Baffle Leak Research Articles

Usefulness of multislice computed tomography angiography to evaluate intravascular stents and transcatheter occlusion devices in patients with d-transposition of the great arteries after mustard repair

Percutaneous interventions can treat long-term complications after Mustard atrial switch operation in patients with d-transposition of the great arteries (d-TGA), but follow-up for these procedures has not been established. Four patients with d-TGA and previous Mustard operation underwent percutaneous placement of covered stents to relieve superior and inferior vena caval baffle obstructions and leaks. At 6 to 13 months, assessment with 16-slice spiral computed tomography identified stent patency as well as lead placement and visualization of additional devices.

Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery

Background This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery. Methods Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months). Results Fifteen-year actuarial survival was 92.9% ± 4.9% for coronary transfer, 40.0% ± 21.9% for subclavian interposition, and 89.9% ± 7.5% for intrapulmonary tunnel ( p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% ± 7.4%). Left ventricular shortening fraction increased from 17.3% ± 6.3% before operation to 34.1% ± 4.6% at last follow-up ( p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery ( r 2 = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% ± 5.2%) and best longitudinal shortening fraction recovery (36.4% ± 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors. Conclusions Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.

Predicting the identity of decompressing veins after cavopulmonary anastomoses

D veins are an important cause of unanticipated cyanosis after cavopulmonary anastomosis (CPA). Venous connections between the superior and inferior vena caval pathways result in cyanosis after bidirectional CPA (hemi-Fontan and bidirectional Glenn), whereas connections between the systemic and pulmonary venous pathways lead to cyanosis after either bidirectional CPA or total cavopulmonary connection (Fontan operation). Previous studies have focused on hemodynamic settings in which decompressing channels are likely to occur after bidirectional CPA. However the spectrum of anatomic variations resulting in significant venous decompression after total as well as superior cavopulmonary connections has not been well characterized. In this study, we defined the anatomy of significant decompressing veins occurring after CPA, determined the incidence of significant decompressing veins by reviewing angiograms in all patients after CPA, and determined whether cardiac anatomy or clinical parameters were predictive of decompressing venous anatomy. The cardiology database from The Children’s Hospital of Philadelphia was reviewed to identify patients with CPAs who had undergone cardiac catheterization between January 1987 and January 1998. Patients whose angiograms could be obtained and were of adequate quality for interpretation were included in the analysis. Echocardiographic diagnoses were reviewed. The anatomy of the underlying heart defect was classified into 1 of 4 categories: (1) hypoplastic left heart syndrome, (2) other functional single right ventricle (including double-outlet right ventricle), (3) functional single left ventricle (including tricuspid atresia and pulmonary atresia), and (4) heterotaxy syndrome. Hemodynamic information including arterial oxygen saturation, pulmonary artery pressures, and ventricular end-diastolic pressures was recorded. Angiograms were reviewed noting anatomy and size of decompressing veins. Magnification correction was based on the known diameter of the angiographic catheter. Decompressing vessels were deemed significant if they were 3 mm in diameter or associated with arterial oxygen saturation 1 SD below the mean for each group (superior or total CPA). In many patients, right azygos veins were intentionally left patent by the surgeon at the time of bidirectional CPA. The significance of decompressing right azygos veins was determined solely by saturation criteria. Finally, in patients with other sources of arterial desaturation after total cavopulmonary connection (baffle leak, fenestration, or partial hepatic vein exclusion), only size was considered in determining the significance of decompressing veins. Differences between groups were evaluated by Student’s t test or Wilcoxon rank-sum test for numerical data, and chi-square distribution for categorical variables. A p value 0.05 was considered statistically significant. Five hundred fifty patients were identified with CPA. Angiograms adequate for review were obtained for 298 of these patients. Hypoplastic left heart syndrome was present in 170 patients (47%), single left ventricle in 101 (28%), single right ventricle in 60 (17%), and heterotaxy syndrome in 32 (8%). Data were collected on 208 patients who had undergone hemi-Fontan or bidirectional Glenn procedures. The median age at catheterization was 19 months (range 4 months to 17 years). Thirty-three of these patients (16%) had significant decompressing veins. Pulmonary artery pressures were higher, and (partially by definition) arterial oxygen saturations were lower in patients with decompressing veins (p 0.05; Table 1). Four types of decompressing veins occurred after bidirectional CPA. Most common were azygos connections; 19 cases accounted for 58% of the total. This type included azygos veins to either the right or left superior vena cava (SVC), hemiazygos veins, and accessory hemiazygos veins (Figure 1A). Left SVC decompressing to a coronary sinus occurred in 8 paFrom the Department of Pediatrics, University of Pennsylvania School of Medicine and Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania. Dr. Rome’s address is: Division of Cardiology, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. E-mail: rome@email.chop.edu. Manuscript received February 19, 2001; revised manuscript received and accepted July 23, 2001. TABLE 1 Hemodynamic Data from Patients With Bidirectional Cavopulmonary Anastomosis

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

Background: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. Methods: Thirty-one patients (19.9 ± 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. Results: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. Conclusions: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues. (J Thorac Cardiovasc Surg 2000;119:340-6)

Intermediate outcome after Mustard and Senning procedures: A study by the Congenital Heart Surgeons Society.

Although atrial switch is rarely performed today except as part of a "double switch" operation, there continues to be interest in the long-term outcome of the procedure because of the many Mustard and Senning survivors who are in follow-up. In contrast to most other reported series on atrial switch, this study by the Congenital Heart Surgeons Society (CHSS) is a prospective multiinstitutional study of patients encountered in a relatively short time frame during the late 1980s. Between 1985 to 1989, 20 centers with surgeons belonging to the CHSS prospectively registered patients <15 days of age presenting with simple or complex transposition of the great arteries (TGA). The patients were assigned to protocol groups based on intent to treat (arterial switch, Mustard, or Senning). Data were abstracted in the Data and Analysis Center, which in most cases, conducted the annual follow-up. Among the 281 patients who had an atrial switch, there were 108 Mustard and 173 Senning procedures. For the combined atrial switch population, the survival at 1 month, 5 years and 10 years was 90%, 85%, and 84%, respectively. Results for the Mustard were better than for the Senning with survival at 1 month, 5 years, and 10 years being 96% versus 86%, 95% versus 80%, 93% versus 78%; (P <.001) for Mustard versus Senning. While the major mortality risk occurred in the first postoperative month for both groups, thereafter, the late rate of death from 1 to 10 years after operation was 0.78%/year Senning versus 0.23%/year Mustard (P <.05). TGA/ventricular septal defect (VSD), lower weight, younger age, cardiac positional anomalies, and procedures on the left ventricle (LV) outflow all correlated significantly with poor outcome. There were 19 reoperations including 2 for right ventricle (RV) failure, 12 for pathway obstruction, and 5 for baffle leak with a 36% overall mortality. Freedom from any pathway obstruction at 10 years was 95%. A permanent pacemaker was required in 21 patients with the significant risk factors including TGA/VSD and a Senning, and previous atrial septectomy. Freedom from a pacemaker insertion at 10 years was 91%. For both Mustard and Senning, 60% of patients were in New York Heart Association (NYHA) functional Class I with the remaining in Class II. This study of atrial switch outcomes by the CHSS suggests that the subgroup undergoing a Mustard procedure had better early and late survival than those undergoing a Senning operation. The overall incidence of baffle pathway obstruction was low though reoperation for this complication carried high risk. Serious arrhythmia requiring medication and the need for a permanent pacemaker were both relatively uncommon. The incidence of severe right heart failure was very low, although only 60% of patients are unrestricted in their activity. Copyright 2000 by W.B. Saunders Company

Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Recovery of Left Ventricular Function After Dual Coronary Repair

Objectives. We reviewed our institutional experience with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) after dual coronary repair to assess preoperative variables predictive of outcome, the time course for postoperative recovery of cardiac function, the short- and long-term complications and our experience with left ventricular assist devices (LVAD) in these patients.Background. Outcome after surgical repair of ALCAPA remains incompletely defined.Methods. The surgical records and echocardiograms of 42 patients were reviewed. Left ventricular function was assessed by fractional shortening z-score (FSz) and stress-velocity index.Results. The overall survival rate was 86%. All six patients who died were <1 year old and died within 3 days of the operation. More severe preoperative mitral regurgitation (MR) was associated with increased mortality, but age, body surface area, preoperative FSz and end-diastolic dimension were not. We used an LVAD for 7 of 28 patients who underwent repair for ALCAPA since its introduction at our institution, with a survival of 5 of 7 patients. The degree of MR improved in 62% of patients and remained unchanged in 38%. Complications included supravalvar pulmonary stenosis (16 of 21 patients) and baffle leaks (11 of 21 patients) with the intrapulmonary baffling technique. Supravalvar pulmonary stenosis developed in 1 of 11 patients after direct coronary reimplantation. Left ventricular function became normalized in all 28 patients with follow-up past 1 year, regardless of preoperative FSz. Of 13 patients who underwent serial postoperative echocardiography, the average time to normalization of function was 2 to 7 months.Conclusions. The degree of preoperative MR was predictive of outcome, whereas the severity of preoperative cardiac dysfunction and ventricular dilation were not. Mild and moderate MR tended to improve without mitral valvuloplasty. Complete recovery from myocardial dysfunction is expected after dual coronary repair of ALCAPA.

Pediatric lung transplantation--are there surgical contraindications?

Lung transplantation has evolved as a successful treatment for end-stage cardiopulmonary disease in children; however, clear guidelines regarding surgical exclusion criteria for pediatric lung transplant candidates have not been well-established. Since December 1994, we have performed 10 bilateral lung transplants and 1 heart-lung transplant in 10 recipients (mean age, 7 years; range, 3 months to 19 years). Indications for transplantation included pulmonary vascular disease (n=6), bronchiolitis obliterans (n=2), bronchopulmonary dysplasia (n=1), graft failure due to viral pneumonitis (n=1), and cystic fibrosis (n=1). Among the 10 patients, 4 were evaluated elsewhere for lung transplantation; of these, 3 were rejected by 1 or more programs because of "high-risk" characteristics. We considered 8 of the 10 patients to have 1 or more "high-risk" characteristics, as follows: previous chest operations other than open lung biopsy (n=6 patients having 1-4 previous operations), ventilator-dependence with tracheostomy and high-dose corticosteroids (n=4), redo lung transplant (n=2), concomitant intracardiac repair (n=6), portal hypertension (n=1), and the use of extracorporeal membrane oxygenation (ECMO) at the time of transplant (n=1). Our standard operative approach was a bilateral thoracosternotomy. Cardiopulmonary bypass was used for explant of the recipient lungs and implant of the donor lungs, and during repair of coexisting congenital heart defects. Aprotinin and fresh whole blood were administered during the procedure to aid in hemostasis. Concomitant procedures were frequently performed and included repair of an intra-atrial baffle leak (prior Mustard procedure), closure of an atrial septal defect, repair of partial anomalous pulmonary venous return, reconstruction of the pulmonary venous confluence, ECMO decannulation, and splenectomy. There were no operative deaths, and no patient required re-exploration for bleeding. One patient had primary graft failure due to adenovirus infection of the donor lungs, and required prolonged mechanical ventilation and eventually ECMO support until retransplantation was performed. The mean hospital stay after transplant was 25+/-13 days (range, 10-56 days). All patients were discharged with a natural airway. Airway complications consisted of one bronchial anastomotic stricture which required dilation, for a complication rate of 5% per anastomoses at risk. One patient required reoperation for stenosis of the superior vena cava. There have been no late deaths, with a mean follow-up of 7+/-4 months (range, 1-13 months). We attribute this 100% operative and short-term survival in these "high-risk" pediatric lung transplant recipients to our operative methods, a multidisciplinary approach to postoperative management, and the enormous physiologic reserve of pediatric patients. Therefore, the standard exclusion criteria used for adult lung transplantation may not be applicable to the pediatric age group. We hope to use these data to expand the use of lung transplantation in pediatric patients.

Intermediate results of the extracardiac fontan procedure

Fourteen children (ages 2 to 14 years) and 1 adult (32 years) have undergone a modification of the Fontan procedure in which an extracardiac lateral tunnel or conduit is used in combination with staged or simultaneous bidirectional Glenn shunt(s). Extracardiac lateral tunnels (n = 9) were constructed using a polytetrafluoroethylene patch (n = 7), pericardial patch (n = 1), or in situ pericardial flap (n = 1). Extracardiac lateral conduits (n = 6) were constructed using nonvalved homografts (n = 2) or polytetrafluoroethylene tube grafts (n = 4). Fenestrations were created in 4 patients (2 each in extracardiac lateral tunnel and extracardiac lateral conduit patients). Aortic cross-clamping was completely avoided in 12/15 patients (aortic cross-clamping in 2 patients for atrial septal defect enlargement and 1 for Damus-Kaye-Stansel procedure). There have been no operative deaths. Prolonged postoperative chest tube drainage (> 2 weeks) has been rare (n = 1). At follow-up (range, 6 to 54 months; mean, 27.5 months), all patients are in New York Heart Association class I or II and remain in normal sinus rhythm. Late protein-losing enteropathy was seen in 1 patient and was successfully treated by percutaneous creation of a stented fenestration from the extracardiac tunnel to the systemic atrium. Late catheterizations reveal unobstructed extracardiac lateral tunnel function and low pulmonary pressures (range, 11 to 13 mm Hg). Advantages of the extracardiac Fontan include (1) avoidance of aortic cross-clamping in most patients, (2) the hemodynamic benefits of total cavopulmonary connection, (3) avoidance of atriotomy and intraatrial suture lines, (4) preservation of sinus rhythm and no arrhythmias at 2 year follow-up, (5) drainage of the coronary sinus to low pressure atrium, (6) allowance for early/late fenestrations, (7) prevention of baffle leaks and intraatrial obstruction, and (8) allowance for growth (tunnel procedures only). We recommend this extracardiac procedure for all suitable patients undergoing surgical conversion to the Fontan circulation.

Postoperative complications after the Mustard operation for complete transposition—a qualitative evaluation with magnetic resonance imaging

AbstractLong-term follow-up studies after intraatrial repair for complete transposition have shown postoperative morphologic and hemodynamic abnormalities in many patients which often require reevaluation and therapeutic intervention. The purpose of this study was to assess, using magnetic resonance imaging, the presence and extent of late postoperative complications in a large cohort of 44 patients undergoing repair with the Mustard procedure. Of these 34 had an intact ventricular septum with or without obstruction of the left ventricular outflow tract, while 10 had an associated ventricular septal defect. Transverse spin-echo and gradient-echo images were acquired of the entire heart from the diaphragm to the bifurcation of the pulmonary trunk. Additional oblique images were acquired for better visualization of venous connections. Abnormal findings were diagnosed by visual inspection of spin-echo and gradient-echo images, and diagnoses were compared to previous findings at cardiac catheterization. On magnetic resonance imaging, a baffle leak was seen in eight patients, two had pulmonary venous obstruction, four had obstruction at the caval veins, 23 had obstruction of the left ventricular outflow tract and 18 had tricuspid regurgitation. Cardiac catheterization showed a leak across the baffle in 16, pulmonary venous obstruction in two, obstruction at the caval veins in four, obstruction of the left ventricular outflow tract in nine, and tricuspid regurgitation in nine. We conclude that combined spin-echo and gradient-echo magnetic resonance imaging provides extensive noninvasive assessment of postoperative sequels and residuals in patients after the Mustard procedure for complete transposition.

1002-87 The Results of Spontaneous and Clamshell Umbrella Closure of the Fenestrated Fontan

The fenestrated Fontan (FF) procedure was performed in 24 consecutive patients (pts) — aged 1 to 13 years (mean 5.4) — with one or more high risk factors in order to reduce operative mortality and morbidity. This study evaluated their outcome. The fenestration was 2.7 mm in 4 pts, 4 mm in 12, and 4.5 mm in 8. One pt (4.2%) died in hospital. One pt died 7 months after FF at cardiac transplantation. Survivors have been followed for 16 to 48 months (mean 34). Early O2 saturations (sats) were 88–92% (mean 90) in 2.7 mm and 69–89% (mean 79.4) in larger FF. All four 2.7 mm, one 4 mm and four 4.5 mm fenestrations closed spontaneously but of these one 2.7 mm (at 2 weeks) and one 4 mm (at 3 years) required recreation of a hole by trans-baffle puncture and balloon dilation because of congestive failure. Nine 4 mm and two 4.5 mm fenestrations were closed with a clamshell umbrella (U). Following U closure, RA pressure increased from 12 ± 1.8 to 14.7 ± 2.4 mmHg (p &lt; 0.01), O2 sats increased from 80.2 ± 6.6 to 93.7 ± 4.6% (p &lt; 0.001), but the artriovenous 02 saturation difference increased from 18.8 ± 4.1 to 25.8 ± 6.7% (p &lt; 0.02). Unusual systemic venous collaterals resulted in R to L shunts in 3 pts and recurrent pericardial effusions in 1; all were successfully coil occluded. Atrial baffle leaks resulted in large R to L shunts in 3 pts after effective fenestration closure with U. One was successfully coil occluded; the other 2 have O 2 sats of 84 and 88% late post U. Clinical follow-up has shown a high incidence of dysrhythmias (atrial tachycardia in 5, 4 requiring amiodarone; sick sinus syndrome in 3; seven of these required a pacemaker). Three pts have had severe congestive failure, one immediately post U closure. Two pts have protein losing enteropathy. Two pts have had strokes: 1 early and minor; the other 4 months after U closure and major. Eighteen of 22 survivors are in NYHA Class 1. The fenestrated Fontan resulted in a low operative and intermediate mortality. Most fenestrations either closed spontaneously or were closed with a clamshell umbrella. Although most patients have a good functional result, dysrhythmias and other late complications are frequent.

985-62 Outcome of Anomalous Origin of the Left Coronary Artery

The outcome after surgical repair of anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA) remains incompletely defined, and transplantation has been advocated for patients with severe left ventricular dysfunction. We reviewed our institutional experience with ALCAPA since 1977 in 38 patients whose repair established a dual coronary circulation (7 early cases with LCA ligation were excluded) to assess whether cardiac function predicts outcome, the use of left ventricular assist device (LVAD), and postoperative complications of the current operative techniques. Surgical techniques included direct coronary reimplantation to the aorta in 11 pts, intrapulmonary baffling (Takeuchi) when the LCA could not reach the aorta directly in 25 pts, and modified Takeuchi in which an extrapulmonary baffle was fashioned from anterior and posterior walls of the pulmonary artery in 2 pts. The operative survival was 91%, 88%, and 100%, respectively (89% overall). All pts who died were &lt; lyr old (3/19 Takeuchi, 1/10 reimplantation). Since its introduction at our institution in 1990, LVAD has been used in 4/16 pts, 1 Takeuchi pt who died and 3 reimplantation pts who all survived. Survival in pts &lt; 1 yr old was not different in those with severe dysfunction (shortening fraction (SF) &lt; 15%,18/20) compared with moderate dysfunction (SF 16–25%, 4/5). Function returned to normal in all pts for whom followup is known past one year, regardless of the severity of preoperative dysfunction. Postoperative complications for Takeuchi pts included baffle leaks in 6/21 pts who underwent postoperative echocardiogram, supravalvar pulmonary stenosis in 13/20 pts for whom followup is known (&lt; 25 mmHg in 9 pts, 26-50 mmHg in 2 pts, &gt; 50 mmHg in 2 pts requiring reoperation), and progressive aortic regurgitation requiring aortic valve replacement in 1 pt. There were no complications in the two modified Takeuchi pts (6 and 12 mos followup) or in reimplantation pts. (1) The degree of LV dysfunction at presentation does not predict survival in pts &lt; 1yr old, (2) current operative survival for ALCAPA does not justify consideration of transplantation, (3) LVAD may improve survival for some patients and should be available for postoperative management, and (4) supravalvar pulmonary stenosis occurs in a majority of Takeuchi pts, some of whom require reoperation; a modified technique may improve this postoperative complication rate.

Transcatheter management of pulmonary venous pathway obstruction with atrial baffle leak following mustard and senning repair

Two patients presenting with pulmonary venous baffle obstruction following Mustard or Senning repair of transposition of the great arteries were successfully treated with percutaneous balloon dilatation. At the time of baffle dilatation, a significant systemic to pulmonary venous Mustard baffle leak was successfully closed with a Rashkind ductal occluder device. Specific features pertaining to the morphology of the baffle defect that allowed successful catheter occlusion are discussed and compared to that found in a Senning patient. The important adjunctive role of transoesophageal echocardiography within the catheterization laboratory is emphasized.

The Extracardiac Total Cavopulmonary Connection For Definitive Conversion to the Fontan Circulation: Summary of Early Experience and Results

Between July 1991 and March 1993, five children (ages 2 to 6 years) with complex congenital heart disease have undergone a new operation for conversion to the Fontan circulation. This procedure combines a bidirectional Glenn shunt with an extracardiac lateral tunnel (ELT) to carry systemic venous return to the pulmonary arteries (PAs). The ELT was constructed so that the circumference consists of Gore-Tex (2/3) and lateral epicardial atrial wall (1/3). The ELT can be performed with all varieties of single ventricle physiology, as in our patients with tricuspid atresia (n = 3), dextrocardia (n = 1), and situs inversus with levocardia (n = 1). PA reconstruction was required in four patients. At follow-up from 1 to 20 months, all patients are in New York Heart Association Class I and in normal sinus rhythm. Postoperative catheterization has revealed low PA pressures (< or = 12 +/- 1 mmHg) and angiography has shown excellent ELT function with brisk flow into the PAs bilaterally. All patients maintain an O2 saturation > 94% on room air. The advantages of this new extracardiac modification of Fontan's operation are: (1) aortic cross-clamping is not usually required; (2) incorporation of lateral atrial wall in ELT allows for growth while permitting construction of a fenestration or adjustable atrial septal defect in high risk patients; (3) absence of atriotomy and intraatrial suture lines may decrease late risk of arrhythmias; (4) early or late baffle leaks cannot occur; (5) intraatrial obstruction from the baffle cannot occur; (6) coronary sinus remains in low pressure atrium; and (7) hydrodynamic benefits of the total cavopulmonary connection are preserved. We recommend this procedure for patients undergoing surgical conversion to the Fontan circulation.

Magnetic Resonance Imaging of Cardiac Function and Morphology in Patients with Transposition of the Great Arteries Following Mustard Procedure

In order to assess the diagnostic possibilities of magnetic resonance imaging (MRI), 30 children (mean age 11.5 years) with d-transposition of the great arteries (TGA) corrected by the Mustard procedure were studied. Patient values were compared to those of 10 healthy volunteers. The most important postoperative abnormalities such as baffle leaks, systemic or pulmonary venous obstructions, left ventricular outflow tract (LVOT) stenosis and tricuspid regurgitation were assessed. Cardiac volumes and muscle mass were measured. Baffle leaks were found in 5 and obstruction of the baffle limbs in 6 patients. On gradient echo images 13 patients showed signs of LVOT stenosis and 12 tricuspid incompetence. Right ventricular end diastolic volumes in patients were significantly higher than the left ventricular volumes, but slightly lower than right ventricular volumes in the normals. In patients with TGA and ventricular septal defect (VSD) (n = 7) right ventricular (RV) volumes were found to be higher than in patients with intact ventricular septum (n = 23) and in normals. In contrast to the normal RV function of patients after Mustard surgery only patients with previous VSD showed a diminished RV ejection fraction. The muscular mass in TGA patients showed a ratio of 1.9:1 between right and left ventricles. In normals the ratio was 1:1.5. Magnetic resonance imaging allows a quantitative insight into the ventricular function and the morphology of the heart after inflow correction. Thus, it supplements the noninvasive evaluation of TGA patients.

The Senning operation for complete transposition: mid-term physiologic, electrophysiologic, and functional results

SummaryWe report analysis of surgical results in 110 consecutive infants and children who underwent atrial repair of simple complete transposition using the Senning operation between February 1978 and May 1990. Mean age at operation was 5.4 months ± 6.1 (range 1 week to 4 years); 75 were less than 6 months old. There were 72 males and 38 females. Operative mortality rate was 5.5%, with one late death. Average follow-up is 48.1 months with 44 followed greater than 3 years, and 27 greater than 5 years. Postoperative cardiac catheterization was performed in 48 patients. Right ventricular ejection fraction averaged 0.52 ± 0.08 and was normal in 28 patients. Response of right ventricular ejection fraction to afrerload stress was abnormal in 12 of 14 patients tested. Right ventricular ejection fraction increased normally during exercise in 6 patients, but was abnormal in 15. Mild tricuspid regurgitation was noted in 12 patients. Mild obstruction of the superior caval vein was noted in 4 patients. Baffle leak requiring reoperation occurred in one patient. Fifty-seven of 90 patients are in sinus rhythm by latest electrocardiogram. Postoperative electrophysiologic studies were performed in 34 patients, and Holter monitoring in 25. Significant arrhythmia occurred in 26 patients: 6 patients required pacemakers for slow junctional rhythm or complete heart block; 20 additional patients have a junctional rhythm. Six patients have delayed sinus nodal recovery time. At last follow-up, 88 children (98%) are in New York Heart Association functional Class I, and 2 (2%) are in Class II. The Senning operation for compete transposition can be accomplished with a low operative and late mortality. Serious baffle complications requiring reoperation are rare. Surviving patients are clinically well, but arrhythmias and depressed right ventricular function may limit their long-term functional status.

Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after an atrial correction procedure

Previous methods used to assess atrial baffle function after correction of transposition of the great arteries have included precordial echocardiography and cardiac catheterization. To evaluate whether single plane transesophageal echocardiography might provide additional information, its findings were correlated with information derived from both precordial echocardiography and cardiac catheterization in 15 patients (14 Mustard procedures, 1 Senning procedure) aged 4.2 to 33 years (mean 16.3). Precordial ultrasound with combined imaging, color flow mapping and pulsed Doppler ultrasound visualized the supramitral portion of the common systemic venous atrium in every case but could identify only superior limb obstruction in three of six patients, mid-baffle obstruction in zero of two and inferior limb obstruction in zero of two patients.Transesophageal studies with use of the same range of ultrasound methods demonstrated superior limb obstruction (severe in four, mild in two) in six of six patients, mid-baffle obstruction in two of two and inferior limb obstruction in two of two patients. The entire pulmonary venous atrium was equally well interrogated by either ultrasound approach, with both identifying three cases (two mild, one moderate) of mid-pulmonary venous atrium obstruction. However, individual pulmonary vein velocity profiles could only be recorded by transesophageal pulsed Doppler ultrasound. Precordial studies identified baffle leaks (1 large, 2 small) in only three patients, whereas transesophageal studies identified 11 such baffle leaks (1 large, 10 small), which were multiple in two patients.It is concluded that transesophageal echocardiography provides a more detailed and accurate assessment of atrial baffle morphology and function than is provided by either precordial ultrasound or cardiac catheterization. Transesophageal echocardiography appears to be the investigative method that derives the most information and that is applicable on an outpatient basis in adolescent and adult patients.

Ten years' experience with the senning operation for transposition of the great arteries: Physiological results and late follow-up

We report our results in 93 consecutive infants and children who underwent atrial repair of simple transposition of the great arteries using the Senning operation between February 1978 and February 1988. Mean age at operation was 5.6 ± 6.3 months (range, 1 week to 4 years); 60 were less than 6 months old. There were 65 boys and 28 girls. Operative mortality was 5.4%, and there has been 1 late death. Average follow-up is 45.1 months with 39 followed more than 3 years and 25 followed more than 5 years. Postoperative cardiac catheterization was performed in 43 patients. Right ventricular ejection fraction at rest averaged 0.50 ± 0.09 and was normal in 26 patients. Response of right ventricular ejection fraction to afterload stress was abnormal in 12 of 14 patients tested. Right ventricular ejection fraction increased normally during exercise in 6 patients, but was abnormal in 15. Mild tricuspid regurgitation was noted in 10 patients. Mild obstruction of the superior vena cava was noted in 4 patients. Baffle leak requiring reoperation occurred in 1 patient. Seventy-two of 80 patients are in sinus rhythm by latest electrocardiogram. Postoperative electrophysiological studies were performed in 34 patients and Holter monitoring was performed in 22. A major arrhythmia occurred in 8 patients: 3 required a pacemaker for functional rhythm or sinus node dysfunction, 2 have symptomatic or inducible supraventricular tachycardia, 2 have functional rhythm, and 1 has sick sinus syndrome. Eight additional patients have delayed sinus node recovery time. At last follow-up, 78 children (97.5%) are in New York Heart Association functional class I, and 2 (2.5%) are in class II. The Senning operation for simple transposition of the great arteries can be accomplished with a low operative mortality. Serious baffle complications requiring reoperation are rare. Surviving patients are clinically well, but arrhythmias and depressed right ventricular function can be a problem with prolonged follow-up.

Senning repair for transposition of the great arteries without patch augmentation of the septum

Forty-three consecutive patients with previous balloon atrial septostomy have undergone a Senning type repair of transposition of the great arteries without patch augmentation of the atrial septum. A technique was used that allows expansion of the atrial septum without the use of additional foreign or autogenous materials. The operative (30-day) mortality rate was 4.6% (2/43) with no late deaths. A mean follow-up of 20 months shows that 83% of the patients are in sinus rhythm and none of these patients have clinically detectable caval or pulmonary venous obstruction or baffle leaks. In patients selected for atrial switch, the Senning operation may be performed without patch augmentation of the septum. Performed by the method described herein, the operation provides predictable early and late results with a low prevalence of arrhythmias. Significant venous obstruction has not occurred.

Fate of Long-Term Survivors of Mustard Procedure (Inflow Repair) for Simple and Complex Transposition of the Great Arteries

Between 1967 and 1976, 106 children with transposition of the great arteries (TGA) (55 simple, 51 complex) survived the Mustard procedure. Late death occurred in 8 patients (1 simple, 7 complex TGA). Cardiac arrhythmia developed in 31 patients, 6 of whom required a permanent pacemaker. Postoperative cardiac catheterization showed mild superior vena cava obstruction in 4 patients, mild pulmonary venous obstruction in 3, and baffle leak in 4. Only 1 of these patients underwent reoperation elsewhere for a baffle leak. Two other patients had reoperation for subpulmonary stenosis and 1, for tricuspid regurgitation. The actuarial survival at 18 years is 92 ± 2.3%, and the event-free survival is 83 ± 3.8% (95% confidence limits). Eighty-seven patients are in New York Heart Association Functional Class I, and 3 are in Class II. The results of this study show that the long-term survival and event-free survival have been satisfactory. Late death was significantly higher in patients with complex TGA ( p = .027). Postoperative arrhythmia was common, but only 6 patients required permanent pacemakers and the incidence of late complications and reoperation has been low.

Two-dimensional echocardiographic localization of residual atrial shunts after the Senning procedure

The most common complication of Mustard repair of transposition of the great arteries is a baffle leak, with incidence rates averaging approximately 20%. 1–3 Although there are fewer follow-up data for the Senning procedure, the incidence of residual atrial shunting is probably similar after both the Mustard and Senning procedures. 4 We investigated whether 2-dimensional (2-D) echocardiography can successfully localize the site of interatrial shunting following Senning repair.