MONDAY AFTERNOON, MAY 8, 1978

2:00 P.M. Scientific Session - Ballroom

7. Thoracic Neuroblastoma in Infants and Children

PHILIP W. CATALANO*, WILLIAM A. NEWTON*,

THOMAS E. WILLIAMS, JR.*, H. WILLIAM CLATWORTHY, JR*

and JAMES W. KILMAN, Columbus, Ohio

Neuroblastoma is the third most common tumor in childhood. Over the past 32 years we have treated 186 patients with neuroblastoma. Sixty-five percent were primary abdominal tumors and 20 percent (38 patients) were primary chest tumors. These 38 tumors occurred in 16 males and 22 females. Three of the males were black, otherwise all patients were white. There were 22 patients (58%) under the age of 2 years with a 90% two-year survival. There were 16 patients (42%) who were two years old or older and of these only 37% (six patients) survived two years after the diagnosis was made. The vast majority of these patients were treated with surgery (debulking-type procedure), and postoperative radiation and chemotherapy. Of the 12 deaths, 11 occurred within two years of initial diagnosis. Patients with most differentiated tumors had a remarkably good survival with no deaths, however, the tumors with lesser differentiation did not stratify enough to draw conclusions as to survival. Staging correlated the least with survival when compared to age or grading. The percent of two-year survivors of Stages I, II, III, IV, IV's were 75%, 82%, 100%, 22% and 80% respectively. Postoperative deaths were reviewed and of the 12 deaths: 2 died of operative complications, 3 others had only node biopsy to confirm diagnosis without debulking procedure, and 1 died of massive gastrointestinal hemorrhage.

In conclusion, 38 documented primary thoracic neuroblastoma patients are reviewed with followup from 2 to 27 years with an average of 9.3 years. We have concluded from this experience that age is the main determining factor influencing survival. Heroic and/or radical surgery is not warranted and is contraindicated in this disease.

*By invitation

8. Conduit Repair for Complex Congenital Heart Disease: Late Follow-up

HERMAN A. HECK, JR. * RICHARD M. SCHEIKEN*,

RONALD M. LAUER * and DONALD B. DOTY,

Iowa City, Iowa

A five year experience with the Rastelli operation including systematic late recatheterization is presented. Twenty-two patients have undergone primary repair and an additional patient has had his obstructed conduit replaced. Mortality for primary repair was 27% (6/22). Eighty-eight percent of survivors are asymptomatic four months to seven years post repair. Of 14 patients who are alive and greater than one year post repair, 13 have undergone recatherization a mean of 37.7 months later. Fourteen conduits are available for review and 11 (78%) reveal varying degrees of obstruction ranging from mild to severe, eight or 57% of these having significant gradients greater than 40 mm.Hg. All five homograft or composite homograft conduits reveal gradients at the homograft valve greater than 40 mm.Hg., two with severe gradients greater than 80 mm.Hg. Six of nine heterograft conduits recatheterized are obstructed proximally though only one has a severe degree of obstruction.

Three of eight patients (37.5%) with large systemic-pulmonary communications of greater than five years duration subsequently exhibited evidence of moderate to severe pulmonary vascular disease.

Experience with the Rastelli operation and late follow-up suggests that: 1) pre-existing shunts or very large collaterals should be considered for repair prior to five years duration, 2) acceptable early mortality and substantial long term palliation can be achieved in cases associated with severely increased pulmonary vascular resistance, 3) factors of growth and/or substernal positioning seem to be responsible for proximal conduit obstruction, 4) insofar as severe obstruction may exist in otherwise asymptomatic patients, routine late recatheterization should be performed on all conduit patients.

*By invitation

9. Mechanism of Sinus Node Damage During Mustard Operation

JOHN H. WITTIG*, MARC DELAVAL *, and

JARDASLAV STARK*, Los Angeles, California

and London, England

Sponsored by James V. Maloney, Jr., Los Angeles, California

We have previously described the damage to the atrial septum which produced postoperative arrhythmias during Mustard operation using epicardial and endocardial mapping in 39 patients. In 13 of 39 patients, detailed sinus node mapping before and after operation demonstrated significant damage to the sinus node with development of sick sinus node disease. This was further investigated in 29 dogs undergoing baffle placement with 20 chronically implanted electrodes in the SA node area. Two immediate and one late iatrogenic mechanism for SA node arrhythmias were noted. Early arrhythmias after baffle placement in 5 of 15 dogs were due to ligation of the sinus node artery. In 2 of 15, placement of the sutures through the epicardially located SA node during suturing of the SVC portion of the intra-atrial baffle produced arrhythmias identical to those seen in our patients. The late sinus arrhythmias were seen when fibrotic scar from the SVC baffle suture line produced sclerotic changes in the sinus nodes in 13 of 15 animals. These arrhythmias developed between 6 weeks and 16 months post-operatively and appear to be similar to the late postoperative arrhythmias seen in the Mustard patients. Awareness of the variations in anatomy of the SA node, its arterial supply and nervous supply is mandatory. Movement of the SVC portion of the intra-atrial baffle into the RA appendage prevents ligation of the SA node artery and nerve. Placement of the baffle suture line away from the sinus node has reduced early arrhythmias to 0 of 14 and late arrhythmias due to sclerosis of the sinus node to 1 out of 14. The application of these operative changes to the Mustard technique can markedly reduce arrhythmias and sudden death in patients.

*By invitation

10. Clinical Implications of Postoperative Unilateral Phrenic Nerve Palsy

JOHN J. MICKELL *, F. JAY FRICKER * ROBERT A. MATHEWS*,

and RALPH D. SIEWERS*, Pittsburgh, Pennsylvania

Sponsored by Henry T. Bahnson, Pittsburgh, Pennsylvania

Unilateral phrenic nerve palsy (PNP) followed 28 (1.48%) of 1891 consecutive cardiac surgical procedures during an 8 year period. Patient age at time of operation ranged from 1 day to 14 years (mean 3.9 years), and 11 were infants under 8 months. Five (18%) had a Blalock-Taussig shunt, 4 (14%) PDA ligation, 4 (14%) a Mustard operation, 3 (11%) ASD closure, 2 (7%) a Waterston anastomosis, and 2 (7%) atrial septectomy. The remaining 8 (29%) were isolated occurrences with a variety of procedures. Though 7 (25%) occurred during 1975, including the 3 following ASD closure, there was no correlation with a specific surgeon or malfunction of electrocautery equipment. PNP complicated 6.7% of atrial septectomies, 5.0% of Blalock-Taussig shunts, 4.4% of Mustard operations, 3.6% of Waterston anastomoses, and 1.3% of ASD closures and PDA ligations. Sixteen (57%) had right PNP and 12 (43%) had left PNP. Five of the 8 (29%) who developed PNP following median sternotomy had a previous atrial septectomy or Blalock-Taussig shunt through a lateral thoracotomy. Radiographic diagnosis of PNP was often delayed during positive pressure ventilation. Diagnosis was confirmed in 18 (64%) by fluoroscopy of the diaphragm during spontaneous breathing. Twelve (43%), including 8 infants, required prolonged mechanical ventilation ranging from 36 hours to 93 days (mean 25 days). Weaning was complicated by atelectasis (8), postextubation stridor (7), CO₂ retention (4) and respiratory arrest (3). Four (14%) required tracheostomy and one underwent diaphragm plication 3 months postoperatively. One died of sepsis and 1 of a subsequent Fon-tane procedure. Radiographic resolution was documented in 18 (64%) from 1 week to 3½ years postoperatively. Radiographic abnormality persisted in 7 (25%) after a follow-up of 1 month to 5 years and all were asymptomatic. PNP significantly affects the morbidity of cardiac surgery especially in infancy. A satisfactory outcome is possible in most cases with prolonged hospitalization for ventilatory support.

INTERMISSION - VISIT EXHIBITS

*By invitation

11. The Senning Operation for Transposition of the Great Arteries

L. PARENZAN*, G. LOCATELLI*, and

M. VILLANI*, Bergamo, Italy

Sponsored by Albert D. Pacifico, Birmingham, Alabama

The reported incidence of the main complications related to the Mustard operation for correction of transposition of the great arteries seems to be quite high: more than half patients develop arrhythmias and about one third develop venous (pulmonary or systemic) obstructions.

For these reasons we have reconsidered the type I (1959) Senning operation. Thirteen children below two years of age (body weight ranging from 3.9 to 12 kg.) have been operated on with this technique at our Institution.

Twelve were D-TGA and one L-TGA. One patient had a complex TGA (large VSD and severe subpulmonary stenosis associated) where as the remaining cases were considered as having simple TGA (small VSD's and mild pulmonary outflow obstructions). All patients survived operation and none suffered from complications.

At the time of the discharge from the Hospital all were in sinus rhythm. Late evaluation (24 hours EKG, cardiac catheterization, ect.) is in course. We believe that the Senning operation is easier to perform than the Mustard operation because of its more standardized technique which respects the internal geometry of the heart. Additional advantages are: 1) the intraatrial conducting pathways are less likely to be damaged. 2) There is minimal or no need for artificial tissues.

*By invitation

12. Total Correction of Transposition of the Great Arteries: Conduction Disturbances in Infants Less Than Three Months of Age

KEVIN TURLEY* and PAUL A. EBERT, San Francisco, California

During the period 1975 through 1977, 49 patients underwent pericardial intra-atrial baffle procedure for correction of transposition of the great arteries (TGA). Thirty-three patients were less than 12 months of age; 22 were infants of three months or less. Twenty-four patients (12 infants) had simple TGA and underwent baffle procedure only. Twenty-one had associated ventricular septal defects (VSD); 16 defects were closed (6 infants) and five left open (3 infants). Five of these 21 patients underwent concomitant relieval of left ventricular outflow tract obstruction (LVOTO). Four patients (one infant) had an intact ventricular septum with LVOTO that was resected at the time of their baffle procedure. Three patients (2 infants) died early postoperatively (6%). There were three late deaths (1 infant), none from sudden arrhythmia. No deaths occurred in patients with simple TGA, intact ventricular septum with LVOTO, or in those with VSD left open.

Surgery was performed utilizing either cardiopulmonary bypass (27) or bypass combined with profound hypothermia and circulatory arrest (22). The latter was employed with single atrial cannulation in 19/22 infants. Operative techniques included the use of a loose intraatrial pericardial baffle and patch enlargement of the systemic atrium. In no patient was the coronary sinus enlarged.

Complete heart block did not occur in any infant. Conducted atrial rhythm was present at discharge in 16 infants, junctional rhythm in two, junctional escape in two. Atrial flutter occurred in two infants prior to discharge, requiring cardioversion in one.

Total correction of TGA utilizing pericardial baffle technique in infants was associated with a low incidence of conduction disturbances. Contributing to this result was avoidance of injury in the region of the coronary sinus, ventricular septum, superior vena cava-atrial junction, and specifically in the area of the sinus node.

*By invitation

13. Factors Influencing the Results of Corrective Surgery for Tetralogy of Fallot with Pulmonary Atresia

OTTAVIO ALFIERI*, JOHN W. KIRKLIN,

EUGENE H. BLACKSTONE*, ALBERT D. PACIFICO

and NEVIN M. KATZ*, Birmingham, Alabama

The hospital mortality (1967-July 1977) in 69 patients with tetralogy of Fallot and congenital or acquired pulmonary atresia was 17%. It was higher in patients less than 48 months of age than in older patients (p=0.06). Five of 48 patients (10%, 70% confidence limit: 6%-17%) with normal-sized or moderately small right and left pulmonary arteries (RPA, LPA) died compared with 7 of 21 patients (33%, CL 22%-47%) in which they were very small or one absent (p=0.02). Right ventricular-pulmonary artery discontinuity was managed with a valved external conduit in 42 patients (hospital mortality 24%, CL 17%-32%), trans-annular patch in 20 patients (hospital mortality 10%, CL 3%-22%) and unfundibular resection with or without valvulotomy in 7 patients (no hospital mortality, CL 0%-24%). Thirteen of the 35 patients with congenital pulmonary atresia undergoing repair with a conduit required catecholamines postoperatively compared with none of the 8 with a transannular patch (p=0.04); postoperative mean PRV/LV was 0.60 in both groups. The survival of patients with a valved conduit was 84% (CL 71%-93%) at and beyond 4 years; 5 of the 32 hospital survivors have required reoperation for conduit obstruction. There have been no late deaths or reoperations among the patients with a transannular patch. Early postoperative pulmonary dysfunction occurred more frequently in patients with large "bronchial" collaterals than in patients with patent ductus arteriosus and/or previous surgical shunts (p=0.003). Ligation of large collaterals did not prevent pulmonary dysfunction and in 1 case resulted in fatal pulmonary infarction. Cold cardioplegia was associated with a higher early postoperative cardiac index than simple cold ischemia (p=0.14). In view of these results, at present the conduit operation is performed only if the main pulmonary artery is absent (which it was in 12.5% in this series) or if the anterior descending coronary artery arises anomalously, large "bronchial" collaterals are not ligated, and cold cardioplegia is used. A preliminary palliative operation is considered if RPA and LPA are very small.

*By invitation

14. Corrective Operation for Double Outlet Right Ventricle Associated with Ventricular Inversion

IMAD TABRY*, DWIGHT C. McGOON, GORDON K. DANIELSON,

ROBERT B. WALLACE, ZEV DAVIS*and

JAMES MALONEY*, Rochester, Minnesota

Twenty-two corrective operations for this unusual condition have been performed, beginning in 1965. All but two of the patients also had pulmonary stenosis (PS) and one of the exceptions had been banded. In all but 1, the cardiac axis pointed inappropriately, and there was situs inversus in 3. Two types of correction were employed. The totally intracardiac repair involved placing the VSD patch between the semilunar valves, plus direct relief of PS. The extracardiac conduit repair involved closure of VSD so as to preserve double outlet RV, and placement of a conduit from pulmonic (morphologic left) ventricle to distal end of divided pulmonary trunk. The table gives results, the average duration of follow up being 45 months.

Three conduits were aortic homografts (the two survivors have required replacement with Hancock conduit (HC), one was a valveless conduit, and the 12 remaining received HC. Heart block occurred in 6, including two among 8 who had successful intra-operative mapping of His bundle. The bundle was not consistantly positioned, being at the postero-caudal, rim of VSD in 4 and antero-cephalad in 4. There have been no hospital deaths in the last 16 operations. Thus correction provides good results, but leaves the ventricles in an inverted relationship functionally as well as anatomically.

*By invitation