Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction.

OBJECTIVE: Assessment of the Lecompte procedure, our repair method of choice in malposition of the great arteries with pulmonary stenosis. METHODS: A retrospective analysis of 42 patients (median age at operation, 1.4 years) operated on between 1986 and 1999 for various forms of great artery malposition, ventricular septal defect, and pulmonary stenosis. Relevant associated findings included the insertion of a tricuspid papillary muscle on the conal septum (nine patients), absence of conal septum (six patients), hypoplasia of a side pulmonary artery (four patients), and hypoplasia of the right ventricle (one patient). A preliminary systemico-pulmonary shunt was created in 28 patients and a cavo-pulmonary anastomosis in one patient. At operation, the conal septum (whenever present) was resected (36 patients), the pulmonary bifurcation was usually translocated over the ascending aorta (37 patients), and the main pulmonary artery was enlarged with a patch of pericardium. A monocusp valve was fashioned within the patch in 40 patients. The follow-up information was complete in 32 patients and ranged from 0.4 to 14 years (mean, 5.4+/-3.2 years). RESULTS: The survival rate at 5 years was 92+/-5%. Three patients died post-operatively (mortality, 7%) and none during follow-up. The freedom from reoperation was 86+/-8 and 51+/-22% at 5 and 10 years, respectively. Six patients were reoperated, all for a pulmonary stenosis. Calcification of the monocusp patch was present in all. Pulmonary stenosis developed in three further patients: one underwent percutaneous dilatation and two are awaiting surgery. No procedural factors thought to have a potential correlation with the development of right ventricular outflow tract stenosis could be individualized on univariate analysis. CONCLUSIONS: The Lecompte procedure, which allows early repair of these defects, provides overall good results. The need to reoperate on the right or left ventricular outflow tract seems reduced in comparison with the Rastelli operation, the other alternative.

Pedicled pericardium for repair of right ventricular outflow tract and pulmonary arterial stenoses in tetralogy of Fallot: a six-year experience.

From June 1988 through June 1994, 20 children with symptomatic tetralogy of Fallot, associated with hypoplastic or stenotic pulmonary arteries in 19 cases, were operated on in our institution. Mean age at operation was 49.5 +/- 43 months (ranging from 10 months to 12.5 years). Mean weight was 13.5 +/- 6.5 kg (range 6.2 to 30 kg) and mean body surface area was 0.6 +/- 0.2 m2. Mean preoperative hematocrit value was 47.6% +/- 11.1%, and mean preoperative arterial oxygen saturation ratio was 75.7 +/- 9.5. Six patients (30%) had prior systemic-pulmonary arterial shunts. Pulmonary arterial stenoses were congenital or iatrogenic (or both) and were located principally on the left pulmonary artery (63%), on the pulmonary bifurcation (15%), or separately on the left and right pulmonary arteries (21%). In all children the pulmonary arterial tree was repaired with the patient's own pedicled pericardium. Mean follow-up is 36 +/- 25.2 months (range 1 to 71 months). Hospital mortality rate was 0%. There was one late death, and two children were lost to long-term follow-up. No child required reoperation. Seventeen children returned for evaluation, consisting of physical examination, electrocardiogram, chest roentgenogram, and Doppler echocardiogram, and 11 underwent late catheterization or magnetic resonance imaging of the chest to evaluate the pulmonary arterial repair. All children were in New York Heart Association class I or II. Right ventricular function was normal by echocardiography in 100% with a mean right ventricular systolic pressure of 39.37 +/- 8.4 mm Hg. Mild to moderate pulmonary regurgitation was present in the majority of patients. The results of pulmonary arterial repair were good in 100%. There was no residual stenosis, and we observed uniform enlargement of the repaired pulmonary arteries over a 5-month to 6-year follow-up period. These results are of particular interest inasmuch as other materials previously used for repair of pulmonary arteries do not grow and may even shrink, resulting in residual stenosis.

"Classic" repair of congenitally corrected transposition and ventricular septal defect.

BACKGROUND: This study examined the results of "classic" repair of congenitally corrected transposition of the great arteries and ventricular septal defect. METHODS: From 1974 to 1994, 52 patients underwent a classic complete repair of lesions associated with congenitally corrected transposition. They were divided into two groups: ventricular septal defect plus left ventricular outflow tract obstruction (group I, 37 patients) and isolated ventricular septal defect (group II, 15 patients). Tricuspid plasty or replacement was performed primarily in 1 patient of group I (3%) and in 8 patients of group II (53%). RESULTS: The overall operative mortality was 15% (8/52 patients), and the incidence of postoperative atrioventricular block was 27% (14/52 patients). Eight patients died secondarily, 5 of heart failure. Survival rates were 83% +/- 6% at 1 year and 55% +/- 14% at 10 years for group I and 86% +/- 9% at 1 year and 71% +/- 12% at 10 years for group II (not significant). Redo tricuspid plasty or replacement was performed in 12 patients. CONCLUSIONS: Results of classic complete repair of lesions associated with congenitally corrected transposition are not satisfactory in our experience because (1) the operative mortality and the incidences of tricuspid valve replacement and atrioventricular block are high and (2) secondary heart failure is frequent. However, a retrospective review of morphologic findings shows that "anatomic" complete repairs would not have been feasible in 6 of our patients.

Diffuse subaortic stenosis: modified Konno procedures with aortic valve preservation.

The Konno procedure provides adequate relief of diffuse subaortic stenosis but requires aortic valve replacement. This may be questionable in patients without aortic valve lesions. Eleven patients had diffuse subaortic stenosis and normal aortic orifice: tunnel subaortic stenosis (6 patients) or diffuse hypertrophic obstructive cardiomyopathy (5 patients). The mean age was 16 +/- 14 years (range: 3 months to 45 years). The mean subaortic gradient was 95 +/- 24 mmHg (range: 60 to 150 mmHg). Two patients had previously undergone resection of discrete subaortic stenosis. Modified Konno procedures were used: aortoseptal approach with aortic annulus division in 5 patients, conal enlargement without aortic annulus division in 6 patients. There was one early death (9%, 70% CL = 1%-27%) and no late deaths. Reoperation was required in two patients: one early for iatrogenic aortic regurgitation and one late for residual ventricular septal defect and mitral regurgitation. The mean follow-up was 3.8 +/- 4.2 years (range: 2 months to 10 years). The survivors were in functional class I (8/10), had a normal left ventricular function (9/10), were in sinus rhythm (10/10) and had left ventricular outflow tract gradients ranging up to 25 mmHg (mean = 6 +/- 10 mmHg). Residual surgery-related defects included ventricular septal defect (one) and aortic regurgitation (one). In patients with diffuse subaortic stenosis and normal aortic orifice, modified Konno procedures with aortic valve preservation are preferable, effective and can be safely performed in infants and children.

Anomalous left coronary artery from the pulmonary artery: results of isolated aortic reimplantation.

Thirty-one consecutive children with anomalous left coronary artery underwent direct aortic reimplantation of the anomalous artery without an associated procedure. There were five deaths (16%; 70% confidence limits, 9% to 26%), three in the hospital and two early (within 3 months). The severity of preoperative left ventricular dysfunction was the only incremental risk factor for mortality: 31% mortality rate among patients with left ventricular shortening fraction of less than 0.20 versus 0% among patients with a left ventricular shortening fraction of 0.20 or more (p = 0.03). There were no late deaths up to 6 years, a survival rate of 84% +/- 7%. Late results were studied in 23 survivors having a follow-up of longer than 12 months. Ninety-six percent were free of symptoms; left ventricular function recovered to normal in all patients; moderate to severe mitral regurgitation decreased to minimal or no regurgitation in most patients (5/7); and the reimplanted anomalous left coronary artery was patent in each patient. Based on this study, we reached five conclusions. (1) Direct aortic reimplantation is technically feasible in most patients with anomalous left coronary artery and yields a high rate of late patency. (2) Left ventricular resection is unnecessary. (3) The mitral valve should not be interfered with at the initial operation, but mitral regurgitation may persist in a few patients and necessitate later operation. (4) In patients with moderate left ventricular dysfunction, the operative risk is low and early operation indicated. (5) In patients with severe left ventricular dysfunction, the operative risk is high; heart transplantation may be suggested, but our current approach favors an immediate corrective procedure.

Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Rastelli or Lecompte procedure?

During a 10-year period (1980 to 1990), 62 patients underwent complete repair for transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction. Twenty-two patients (35%) (mean age 8.1 +/- 7.2 years) underwent the Rastelli operation: The ventricular septal defect was enlarged anteriorly in eight patients, and right ventricular-pulmonary artery continuity was established with an extracardiac valved (9/22) or nonvalved (13/22) conduit. Forty patients (65%) (mean age 3.3 +/- 3.2 years) underwent the Lecompte modifications: The conal septum was extensively excised when present (30/40), anterior translocation of the pulmonary bifurcation was performed in 32 patients, and right ventricular-pulmonary artery continuity was established by direct anastomosis without a prosthetic conduit. There were seven early deaths (11%; 70% confidence limits, 7% to 17%): two after the Rastelli procedure (9%; 70% confidence limits, 3% to 20%) and five after the Lecompte operation (12.5%; 70% confidence limits, 7% to 20%). Four patients were lost to follow-up, yielding a 93% complete follow-up (mean follow-up 55 months). There were two late deaths (one in each group). Actuarial probability of survival (+/- standard error) at 5 years was 83% +/- 9% after the Rastelli operation and 84% +/- 6% after the Lecompte procedure. All long-term survivors (except one in the Rastelli group) were in functional class I. Five patients in the Rastelli group underwent late reoperation for obstruction of the extracardiac conduit (28%; 70% confidence limits, 16% to 42%). Three late reoperations (10%; 70% confidence limits, 4% to 19%) were required after the Lecompte operation (one for residual ventricular septal defect and two for residual pulmonary outflow tract obstruction). At most recent examination, residual pulmonary outflow tract obstruction was present in seven patients of the Rastelli group (39%; 70% confidence limits, 26% to 53%) and in six patients of the Lecompte group (19%; 70% confidence limits, 12% to 29%). The combined likelihood of reoperation for pulmonary outflow tract obstruction and residual pulmonary outflow tract obstruction was significantly higher in the Rastelli group (67% versus 26%; p = 0.005). Both procedures provide satisfactory early and late results. The Lecompte operation allows complete repair in infancy, is feasible in patients with anatomic contraindications to the Rastelli operation, and may reduce the need for reoperation and the prevalence of residual pulmonary outflow tract obstruction.

[Tumors of the heart in newborn infants and infants. Apropos of 5 cases]. / Tumeurs du coeur du nouveau-né et du nourrisson. A propos de cinq cas.

Between February 1985 and March 1987, 5 children underwent resection of primary cardiac neoplasms, 3 of them in the first days of life and 2 before the age of 6 months. Routine echocardiographic follow-up of pregnancies allowed detection of cardiac tumors in 2 foetuses 30 and 36 weeks old. In 3 children the diagnosis was suspected by the discovery of cardiac murmur or congestive heart failure. The first case was a hemangioma, originating from outside the left ventricular wall, and was resected without cardiopulmonary bypass (CPBP). The other case was a pseudomyxoma, spreading extensively in to the right atrium. The third case was a rhabdomyoma arising from the pulmonary infundibulum with clinical manifestations of tuberous sclerosis. The last two patients had intraseptal lesions, just above the aortic valve; complete resection was therefore impossible, particularly in one patient with multiple tumors. There was one death related to congestive heart failure. The remaining four survivors were followed up for an average of 18.4 months (+/- 12.9) and all were in functional class I. Echocardiographic follow-up showed evidence of a residual subaortic lesion in one asymptomatic patient.

Modified Blalock-Taussig shunts: results in infants less than 3 months of age.

The optimal procedure for shunting palliation in cyanotic infants remains to be determined. Sixty-two infants less than 3 months of age underwent 63 modified Blalock-Taussig shunts. Their age range at operation was 1 to 84 days (mean, 16 +/- 20 days). Shunts were constructed using 5-mm polytetrafluorethylene tubes in 20 patients and 4-mm polytetrafluoroethylene grafts in 43 patients. There were 13 early deaths (21%; CL, 15% to 27%) of which three deaths (5%; confidence limits, 2% to 9%) were shunt related. The survivors were followed up from 6 to 53 months (mean, 29 +/- 12.5 months). Shunt failure (occlusion, inadequate palliation) occurred in 27 patients. The overall probability rate of adequate shunt function was 58% +/- 8% at 2 years. Univariate and multivariate analyses showed that the size of the graft was a risk factor of shunt failure. Severe distortion of the pulmonary arterial branch was noted in 12 patients. The inferences are: (1) modified Blalock-Taussig shunts provide satisfactory early palliation but late shunt failure is frequent; (2) similar results should be obtained with other shunting procedures; and (3) the optimal procedure should be selected for each cyanotic infant on an individual basis.

Primary definitive repair of interrupted aortic arch with ventricular septal defect.

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.

[Heart tumors in newborn infants and infants. Apropos of 5 cases]. / Tumeurs du coeur du nouveau-né et du nourrisson. A propos de cinq cas.

Between February 1985 and March 1987, 5 children underwent resection of primary cardiac neoplasms, 3 of them in the first days of life and 2 before the age of 6 months. Routine echocardiographic follow-up of pregnancies allowed detection of cardiac tumors in 2 foetuses 30 and 36 weeks old. In 3 children the diagnosis was suspected by the discovery of cardiac murmur or congestive heart failure. The first case was an hemangioma, originating from outside of the left ventricular wall, which could be resected without cardiopulmonary bypass (CPBP). The other case was a pseudomyxoma, spreading extensively inside the right atrium. The third case was a rhabdomyoma arising from the pulmonary infundibulum with clinical manifestations of tuberous sclerosis. The last two patients had intraseptal lesions, just above the aortic valve; complete resection was therefore impossible, particularly in one patient with multiple tumors. There was one death related to congestive heart failure. The remaining four survivors were followed up for an average of 18.4 months (+/- 12.9) and all were in functional class I. Echocardiographic follow-up showed evidence of a residual subaortic lesion in one asymptomatic patient.

Aortic coarctation with hypoplastic aortic arch. Results of extended end-to-end aortic arch anastomosis.

Between 1980 and 1986, 80 infants (less than or equal to 3 months old) with symptomatic aortic coarctation and associated severe tubular hypoplasia of the transverse aortic arch underwent surgical treatment. Extended end-to-end aortic arch anastomosis was used in an attempt to correct both the isthmic stenosis and the hypoplasia of the transverse arch. After complete excision of the coarctation tissue, a long incision was made in the inferior aspect of the aortic arch, which was then anastomosed to the obliquely trimmed distal aorta. Pure coarctation was present in 17 patients (group I); 24 infants had an additional ventricular septal defect (group II), and 39 patients had associated complex heart disease (group III). The overall early mortality rate was 26% (confidence limits 21% to 32%) (18% in group I, 17% in group II, and 36% in group III). The early risk declined with time and was 18% (confidence limits 12% to 26%) for the last 2 years (seven deaths in 39 patients). Follow-up was 100% for a mean of 19 months. Actuarial survival rate at 3 years was 82% for group I, 78% for group II, and 32% for group III. Recurrent coarctation (gradient greater than or equal to 20 mm Hg) occurred in six operative survivors (10%, confidence limits 6% to 16%) and necessitated reoperation in three. Freedom from recoarctation at 4 years was 88%. Because extended end-to-end aortic arch anastomosis provides adequate correction of the aortic obstruction and entails a low risk of restenosis, it is our procedure of choice in infants with coarctation and severe hypoplasia of the aortic arch.

Coarctation of the aorta in infants: which operation?

In this series, 178 infants (age, less than or equal to 3 months old) underwent repair of aortic coarctation. Pure coarctation was present in 63 patients (Group 1), 47 infants had additional ventricular septal defects (Group 2), and 68 patients had associated complex heart disease (Group 3). Subclavian flap angioplasty was used in 26 patients, limited resection and end-to-end anastomosis in 45 patients, extended resection and end-to-end anastomosis in 99 patients, and miscellaneous procedures in 8 infants. The early mortality was 8% for the first group, 11% for the second group, and 37% for the third group (p less than 0.001). Mean follow-up was 32 months and included 97% of patients. Actuarial survival at five years was 90% for the first group, 84% for the second group, and 40% for the third group. Recoarctation occurred in 15 operative survivors (11%); 7 necessitated reoperation. Freedom from recoarctation at five years was 89% after subclavian flap angioplasty, 81% after end-to-end anastomosis, and 86% following extended resection and end-to-end anastomosis. Early mortality and late results were not influenced by the type of coarctation repair but were determined by the clinical status and the presence of associated major cardiac anomalies. These results suggest that the surgical procedure should be individualized for each infant to optimize the aortic anatomy.

Anomalous left coronary artery from the pulmonary artery in infants. Which operation? When?

The surgical management of anomalous left coronary artery from the pulmonary artery in infants and small children remains controversial, because the ideal surgical procedure and the optimal time for operation are yet to be determined. From 1977 to 1985, 22 patients less than 4 years of age (mean age 18.2 months) underwent direct aortic reimplantation of the anomalous left coronary artery. There were five operative deaths (23%, confidence limits 13%-36%). The determinant risk factor of early mortality was the severity of preoperative left ventricular dysfunction (p = 0.05), not age at operation (p = 0.64) or preoperative clinical status (p = 0.36). There were not late deaths (mean follow-up 38 months). All survivors but one were symptom free. The reimplanted anomalous left coronary artery was patent in each reevaluated case (9/17). Left ventricular function improved significantly in all survivors. Moderate to severe preoperative mitral incompetence lessened in all patients but one, without mitral valve repair. When technically feasible, direct aortic reimplantation of the anomalous left coronary artery is an attractive procedure because it offers a high rate of patency and avoids the potential drawbacks of procedures involving autogenous venous or arterial tissue. Optimal intraoperative myocardial preservation and institution of temporary left ventricular assistance at the end of the operation may decrease the operative risk. Left ventricular function nearly always recovers after successful revascularization, and resection of left ventricular myocardium is rarely indicated, if ever. Mitral incompetence almost always lessens, and the mitral valve should not be repaired at initial operation; however, residual mitral incompetence may necessitate reoperation in a few cases. In infants with moderate left ventricular damage (usually asymptomatic with medical therapy), surgical treatment should be delayed until 18 to 24 months of age so that it can be performed with a low operative risk. Infants with severely impaired left ventricular function and persistent congestive heart failure should probably undergo operation as soon as the diagnosis has been made.

Late results after left-sided cardiac valve replacement in children.

Selection of types of cardiac valve substitutes for children remains controversial. Between 1976 and 1984, 166 children, 15 years of age or younger, underwent aortic (N = 53) or mitral valve replacement (N = 90) or both (N = 23). Biological prostheses were used in 84 patients and mechanical prostheses in 71; both a mitral bioprosthesis and an aortic mechanical valve were used in 11 patients. The overall early mortality was 9%. Mean follow-up intervals were 4.1 years for the bioprosthesis group, 3.3 years for the mechanical valve group, and 3.5 years for the group receiving both. The 7 year survival rates (+/- standard error) were 63% +/- 6% in the bioprosthesis group and 70% +/- 7% in the mechanical valve group (p = NS). After aortic valve replacement the 7 year survival rates were 66% +/- 14% (bioprosthesis group) and 77% +/- 9% (mechanical valve group) (p = NS); after mitral valve replacement the rates were 65% +/- 7% (bioprosthesis group) and 54% +/- 17% (mechanical valve group) (p = NS). The incidence of thromboembolic events was 0.6% +/- 0.4% per patient-year in the bioprosthesis group (none after aortic valve replacement, 0.8% +/- 0.6% per patient-year after mitral valve replacement) and 1.4% +/- 0.8% per patient-year in the mechanical valve group (0.7% +/- 0.7% per patient-year after aortic valve replacement, 4.0% +/- 2.8% per patient-year after mitral valve replacement) (p = NS). The linearized rates of reoperation were 10.4% +/- 1.8% per patient-year (bioprosthesis group) and 2.3% +/- 1.0% per patient-year (mechanical valve group) (p less than 0.001). The 7 year probability rates of freedom from all valve-related complications were 43% +/- 6% in the bioprosthesis group and 86% +/- 4% in the mechanical valve group (p less than 0.001). In the aortic position, a mechanical adult-sized prosthesis can always be implanted, and satisfactory long-term results can be anticipated. In the systemic atrioventricular position, the results are less than satisfactory with either type of prosthesis; every effort should be made to preserve the natural valve of the child.

[Isolated coarctations of the aorta. Early repair by aortoplasty with the left subclavian artery. 15 cases]. / Coarctations isolées de l'aorte. Réparation précoce par plastie avec l'artère sous-clavière gauche. Quinze observations.

When medical treatment is ineffective, isolated aortic coarctation must sometimes be surgically repaired during the first months of life. For these early operations the Crafoord resection-anastomosis technique seems to be disappointing, with a high recurrence rate. Between 1979 and 1983, 15 infants under 6 months of age underwent repair of their coarctation by a different technique: longitudinal aortoplasty using the left subclavian artery. One child died of septic rupture of the aorta, and another was lost sight of. The remaining patients were examined 35 +/- 15 months after surgery. The blood pressure usually returned to normal with no pressure gradient, at rest, between the right upper limb and the lower limbs. One child, however, had a slight (10 mm Hg) residual gradient corresponding to discreet alterations at two-dimensional echocardiography. These good results at rest after aortoplasty seem to be better in medium term than those of resection-anastomosis. The left subclavian flap aortoplasty appears to be the technique of choice in children under 6 months for the treatment of isolated coarctation.

Cor triatriatum and total anomalous pulmonary venous connection: a rare, surgically correctable anomaly.

Two cases of a very uncommon congenital association (cor triatriatum and total anomalous pulmonary venous connection) are presented. This association should be suspected in any infant with signs of pulmonary venous obstruction.