TUESDAY MORNING, MAY 1,1979

8:30 A.M. Scientific Session - Ballroom

15. Management of Symptomatic Tetralogy of Fallot in the First Year of Life

WILLIAM Y. TUCKER*, KEVIN TURLEY*, DANIEL J. ULLYOT,

and PAUL A. EBERT, San Franciso, California

The surgical management of tetralogy of Fallot (TOP) in infants is debatable. The questions of total correction versus palliation, and the type of palliative procedure, remain controversial.

During the past 3½years, 28 infants, ages 12 months or less, with symptomatic TOP underwent either total correction (21 infants) or palliation by relieving the pulmonary stenosis with a right ventricular outflow tract (RVOT) patch (7 infants). The age range of these infants was two days to 12 months, with a mean of 5.8 months. There were four hospital deaths in the entire group (mortality 14.3%), with three deaths occurring in the total correction group (14.3%) and one in the palliation group (14.3%).

The right pulmonary artery diameter to ascending aortic diameter ratio (PA:AO ratio) was measured on the anteroposterior cineangeo-gram and calculated for all patients. Nineteen of 21 patients in the total correction group had PA:AO ratios of greater than 1/3. All patients in the palliation group had PA:AO ratios of less than 1/3 and six of seven infants had PA:AO ratios of less than 1/4. The two patients in the total correction group with PA:AP ratios of less than 1/3 accounted for two of the three operative deaths in that group.

Three of four patients receiving palliation with a RVOT patch who have been recatheterized have shown an increase in their PA:AO ratio from less than 1/4 to greater than 1/3.

If the PA:AO ratio is greater than 1/3, total correction can be undertaken with good functional results and low mortality irrespective of age. Infants with PA: AO ratios of less than 1/3 should be palliated. We prefer palliation by relieving pulmonary stenosis with a RVOT patch whenever possible as this allows for growth of the main and branch pulmonary arteries.

*By invitation

16. Repair of Double Outlet Right Ventricle

ROBERT W. STEWART*, JOHN W. KIRKLIN,

ALBERT D.. PACIFICO, EUGENE H. BLACKSTONE*, and

LIONEL M. BARGERON, JR. *, Birmingham, Alabama

We have reviewed the 61 patients(pts) with double outlet right ventricle (DORV) and concordant atrioventricular connection, repaired between 1967 and July 1978, to define the determinants of the high hospital mortality (21 deaths, 34%, 70% CL 28%-42%) and ways of reducing it. 2(12%, CL 4%-27%) deaths occurred in the 16 relatively uncomplicated cases (2/14 in pts with subaortic or doubly committed VSD and intraventricular repair and 0/2 in pts with Lincoln-Danielson type DORV). One death was from faulty patch geometry and 1 from postoperative error. Age at repair was not a determinant of risk, nor was presence or absence of pulmonary stenosis (p for difference = 0.7). Surgical enlargement of the VSD as part of the repair did not increase risk (p = 0.9). However, previous surgery was an incremental risk factor (14 deaths among 30 secondary repairs, 47%, vs. 7 of 31 with primary repair 23%, p < 0.05), as was use of a transannular patch or non-valved conduit in patients with pulmonary stenosis (5 deaths in 6 pts, 83%) vs. a valved external conduit (1 of 8, 12%) (p = 0.01). When the VSD was non-committed (A-V canal type 2, muscular 2), risk was high with 2(50%, CL 18%-82%) deaths after repair which included intraventricular tunneling and a valved external conduit. 2(50%) of 4 pts with DORV and complete A-V canal died. The Taussig-Bing type of DOR V is a very different malformation. 1 patient in this group, who died, had complete A-V canal, and 24 subpulmonary VSD. Of these, one patient, who survived, had only intraventricular rerouting, 2(20%, CL 7%41%) died among 10 in whom the Rastelli repair was used, as did 6(46%, CL 30%-64%) of 13 in whom the VSD was closed and a Mustard repair done (p = 0.3). Late postoperative results were good except in the Taussig-Bing DORV group, in which 5 of the 6 hospital survivors with the Mustard repair died late postoperatively as did 3 of the 8 with valved external conduits (p=0.3). We conclude that all types of DORV can be repaired, that the first operation should be a complete repair, that a completely intraventricular repair with a precisely tailored tunneling patch (described in the report) should be done whenever possible, that when necessary a valved external conduit should be used rather than transannular patching, that the VSD should be enlarged anteriorly when necessary for the repair, and that the Taussig-Bing type of DORV should usually be treated by a completely intraventricular repair, or the Rastelli technique.

*By invitation

17. Surgical Technique to Reduce the Risk of Complete Heart Block Following Qosure of Ventricular Septal Defect in Atrioventricular Discordance

MARC R. DELEVAL*†, PEDRO BASTOS*, JAROSLAV STARK*,

JAMES F. N. TAYLOR *, ROBERT H. ANDERSON*, and

FERGUS J. McCARTNEY*, London, England

Sponsored by John W. Kirklin, Birmingham, Alabama

Complete heart block (CHB) remains a complication of closure of ventricular septal defects (VSD) in patients (pts) with atrioventricular discordance (AVD). The location of the A-V node and bundle, the presence of a conduction tissue sling in some pts and the position of the bundle and its bifurcation on the morphologic left side all contribute to surgical CHB. To reduce the risks of injury to the bundle and its bifurcation, a technique of placement of the VSD patch on the morphologic right side without opening the systemic ventricle was used in 12 consecutive pts operated since 1975. Ages ranged from 5 months to 15 years (mean 12.1 years). All had AVD and VSD. Associated anomalies included: situs inversus (2 pts), dextrocardia (3 pts), ventriculo-arterial discordance (7 pts), double outlet right ventricle (4 pts), pulmonary stenosis (9 pts), pulmonary atresia (1 pt), pulmonary venous return anomalies (2 pts), tricuspid valve regurgitation (2 pts) and subaortic stenosis (1 pt). The VSD was closed through the mitral valve or the left ventricle in 11 pts. All stitches were placed through the VSD on the morphologic right side of the septum using continuous or interrupted sutures. In 1 pt with AVD and ventriculo-arterial concordance, the patch was sewn through a right ventriculotomy. Associated anomalies were dealt with appropriately, including the insertion of an external valved conduit in 10 pts. 2 pts were in AV dissociation before cardi-otomy, of which 1 died on the table and 1 other remained in AV dissociation with a ventricular rate > 100/min. 10 pts were in sinus rhythm at completion of the repair and remained in continuous sinus rhythm, except for one transient episode of CHB in 1 pt and one episode of nodal rhythm on 24-hour monitoring in another pt. There were 2 early and 2 late non-arrhythmic deaths. Postoperative electrocardio-graphic features of morphologic right bundle branch block were present in 6 pts and none showed evidence of left bundle branch block. Thus, the technique has produced no serious conduction disturbances.

†Dr. de Leval was the 24th EVARTS A. GRAHAM MEMORIAL TRAVELING FELLOW (1973-74).

*By invitation

18. Modified Fontan Operation for Univentricular Heart and Complicated Congenital Lesions

ALAN W. GALE*†, GORDON K. DANIELSON,

DWIGHT C. McGOON, ROBERT S. WALLACE and

DOUGLAS D. MAIR *, Rochester, Minnesota

Since May 1974, 15 patients (pts) have undergone right atrio-pulmonary shunts combined with closure of artrial septal defect and patch closure of the right atrioventricular (A-V) valve for congenital cardiac anomalies other than tricuspid atresia. Eleven pts had a univentricalar heart (type AIII, 7 pts; type All, 2 pts; type AI, 1 pt; type C, 1 pt). One pt had corrected transposition, pulmonary stenosis and a straddling A-V valve and another had double outlet right ventricle with non-committed ventricular septal defect and pulmonary stenosis. Two pts had dextrocardia, inverted ventricles, ventricular septal defect, pulmonary stenosis, and transposition of the great arteries, one with straddling A-V valve and one with common A-V orifice. One pt had tricuspid valve hypoplasia. The atrial septal defect was closed by suture in 8 pts early in the series but subsequently was closed with a patch.

There were 3 early deaths, all in the univentricular heart group (the first 2 pts in 1974 and 1975 and one in 1978). Early complications of fluid retention and renal failure requiring dialysis occurred in 2 pts. Pleural effusions were uniform. Complete heart block occurred in 3 pts (2 permanent, one transient).

On follow-up, dehiscence of the patch closure of the tricuspid orifice occurred in one pt who underwent successful reoperation. One pt with dextrocardia died suddenly at home of unknown cause. The remaining pts are improved, compared to their preoperative status.

The modified Fontan procedure offers an alternative palliative operation which may be applied to those patients with severe pulmonary stenosis and normal pulmonary artery pressure and size. This procedure is especially appealing for those patients in whom septation or complete repair cannot be performed or is too risky. The long-term effects of systemic venous hypertension require further elucidation.

†Dr. Gale was the 28th EVARTS A. GRAHAM MEMORIAL TRAVELING FELLOW (1977-78).

INTERMISSION - VISIT EXHIBITS

*By invitation

19. Pulmonary Atresia with Intact Ventricular Septum - A 16 Year Experience

ANTHONY L. MOULTON*, FREDERICK O. BOWMAN, JR.,

RICHARD N. EDIE, CONSTANCE HAYES*, KENT ELLIS*

and JAMES R. MALM, New York, New York

Infants with pulmonary atresia and intact ventricular septum (PA & IVS) usually require urgent surgical intervention. 28 of 32 patients with this anomaly seen at the Columbia-Presbyterian Medical Center between 1962 and 1978 had palliative operations within the first three days of life. 6 underwent a closed pulmonary valvotomy alone without a survivor; 5 had only a systemic to pulmonary artery shunt with 3 survivors. Based on this experience, 17 had a combined procedure of valvotomy and shunt with 14 survivors. 3 patients died before operation and 1 recently had a definitive right ventricular outflow patch using cardiopulmonary bypass.

Eight patients subsequently have had corrective open heart procedures with 5 long term survivors ranging from 2 to 10 years. Repair employed an aortic homograft unicusp in 5 and a Hancock valve conduit in 3. Five patients are presently awaiting surgery.

We conclude that the initial surgical management of these critically ill infants must not only increase pulmonary blood flow but in addition provide an opportunity for right ventricular growth. Thus we continue to advocate the combined procedure of a valvotomy plus a shunt to provide adequate palliation and allow for definitive correction at a later date.

*By invitation

20. Technique, Indications and Clinical Use of 24-Hour Esophageal pH Monitoring

TOM R. DeMEESTER, CHING-I WANG*, JORGE A. WERNLY*,

CARLOS A. PELLEGRINI*, ALEXANDER G. LITTLE*,

LAWRENCE F. JOHNSON* and DAVID B. SKINNER,

Chicago, Illinois

Dissatisfaction with symptomatology, radiology, esophageal manometry, and intraesophageal pH testing to diagnose gastroesophageal reflux, led us to develop continuous 24-hour esophageal pH monitoring as an objective test for reflux. Over the past six years, 393 patients have undergone 24-hour pH monitoringin addition to endoscopy, manometry, and standard acid reflux test. The patients were divided into eight different groups based on their presenting symptoms and indications for 24-hour pH monitoring. 1) 105 patients had typical reflux symptoms with endoscopic evidence of esophagitis (85) or stricture (20), and were candidates for anti-reflux surgery due to failure of medical therapy. The test confirmed abnormal reflux in 77 to 85 patients, without stricture, and indicated those most at risk for developing stricture (58), and those likely to develop the post-operative gas bloat syndrome (4). 15 patients had abnormal reflux only in the supine position. The 8 patients with a normal test had either a borderline test result or another etiology for esophagitis. 2) 94 patients had typical reflux symptoms in the absence of endoscopic esophagitis. Abnormal reflux was confirmed by 24-hour pH monitoring in 53 patients. In the remaining patients, other causes for symptoms were subsequently identified. 3) 18 patients had a variety of respiratory symptoms; in 11 the respiratory symptoms were proven to be secondary to occult reflux by 24-hour pH monitoring. 4) 13 patients had atypical chest or abdominal pain; in 6 the pain was proven to be secondary to abnormal reflux by 24-hour pH monitoring. 5) 48 patients with other diseases of the chest and abdomen also had symptoms suggestive of reflux. In 27, the presence of abnormal reflux was detected by 24-hour pH monitoring and directed appropriate therapy. 6) 31 children, who were unable to communicate their symptoms, and who had a history of recurrent pneumonia, asthma, repeated vomiting, apnea, or failure to thrive were tested for the presence of reflux. In 21, abnormal reflux was documented as the cause of their symptoms by 24-hour pH monitoring and indicated anti-reflux surgery. 7) 28 patients had a motility disorder, 13 of whom had untreated achalasia. Abnormal reflux was recognized by 24-hour pH monitoring as a cause for, or resulting from, the motility disorder in 17 patients. 8) 56 patients had recurrent reflux symptoms after esophageal surgery. Abnormal reflux was demonstrated by 24-hour pH monitoring in 10 of 16 patients who had a previous myotomy for achalasia. 46 patients had a previous hiatal hernia repair and recurrent reflux was documented by 24-hour pH monitoring in 25.24-hour pH monitoring had a higher sensitivity and specificity than the standard acid reflux test or a DBS pressure less than 10 mm. of Hg. for the diagnosis of abnormal reflux. Based on this experience, 24-hour pH monitoring has emerged as a clinically useful technique for the evaluation of esophageal pathology, and significantly contributes to the clinical management of patients by selecting those who will benefit from anti-reflux surgery.

*By Invitation

21. Realistic Expectations Following Aggressive Surgical Treatment of Acute and Chronic Aortic Dissections - Experience with 111 Patients over a 14 Year Period

D. CRAIG MILLER*, EDWARD B. STINSON*,

PHILIPS. OYER*, STEPHEN J. ROSSITER*, BRUCE A. REITZ*,

RANDALL B. GRIEPP*, and NORMAN E. SHUMWAY,

Stanford, California

Current therapy of aortic dissections remains unstandardized due to the rarity of these catastrophic events and the lack of prospective trials. This data-bank study defined the long-term results (mean follow-up = 5 years(YR), maximum = 14 YR, cumulative = 318 PT-YR) of surgical treatment of 111 unselected patients (PTS). Forty-eight PTS were classified acute Type A(AC-A), 26 were chronic Type A(CH-A), 18 were acute Type B(AC-B), and 19 were chronic Type B(CH-B). Mean age was 57 YR. Regardless of moribund status or age, no acute PTS were denied surgery. Aortic valve replacement (AYR) was performed in 10% of the AC-A PTS and 35% of the CH-A PTS. The intimal tear was resected in 68% of PTS. Reoperation rate was 3.8%/PT-YR; redis-section rate was 0.3%/PT-YR. No PTS were lost to follow-up.

Operative mortality(OP MORT) and 8 YR actuarial survival(± SEM) were 38% and 33 ± 10% for AC-A, 15% and 49 ± 12% for CH-A, 50% and 40 ± 13% for AC-B, and 21% and 43 ± 14% for CH-B PTS. OP MORT was statistically(p < 0.05) greater for those ACUTE PTS with new paraplegia, AC-B PTS who had failed medical therapy, and several other selected PT subsets. Twenty-nine percent of the hospital deaths and 69% of the late deaths were cardiovascular or cerebrovascular in nature. Whether AYR had been performed or the intimal tear had been resected did not significantly(p >0.05) correlate with OP MORT, late functional result, late reoperation, or late mortality. No redissections occurred among Type A PTS in the preserved rim of supra-anular aorta. Eight year actuarial survival probability for those AC-A PTS discharged from the hospital was 53 ± 15%; for CH-A, 58 ± 14%; for AC-B, 80 ± 18%; and CH-B, 54 ± 16%. Although sustaining slightly higher OP MORT, the AC-B PTS experienced the least late attrition.

No medical or surgical study heretofore has included follow-up of this duration and magnitude with which to define the postoperative "natural" history of aortic dissections. These results should be interpreted as unduly pessimistic due to the totally unselected nature of the study population and lower contemporary OP MORT. These long-term results support a philosophy of early emergency surgery for both AC-A and AC-B PTS. Furthermore, we continue to urge adaption of this simplified functional classification system(based on involvement of the ascending aorta irrespective of the site of tear) since the biological behavior of aortic dissections is primarily predicated on this concept.

*By invitation

22. Treatment of Transverse Aortic Arch Aneurysm

E. STANLEY CRAWFORD, SALWA A. SALEH*,

JOHNS. SCHUESSLER*, and RAUL GARCIA-RINALDI*,

Houston, Texas

Surgical treatment of aneurysms involving the transverse segment of aortic arch is difficult and associated with high mortality and morbidity. The latter is primarily related to techniques of cerebral protection employed during the period of aortic branch occlusion needed for reconstruction. Recent reports suggest the superiority of deep hypothermia and circulatory arrest; however, the mortality reported varies from 16-25%. This report is concerned with treatment of 25 patients with arch lesions in whom cerebral perfusion was selected based upon the extent and location of aneurysm. In 11, the lesion involved the distal arch and descending aorta requiring replacement of the subclavian artery. Cerebral perfusion techniques were not employed in these cases even though the left common carotid artery was temporarily clamped in most cases. All patients survived without neurologic problems. The aneurysm involved two or all three vessels in 13. Cerebral perfusion was maintained by dacron shunts in 4 of these patients in whom the proximal ascending aorta was not involved and normothermic cardiopulmonary bypass techniques in 9 patients with total involvement of the ascending aorta. Death occurred in one of the former from graft disruption and one of the latter from cerebral damage. The perfusion technique in the latter patient was that employed early in the experience when high pulsatile flow rates (250-350cc per minute per vessel) were used. Low flow rates (60-75cc per vessel) with low pulse amplitude were employed in the last 5 patients. All survived without complication, suggesting this to be a very satisfactory method of cerebral protection and complications associated with deep hypothermia and circulatory arrest are avoided.

11:15 Address of Honored Speaker

CARDIOTHORACIC SURGERY IN THE ANTIPODES

Sir Brian Barratt-Boyes Auckland, New Zealand

*By invitation