Truncus arteriosus: ten-year experience with homograft repair in neonates and infants.

BACKGROUND: Early complete repair of truncus arteriosus with homograft reconstruction of the right ventricular outflow tract was performed and long-term results were evaluated. METHODS: Review of 46 consecutive patients with truncus arteriosus who underwent primary correction between June 1987 and May 1997 was performed. Ages ranged from 21 days to 7.2 years (median, 62 days) and weights from 1.8 to 21.5 kg (median, 3.4 kg). Ten patients were operated on before 1 month of age, 20 between 1 and 3 months, 13 between 3 months and 1 year, and 3 at an older age. Associated cardiac conditions were encountered frequently, the most common being coronary artery anomalies (n = 16), truncal valve incompetence (n = 12), and interrupted aortic arch (n = 5). The right ventricular outflow tract was reconstructed with an aortic (n = 28) or a pulmonary homograft (n = 18). RESULTS: There were two hospital deaths (4.3%). Both patients had severe truncal valve regurgitation and interrupted aortic arch together with other unfavorable conditions. Survival in uncomplicated truncus arteriosus was 100%. Follow-up was from 3 months to 10 years (mean, 36 months). There was one late death 4 months after the initial repair, presumably because of cardiac reason. Actuarial survival was 93% at 4 months and beyond. Actuarial freedom of reoperation in the 27 hospital survivors with aortic homografts was 43% at 75 months; it was 73% at 62 months in the 17 patients surviving with pulmonary homografts. CONCLUSIONS: Neonatal or early infancy complete repair is the treatment with the best potential for survival. The homograft remains our conduit of choice to establish continuity between the right ventricle and the pulmonary artery. Management of severe truncal valve incompetence remains a surgical challenge.

The fate of small-diameter homografts in the pulmonary position.

BACKGROUND: Limited durability is expected for small homograft valves that are used to correct congenital cardiac disease. METHODS: All 76 homograft valves with an internal annulus diameter ranging from 8 to 13 mm that were implanted from 1987 through 2000 in the pulmonary position were retrospectively analyzed. In each case, homograft size was normalized to the patient's body surface area: z-value. For 93% (14 of 15) of the 8 to 9 mm grafts, z was less than 2. For 56% (5 of 9) of the 10 mm grafts and 98% (51 of 52) of the 11 to 13 mm allografts, z was greater than 2. Survival and freedom from complications were estimated by the Kaplan-Meier method. Homograft failure was defined as homograft replacement or late death; significant dysfunction, as homograft obstruction with an echo-Doppler gradient greater than 50 mm Hg or grade III or IV valvular insufficiency. The log-rank test was used to compare outcomes. RESULTS: Seven patients died early after operation; three, late. Survival was 86.5% +/- 3.8% at 1 year and remained stable during the succeeding years. Freedom from failure for all homografts was 90.6% +/- 3.7%, 71.8% +/- 6.9%, and 61.8% +/- 9.0% at 1, 5, and 10 years, respectively. Corresponding freedom from significant dysfunction was 87.6% +/- 4.1%, 51.2% +/- 7.4%, and 10.1% +/- 8.3%. The smaller homografts (z less than 2) failed and deteriorated faster (p < 0.0001): only 32.1% +/- 13.0% were still functioning at 24 months. The larger grafts (z at least 2) retained function for the first 4 years, and 73.7% +/- 10.4% had not yet failed at 10 years. CONCLUSIONS: Smaller (z less than 2) homografts (the great majority of 8 to 9 mm grafts) have to be replaced early, usually within 2 years of implantation. Larger (z at least 2) grafts (nearly all 11 to 13 mm grafts) show remarkable durability and are suitable valved conduits for establishing right ventricle to pulmonary artery continuity in neonates and young infants.

Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants.

OBJECTIVE: To present our 17-year experience of surgical repair of total anomalous pulmonary venous drainage (TAPVD) in 71 consecutive neonates and infants, with particular emphasis on the role of preoperative pulmonary venous obstruction (PVO), the management of postoperative pulmonary hypertensive crises and the long-term results. PATIENTS AND METHODS: From July 1977 to May 1994, 36 newborns and 35 infants, aged between 1 and 300 days, had repair TAPVD in our Department. Fifty-four patients had decompensated heart failure, whereas three were moribund. The anomalous drainage was supracardiac in 32 (45%), cardiac in 17 (24%), infracardiac in 17 (24%) and mixed in 5 infants (7%). Major associated cardiac anomalies coexisted in nine cases. Forty-five (63%) exhibited signs of PVO. The repair was performed under moderate hypothermia in 15 and deep hypothermic arrest in 56 patients. In case of postoperative pulmonary hypertensive crises, artificial hyperventilation with high oxygen concentration, sedation and relaxation, inotropic support and afterload reduction with phentolamine were applied selectively. The actual follow-up is based on functional symptomatology and cardiac echo-doppler studies. RESULTS: Six children (8.5%) died early: four perioperatively, two on the 2nd and 10th postoperative day, respectively. Poor preoperative condition, operation before 1987 and preoperative PVO were the main incremental risk factors for death. No death occurred among the last 38 cases. There were complications in 45 patients, in particular pulmonary hypertensive crises in 27. Two patients died late, after 80 and 118 days, respectively, of progressive intrapulmonary vein fibrosis: the second despite reoperation. The risk of late death approaches zero after 6 months. Among the 63 survivors, 56 (86%) are asymptomatic and without medication, 3 months-17 years after operation. Five have mild symptoms with ordinary physical activity. Two are lost to follow-up. CONCLUSIONS: Early repair of TAPVD with aggressive management of pulmonary hypertensive crises carries low operative mortality nowadays. Preoperative PVO as a risk factor has been neutralized since 1987. Long-term results are gratifying: no late death after 6 months, no reoperation and functional good results. Progressive pulmonary vein fibrosis remains an unpredictable rare cause of death within the 1st year after surgery.

Transposition of the great arteries associated with ventricular septal defect: surgical results and long-term outcome.

OBJECTIVES: To identify potential risk factors influencing early and late outcome following the arterial switch operation (ASO) for transposition of the great arteries associated with ventricular septal defect including double-outlet right or left ventricle. METHODS: All patients who underwent ASO in our department until August 2000 (n=105) were included in this study. There were 77 transpositions of the great arteries with ventricular septal defect, 22 Taussig-Bing hearts and six patients with double-outlet morphology. The median age at operation was 24 days. Aortic arch obstruction was present in 25 patients; in 13 of these patients, a repair with aortic arch reconstruction was done before ASO. The usual coronary artery pattern was present in 59% of the patients. In six patients, we found an intramural course of at least one coronary artery. The ventricular septal defect was closed with a patch through the right atrium (n=35), the aorta (n=25), the pulmonary artery (n=25) or the right ventricle (n=3); in 17 patients a combined approach was necessary. RESULTS: There were five hospital deaths (4.7%, 95% confidence limit 2-11%). The median duration of follow-up was 72 months. Fourteen patients underwent 15 reoperations 33 months after repair (median), eight for right ventricular outflow tract obstruction or neopulmonary stenosis. Four late deaths occurred, two due to complications related to coronary artery anomalies. Statistical analysis revealed no significant risk factor whatsoever correlating with death or need for reoperation. Survival after 12 years was 91.6%, and freedom from reoperation was 82.6%. Latest follow-up data showed that 13% of patients were in NYHA class II and/or required medical treatment; 87% were in NYHA class I. CONCLUSIONS: ASO associated with patch closure of ventricular septal defect can be performed early in life with a low risk of mortality (<5%), low incidence of reintervention (<15%) and promising long-term outcome.

Closure of the zone of apposition at correction of complete atrioventricular septal defect improves outcome.

OBJECTIVE: Outcome after correction of atrioventricular septal defect depends to a great deal on the postoperative function of the left atrioventricular valve. The related role of the zone of apposition ('cleft') has been debated: should it be closed (bileaflet repair) or should it be left untouched (trileaflet repair)? This study aims to answer the question by comparing the outcome of patients treated according to these two approaches. METHODS: We reviewed all our patients who underwent repair of complete atrioventricular septal defect from 1984 to 1997 and selected those in whom the closure of the zone of apposition in principle would have been possible. Two groups with similar characteristics were constituted: group I (n=63), where the zone of apposition was deliberately not closed as part of a trileaflet repair (postoperative open zone of apposition) and group II (n=96), where it was electively closed as part of a bileaflet AV valve repair (closed zone of apposition). Since we changed from a trileaflet to a bileaflet repair in 1987, the two groups differ in terms of size and length of follow-up. Outcome was compared with regard to survival and freedom from reoperation for left atrioventricular valve incompetence. Late atrioventricular valve function was evaluated by Echo-Doppler. For statistical analysis, we used Chi-square or Fisher's exact test, the Mann-Whitney test and the log-rank test for comparison of Kaplan-Meier curves. The difference was considered statistically significant with a P-value of 0.05 or less. RESULTS: Early mortality was 9.5% (6/63) in group I and 3.1% (3/96) in group II (P=0.16). Actuarial survival after 1, 4 and 8 years was 80.4, 68.4 and 64.8%, respectively, for group I. Actuarial survival for group II was 94.7, 92.1 and 92.1% (P=0.0002). Freedom from reoperation for left atrioventricular valve regurgitation was 90.2, 85.6 and 77.8% for group I at the same time interval. It was a constant 97.9% for group II (P=0.0016). At reoperation, left atrioventricular valve regurgitation was present through the open zone of apposition in 63% of group I cases. The follow-up is 96% (126/131) complete. An increase in degree of left atrioventricular valve incompetence was noted in 28% (11/39) of group I cases and in 9% (8/87) of group II cases (P=0.0131). CONCLUSION: This study demonstrates the advantage of closing the zone of apposition ('cleft') as part of repair of complete atrioventricular septal defect. Survival, freedom from reoperation for left atrioventricular valve incompetence and over-all outcome were more favourable in patients of group II. The zone of apposition should be surgically addressed whenever the morphology of the left atrioventricular valve allows for closure without producing stenosis.

[Prolonged endotracheal intubation after open heart surgery under the age of one year]. / Intubation endotrachéale prolongée après chirurgie à coeur ouvert avant l'âge d'un an.

The aim of this study was to determine the impact of prolonged endotracheal intubation after open heart surgery in neonates and babies with respect to the cardiac diseases most frequently responsible and the reasons for delayed extubation and the related mortality. Intubation was considered to be prolonged if lasting over 48 hours. This was a retrospective study of all cases of open heart surgery performed before one year of age between 1991 and 1996. Prolonged intubation was noted in 43.1% of cases (266/617). The frequency was much higher than in children over 1 year of age: 8.3% (56/725). Truncus arteriosus (93.5%: 29/31), obstructed total anomalous venous drainage (93.3%: 14/15). Taussig-Bing anomaly (83.3%: 5/6), interruption of the aortic arch (78.9%: 15/19), double outlet right ventricle (61.1%: 11/18) and transposition of the great arteries (50.2%: 107/213) were the commonest responsible malformations. The reasons for delayed extubation, often multiple, were established in 222 cases: cardiogenic shock or circulating failure in 155 cases, pulmonary dysfunction in 142 cases, surgical complications in 65 cases and neurological complications in 14 cases. Twelve patients died: 5 of congestive heart failure, 3 of pulmonary hypertension, 2 of septic shock and 1 of diffuse stenosis of the pulmonary veins. The mortality rate was 4.5% (12/266) in cases of prolonged intubation and 5.2% (32/617) for all infants operated during the first year of life. The authors conclude that a large number of infants undergoing open heart surgery undergo prolonged intubation because of their poor preoperative status and the particularly severe effects of cardiopulmonary bypass at this age. However, the associated mortality was low in this series.

[Heart surgery under cardiopulmonary bypass during the neonatal period remains a high-risk procedure]. / La chirurgie cardiaque sous circulation extracorporelle dans la période néonatale comporte encore un risque non négligeable.

Congenital Heart disease with a poor prognosis has to be operated early but with an acceptable surgical risk and a good chance of survival. The aim of this study was to analyse the indications, the operative mortality and medium-term survival of neonates undergoing cardiac surgery under cardiopulmonary bypass from 1991 to 1998. Three hundred and twenty nine operations were programmed in 326 neonates, 18% (329/1805) of all open heart surgical procedures. Anatomical detransposition of the great arteries was the commonest operation (N = 226). Correction was complete (biventricular) in 97% of cases (317/326). Twenty-eight neonates died. The operative mortality was 8.5%, much higher than that of children of 3 months or over (1.5%; p < 0.0001). There were 8 late deaths, all in the first postoperative year. The overall medium-term survival rate was 88.9 +/- 1.7%. It was 100% after correction of truncus arteriosus without interruption of the aortic arch; 94.2 +/- 1.5% after anatomical detransposition; 85.7 +/- 9.4% after commissurotomy of aortic stenosis; 79.2 +/- 8.3% for all the complex forms of interruption of the aortic arch; 75 +/- 9.7% for total anomalous pulmonary venous drainage and 42.9 +/- 18.7% after the Norwood palliative procedure of hypoplastic left heart syndrome. The authors conclude that early cardiac surgery saves the large majority of neonates suffering from complex congenital cardiac disease with poor prognosis. Survival stabilises one year after the operation. Other techniques or treatments are necessary to lower present surgical risk (8.5%) to that of surgery under cardiopulmonary bypass of children over 3 months of age (1.5%).

[Surgical repair of common truncus arteriosus with homograft. Short- and mid-term results]. / Réparation chirurgicale du tronc artériel commun à l'aide d'homogreffes. Résultats à court et à moyen terme.

Between July 1987 and July 1993, 25 consecutive children with truncus arteriosus underwent complete surgical correction with homografts. Nineteen were under 6 months of age and 4 had an associated interruption of the aortic arch. Ten pulmonary and fifteen aortic homografts were implanted. They were cryopreserved with the exception of two, fresh aortic homografts. The diameters of the homografts varied from 8 to 19 mms. Two children who had an interruption of the aortic arch, aged 24 and 31 days, died in the peroperative period. The postoperative course was uncomplicated in only 3 cases; 20 patients had complications, including 9 cardiogenic shocks and 8 pulmonary hypertensive crises. The medium-term results included one death 4 months after surgery in an infant with an interruption of the aortic arch, and 2 successful homograft replacements 3 and 12 months after the initial repair for mycotic infection in 1 case and valvular stenosis in the other. The mean follow-up of the other 20 patients was 23 months: 14 were in NYHA functional Class I, 4 are on the waiting list for replacement of their homograft for obstruction after an average period of 47 months. The authors strategy is to perform surgical correction of truncus arteriosus very early: immediately in the neonatal period in cases with uncontrollable cardiac failure, at 6 months at the latest. Small homografts make this aggressive attitude feasible, but with an operative mortality in the first 6 months of life of 10.5% (2/19).(ABSTRACT TRUNCATED AT 250 WORDS)

Integrated home monitoring predicts lead failure in a pacemaker dependent 4-year-old girl.

A 4-year-old girl with post-surgical complete atrioventricular block received an epicardial dual chamber pacemaker system. During further growth intermittent exit block occurred, first misinterpreted as neurological seizures. The epicardial lead was replaced using a transvenous approach, and a pacemaker with an integrated home monitoring facility was implanted. After her discharge, a rise in the pacing threshold automatically initiated an event message. On the basis of this information, the patient was called in and imminent dislodgement of the ventricular lead was diagnosed by x-ray. The lead was repositioned and was found stable over 1-year follow-up.