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Correction of truncus arteriosus with truncal valvar stenosis or insufficiency using two homografts.

Surgical correction of truncus arteriosus requires the creation of right ventricular to pulmonary artery continuity and closure of the ventricular septal defect. A variety of conduits have been used including valved and nonvalved. Despite a significant incidence of truncal valvar stenosis and insufficiency, this valve has seldom been replaced. We present 4 cases of truncus arteriosus with truncal valvar stenosis or insufficiency that were repaired using two valved homografts: one to create the pulmonary outflow tract and the other to replace the abnormal truncal valve. Two of these patients are doing well after 4 months. Another child survived the operation and did well for 2 months when she died suddenly. The last child died 14 hours postoperatively from low cardiac output syndrome secondary to diabetic hypertrophic cardiomyopathy. When truncal valvar abnormalities are present, the primary repair of truncus arteriosus in an infant should include replacement of the truncal valve. Total correction can be successfully achieved using two valved homografts, resulting in long-term palliation and freedom from thromboembolic events and the use of anticoagulants.

Abnormalities, Multiple↗

Repair of truncus arteriosus in infancy.

Improvements in myocardial protection, surgical technique, and postoperative care have decreased operative mortality for neonatal repair of truncus arteriosus. Primary repair of truncus arteriosus in infancy without prior pulmonary artery banding is currently the preferred approach. During the period from 1982 to December 1990, 32 patients under the age of 12 months underwent surgical correction of truncus arteriosus at UCLA. The average age was 3.5 months (range, 12 days to 12 months). Three patients had interrupted aortic arch. Early mortality for the entire group was 15.6% (5/32); for those older than 1 month early mortality was 7% (2/28). In the past 4 years, early mortality has decreased to 8.3% (2/24); both of these patients had interrupted aortic arch. Excluding patients with interrupted aortic arch, there were no early deaths in the last 22 patients (1986 to 1990). Late mortality overall was 7.4% (2/27). In a mean follow-up of 73 months (range, 40 to 110 months), 71% (5/7) of the survivors with Dacron porcine-valved conduits required conduit replacement secondary to obstruction. In a mean follow-up of 36 months (range, 1 to 89 months), only 14% (3/21) of the patients with homografts required replacement secondary to obstruction.

Age Factors↗

Successful treatment of acute postoperative pulmonary hypertension with nifedipine.

We report the successful use of nifedipine in the treatment of acute pulmonary hypertension in an infant after a cardiac operation. This patient had undergone total surgical correction of his truncus arteriosus malformation. He had signs of severe pulmonary artery hypertension unresponsive to hyperventilation, oxygenation, sedation, and a myriad of vasodilators. Nifedipine, 0.2 mg/kg every 4 hours, effectively treated his pulmonary artery hypertension and allowed for a smooth postoperative course and positive outcome.

Humans↗

Fate of small homograft conduits after early repair of truncus arteriosus.

Neonatal repair of truncus arteriosus is being performed in a number of centers, often with the use of small homograft conduits. The fate of the homograft and the risk of replacement were the subjects of this study. Between January 1987 and October 1991, 43 infants aged less than 3 months (range, 3 to 90 days) underwent primary repair of truncus arteriosus including implantation of a valved homograft conduit (diameter, 7 to 12 mm). Twenty-nine had follow-up of more than 6 months (range, 6 to 65 months; mean, 21.9 months). After a mean period of 31 months (range, 8 to 65 months), 7 patients showed obstruction with right ventricular pressures at least 75% systemic and underwent either a conduit change (n = 5) or a patch augmentation (n = 2). Mean cardiopulmonary bypass time at reoperation was 99 minutes; mortality was zero. Five other children are known to have a right ventricular pressure of 50% to 60% systemic, 2 having undergone balloon dilation. Statistical comparison of the patients with conduit reoperation or high right ventricular pressure (n = 12) with the rest of the population (n = 17) revealed an elevated pulmonary artery to right ventricular pullback gradient on postoperative day 1 after the repair (7.7 versus 1.3 mm Hg; p = 0.001) and choice of an aortic over a pulmonary homograft (100% versus 64.7%; p = 0.065) as significant risk factors. Age and weight at repair, postoperative pulmonary artery pressure, length of follow-up, and size of the homograft showed no significant differences between the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗

Anterior pulmonary translocation for repair of truncus arteriosus with interrupted arch.

A newborn was found to have truncus arteriosus and an interrupted aortic arch, and underwent primary repair. The patient did well initially, but, by 8 weeks postoperatively, showed evidence of severe compression of the right pulmonary artery. At reoperation, the pulmonary artery was found to be compressed by a large aortic root (truncal root) and the retroaortic area was narrowed. The pulmonary artery bifurcation was therefore translocated anteriorly to alleviate the compression.

Aorta, Thoracic↗

Novel technique for extending the use of allografts in cardiac operations.

Small cryopreserved allografts for use in neonates and infants are increasingly difficult to find. We describe a technique for surgically reducing the size of the more readily available large-diameter allografts to make them conform to size requirements appropriate for neonates and infants. This technique involves a longitudinal incision of the allograft from its muscular annulus to its distal orifice with the excision of a single valve leaflet. The two commissural posts of the excised leaflet and the wall of the allograft are then reapproximated with fine suture. Four patients ranging in age from 14 days to 11 months (mean age, 5.2 months) and ranging in weight from 3 to 8.4 kg (mean weight, 5.2 kg) underwent reconstruction of the right ventricular outflow tract using this surgical technique. Two patients underwent repair of truncus arteriosus (age, 14 days and 16 days) and two patients underwent Rastelli operations (age, 9 months and 11 months). The mean follow-up time was 22.5 months (range, 14 to 28 months). All 4 patients are alive and well. One patient requires digoxin and furosemide for moderate quadricuspid truncal valve insufficiency. Serial echocardiography documents mild allograft stenosis in 2 patients and trivial to mild allograft insufficiency in 2 patients. Because handling characteristics and lower bleeding risks render allograft conduits preferable to synthetic conduits and, furthermore, because conduit insertion of any type in the neonate will eventually be outgrown and require replacement, we find this technique to be justifiable as a therapeutic option. In the short term, the technique results in excellent functional results and provides an alternative to synthetic conduits when an appropriate sized allograft is unavailable.

Female↗

Salvage of infected truncus repair using rifampicin-impregnated gelatin-sealed graft.

An infant suffered sternal infection and ventricular septal patch dehiscence after a truncus arteriosus repair. Successful reoperation used rifampicin-impregnated gelatin-sealed Dacron to repair the recurrent ventricular septal defect and replace the contaminated conduit. This was an effective solution to a potentially lethal surgical complication.

Blood Vessel Prosthesis↗

Truncus arteriosus with interrupted aortic arch: successful correction using autologous flap.

A newborn baby with type II truncus arteriosus and type B interrupted aortic arch was successfully treated by creating a pulmonary tract using autologous flap made from truncal wall without excision of the pulmonary artery and by reconstructing the aortic arch with direct anastomosis. This method provided excellent hemodynamics with wide reconstruction of the pulmonary tract without conduit.

Anastomosis, Surgical↗

Truncus arteriosus with total anomalous pulmonary venous return and interrupted arch.

An infant with truncus arteriosus, total anomalous pulmonary venous return of the supracardiac type and interrupted aortic arch distal to the left subclavian artery is presented. One-stage repair was performed in the newborn period using a 12-mm aortic homograft. Recovery was complicated by congenital stenosis of the left main bronchus. The cardiovascular status remains stable 2 years after repair.

Aorta, Thoracic↗

Anterior pulmonary translocation without conduit for the repair of truncus arteriosus.

A technique with autologous tissue for the correction of type III truncus arteriosus is described. The truncal root was excised as a cylinder that incorporated pulmonary arteries and that was translocated anterior to the ascending aorta. The proximal section of the cylinder was closed and the pulmonary tract was reconstructed with anastomosis of a widely opened distal section to the right ventricle. Autologous pericardium was sutured to the entire surface of the pulmonary tract.

Anastomosis, Surgical↗

Early pulmonary homograft failure from dilatation due to distal pulmonary artery stenosis.

Early progressive pulmonary homograft insufficiency developed in an 11-month-old infant after repair of truncus arteriosus because of dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia. Before repair, the pulmonary artery branches were discontinuous, with the right pulmonary artery being somewhat hypoplastic and originating from the trunk, and the left pulmonary artery supplied by a modified Blalock-Taussig shunt created in the newborn period. At repair, a pulmonary homograft was used to connect the branches. Progressive cardiomegaly and oxygen dependance occurred 3 weeks postoperatively. Cardiac catheterization showed systemic right ventricular pressure, severe homograft insufficiency, and residual distal pulmonary artery stenosis and hypoplasia. On reoperation at 3 months postoperatively, the homograft annulus diameter increased from 14 mm to 16 mm. Dilatation and insufficiency probably occurred because the right ventricle and homograft distal to the obstruction functioned as a unit during systole. The problem might have been minimized with the use of aortic homograft, which is thicker, or annular reinforcement with a synthetic material.

Constriction, Pathologic↗

Stentless xenograft valve in a truncus arteriosus communis.

We report the use of a recently developed stentless porcine valve in a 2-year-old child with truncus arteriosus communis (type I). The child had received no previous surgical palliation. Despite a markedly increased pulmonary arteriolar resistance the right ventricle was connected to the pulmonary artery with a homograft. The postoperative course was complicated at first by right ventricular failure due to pulmonary hypertension and secondarily by left ventricular failure caused by rapidly increasing truncal valve incompetence. Because we did not have a suitable homograft at hand we replaced the truncal valve with a stentless porcine xenograft valve.

Bioprosthesis↗

Correction of truncus arteriosus with autologous arterial flap in neonates and small infants.

BACKGROUND: This study describes the results of techniques using the autologous truncal wall and part of the pulmonary artery for correction in anticipation of the growth of the pulmonary tract in patients with truncus arteriosus. METHODS: Seven consecutive patients with truncus arteriosus were reviewed. The posterior wall of the pulmonary tract was obtained by anastomosing the lower edge of the truncal arteriotomy to the upper corner of the ventriculotomy from the truncus in types I and II. Anterior translocation of the pulmonary artery was performed in a type III. A pericardial patch with or without a monocusp was placed to complete the right ventricular outflow tract. RESULTS: There were two hospital deaths, one of which was unrelated to a cardiac problem. Postoperative right-to-left ventricular peak pressure ratio was less than 0.55. There was one left pulmonary stenosis due to monocusp adherence in the late postoperative period. The sizes of the pulmonary tract at anastomosis were between 107% and 166% of the normal value between 7 months and 3.8 years of follow-up. CONCLUSIONS: The use of autologous arterial wall instead of a conduit is recommended for the repair of truncus arteriosus to expect growth of the pulmonary tract.

Follow-Up Studies↗

Common arterial trunk associated with absence of one atrioventricular connexion.

An unusual case of common arterial trunk is described. The right pulmonary artery is supplied by the ascending trunk and the left pulmonary artery arises via an arterial duct from the left brachiocephalic trunk. Although common arterial trunk can exist with any atrioventricular connexion, the absence of one atrioventricular connexion (tricuspid atresia) is the other rarity of this case.

Heart Atria↗

Common arterial trunk arising exclusively from the right ventricle with hypoplastic left ventricle and intact ventricular septum.

A common arterial trunk is a rare congenital anomaly usually accompanied by other intracardiac malformations. The association with hearts with a univentricular atrioventricular connexion is particularly uncommon. We report a case of a common arterial trunk occurring in a heart with absent left atrioventricular connexion. The trunk arose exclusively from the right ventricle, the left ventricle was incomplete and the ventricular septum was intact. Other associated malformations were an interrupted aortic arch and an atrial septal defect.

Aorta, Thoracic↗