardiovascular status. The echocardiographic serial postoperative studies (up to 52 months) showed no significant residual mitral stenosis and normal global and regional function of the left ventricle in all but one patient.
A rare aortic malformation, the persistence of the fifth aortic arch (PFAA) associated with coarctation of the aorta, is reported in a 3-month-old male infant who underwent an emergency surgical intervention at 10 days of life, to relieve a severe aortic coarctation by pericardial patch technique. A successful balloon angioplasty was performed 2 months later eliminating a recoarctation. The gradient fell from 77 mmHg to 0, but a definitive surgical intervention for recoarctation was necessary 14 months after angioplasty.
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.
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We analyzed the findings in 23 patients with atresia of the left atrioventricular valve and a patent aorta seen in the period from January 1980 to July 1989. Having divided the cases according to the anatomical findings, we made a subsequent analysis of the clinical and surgical results with the aim of establishing the management most likely to diminish risks, still high, in treatment of this complex anomaly. From the anatomical viewpoint, three variants were observed. In the first, made up of 15 cases, there was absence of the left atrioventricular connexion. The characteristic finding in the second group, with five cases, was an imperforate left atrioventricular valve in the setting of concordant atrioventricular connexions. The third group, of these cases, was dominated by the presence of isomerism of the atrial appendages, both appendages being of left morphology in one case, and of right morphology in the other two. Further anatomical variation was then found in each group. Nine of the 15 with absence of the left atrioventricular connexion had the right atrium connected to a dominant left ventricle in presence of a rudimentary and incomplete right ventricle associated with discordant ventriculo-arterial connexions, all of them being in usual atrial arrangement and three with pulmonary stenosis. The remaining six in this first variant had the right atrium connected to a dominant right ventricle. In the five patients with imperforate left atrioventricular valves, two had discordant and three had concordant ventriculo-arterial connexions. In the three cases with isomerism, two had absence of the left atrioventricular connexion, with a dominant right ventricle. The last patient had an imperforate left atrioventricular valve and a discordant ventriculo-arterial connexion. From the functional viewpoint, there were 14 patients (10 with absence of an atrioventricular connexion, four with imperforate atrioventricular valve) with congestive heart failure and nine patients (five from the first, one from second, and three from the third variant) with hypoxia. Long-term follow-up (median 16.4 months--varying from 1 to 41 months--in the group with congestion and 27.7 months--varying from 12 to 57 months--in those with hypoxia) showed favorable clinical evolution in 11 (91%). We conclude that an anatomico-functional division can point towards the most appropriate management in this complex anomaly.
Three infants with anomalous origin of left coronary artery, being two from the right pulmonary artery and one from the left, without other congenital defects, ages of 5, 15 and 3 months old, presented with early congestive heart failure due to anterolateral myocardial infarction. Anatomical characteristic was peculiar in two cases since the left coronary artery, before the anastomosis with the right pulmonary artery, presented a wy, 15 mm extension path within the aortic wall which has facilitated the surgical correction by the proximity of these structures. In the third case, the left coronary artery was anastomosed with the left pulmonary artery, 2 cm from its origin and anterior to the left atrium being the surgical correction by direct implant at the ascending aorta. Post-operative follow-up, 36, 30 and 33 months respectively, showed clinical and laboratorial normalization except for the second case in which ST segment remained upward due to ventricular aneurysm. Myocardial recovery, even after extensive infarction, in this anomaly, emphasizes the need for earlier diagnosis and management.
Common arterial trunk is a rare congenital anomaly, usually found in the setting of a concordant atrioventricular connexion. The association of a common arterial trunk with absence of the right atrioventricular connexion is very rare, having, as far as we know, been reported only twice in the literature. In the case reported here the pulmonary arteries originated separately from the posterior wall of the arterial trunk, and the trunk overrode the septum between the dominant left- and the right-sided rudimentary right ventricle. We discuss the electrocardiographic features which can help in the diagnosis of this malformation.
PURPOSE: To study the hypoplastic left heart syndrome (HLHS) emphasizing clinical aspects and therapeutic measures. PATIENTS AND METHODS: Forty patients, 24 male, ages ranging between one to 120 days (mean six days). Diagnosis was established by clinical elements, eletrocardiogram, chest X-ray and Doppler echocardiogram. Eight patients underwent cardiac catheterization. In 21 patients anatomical diagnosis was confirmed by necropsy. RESULTS: Twenty-two patients (55%) died, in spite of all possible clinical measures. In 16 (40%) palliative surgery was performed, Norwood technique in 14 and pulmonary artery banding with Dacron graft interposition between pulmonary trunk and brachiocephalic trunk in two. Immediate postoperative results were favorable in three patients (Norwood technique). Both patients underwent the other described technique died at 12th and 30th postoperative day due to infection. CONCLUSION: HLHS is a severe cardiac anomaly with a fast downhill evolution mostly at first week of life. The good results depend basically of early diagnosis as well as of an adequate clinical management for an appropriate time in indication of the palliative surgical technique.
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Two patients with transposition of the great arteries submitted to Jatene surgery at ages of 6 days and 6 months respectively, presented in the postoperative period stenosis of the aorto-pulmonary anastomosis. The diagnosis was made three and six years after the surgery. In both patients successful percutaneous transluminal angioplasty (PTA) was performed, respectively six months and immediately after the diagnosis. Pressure gradients decreased from 83 mmHg to 24 mmHg in the first case, and from 76 mmHg to 13 mmHg in the other case, with clear improvement of the angiographic image. Complications of the procedure did not occur in any case. This application for PTA was not previously reported and should be considered as an alternative to the surgical correction of the "new" pulmonary trunk stenosis in the Jatene surgery.
A new corrective operation for truncus arteriosus without the use of an extracardiac conduit was performed in seven patients with truncus type I (six patients) or type II (one patient) aged from 2 to 9 months. The common truncus arteriosus was septated with a patch into aortic and pulmonary segments and the ventricular septal defect was closed through a ventriculotomy. A direct anastomosis between the pulmonary arteries and the right ventricle was performed, the anterior wall being constructed with a patch with a monocusp valve. There was one death in the immediate postoperative period. In the surviving six patients the postoperative right ventricular/left ventricular peak systolic pressure ratio was less than 0.51 in five and 0.60 in one with a residual ventricular septal defect. All are in functional class I between 1 and 14 months after the operation. On the basis of these results, we propose this technique for patients with truncus type I or II in the first year of life.
Transluminal balloon angioplasty has been successfully used in the treatment of various congenital heart diseases, specially in pulmonary valve stenosis. Dilation angioplasty has also been used in venous obstructions in children, but the results have been contradictory. This report describes the case of a three-month-old female infant with obstructed total anomalous pulmonary venous return into the right superior vena cava. Percutaneous angioplasty was performed with two 4 mm diameter balloon catheters (Simpson Ultra Low Profile), with success. Right ventricular systolic pressure decreased from 68 to 49 mmHg and mean pulmonary vein pressure decreased from 41 to 17 mmHg. There was significant clinical improvement. Although it would be an unique experience in our Department, authors suggest that transluminal balloon angioplasty could be an initial therapeutic alternative in infants with obstructive total anomalous pulmonary venous return, a high-risk group for total surgical repair.
Prostaglandin E1, used since 1975, has changed favorably the clinical and surgical prognosis of neonates with congenital heart defects, mainly those with a variety of ductus-dependent defects. Due to recent modifications that have been observed with this drug as far as dosage, side effects, duration of venous infusion and the response of the different cardiac anomalies, this study was undertaken on 47 neonates evaluated with drug infusion between December 1985 and April 1988. The ages of the patients varied from 12 hours to 70 days (median age of 10.3 days), body weight ranged from 1990 to 4430 g (median of 3005 g). The average dose corresponded to 0.021 mcg/kg/min, varying between 0.013 to 0.0089 mcg/kg/min. The therapy was considered effective in 36 (76.5%) patients, evaluated by clinical improvement, increase of arterial oxygen saturation greater than 15 vol. O2% and increased ductus diameter measured by echocardiographic study. In the correlation between the therapeutic result and the patient age, the greatest elevation of arterial oxygen saturation occurred until 21 days of age, especially up to 7 days of age where the elevation in this period was of 24.5 vol. O2%. The cardiac defects that best responded to PGE1 were pulmonary atresia with or without ventricular septal defect, Ebstein's anomaly, tricuspid atresia, pulmonic stenosis, double outlet right ventricle, and those that the arterial oxygen saturation increase was less than 10 vol. O2% such as the hypoplastic left heart syndrome, tetralogy of Fallot and transposition of the great arteries. (ABSTRACT TRUNCATED AT 250 WORDS)
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