Long-term results of atrial correction for transposition of the great arteries. Comparison of Mustard and Senning operations.

Few data exists on the differences in long-term outcome between Mustard and Senning operations. We reviewed available data of all hospital survivors of these operations and assessed risk factors for late death and sinus node dysfunction. Of those patients undergoing the Mustard operation, 60 were hospital survivors (46 simple transposition, 14 complex); of those patients undergoing the Senning operation, 62 were hospital survivors (43 simple, 19 complex). Median duration of follow-up was 16 years (maximum 25 years) for Mustard operation, 11 years (maximum 20 years) for Senning operation. No reoperations were done, except for pacemaker implantation. No differences were found between the two groups with regard to baffle-associated problems, right ventricular failure, sudden death (6% in both groups), and functional status at final follow-up (New York Heart Association class I or II, except for four patients). For patients undergoing the Mustard operation, survival at 16-year follow-up was 91% with simple transposition and 60% with complex transposition (p = 0.027); for both groups of patients undergoing the Senning operation, survival at 16-year follow-up was 78%. Survival in the absence of rhythm disturbance at 16-year follow-up was 18% for Mustard operation and 53% for Senning operation (p < 0.001). In multivariate analysis, significant independent risk factors for late death turned out to be complex transposition (versus simple) and active arrhythmias. The only significant risk factor for the occurrence of sinus node dysfunction was the Mustard operation. We conclude that apart from the difference in the loss of sinus rhythm, no differences were found in the long-term clinical results of the two types of operations.

The arterial switch operation. An eight-year experience.

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.

Developmental considerations of mitral valve anomalies.

Atrioventricular valve development is described as following the general principle of junctional invagination and myocardial undermining. We have added considerations of the topographic relations of the developing mitral valve to these principles. Two groups of malformations are then distinguished: the first comprises disturbances of the general principle, the second is related to topographic abnormalities. It is shown that the atrioventricular septal defect, the straddling mitral valve and the isolated mitral cleft all have their own specific developmental backgrounds.

Tricuspid atresia. Morphology of the outlet chamber with special emphasis on surgical implications.

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.

Growth of an aorta-coronary anastomosis. An experimental study in pigs.

The influence of growth of an aorta-coronary anastomosis, comparable to the coronary translocation anastomosis during the arterial switch operation, was studied in pigs. The anastomosis between the right coronary artery and the aorta did not grow, and this lack of growth may result in stenosis. With another technique, by which the coronary artery was excised with a cuff of aortic wall, the effects caused by absence of growth were circumvented and a normal-sized coronary ostium was present after growth. However, when no cuff was used, stenosis occurred at the suture line and caused growth retardation of the animal as well as histologic damage to the right ventricle.

Nine years' experience of physiological correction of tricuspid atresia: long-term results and current surgical approach.

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.

Horizontal submammary skin incision for median sternotomy.

Usual techniques in open-heart operations leave a vertical median skin scar. Especially for women this is rather embarassing since it looks ugly. The use of horizontal submammary skin incision for access to the heart is described here. Although obtaining the usual midsternal exposure takes some extra time, long-term results have shown the value of this approach and its benefit to the patient. The surgical technique is simple, and wound healing, in the long run, is unimpaired.

Differential atrial filling after Mustard and Senning repairs. Detection by transcutaneous Doppler ultrasound.

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.

Anatomical correction of transposition of great arteries with persistent ductus arteriosus. One year after operation.

The postoperative data are described of a boy who had a 'switch' operation for transposition of the great arteries with persistent ductus arteriosus more than a year before. The child is living a normal active life. Electrocardiography, echocardiography, and angiocardiography show persistent abnormality of structure and function of the right ventricle but no evidence of reduced coronary blood flow, coarctation at the suture lines of the great arteries, or aortic regurgitation. Reference is made to an identical case operated upon 2 1/2 years ago.

Posttraumatic bronchial stenosis.

Bronchial rupture and its sequelae are discussed with reference to data from the literature. Special attention focuses on the pathophysiology and treatment of post-traumatic bronchial stenosis, and in particular on restoration of the ventilation even at operations performed long after the original injury. Two cases are described: one in which the stenosis was abolished a year after the injury, after an unsuccessful primary operation performed elsewhere, and one in which the stenosis was successfully abolished five years after the original injury.

Ventricular septal defect associated with left ventricular outlfow tract obstruction below the defect.

Muscular subaortic stenosis associated with ventricular septal defect (VSD) is a rare but important anomaly. Two types of left ventricular outflow tract (LVOT) obstruction should be distinguished on the basis of morphologic as well as hemodynamic differences, depending on whether the stenosis is localized above (Type I) or below (Type II) the defect. The five cases presented are all in the latter category. In the first two cases the correct diagnosis was not made until after repair of the VSD. Two patients had previously undergone banding of the pulmonary artery (PAB). Marked carotid shudder and a vertical QRS axis in the frontal plane were presented in all cases. For the diagnosis to be established by catheterization, the catheter tip must be maneuvered into the apical part of the left ventricular cavity in order to detect the stenosis on the withdrawal curve. It is difficult to visualize the stenosis by angiography. The surgical approach through the VSD via a right atriotomy is highly recommended for Type II subaortic stenosis.

The trousers-shaped baffle for use in the Mustard operation.

The trousers-shaped baffle has been found very useful in intraatrial correction of transposition of the great arteries. Reasons are given to explain the particular form of the patch, and details of the operative technique are described.

Revival of the Senning operation in the treatment of transposition of the great arteries. Preliminary report on recent experience.

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.

Prosthesis endocarditis: treatment of a case occurring five years after a Rastelli-Ross operation.

A severe staphylococcal septicemia originating from an unknown focus occurred in a 17-year-old patient who had undergone a Rastelli-Ross operation 5 years earlier. The clinical course was complicated by extensive bilateral pneumonia, diffuse intravascular coagulation, and glomerulonephritis. After 4 weeks of intensive conservative treatment, including a daily regimen of 16 Gm. of cloxacillin, the patient was operated upon for a rapidly progressive false aneurysm, which had resulted from dehiscence of the anastomosis between the prosthesis and ventricle. The excised prosthesis proved to be sterile. The postoperative course was uneventful. Cloxacillin treatment was continued for 6 months, initially parenterally and later orally. After discontinuation of therapy, no signs of infection have occurred. Right-sided intracardiac or intravascular prosthetic material may be particularly susceptible to infections originating from the body surface.