TUESDAY AFTERNOON, MAY 4, 1982

2:00 p.m. Scientific Session - Assembly Hall

25. Pulmonary Outflow Tract Reconstruction Without Prosthetic Conduit

YVES LECOMPTE*, JEAN-YVES NEVEUX*,

FRANCINE LECA*, LUCIO ZANNINI*

and YVETTE DUBOYS*

Paris, France

Sponsored by: ALBERT STARR, Portland, Oregon

The use of prosthetic conduits in the reconstruction of the pulmonary outflow tract in infants or small children is a palliative procedure associated with well known drawbacks.

Our previously reported experience of 9 cases of anatomical correction of transposition of the great arteries (T.G.A.) by positioning the pulmonary bifurcation anterior to the ascending aorta stimulated us to extend this concept to correct other complex congenital anomalies: T.G.A. with ventricular septal defect (V.S.D.) and pulmonary stenosis (P.S.), truncus arteriosus, and pulmonary atresia with V.S.D.

In T.G.A. with V.S.D. and P.S., the technique comprised the resection of the conal seplum, the suturing of an interventricular patch establishing continuity between the left ventricle and the aorta, and the direct implantation of the pulmonary artery on the right ventricle after positioning the pulmonary bifurcation anterior to the aorta. This technique was feasible even in small infants and cases of small V.S.D. Eleven patients, from four months to six years of age were corrected using this technique, with four deaths and seven good short term results (3 months to 1 year).

In truncus arlcriosus, anatomical correction was achieved by direct implantation of the pulmonary artery on the right ventricle after positioning the pulmonary bifurcation anterior 10 the ascending aorta. 4 children aged from 4 months to 5 years were operated upon using this technique with 2 deaths probably due to severe pulmonary regurgitation, a complication which should be prevented in future cases by systematic implantation of a mono-cusp valve.

In pulmonary atresia with V.S.D. and absent pulmonary trunk, the continuity between the right ventricle and the pulmonary branches was established by using an arterial tube resected from the ascending aorta. This technique was successfully used in one child with extremely small pulmonary branches.

These preliminary results led us to conclude that many complex congenital cardiac anomalies can be effectively treated without prosthetic conduit.

*By invitation

26. Use of Sub-Pulmonary "Ventricular" Chamber in the Fontan Operation

MARC ROGER DE LEVAL*, CATHERINE BULL*,

JAROSLAV STARK* and FERGUS MACARTNEY*

London, England

Sponsored by: DWIGHT C. McGOON, Rochester, Minnesota

In order to assess the value of incorporation of a sub-pulmonary "ventricular" chamber into a Fontan type repair, the haemodynamics of 13 patients (pts) with this type of repair were compared with 17 pts who had an atrio-pulmonary connection. The methods included analysis of pressure tracings (all pts) and relation of stroke work to filling pressures at completion of the repair (9 pts) detection of valve closure and opening by two-dimensional and pulsed Doppler echocardiography (10 pts) and postoperative cardiac catheterization (8 pts). Immediately post-operatively, mean right atrial (RA pressure was equal to or higher than mean pulmonary artery (PA) pressure in all pts, regardless of the incorporation of a sub-pulmonary "ventricular" chamber. The pressure difference increased as filling pressure was increased. Thus while the Starling curve for the left ventricle had a positive slope, that for the right heart had a negative one. There was an ‘a' wave in the PA pressure trace indicating an atrial dependent circulation in all pts but one. The exception was in a pt with an atrio-ventricular conduit but residual pulmonary stenosis who died in low cardiac output. In all pts with an atrio-ventricular connection, ventricular systole opened the pulmonary valve, produced a V wave in the PA pressure, and closed the conduit valve. By contrast, the conduit valve was observed to close in only one patient with an atrio-pulmonary conduit.

In one pt with an atrio-ventricular connection re-studied a year after repair, the RA pressure was normal and lower than PA pressure. The ‘a' wave in his PA pressure had disappeared indicating a ventricular dependent circulation, resulting from ventricular growth.

We conclude that immediately post-operatively the right heart is functioning more as a conduit for a circulation mainly driven by the left ventricle than as a pumping chamber assisting it. The main benefit of an atrio-ventricular connection is that it introduces a functioning conduit valve into the right heart though this benefit is outweighed if its insertion results in an obstructed pathway from RA to PA and compromises the necessary RA contribution to pulmonary blood flow. Post-operative right ventricular growth can convert an atrial dependent circulation into a ‘normal' ventricular dependent one.

*By invitation

27. Repair of Tricuspid Atresia in 100 Patients

FRANCIS M. FONTAN, CLAUDE DEVILLE*,

JAN QUAEGEBEUR*, JAAP OTTENKAMP*,

ALAIN CHOUSSAT* and GERARD A. BROM

Bordeaux, France and Leiden, The Netherlands

From April 1968 to September 1981, 100 patients (pts) had surgical repair of tricuspid atresia (T.A.), at a mean age of 9 years 5 months (extreme 20 months to 36 years). 72 procedures of palliative surgery had been previously performed in 62 pts. In pts with ventriculo-arterial concordance (VAC), a non valved Dacron conduit or an aortic valve homograft (AVH) was interposed between right atrium (R.A.) and right ventricular outlet chamber; in 32 pts with ventriculo-arterial discordance (VAD) AVH established the continuity between R.A. and pulmonary arteries (P.A.); in addition to closure of atrial septal defect in all pts, and of ventricular septal defect when necessary, in VAC, other procedures were performed occasionally: pulmonary valve commissurotomy (2 pts), P.A. trunk enlargement (2 pts) mitral valve replacement (1 pt), inferior vena cava valvulation with A.V.H. (8 pts). The hospital mortality for the entire group was 12%, 7% in VAC (5 pts), 21.8% in VAD (7 pts), mainly due to P.A. hypertension (5 pts); from 1974 to 1981, hospital mortality was strikingly reduced in 92 pts (8 deaths, 8.7%, CL 6%-13%). There were 5 late deaths due to septicemia (1), heart failure (2), sudden death (1), reoperation for residual shunt (1). Reoperation was performed in 10 pts for residual shunts (6) dacron conduit obstruction (1) heart failure (2) chylothorax (1) with 2 deaths; there was no reoperation in the last 33 pts. Actuarial survival rate at 10 years is 85%. Late results are good in the majority of the pts. Control cardiac catheterization was performed in 46 pts from 1 month to 6 years postoperatively: mean R.A. pressure was 16 ± 4 mm Hg, no significant systolic pressure gradient was found between R.A. and P.A.; in pts with AVH between R.A. and outlet chamber, peak systolic pressure were higher in outlet chamber and P.A. than in R.A.; arterial oxygen saturation was 92.2% ± 5, mean left ventricular ejection fraction was 62%; enlargement of outlet chamber was noted in VAC. Exercise tests were performed in 20 pts; the performance was between 60% and 100% of normal in 13 pts, less than 50% in 2, and better in pts with AVH conduit. This review suggests that current immediate and long term results are satisfactory and permits to consider with confidence surgical repair of T.A.

3:00 p.m. Intermission - Visit Exhibits

*By invitation

3:45 p.m. Scientific Session - Assembly Hall

28. Repair of A-V Canal Malformation in the 1st Year of Life

HARVEY W. BENDER, JOHN W. MAMMON*,

STEVE G. HUBBARD*, JAN MUIRHEAD*

and THOMAS P. GRAHAM*

Nashville, Tennessee

Disappointing results with pulmonary artery banding and subsequent correction led to the decision in 1977 that all infants presenting to our hospital with A-V canal and evidence of severe heart failure, lack of growth, or of pulmonary hypertension should receive early operative correction. Since that time, twenty-five consecutive infants have been operated. All had refractory heart failure. Average age at operation was 18 (3-38) weeks and average weight was 4.4 (2.3-8.8) kg. Only two patients were greater than six months of age at operation. Preoperative peak pulmonary artery pressure was 81 ± 3.3 mm Hg which was equal to systemic arterial pressure in all cases. Mean pulmonary-systemic resistance ratio was .28 ± .05. Fifteen patients had greater than mild mitral regurgitation and five had a patent ductus arteriosus. Three had significant associated malformations. Profound hypothermia and circulatory arrest was utilized in all patients. Common atrioventricular valve tissue was divided and valvular integrity was insured by resuspension to a single dacron patch which closed both the atrial and ventricular defects. Operative mortality occurred in two patients (8%) both with associated defects (1-TAPVC, 1-coarctation). One patient required a permanent pacemaker for surgical heart block. Late mortality occurred in one patient with associated PAPVC, and one patient has had a pacemaker implanted. Survivors have been followed for an average 23 (7-47) months. All patients have returned to normal growth and development postoperatively. All cardiac medications have been discontinued in 14 of 22 patients.

Operative repair of complete A-V canal can be performed in infancy with low operative and late mortality and will relieve signs and symptoms of heart failure and allow infants to experience more normal growth and development. On the basis of this experience, it appears to be unnecessary to delay operative correction with the known increased risk of the development of pulmonary hypertension.

*By invitation

29. The A-V Canal Defects: Relation of Morphology, Function, and Surgical Technique To Early and Late Results

MARKUS R. STUDER*, EUGENE H. BLACKSTONE*,

JOHN W. KIRKLIN, ALBERTO. PACIFICO,

BENINGO SOTO*, GEORGE K. T. CHUNG*

and LIONEL M. BARGERON, JR.*

Birmingham, Alabama

For the 271 patients with A-V canal defects operated upon by us between 1967-1981, and while blinded to knowledge of the patient outcome, we have re-analyzed morphology with a modification of Anderson's concepts, applied this and an evaluation of ventricular dominance and left-sided A-V valve function to re-analysis of the cineangiograms, and related these and the evolving surgical technique to in-hospital death (47 patients, 17%) and followup results (15 late deaths, actuarial survival of hospital survivors 95.3"% at 12 months and 91% at 10 years; 16 reoperations, actuarial incidence 95% at 12 months, 92% at 10 years; and a minimum of 23 patients, 9%, with important P.O. left A-V valve incompetence; categories not mutually exclusive). Heart block developed after repair in 6 patients (2.2%) of whom 2 had no interventricular communication and had left A-V valve replacement and one had multiple VSD's. Variables relating to results are in the table.

Continuously evolving surgical technique (altered suture siting to more securely avoid A-V node and His bundle; "3-leaflet" concept of the left A-V valve; 2 patches when VC present, sandwiching A-V valve between them; functional valve analysis at operation and addition of annuloplasty) have decreased hospital mortality with time (p<0.05), decreased the incidence of valve dehiscence (p<0.05), but have not decreased the overall incidence of important postoperative LAVI in patients with important preoperative LAVI (the latter present in 21, 15%, of 143 patients without VC and in 27, 22%,of 125 with VC).

*By invitation

30. Double Outlet Right Ventricle - Surgical Results 1970-1980

JOHN P. JUDSON*, GORDON K. DANIELSON,

FRANCISCO J. PUGA * and DWIGHT C. McGOON

Lubbock, Texas and Rochester, Minnesota

Between January 1, 1970, and January 1, 1980, 62 consecutive patients who underwent repair of double outlet right ventricle were reviewed. Patients with subpulmonary ventricular septal defect (Taussig-Bing complex), complete atrioventricular canal, atrioventricular discordance, and univentricular heart were excluded. Associated defects in 54 patients included restrictive ventricular septal defect, multiple ventricular septal defects, two-chambered right ventricle, pulmonary atresia, atrial septal defect, coronary artery anomalies, bilateral superior venae cavae, juxtaposed atrial appendages, patent ductus arteriosus, dextrocardia, right aortic arch, subaortic stenosis, tricuspid regurgitation, and stradding of the mitral valve. There were 46 patients with pulmonary stenosis and 16 patients without pulmonary stenosis; of these, 36 were male and 26 female. Age at operation ranged from 8 months to 37 years (mean 9.0 years). The operative mortality was 5 (11%) in cases with pulmonary stenosis, 4 (25%) in cases without pulmonary stenosis, and 9 (14.5%) overall. Mortality was often related to associated anomalies. Causes of death included low cardiac output, infection, pulmonary hypertension, and technical problems (2 patients each), and right heart failure (1 patient). During the follow-up period of 6 months to 8 years, the late mortality was 17% in cases with pulmonary stenosis, 19% in cases without pulmonary stenosis, and 18% overall. Two late deaths were of noncardiac causes, but the remainder occurred 2 months to 6'/2 years postoperatively from arrhythmias. Late results in the majority of survivors are good to excellent. While the operative mortality for double outlet right ventricle continues to decrease, the late mortality is of concern. The problem of late arrhythmias requires further study and analysis.

*By invitation

31. Surgery For Congenital Valvular Aortic Stenosis: A Twenty-Three Year Experience

JAY L. ANKENEY, THOMAS S. TZENG*

and JEROME LIEBMAN*

Cleveland, Ohio

From 1958 through 1980, 70 consecutive patients, 2 to 20 years of age, were operated upon for congenital valvular aortic stenosis. The primary indication for surgery was development of symptoms in 53 patients - decreased exercise tolerance (22), chest pain (21), syncope (5) and SOB )5), ST and T abnormality in the electrocardiogram (ECG) in 13 and progressive LVH in 4. Pre-operative catheterization in 57 patients, in which the left ventricle was entered, showed an average left ventricular-aortic (LV-AO) systolic gradient of 85.3 mm. Hg. (± 36.3 S.D.). Only 9 patients had a gradient less than 50 mm. Hg.

Valvulotomy was performed in all patients, resulting in a bicuspid valve in 36 and a tricuspid valve in 33. In the 3-leaflet group, vestigial commissures were opened in 17 and a third leaflet was created in 16 by incising a common leaflet and using an everting suture near the ring to prevent aortic regurgitation. Twelve patients had insignificant diastolic murmurs in the immediate postoperative period. There were 2 operative deaths (2.8%); one due to technical failure of the valvulotomy, necessitating attempted valve insertion, and the other after sudden cardiac arrest on the third postoperative day.

All 68 surviving patients have been followed from 1 to 23 years (mean 10.5). Postoperative catheterization in 38 patients showed an average LV-AO systolic gradient of 38.7 mm. Hg. (± 27.2 S.D.). The patients' postoperative ECG showed improvement in 39 and deterioration in 7. There were 4 late deaths: one was a consequence of SEE and 3 were non-cardiac in origin - drowning, car accident, and neuroblastoma. Eleven patients underwent reoperation 2 to 22 years postoperatively (mean 10.5). Indications for re-operation were recurrent stenosis in 9, dissecting aneurysm in 1, and aortic regurgitation in 1. In 4, a second valvulotomy was done, and in 7 the valve was replaced. There were 2 operative deaths, both occurring in patients undergoing emergency reoperation: one for cardiogenic shock, and the other for acute dissecting aorta aneurysm. Another died suddenly 9years after the second operation. Of the 53 patients surviving the initial valvulotomy without requiring reoperation 49 are Class I and 4 are Class II. Only 2 patients receive digitalis; one for mild aortic regurgitation and the other for supraventricular arrhythmia.

Our experience indicates that aortic valvulotmy is an effective, palliative operation for congenital valvular aortic stenosis in children, and valve replacement, if necessary, can be delayed for many years.

4:45 p.m. Executive Session - Assembly Hall

7:00 p.m. President's Reception - Heard Museum

*By invitation