[Balloon dilatation treatment of congenital heart defects in children].
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Biomedical subjects
Publications and source records attributed to J Rohmer.
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Several reports have shown that high-quality magnetic resonance (MR) images can be obtained in patients with congenital heart disease. However, little attention has been paid to the segmental analysis of cardiac MR images. The authors believe that this approach is essential in the evaluation of complex congenital cardiac abnormalities. The segmental approach is based on the morphologic identification of the different cardiac compartments and the type of connection among these compartments. The authors applied the basic principles of the segmental approach to the interpretation of MR images obtained in a group of patients with levotransposition of the aorta. Results indicate that this logical step-by-step method can be used to advantage in the accurate analysis of MR images obtained in patients with complex congenital cardiac disease. In this report, the authors discuss the results obtained when the segmental approach is used to interpret cardiac MR images.
Sixty-six patients (23 neonates with transposition of the great arteries and intact ventricular septum, 33 infants and children with transposition and a large ventricular septal defect, and 10 with double-outlet right ventricle with a subpulmonary ventricular septal defect have received an arterial switch repair since 1977. Eight (one with transposition and intact ventricular septum, six with transposition and ventricular septal defect, and one with double-outlet right ventricle with subpulmonary ventricular septal defect) died in the hospital. All other patients have had follow-up as of June, 1985. Including the hospital deaths, the 11 month actuarial survival rate for the entire group was 81%, and no deaths have occurred among the 33 patients alive at that time and traced as long as 8 years. The hazard function for death has only a single early phase, and its 70% confidence limits overlap the hazard function of a matched general population by 12 months after the operation. Incremental risk factors for death included low birth weight (but not weight or age at operation), transposition of the great arteries with large ventricular septal defect, double-outlet right ventricle with subpulmonary ventricular septal defect, and presence of a patent ductus arteriosus. Coronary artery morphology and position of the great arteries were not risk factors. Long aortic cross-clamp time was possibly (p = 0.11) a risk factor. Early date of operation was a risk factor (p = 0.004); thus, predicted 1 year survival rate, including hospital deaths, after the arterial switch operation in 1985 is 99.9% (70% confidence limits 99.0%-100%) for neonates with transposition and intact ventricular septum and 99.7% (98.4%-99.9%) for those with transposition and a large ventricular septal defect or double-outlet right ventricle. The late functional status was excellent, and the rhythm was sinus in 96% of the 55 surviving patients. A formal comparison with the results of the atrial switch repair indicates that the arterial switch repair is superior.
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Percutaneous pulmonary valvuloplasty was performed in 27 patients with congenital pulmonary valve stenosis. A fall in the transvalve gradient of at least 15 mm Hg occurred in 22 patients. In five there was little change in the severity of the stenosis; in three of these the pulmonary valve was dysplastic. None of the successfully treated patients had a dysplastic valve. The two other failures, early in the series, were probably due to inadequate balloon size. In one patient the procedure was performed twice, with a successful result from the second dilatation with a larger balloon. Follow up studies in a further six patients showed no evidence of restenosis in those who had been successfully treated and no late improvement in the remainder. There were no important complications. Percutaneous pulmonary valvuloplasty should be the initial treatment for congenital pulmonary valve stenosis, although when the valve is dysplastic the result is less likely to be satisfactory.
21 patients are described with either the pulmonary or the systemic circulation dependent on the patency of the ductus arteriosus. Treatment with Prostaglandin type E (PGE) was instituted to improve tissue oxygenation. Special PGE 2. It is concluded that PGE can be life-saving in critically ill newborns but that complications, especially episodes of apnoea, are frequent. Little is known about the possible sequelae of long-term oral PGE 2-treatment.
An anatomic study of 28 heart specimens with tricuspid atresia showed 15 hearts with ventriculoarterial concordance and a right-sided anterior outlet chamber. Of these 15 hearts, 12 showed evidence of restricted pulmonary blood flow. In eight of these 12 specimens, an anterior deviation of the outlet septum, relative to the primary septum, was present. This deviation caused a narrowing inside the outlet chamber and functioned as infundibular pulmonary stenosis. The outlet chambers of the 15 hearts with ventriculoarterial concordance were compared with those of 13 hearts with tricuspid atresia and ventriculoarterial discordance and also a right-sided anterior outlet chamber. In the latter group of 13 hearts, five showed evidence of restricted pulmonary blood flow, at least partially caused by posterior deviation of the outlet septum relative to the primary septum. A clear difference in outlet chamber morphology was found without exception between specimens with and without ventriculoarterial concordance. Clinical data in three long-term survivors of the Fontan procedure demonstrate that the special morphology of the outlet chamber can pose surgical problems. Especially in patients with ventriculoarterial concordance, when the outlet chamber is incorporated into the pulmonary circuit, the surgeon must give special attention to the specific morphology to prevent undesirable sequelae.
An anatomical study of 34 specimens presenting with tricuspid atresia, in which particular emphasis was devoted to the atrioventricular junctional area, revealed 5 cases with an imperforate valve. In 1 heart the valve was completely committed to the right ventricle. In the other 4, however, the membrane was related not only to the right ventricle but also to the left ventricle. A hypoplastic right ventricular inlet portion was found in these 4 specimens. In 2 cases the tricuspid membrane overrode an inconspicuous inlet septal defect. In the other 2 hearts, as studied macroscopically, no defect was present underneath the membrane, which was connected to both right and left ventricles. These 4 specimens also had other ventricular septal defects. In classical tricuspid atresia, characterized by absence of the inlet portion of the right ventricle, the inlet septum is absent. In contrast, these 4 hearts did have an hypoplastic inlet septum.
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Venous switch operations for simple transposition of the great arteries are being carried out in increasingly younger patients. To guarantee sufficient growth of the reconstructed atria into adulthood it is advantageous to use as little prosthetic material as possible. We therefore compared the results in 36 patients who underwent the Mustard operation in which a large amount of foreign material is used with the results in 36 patients who underwent the Senning operation in which a small amount, if any, foreign material is used. The Senning operation resulted in a lower hospital mortality and a lower incidence of dysrhythmias.
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Fontan's correction has been performed without early death in 24 consecutive patients with tricuspid atresia. Eighteen patients had ventriculoarterial concordance (group I) and six discordance (group II). Late death related to infections occurred in three patients. The follow-up time in group I (16 survivors) ranges from 1 year 10 months to 7 years 5 months (mean 3 years 8 months) and in group II (five survivors) from 2 years 1 month to 5 years 6 months (mean 3 years 6 months). Early postoperative cardiac catheterisation showed arterial pulsatile pressure recordings in the pulmonary artery only in two patients in group I, in whom a valved conduit was used to connect the right atrial appendage with the outlet chamber. Patients with a valved conduit connected with either the pulmonary artery (group II) or the outlet chamber (two patients of group I) showed better preservation of right atrial contractions angiocardiographically and scored better in exercise tests than did patients with a non-valved conduit (14 patients of group I). In the latter patients evidence of regurgitant bloodflow from the outlet chamber into the right atrium was found. Reoperation was necessary in two patients. The outlet chambers showed a substantial increase in diameter after operation. No disturbances of kidney or liver function were found up to seven years after operation. The clinical condition has improved considerably in all survivors. It is concluded that the use of a valved conduit is preferable for connecting the pulmonary artery directly in cases of tricuspid atresia with ventriculoarterial discordance or the outlet chamber when there is ventriculoarterial concordance.
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The dominance of Mustard's operation for transposition of the great arteries has been challenged by the recent revival of Senning's repair because it promises better long-term results in terms of venous obstruction and atrial haemodynamics. These hypotheses were tested by recording jugular venous flow waveforms transcutaneously in 24 postoperative patients with simple complete transposition using a bidirectional Doppler blood velocimeter. Eight patients had undergone Mustard's operation and 16 the Senning alternative; all had previously had a postoperative cardiac catheterisation. Both groups of patients had similar left ventricular, pulmonary arterial, and systemic venous atrial pressures. No child showed any evidence at catheterisation of either mitral regurgitation or of superior vena caval pathway obstruction. These two findings were endorsed by the transcutaneous Doppler recordings. Jugular venous flow in normal children exhibits two maxima, one of atrial filling during ventricular systole, the other of ventricular filling occurs once the tricuspid valve has opened. Both operative procedures diminished the size of the former phase, but the Mustard did so more. After Mustard's operation forward flow during the atrial filling phase was absent in approximately half the cardiac cycles recorded, and severely diminished in the rest. By contrast, there was approximately a 90 per cent appearance of atrial filling waves after Senning's operation which also provided significantly better atrial function than Mustard's procedure in terms of peak velocity of blood entering the atrium and total atrial filling. It is therefore concluded that both procedures compromise atrial volume and compliance but Senning's repair to a much lesser extent.
Takedown and reconstruction of a previous Glenn anastomosis at the time of repair was performed in 1 child with pulmonary atresia and ventricular septal defect and in 3 children with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. All 4 survived and showed excellent clinical results. The surgical technique and postoperative hemodynamic and lung scintigraphic data are presented.
This preliminary report presents recent experience with the Senning operation for transposition of the great arteries. Reasons are given why the traditional Mustard procedure was abandoned in favour of the Senning operation. Technical details are presented and the advantages and disadvantages of the procedure are discussed.
In animal experiments regurgitant flow through competent and incompetent pulmonary valves was evaluated with electromagnetic flow velocity catheters by using the ratio of the separately integrated backward and forward flow velocity signals. This ratio was obtained with a special purpose analog computing circuit set by an in-vitro zero flow reference signal, which was in good agreement with in-vivo zero flow signals.