[Clinicopathological sessions; total persistence of the common arterial trunk].
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From January 1980 to March 1983, 73 distressed newborns and young infants, in whom congenital cardiac defects were suspected, were investigated by two-dimensional echocardiography. The diagnosis in these infants could be re-established later by cardiac catheterization and cineangiocardiography, operation or autopsy. Correct diagnoses were set in 97% of the patients (71 out of 73). Specific echocardiographic criteria for the diagnosis of the individual defects are discussed.
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From 1976 through 1981, 8 corrective operations for truncus arteriosos communis type I have been performed. The patient's ages ranged from 2 months to 4 1/2 years; 2 of the children had previously undergone banding of the pulmonary artery. Intracardiac correction consisted in closure of the ventricular septal defect (VSD) and disconnection of the pulmonary trunk from the aorta with reconstruction of the right ventricular outflow tract using a valved Dacron conduit. One 3 1/2-year-old child died postoperatively because of right heart failure. In this child the pulmonary vascular resistance had risen to 13 U x m2 despite banding of the pulmonary artery in infancy. All other children have survived the operation without major complications and are in good condition. Postoperative follow-up (re-catheterization in 6 out of 7 survivors) showed a faultless function of the conduits in all instances. Persistence of pulmonary hypertension was ascertained in one patient. According to these findings, which are in agreement with the experience of others, it is concluded that primary correction of truncus arteriosus should be undertaken in early infancy prior to development of pulmonary vascular disease.
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OBJECTIVES: The aim of this study was to analyze the relation between anomalies of the heart and aortic arch arteries in near-term rat fetuses exposed to the chemical bis-diamine. BACKGROUND: Bis-diamine is known to induce cardiovascular anomalies. METHODS: Bis-diamine was given orally to normal pregnant rats, and the 65 fetuses were examined under a dissecting microscope after formalin fixation. RESULTS: There were 26 rat fetuses (40%) with a ventricular septal defect in the perimembranous portion, of which 14 (22%) had tetralogy of Fallot, 4 (6%) had truncus arteriosus and 8 (12%) had a relatively small defect with no other major anomalies. In 44 fetuses (68%) the middle latitudinal muscle bundle of the ventricular septum was continuous with the right ventricular free wall. There were, isolated or in association, a double- or right aortic arch in 6 fetuses (9%), aberrant subclavian arteries in 9 (14%), right ductus arteriosus in 12 (18%) and agenetic ductus in 4 (6%). The cross-sectional area of the ductus, as corrected by that of the aortic isthmus, was abnormally small in 47 rats (72%). The rat fetuses with a septal defect or abnormal myocardial architecture, or both, usually had a small ductus; it was very small or absent in those fetuses with tetralogy of Fallot. Of the four fetuses with truncus arteriosus, two had a vestigial vasculature on the truncus root and three had a rudimentary infundibulum. CONCLUSIONS: The cardinal defect may be the anomalous and reduced development of the sixth arch arteries, which by imposing pressure overload on the fetal right ventricle, may have led to either or both the persistence of ventricular septal defect as a vent or the formation of myocardial architecture favorable for the generation of pressure in the right ventricle.
A two-month-old girl with heart failure from truncus arteriosus (Collett & Edwards Type II) underwent a total correction by the Rastelli procedure using 12-mm-diameter Hancock valved conduit. The orifice of pulmonary arteries was closed from inside without detachment of the pulmonary artery from truncus. The distal anastomosis of the conduit was made to left pulmonary artery. Primary sternal closure was difficult and delayed closure was performed using splint with a resin plate. The skin was primarily closed using bilateral advancement myocutaneous flaps. Complete closure of the sternum was made on the 11th postoperative day. A rotation flap of the right abdominal rectal muscle was used to cover the partially necrotic skin over the sternum. The patient had persistent respiratory and cardiac problems, but was discharged 14 months after surgery.
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A palliative procedure in patients with pulmonary atresia with VSD should improve arterial oxygen saturation, allow growth and development, and promotes a uniform dilatation of the pulmonary arteries. Thirty-three severely symptomatic patients, ranging from 5 days to 24 mths of age and from 2,4 to 11 kg of weight underwent a variety of palliative operations, including the ascending aorta-right pulmonary artery shunt (intrapericardial: 15 cases; extrapericardial: 14 cases), the Blalock shunt (4 cases). In two cases, both with absence of the left pulmonary artery, a Waterston shunt was performed using cardiopulmonary by-pass. The operative mortality was 20%. In two patients an additional shunt procedure was deemed necessary due to persistent cyanosis. All the survivors improved in terms of arterial oxygen saturation, growth and development. There were no late deaths (mean follow-up: 23 months).
The roentgenographic presentations of 11 newborn infants with hypoxemia secondary to pulmonary vasospasm and subsequent right-to-left shunting of blood through the foramen ovale and/or ductus arteriosus (persistent fetal circulation) are described (P. F. C. Syndrome). One infant had radiographically normal lungs, while ten had pulmonary parenchymal abnormalities including hyaline membrane disease [4], meconium aspiration syndrome [4], or an ill defined pattern of retained lung fluid [2]. The roentgenographic appearance of the lungs, however, was discordant with the severe hypoxemia observed in most. Heart size was variable but some degree of cardiomegaly was commonly present. Tolazoline, a potent vasodilator, was useful diagnostically and may have resulted in increased survival. An expanded clinical and roentgeonographic concept of the PFC syndrome is suggested.
One of conjoined thoracopagus twins, with separate hearts and a common pericardial sack, presented with respiratory distress because of a persistent arterial duct causing congestive heart failure in the neonatal period. Surgical ligation of the duct was performed prior to subsequent separation, with an excellent outcome.
A case of persistent left superior vena cava associated with rheumatic mitral stenosis in a 27 year old woman is described. Comments are made of the echocardiographic findings of a space free of echos located at a level behind the back wall of the left ventricle, at a level of the mitral valve. Echocardiographic Mode and two-dimensional technics were used in the observation of this image. Other pathologies, in which is possible to obtain similar images are also discussed, as well as the value of using echocardiographic contrast studies in the differential diagnosis.
The value of axial cineangiography in several forms of congenital heart disease serves as an illustrated supplement to Section I. These techniques visualize defects in the entire ventricular and atrial septum. In persistent atrioventricular (A-V) canal, it is possible to visualize all parts of both septa, status of the A-V valves (two valves versus a common A-V valve), and if a common A-V valve, its degree of override. In tetralogy of Fallot, the bifurcation of the pulmonary trunk, entire ventricular septum and coronary arteries are vividly shown. The presence of true and confluent pulmonary arteries versus systemic or bronchial arteries in pseudotruncus is clearcut. In double outlet right ventricle or in transpositions with or without double outlet right ventricle, the mitral valve-semilunar valve relationships, the left ventricular outflow tract, subpulmonary region and a straddling tricuspid valve are well demonstrated. In asymmetric septal hypertrophy, biventricular angiography may be circumvented.
OBJECTIVES: To determine rates of reintervention after repair of common arterial trunk in the neonatal and early infant periods. BACKGROUND: With improving success in the early treatment of common arterial trunk, the need for reinterventional procedures in older children, adolescents and adults will become an increasingly widespread concern in the treatment of these patients. METHODS: We reviewed our experience with 159 infants younger than four months of age who underwent complete primary repair of common arterial trunk at our institution from 1975 to 1998, with a focus on postoperative reinterventions. RESULTS: Of 128 early survivors, 40 underwent early reinterventions for persistent mediastinal bleeding or other reasons. During a median follow-up of 98 months (range, 2 to 235 months), 121 reinterventions were performed in 81 patients. Actuarial freedom from reintervention was 50% at four years, and freedom from a second reintervention was 75% at 11 years. A total of 92 conduit reinterventions were performed in 75 patients, with a single reintervention in 61 patients, 2 reinterventions in 11 patients and 3 reinterventions in 3 patients. Freedom from a first conduit reintervention was 45% at five years. The only independent variable predictive of a longer time to first conduit replacement was use of an allograft conduit at the original repair (p = 0.05), despite the significantly younger age of patients receiving an allograft conduit (p < 0.001). Reintervention on the truncal valve was performed on 22 occasions in 19 patients, including 21 valve replacements in 18 patients and repair in 1, with a freedom from truncal valve reintervention of 83% at 10 years. Surgical (n = 29) or balloon (n = 12) reintervention for pulmonary artery stenosis was performed 41 times in 32 patients. Closure of a residual ventricular septal defect was required in 13 patients, all of whom underwent closure originally with a continuous suture technique. Eight of 16 late deaths were related to reintervention. CONCLUSIONS: The burden of reintervention after repair of common arterial trunk in early infancy is high. Although conduit reintervention is inevitable, efforts should be made at the time of the initial repair to minimize factors leading to reintervention, including prevention of branch pulmonary artery stenosis and residual interventricular communications.
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