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Dysrhythmias following the Mustard and Senning operation for transposition of the great arteries.

The prevalence and nature of late dysrhythmias following operative repair of transposition of the great arteries were evaluated in 32 children using pre- and postoperative standard electrocardiograms and postoperative Holter monitoring. The Mustard procedure was performed in 17 patients, the Senning procedure in 15 patients. The mean age at operation was 40 +/- 35 months in the Mustard and 9 +/- 6 months in the Senning group. The mean interval between operation and Holter monitoring 7 +/- 2 years in the Mustard and 1.6 +/- 1 years in the Senning group. All patients had sinus rhythm preoperatively. Following operation, the incidence of sinus rhythm increased from 59% in the Mustard and 87% in the Senning group in the first postoperative week to 76% in the Mustard and 100% in the Senning group at the end of follow-up (mean 4.6 +/- 3.5 years). Holter monitoring revealed dysrhythmias or conduction abnormalities in 12 patients (9 Mustard, 3 Senning). A permanent pacemaker had to be inserted in 2 Mustard patients because of complete atrioventricular block and sinus bradycardia with sinus arrest, respectively. Results indicate a prevalence of late postoperative dysrhythmias of 20% in the Senning and of 59% in the Mustard group (p less than 0.05). However, the significantly longer mean follow-up period of the Mustard group precludes a direct comparison between the 2 types of repair.

Arrhythmias, Cardiac

Sinus node shift after the Senning procedure compared with the Mustard procedure for transposition of the great arteries.

To investigate the nature of the dominant intrinsic cardiac pacemaker activity after the Senning procedure, endocardial mapping of the systemic venous atrium was accomplished a mean of 13 months after operation in 10 patients, aged 22 +/- 6 months. Multiple endocardial sites were measured to find the earliest atrial electrical activity timed back from the QRS complex. These data were compared with data from endocardial mapping performed in 6 patients late after the Mustard procedure. In 8 of 10 patients who had undergone the Senning procedure, the earliest activation time, corresponding by definition to the origin of the intrinsic pacemaker, was located in the inferior medial portion of the superior limb of the systemic venous atrium. In the other 2 patients who had undergone the Senning procedure, the earliest activity was in the high superior limb of the baffle at its junction with the superior vena cava. In contrast, the earliest activity in all patients who underwent the Mustard operation was at the junction of the superior vena cava and the superior limb of the systemic venous atrium. In response to programmed extrastimulation, the electrophysiologic behavior of the intrinsic pacemaker in the Senning group was abnormal compared with known normal sinus node (SN) data in only 3 of 10 patients, whereas all patients in the Mustard group had SN dysfunction. Abnormal SN function was noted in both patients in the Senning group, in whom a shift in the position of the earliest endocardial activation point was not seen.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Pacing, Artificial

Operative treatment of TGA: comparison of Senning's and Mustard's operation in patients under 2 years.

Sixty-one patients with simple TGA operated on below 2 years of age using atrial inversion techniques were reported. The results of 27 Senning versus 34 Mustard procedures were compared. Postoperative observation time averaged 32 months after the Mustard operation and 12 months after the Senning operation. Early mortality rate was 3% (one patient) in the Mustard group and 7.4% (two patients) after the Senning operation. Sinus rhythm persisted in 20 out of 27 Senning patients and in 30 out of 34 Mustard patients. No baffle obstruction has been registered. Operation results and patient follow-up so far do not favor one technique. The Senning operation rarely requires patch material. Therefore, growth of the atria may be less disturbed.

Cardiac Catheterization

Bacillus subtilis SenS exerts its activity through a site in the 5' flanking region of the aprE promoter.

The Bacillus subtilis gene senS, when present in high copy number, stimulates the expression of several extracellular protein genes during the onset of stationary phase, e.g. aprE. A novel integration vector, pINT, was constructed for transcription expression studies; it employed a unique method of promoter insert production for fusion with the lacZ reporter gene. Deletions were made of the 5' flanking region of the aprE promoter to localize the site responsible for SenS-mediated enhancement activity. pINT was used translationally fuse aprE promoter deletion fragments with the lacZ reporter gene. A site between -177 and -415 with respect to the aprE start site of transcription was found to be required for the maximal SenS-mediated transcription increase from the aprE promoter. A multicopy vector containing the senS coding region without its native negative regulation was highly unstable in B. subtilis; this was due to the expressed senS insert.

Bacillus subtilis

Arrhythmia and late mortality after Mustard and Senning operation for transposition of the great arteries. An eight-year prospective study.

Arrhythmia and late sudden death are recognized complications of intraatrial repair of transposition of the great arteries. We performed a prospective study over 8 years in 100 consecutive hospital survivors who underwent Mustard (46 patients) or Senning (54 patients) operations between 1978 and 1982. Arrhythmia was analyzed by preoperative, postoperative, and serial follow-up Holter monitoring and standard electrocardiograms and was related to clinical outcome. Before repair, all patients were in sinus rhythm with a low incidence of arrhythmia. After repair, there was a gradual decrease in stable sinus rhythm during follow-up so that at a mean of 7 years after operation only 56% of patients having the Senning operation and 66% having the Mustard, with simple transposition, were in stable sinus rhythm on Holter monitoring. There was no significant difference between the two operations. Eleven patients (five after Senning [two simple, three complex], six after Mustard [five simple, one complex]) died during follow-up, four suddenly (two after Senning, two after Mustard). However, loss of sinus rhythm or the presence of arrhythmia on standard electrocardiograms or Holter recordings did not identify patients at risk for increased morbidity or mortality. Thus, even with current surgical techniques, gradual loss of sinus rhythm occurred after both Mustard and Senning operations. Because late death could not be predicted by electrocardiographic analysis, an alternative approach involving detailed hemodynamic and electrophysiologic measurements may be required to identify high-risk patients.

Arrhythmias, Cardiac

Comparison of ventricular function after Senning and Jatene procedures for complete transposition of the great arteries.

Postoperative right (RV) and left ventricular (LV) volume characteristics in patients with complete transposition of the great arteries were studied to compare ventricular function after Senning and Jatene procedures and to analyze RV dimensional change during systole in patients after the Senning procedures. RV end-diastolic volume (EDV) was 181 +/- 74% of normal (mean +/- standard deviation) and RV ejection fraction (EF) was 0.48 +/- 0.09 in 15 patients who underwent the Senning procedure. In 9 patients who underwent the Jatene procedure, LVEDV was 152 +/- 27% of normal and LVEF was 0.61 +/- 0.09. One patient with aortic regurgitation, 1 with aortic regurgitation and residual ventricular septal defect, and 1 with aortic regurgitation and generalized LV wall hypokinesia of unknown cause had large LVEDVs. Pulmonary ventricular EDV and EF were within normal ranges except in the patients with persistent pulmonary hypertension, who had large EDVs and low EFs regardless of the anatomic type of ventricle, either the left or right. The study of RV dimensional change in the Senning group showed a reduced systolic shortening of the anteroposterior diameter compared with the preoperative transposition of the great arteries and normal. This reduced shortening may be related to postoperative adhesion of the RV free wall to the anterior chest wall and fixation of the atrium secondary to the intraatrial repair. In conclusion, systemic ventricular function after intraatrial repair for complete transposition of the great arteries is depressed by unavoidable residua and sequelae: persistent RV hypertension, anatomy of the right ventricle and, possibly, postoperative adhesions.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Surgical Procedures

Senning's procedure for transposition of the great arteries.

The outlook for children with transposition of the great arteries (TGA) improved dramatically with the advent of the atrial repair. This procedure, first successfully performed by Ake Senning, followed years of unsuccessful attempts at correction by a number of surgeons using a variety of techniques. Senning's procedure expanded on the concept experimentally proposed by Albert of redirecting venous return at the atrial level to achieve physiological correction. The Senning procedure was largely abandoned when Mustard's technique was introduced in 1964, but has enjoyed a resurgence as a number of its potential advantages became more fully appreciated. Today, patients with TGA are increasingly undergoing repair by the arterial switch technique. Not all patients, however, are suitable candidates for this approach, and its success will be measured against the ingenious procedure described by Senning more than a quarter of a century ago.

Child

Modified Senning operation for treatment of transposition of the great arteries.

Since February, 1978, 42 infants ranging in age from 15 days to 16 months (mean age 6 months) and weighing between 2.0 and 9.0 kg (mean weight 5.8 kg) underwent a modified Senning I operation. Eleven (26%) underwent operation during the first 3 months of life. Twenty-nine patients had dextro-transposition of the great arteries (d-TGA) and an intact ventricular septum (Group I), and 13 patients had d-TGA and a large ventricular septal defect (VSD) (Group 2). In addition to the Senning I procedure, 13 patients had transatrial closure of their VSD, eight had ligation of a patient ductus arterioses, two had removal of a pulmonary artery band, and four had trans-pulmonary artery resection of short-segment subpulmonary stenosis. Modification of the original Senning operation included (1) patch augmentation (pericardium or Gore-Tex) of the atrial septal flap and (2) pericardial patch enlargement of the pulmonary venous pathway. One patient in Group 1 (3%) and two patients in Group 2 (15%) died after operation. A 2-week-old infant (Group 1) was treated with prostaglandin E1 (PGE) for 2 weeks before operation. The other hospital deaths (Group 2) occurred in a 2-month-old infant with advanced ischemic damage to the right ventricle and a 4-month-old child with multiple VSDs and Grade IV pulmonary vascular disease. No caval gradients were found after modification of the right atrial incision. Two patients died later from pulmonary venous obstruction, one during attempted recatheterization and the other after repair of the pulmonary venous obstruction. One patient had transient complete heart block, and four were discharged in junctional rhythm. Thirty-four patients (87%) were in regular sinus rhythm when released from the hospital. Postoperative catheterizations in eight patients showed no significant gradients in six and severe pulmonary venous obstruction in two (late deaths). More late postoperative results are required, including postoperative catheterization and electrophysiological studies, before the relative merits of the Senning versus the Mustard operation can be assessed.

Cardiopulmonary Bypass

Results of the Senning procedure in infants with simple and complex transposition of the great arteries.

Because of interest in the arterial switch operation, recent experience with the Senning operation in patients with simple and complex transposition of the great arteries (TGA) was examined. Between 1982 and 1985, 35 patients with simple TGA (group I) and 10 patients with complex TGA (group II) underwent a Senning operation. Mean duration of follow-up was 14 months for group I and 24 months for group II. In group I, 1 patient died early and no patient died late; infrequently, right ventricular dysfunction, tricuspid regurgitation, baffle obstruction or arrhythmias occurred. In group II, no patient died early and 3 died late. In addition, many patients required prolonged digoxin therapy. Because the arterial switch operation has a high early mortality risk and an undetermined long-term morbidity and mortality risk, the Senning operation (or other venous switch operations) is considered the preferred surgical approach for simple TGA. In contrast, an arterial switch operation is performed in patients with complex TGA (without subpulmonic obstruction). Comparison of the early and late morbidity and mortality of the arterial vs the venous switch operation may be helpful in selecting the appropriate surgical approach to complex TGA.

Female

Electrophysiological studies after the Mustard and Senning operations for complete transposition. Do they have prognostic value?

Overall, long-term survival after the Mustard or Senning operation is good, but late arrhythmias remain a concern. Whether postoperative electrophysiological investigations can identify patients at risk of developing serious arrhythmias is unknown. In this study, 16 children who underwent electrophysiological study after the Mustard or Senning operation for complete transposition (the combination of a concordant atrioventricular and a discordant ventriculo-arterial connexion) were followed up for one month to 9 years (mean 4.33 years) after this investigation. At the electrophysiological study, sinus nodal dysfunction was diagnosed in 9/16 patients, and abnormalities of atrial conduction and refractoriness in 7/13. During follow-up, 7 children developed clinical evidence of the sick sinus syndrome. When considering corrected sinus node recovery time as the only electrophysiological parameter, the sensitivity of predicting sick sinus syndrome from the electrophysiological study was 42%, and specificity 66%. The sensitivity increased to 71% if additional electrophysiological criteria of sinus node dysfunction were included, such as sinuatrial conduction time or sinus node entrance block, but specificity dropped to 55%. Atrioventricular conduction disturbances were rare. Thus, abnormalities at electrophysiological studies after the Mustard or Senning operations are frequent, but identification of patients at risk of developing sick sinus syndrome remains difficult.

Arrhythmia, Sinus

Technical advances in the Senning operation.

Since March 1978, 59 patients with transposition of the great arteries (TGA) ranging in age from 2 months to 6 years have undergone the Senning operation. Forty-six patients (77.9%) had transposition with intact ventricular septum, 8 of them with patent ductus arteriosus; 8 patients (13.5%) had TGA with ventricular septal defect (VSD), 3 of them with previously performed banding; 4 patients (6.7%) had TGA with VSD and pulmonary stenosis (PS), and one (1.6%) had TGA with PS. Prior to correction, 38 cases had undergone a Rashkind septotomy and 15 had had a previous Blalock-Hanlon septectomy. During this time we have introduced some technical modifications of the original Senning operation: (A) right atrial incision, (B) large left atrial wall window, (C) infrasinal suture of the left atrial flap, (D) avoidance of damage to the hypothetical anterior atrioventricular conduction bundle, and recently, in 12 patients, we have used (E) an inverted left atrial appendage as an interatrial flap. There were 9 hospital deaths (15.2%) caused by low cardiac output (4 cases), severe hypoxemia and low cardiac output (4 cases) and one patient with pulmonary venous drainage obstruction died following reoperation. Two patients developed neurological complications that disappeared without sequela. Another complication was a right chylothorax (4 patients), which improved without the necessity of surgical repair. Eighteen patients developed a junctional rhythm for a period over 24 hours. We think that the technical modifications which we present here, partially contribute to improving the Senning operation.

Cardiac Output, Low

The Senning procedure for repair of d-transposition of the great arteries.

We report our results with the Senning procedure for repair of d-transposition of the great arteries (d-TGA) including techniques that we feel facilitate the operation. When the atrial septal flap is deficient at the site of an atrial septal defect, the flap is filleted to enlarge it using living tissue. This also has the effect of thinning the septal flap which enlarges the left pulmonary vein pathway located posterior to the floor of the new caval tunnels. Another technique includes the use of the upper end of the sulcus terminalis in the construction of the roof of the new caval tunnels. The upper posterior rim of the right atriotomy is left external to this suture line and can be used in construction of the new pulmonary venous pathway. The latter suture line is inferior to the SA node and can be placed expeditiously and with less chance of damaging the SA node. Use of this technique has resulted in a reduced incidence of late arrhythmias. Following the Senning procedure in 40 patients there were no hospital deaths, but there were two late deaths. The Senning operation is currently a very satisfactory alternative for repair of d-TGA when arterial switch repair cannot be performed.

Anastomosis, Surgical

Pulsed Doppler echocardiographic assessment of the pulmonary venous pathway after the Mustard or Senning procedure for transposition of the great arteries.

Pulsed Doppler evaluation of pulmonary venous flow was performed in three groups of patients who had undergone either a Mustard or Senning procedure. Group I consisted of 43 patients in sinus rhythm who had undergone the Mustard procedure 9 months or more before the Doppler examination and 16 who were evaluated immediately after surgery. Group II consisted of 12 patients in sinus rhythm who had undergone the Senning procedure 9 months or more before the Doppler evaluation. Group III consisted of eight patients with mid baffle obstruction of the pulmonary venous atrium and seven with isolated stenosis of the pulmonary vein of which all but one had previously had associated mid baffle obstruction. In those without obstruction, the pulmonary venous flow pattern mirrored the left atrial pressure trace, with peak forward flow occurring during the x and y descent. Obstruction produced a specific high-velocity turbulent pattern, whether at the mid baffle or pulmonary venous level. This technique provides reliable noninvasive information about pulmonary venous flow after the Mustard or Senning procedure.

Child

Surgical management of late right ventricular failure after Mustard or Senning repair.

BACKGROUND: Information on surgical management and outcome in patients who develop symptomatic right ventricular failure after prior Mustard or Senning operations is limited. METHODS AND RESULTS: From March 1987 to March 1991, 10 patients 3.6-23.5 years old (median, 7.0 years) with transposition of the great arteries and prior Mustard (six patients) or Senning (four patients) repairs (performed at ages 2 months to 5 years; median, 6 months) underwent surgical intervention for symptomatic right ventricular failure. In five of 10 patients, anatomic correction with either an arterial switch operation (three patients) or a pulmonary artery-to-aorta anastomosis and right ventricle-to-pulmonary artery conduit (two patients) was performed. Before anatomic correction in these five patients, four of five patients had a pulmonary artery band to prepare the left ventricle. The interval between preparation and correction ranged from 8 days to 12 months (median, 2 months). One patient died after an arterial switch operation. In the remaining five patients, coexisting left ventricular dysfunction precluded anatomic correction; all five patients survived cardiac transplantation. Survival for the entire group of 10 patients is 90%, and the median postoperative hospital stay was 17 days. During follow-up (12-62 months; median, 27 months), there were no deaths. Neoaortic insufficiency after anatomic correction was common (mild in one patient, moderate in two patients, and severe in one patient who required aortic valve replacement 4 months after surgery). In the transplantation group, one patient developed lymphoma 3 months after transplantation but is currently in remission after reduction of immunosuppression. CONCLUSIONS: In patients who develop late right ventricular failure after Mustard or Senning repair, surgical intervention with either anatomic correction or cardiac transplantation can be done with acceptable morbidity and low mortality. Neoaortic valve insufficiency demands close follow-up after anatomic correction.

Aorta, Thoracic

[Correction of funnel chest using the Senning-Johanson method].

Between 1962 and 1987 52 patients underwent surgical reconstruction of pectus excavatum at the University Hospital of Zurich using the Senning-Johanson method, first published in 1974. The technique of Senning-Johanson consists of a Y-shaped sternotomy and a transsection of the involved ribs from inside of the thoracic cage, thereby avoiding a dissection of the anterior chest wall. The average age of the 36 men and 16 women was 20 years (range 12-33). The internal sterno-vertebral distance, which we consider to be a good parameter for determining the anatomic dimension of a funnel-chest, was extended by the operation from an average of 5.8 cm (range 2.5 to 10) to 10.1 cm (range 6.1 to 14) or 74% of the original value. The physical working capacity in sitting position was improved by 10%. The vital capacity was preoperatively normal and was not changed by the operation. Thirty-seven patients considered the operative result to be good, in twelve it was satisfactory, and in three bad. The funnel-chest repair using the Senning-Johanson method can be recommended as a successful procedure with relatively few complications for the adult patient.

Adolescent

Evaluation of patients undergoing senning repair with two-dimensional contrast echocardiography.

The postoperative anatomy of the Senning interatrial diversion was defined by two-dimensional contrast echocardiography in ten patients. A modified apical four-chamber view proved most valuable, allowing for simultaneous visualization of both the systemic venous atrium and the pulmonary venous atrium. The anatomy was confirmed with two-dimensional contrast echocardiography utilizing catheters placed in the systemic venous atrium and pulmonary venous atrium at the time of surgery. In addition, in several of the patients, we were able to detect atrial or ventricular level shunts which were not clinically apparent. One patient who was judged to have a significant shunt by two-dimensional contrast echocardiography had no anatomic site found at autopsy to explain the shunting. We conclude that two-dimensional contrast echocardiography can define the postoperative anatomy following Senning repair allowing for immediate and future evaluation. Shunting at the atrial and ventricular levels can also be detected, but the method is very sensitive and difficult to quantitate or correlate clinically.

Child, Preschool

Hemodynamic and electrophysiologic results of the Senning procedure for transposition of the great arteries.

Of 24 patients, aged 6 days to 24 months, undergoing the Senning procedure for transposition of the great arteries, 2 patients died perioperatively (8% operative mortality): 1 patient, a neonate, from sepsis and 1 patient, born prematurely and with multiple anomalies, from congestive heart failure. One patient died late postoperatively from noncardiac causes. The 21 survivors are clinically well, and in 20 complete hemodynamic and electrophysiologic data were obtained by cardiac catheterization a mean of 13 months after repair. All patients have normal systemic arterial oxygen saturation. Left ventricular function and pulmonary artery pressures are normal in all. None had pulmonary venous obstruction. Narrowing at the junction of the superior vena cava and systemic venous atrium with mean pressure differences of 5 mm Hg or more was found in 4 of the 20 patients, but was clinically manifest in only 1 patient. No abnormality of atrioventricular conduction was seen in response to programmed electrical stimulation. Sinus node dysfunction was present in 6 patients, with abnormalities of both automaticity and sinoatrial conduction. Among these 6 patients were the 5 who were younger than 5 months at operation. The Senning procedure generally results in excellent hemodynamic and electrophysiologic status in patients who undergo operation after the newborn period. Identification of sinus node dysfunction, seen in patients in whom the procedure is performed in the first few months of life, is of concern and identifies a need for close follow-up of sinus node function in this cohort of patients.

Cardiac Pacing, Artificial

Doppler evaluation of superior caval venous pathways after Mustard and Senning operations.

Doppler waveforms from the superior caval vein were analysed to evaluate the patency of superior caval venous pathways following venous redirection (Mustard and Senning) operations for complete transposition. The group consisted of 26 unselected survivors of Mustard (9 patients) and Senning operations (17 patients). Patients were examined a mean of 5.9 (range 0.1-15.3) years following operation and their age at study ranged from 0.4-25.3 years, mean 7.3 years. Doppler waveforms were correlated with digital subtraction angiograms, which were performed in every patient within 24 hours of the Doppler study. Totally occluded superior caval venous pathways were identified in 3 patients with digital subtraction angiography. The remaining patients had angiographically patent pathways. In the 23 patients with patent pathways, Doppler waveforms demonstrated an initial systolic peak smaller than a second diastolic peak. In the 3 patients with totally occluded pathways, the pattern of the waveform was reversed, with the systolic larger than the diastolic peak. Doppler examination of the superior caval vein is a quick and simple bedside method of accurately determining patency of superior caval venous pathways after atrial redirection procedures for complete transposition.

Adolescent