The Mustard procedure in transposition of the great arteries associated with juxtaposition of the atrial appendages with and without dextrocardia

Left-sided juxtaposition of the right atrial appendage (LJRAA) was seen in 10 patients in a series of 361 consecutive Mustard procedures for transposition of the great arteries (TGA). Dextrocardia complicated LJRAA in four cases. Right atrial capacity and free atrial wall size were smaller than normal in all patients, and a Mustard intra-atrial baffle repair was performed in all instances. Direct caval cannulation or cannulation of either atrial appendage facilitated intra-atrial repair. Nine patients in whom the pulmonary venous atrium was enlarged with a patch survived. One child who did not have a right atrial patch died 1 hour postoperatively of pulmonary edema. Two late deaths occurred 1 year postoperatively. Seven children survived and are well. In one child, superior vena caval baffle obstruction is controlled by digoxin and diuretic therapy. TGA associated with juxtaposition of the atrial appendages (JAA) and dextrocardia may be successfully repaired by the Mustard procedure, provided that the tricuspid valve and right ventricle are normal, an adequate superior vena caval channel is created, and patch enlargement of the pulmonary venous atrium is undertaken.     

Continuous murmur following Mustard operation for transposition of the great arteries. A sign of pulmonary venous obstruction

Three patients developed severe pulmonary venous obstruction following Mustard operation for transposition of the great arteries. Each patient had a soft continuous murmur with distinct diastolic accentuation at the low left sternal border or xiphoid area. Simultaneous recording of intracardiac sound and pressure in one patient showed that the murmur originated at the site of obstruction in the surgically-constructed pulmonary venous atrium. Selective cineangiograms demonstrated the baffle to be the cause of the obstruction. A continuous murmur in a patient following Mustard operation may suggest significant pulmonary venous obstruction.     

Simple d-transposition of the great arteries. Results of early balloon septotomy followed by two-stage surgical correction

The follow-up of 44 patients with simple d-transposition of the great arteries is presented. All had balloon atrial septotomy shortly after birth. If an operation was needed at or before one year of age, atrial septectomy was carried out. Correction was done between the ages of 19 and 64 months. Of the 44 patients, 34 (77 per cent) are living and doing well (27 after the Mustard procedure), 3 (7 per cent) are lost to follow-up, and 7 (16 per cent) are dead. Death was due to technical problems during initial catheterization and balloon septotomy in 2 patients, occurred after septectomy in one (3 per cent), and after the Mustard procedure in one (4 per cent). There was one sudden inexplicable death in a 1 1/2-month-old patient, and 2 patients died with pulmonary vascular obstructive disease. Because of the relatively low over-all mortality and morbidity and the low surgical mortality rate for both palliation and correction, we believe that two-stage surgical correction of transposition of the great arteries is preferable to early one-stage correction in the infant.     

Late postoperative hemodynamic results and cineangiocardiographic findings after Mustard atrial baffle repair for transposition of the great arteries

Forty-nine patients with transposition of the great arteries who underwent a Mustard atrial baffle repair between 1964 and 1971 were assessed late postoperatively. There have been five late deaths: two related to baffle obstruction, two from noncardiac causes, and one sudden and unexpected. Hemodynamic data were available in 42 patients and autopsy in four. Obstruction of the lower venous channel was not encountered. Three patients had severe obstruction of the upper venous channel and in four there was mild restriction. Two patients had severe pulmonary venous obstruction resulting in late death; mild asymptomatic obstruction could not be excluded with certainty in six patients. Tricuspid incompetence was infrequently encountered in patients with an essentially intact ventricular septum. Left ventricular outflow tract obstruction was found in eight patients. In three it was present after satisfactory but incomplete surgical relief and in five it had not been recognized prior to operation. In only one of the latter patients was the obstruction important. Seventeen patients were operated on prior to one year of age. While baffle obstruction was confined almost entirely to these patients, the five youngest patients, aged one to nine weeks at operation, had adequate pulmonary and caval compartments at restudy two to three years later.     

Progresses in the study of general surgery in China, 1997

Late systemic venous baffle obstruction after Mustard repair for complete transposition of the great arteries is a recognized complication. Balloon-expandable intravascular stents have previously been used to relieve systemic baffle narrowing in children. We report a successful stent implantation to relieve symptomatic superior vena cava obstruction and baffle dehiscence after Mustard repair in an adult patient. She had been turned down for surgery due to right ventricular dysfunction. Eighteen months after the procedure, she remains symptomless.     

Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage

BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS: Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described. RESULTS: There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome. CONCLUSIONS: Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.     

Anomalous pulmonary venous return in the staged palliation of functional univentricular heart defects

BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic, pulmonary, or systemic and pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS AND RESULTS: Between March 1990 and March 1997, 32 patients with functional single ventricle and anomalous pulmonary venous return underwent operation at our institution. Five of 25 patients who underwent neonatal palliation died in the early postoperative period, all of whom had obstructed anomalous pulmonary venous return. Twenty-one patients have undergone bidirectional cavopulmonary shunt, including 7 in whom this was the primary palliative procedure. There was one early and two late deaths after the bidirectional Glenn procedure, two in patients with asplenia syndrome and none in patients with previously obstructed pulmonary venous return. Seven patients have undergone Fontan completion, 5 with an extracardiac conduit. There was one early death and one take-down to a classic Glenn shunt, both in patients who did not undergo the extracardiac conduit Fontan operation. CONCLUSIONS: Anomalous pulmonary venous return can significantly complicate the management of the single-ventricle patient, with the major impact on survival coming in the neonatal period. Palliation with the aim of performing an extracardiac conduit Fontan procedure allows greater latitude and more streamlined management in this group of patients.     

Laparoscopically assisted abdominal aortic aneurysm repair: first 20 cases

PURPOSE: Laparoscopic surgery decreases postoperative pain, shortens hospital stay, and returns patients to full functional status more quickly than open surgery for a variety of surgical procedures. This study was undertaken to evaluate laparoscopic techniques for application to abdominal aortic aneurysm (AAA) repair. METHODS: Twenty patients who had AAAs that required a tube graft underwent laparoscopically assisted AAA repair. The procedure consisted of transperitoneal laparoscopic dissection of the aneurysm neck and iliac vessels. A standard endoaneurysmorrhaphy was then performed through a minilaparotomy using the port sites for the aortic and iliac clamps. Data included operative times, duration of nasogastric suction, intensive care unit days, and postoperative hospital days. Pulmonary artery catheters and transesophageal echocardiography were used in seven patients. For these patients data included heart rate, pulmonary artery systolic and diastolic pressures, mean arterial pressure, central venous pressure, pulmonary capillary wedge pressure, cardiac index, and end diastolic area. Data were obtained before induction, during and after insufflation, during aortic cross-clamp, and at the end of the procedure. RESULTS: Laparoscopically assisted AAA repair was completed in 18 of 20 patients. Laparoscopic and total operative times were 1.44 +/- 0.44 and 4.1 +/- 0.92 hours, respectively. Duration of nasogastric suction was 1.3 +/- 0.7 days. Intensive care unit stay was 2.2 +/- 0.9 days. The mean length of hospital stay was 5.8 days excluding three patients who underwent other procedures. There were two minor complications, one major complication (colectomy after colon ischemia), and no deaths. For the eight patients who had intraoperative transesophageal echocardiographic monitoring, no changes were noted in heart rate, pulmonary artery systolic pressure, pulmonary capillary wedge pressure, and cardiac index. Pulmonary artery diastolic pressure and central venous pressure were greatest during insufflation without changes in end-diastolic area. Volume status, as reflected by end-diastolic area and pulmonary capillary wedge pressure, did not change. CONCLUSIONS: Laparoscopically assisted AAA repair is technically challenging but feasible. Potential advantages may be early removal of nasogastric suction, shorter intensive care unit and hospital stays, and prompt return to full functional status. The hemodynamic data obtained from the pulmonary artery catheter and transesophageal echocardiogram during pneumoperitoneum suggest that transesophageal echocardiography may be sufficient for evaluation of volume status along with the added benefit of detection of regional wall motion abnormalities and aortic insufficiency. Further refinement in technique and instrumentation will make total laparoscopic AAA repair a reality.     

The pharmacokinetics of pancuronium bromide in infants, children and adults

Laparoscopic retroperitoneal lymph node dissection is a new surgical procedure used to enhance staging in men with clinical stage I nonseminomatous germ cell tumors of the testis. The procedure has been performed in a limited number of patients at several centers with extensive laparoscopic experience. Laparoscopic retroperitoneal lymphadenectomy is a technically demanding procedure which can be successfully completed in the majority of patients. However, the risk of complications is greater than in patients who undergo standard open retroperitoneal lymph node dissection. The primary advantage of a laparoscopic approach is shortened hospitalization and rapid return to normal activity. The role of laparoscopy in the management of patients with testis malignancy has not been defined. The use of this staging procedure may help minimize the need for surveillance studies following surgery and may be best utilized in men with a lower likelihood of nodal metastases. Ultimately, prospective study in large groups of patients will be necessary to determine the role of laparoscopic retroperitoneal lymph node dissection in patients with testis cancer.     

A non-hypervariable human minisatellite strongly stimulates in vitro intramolecular homologous recombination

BACKGROUND: Late morbidity and mortality after the Fontan operation are largely due to atrial arrhythmias, ventricular failure, and thrombus formation. The extracardiac Fontan procedure avoids extensive atrial manipulation and suture lines, theoretically minimizing the impetus for these events. We examined our experience with the extracardiac Fontan operation with particular attention to thromboembolism and arrhythmias. METHODS AND RESULTS: We retrospectively reviewed the medical and surgical records of all 16 patients who underwent an extracardiac Fontan operation between July 1993 and May 1996. Fifteen patients (94%) were in sinus rhythm before the operation. In the immediate postoperative period, seven (44%) had arrhythmias consisting of accelerated junctional rhythm and ectopic atrial rhythm. No associated hemodynamic compromise and no early deaths occurred. Patients were followed up for 3 to 34 months after the Fontan operation. Arrhythmias were detected in eight patients (50%) on surface electrocardiograms, and seven (44%) showed evidence of sinus node dysfunction on 24-hour Holter monitor studies. Thrombi were found in three patients (19%). All patients were asymptomatic, with no evidence of conduit obstruction by echocardiogram. CONCLUSIONS: The incidence of hemodynamically significant tachyarrhythmias appears to be reduced after the extracardiac Fontan operation. A significant percentage of patients have evidence of sinus node dysfunction, suggesting the presence of other surgical or nonsurgical factors responsible for this finding. Our incidence of thrombotic events is similar to previous reports with other Fontan modifications. It appears to be a reasonable option to maintain these patients on anticoagulation indefinitely.     

Pacemaker syndrome without a pacemaker

Circulatory consequences of cardiac arrhythmias are not always evident. Proper interpretation of the clinical symptoms in certain cases requires assessment of the patients' other hemodynamic characteristics. The authors present the case of a patient with left ventricular hypertrophy, who developed severe circulatory failure at the time of artrioventricular dyssynchrony in association with junctional rhythm. Analogy between the circulatory consequences of the junctional rhythm and ventricular pacing was documented by hemodynamic measurements. The patient was subsequently treated by implanting an atrioventricular pacemaker.     

Venous valve repair: early results in fifty-two cases

PURPOSE: Chronic deep venous insufficiency, usually secondary to the postthrombotic syndrome, is due to primary valve failure in approximately 15% of cases. In these cases surgical repair of the valvular mechanism may be indicated. METHODS: Fifty-two limbs in 42 patients were treated with superficial femoral vein valvuloplasty after appropriate investigation. Adjunctive operations on the superficial or perforating veins were performed on 49 limbs during the same hospital admission. RESULTS: Of the 27 patients who have completed one year of follow-up, 85% are free of reflux on duplex scanning and 68% have had normalization of venous refilling times. Of 11 limbs with venous ulceration followed up for more than 1 year, only one has had a recurrent ulcer (9%). In patients without ulceration the procedure has been successful in alleviating symptoms of venous insufficiency. CONCLUSIONS: We conclude that the procedure, in conjunction with appropriate superficial venous operation, is effective in selected patients with deep vein reflux whose conditions remain uncontrolled by conservative measures. At 1 year, 85% of valvuloplasties in this series remained competent.     

Cardiology. acute severe mitral regurgitation

Acute mitral regurgitation occurs as a complication of myocardial infarction in perhaps 1% of cases. The characteristic clinical findings include a new systolic murmur occurring in the setting of sudden development of acute congestive heart failure, hypotension, and shock. S3 and S4 gallops and sinus rhythm usually are present, and gross left ventricular or left atrial enlargement usually is not evident radiologically. The immediate and long-term prognoses for patients with acute severe mitral regurgitation are poor without treatment. Although medical therapy can reverse temporarily some of the hemodynamic aberrations, it does not influence survival or eliminate the need for surgical treatment. Surgery consisting of mitral valve replacement and possibly simultaneous coronary revascularization appears to offer some hope in increasing the survival rate among these patients. When acute severe mitral regurgitation is secondary to rupture of chordae tendineae in circumstances other than coronary artery disease, the outlook is not as grim. These patients may show signs and symptoms of congestive heart failure for a few weeks or, at times, many months. Again, sinus rhythm usually is present, with slight left ventircular enlargement, a relatively normal left atrial size, and markedly elevated left atrial and pulmonary arterial pressures. This constellation of findings is indicative of a large mitral regurgitant flow with preservation of left ventricular function and suggests that the patient is likely to benefit greatly from surgical treatment. The prognosis for such patients is much better than it is for the patients with acute mitral regurgitation secondary to coronary artery disease.     

Three unusual complications resulting from attempted repair of partial anomalous pulmonary venous drainage

The correction of shunts resulting from partial anomalous pulmonary venous drainage has become an accepted surgical procedure. Surgical complications, other than those that were purely postoperative, have been rare. The present report details the case histories of three patients with unusual complications resulting from this type of surgery. Unilateral pulmonary venous obstruction and repeated infections occurred in one patient. In another, obstruction of the superior vena cava resulted. In the third patient, an indaequate operation was performed when the site of partial anomalous pulmonary venous drainage into the coronary sinus was not recognized initially at the time of surgery.     

Surgical stratification of patients with atrial fibrillation secondary to organic cardiac lesions

BACKGROUND: While the maze procedure does not always eliminate atrial fibrillation (AF) secondary to organic cardiac lesions, concomitant performance of the procedure is associated with increased surgical complexity and potential risks. METHODS: To stratify the surgical approach for patients with AF secondary to underlying cardiac lesions, we analyzed 24 preoperative and perioperative variables in 115 consecutive patients with AF undergoing a modified maze procedure combined with valvular intervention (101), repair of congenital anomalies (13) and coronary revascularization (1). RESULTS: Patients who remained in AF (18) compared to patients with restored atrial rhythm (97), had a higher incidence of giant left atrium (56% vs 10%, P < 0.0001), larger cardiothoracic ratio (70 +/- 13 vs 62 +/- 8%, P = 0.001) and left atrial dimension (64 +/- 12 vs 55 +/- 12 mm, P = 0.004), a longer history of AF (13.7 +/- 6.8 vs 8.3 +/- 6.9 years, P = 0.003) and lower f-wave voltage (0.10 vs 0.15 mV, P = 0.004). Multivariate logistic regression analysis of 24 preoperative and perioperative variables identified the presence of giant left atrium, cardiothoracic ratio and age at operation as the significant risk factors predisposing patients to persistent postoperative AF. Retrospective estimation identified 73 (63.5%) patients with a high probability of atrial defibrillation (97.3%) and 42 (36.5%) patients with a high risk of failure (38.1%). Regardless of the preoperative risk analysis or the performance of left atrial plication, every patient with a postoperative left atrial dimension less than 40 mm or cardiothoracic ratio below 55% was successfully defibrillated. CONCLUSION: The results suggest performing the maze procedure before "risk factors" develop for patients with predicted maze-amenable AF. While omitting the maze procedure may be prudent for patients with suspected maze-refractory AF, the simultaneous reduction of left atrial size may offset the increased risk from preoperative size factors. A prospective study seems warranted to examine the effects of left atrial plication on postoperative rhythm.     

The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children

AIMS: To assess outcomes of anatomical repair (double switch procedure) in infants and children with congenitally corrected transposition of the great arteries. METHODS AND RESULTS: Between September 1993 and August 1996, 17 patients with congenitally corrected transposition underwent surgery at UCSF. Anatomical repair was performed in 11 of these patients, at ages ranging from 4.8 months to 7.8 years (median 3.2 years). The remaining six patients did not undergo anatomical repair due to unfavourable anatomy (n = 2), prior conduit repair (n = 2), biventricular dysfunction (n = 1), and isolated complete atrioventricular block (n = 1). The 11 patients who underwent anatomical repair make up the study group for the present report. All 11 patients had a malalignment ventricular septal defect, while pulmonary outflow tract obstruction was present in nine patients and significant tricuspid valve pathology or dysfunction was present in five. Anatomical repair was achieved with a Senning (n = 7) or a Mustard (n = 4) procedure combined with an arterial switch operation plus ventricular septal defect closure (n = 4), or a Rastelli procedure with left ventricle to aortic baffle and right ventricle to pulmonary artery conduit (n = 7). There was one early death and no patients developed surgical complete atrioventricular block. At a median follow-up of 22 months, there were no late deaths. Two patients required a total of three late reoperations, and all patients were asymptomatic on no cardiac medication. Follow-up echocardiography revealed normal biventricular function in all patients. CONCLUSIONS: Anatomical repair of corrected transposition can be achieved with low rates of early mortality and surgical heart block, and favourable mid-term results. Long-term follow-up will be necessary to determine if the double switch approach improves the natural history of corrected transposition when compared to less aggressive surgical approaches that leave the right ventricle in the systemic circulation.     

Multifactorial index of cardiac risk in noncardiac surgical procedures

To determine which preoperative factors might affect the development of cardiac complications after major noncardiac operations, we prospectively studied 1001 patients over 40 years of age. By multivariate discriminant analysis, we identified nine independent significant correlates of life-threatening and fatal cardiac complications: preoperative third heart sound or jugular venous distention; myocardial infarction in the preceding six months; more than five premature ventricular contractions per minute documented at any time before operation; rhythm other than sinus or presence of premature atrial contractions on preoperative electrocardiogram; age over 70 years; intraperitoneal, intrathoracic or aortic operation; emergency operation; important valvular aortic stenosis; and poor general medical condition. Patients could be separated into four classes of significantly different risk. Ten of the 19 postoperative cardiac fatalities occurred in the 18 patients at highest risk. If validated by prospective application, the multifactorial index may allow preoperative estimation of cardiac risk independent of direct surgical risk.     

Laparoscopic cholecystectomy during pregnancy is safe for both mother and fetus

The use of laparoscopic cholecystectomy in pregnant women has been slow to gain wide acceptance for two reasons: one is the potential for mechanical problems related to the pregnant uterus and the other is fear of fetal injury resulting from instrumentation or the pneumoperitoneum. To assess the effects of laparoscopic cholecystectomy on both the mother and the unborn fetus, we reviewed our surgical experience over a 5-year period analyzing indications for the procedure along with complications and outcome. During this 5-year period, 22 patients ranging in age from 17 to 31 years underwent laparoscopic cholecystectomy during pregnancy. Gestational ages ranged from 5 to 31 weeks with two patients being in the first trimester, 16 in the second, and four in the third. The primary indications for surgical intervention were persistent nausea, vomiting, pain, and inability to eat in 17 patients, acute cholecystitis in three, and choledocholithiasis in two. In all patients a pneumoperitoneum was established by means of a closed technique starting in the right upper quadrant of the abdomen. Two of the 22 patients also underwent successful transcystic common bile duct exploration with removal of common duct stones. All 22 patients survived the surgical procedure without complications, and there were no fetal deaths or premature births related to the procedure. Based on the preceding results, it would appear that laparoscopic cholecystectomy during pregnancy is safe for both the mother and the unborn fetus. Indications for this procedure should include stringent criteria such as unrelenting biliary tract symptoms or the complications of cholelithiasis. If at all possible, when laparoscopic cholecystectomy is indicated, it should be performed either in the second trimester or early in the third.     

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.     

Cardiac output response to dynamic exercise after atrial switch repair for transposition of the great arteries

Results from this study showed that patients who underwent successful operation for transposition of the great arteries had no appropriate increase in stroke volume in response to exercise of a nature similar to common recreational activities. The impairment, most likely due to disturbances in both venous return and ventricular systolic function, is compensated for by an increase in peripheral oxygen extraction; however, this increase may not be adequate with maturation or during prolonged exercise when cardiovascular constraints are more important.