Total correction of truncus arteriosus with severe truncal valve insufficiency in neonate]

The prognosis of infants with truncus arteriosus associated with severe truncal valve insufficiency is quite poor. Total correction was successfully performed in a neonate with such a complicated anomaly. The patient was 21 days old female with anuria due to severe congestive heart failure preoperatively in spite of medical treatment. She underwent Rastelli operation and pulmonary artery was reconstructed using autologous pericardial 3 valved conduit. Truncal valve was 4 cusps with the malformed nodular margins and one cusp had cleft. This cleft was closed suturing the cleft cusp and adjacent cusp each other and annuloplasty was added in 4 commissures. She survived and her truncal valve insufficiency was still mild at 2 years post-operative period. Although total correction with truncal valve repair for such a severely ill neonate and young infant with truncus arteriosus as this patient has not been reported, our experience suggests that severe truncal valve insufficiency could be fairly well repaired by valvulo-annuloplasty.     

Truncal Valve Repair: Initial Experience in Neonates

Background. The identification of moderate to severe preoperative truncal valve regurgitation has been synonymous with significant postoperative mortality after neonatal repair of truncus arteriosus. Spurned by the deficiencies of current truncal valve substitutes surgeons are once again reexamining the option of truncal valve reparative techniques.

Methods. From May 1996 until June 1997, 8 children underwent correction of truncus arteriosus. A retrospective analysis was conducted.

Results. There was one in-hospital death secondary to a postoperative massive coronary air embolism. Moderate-to-severe truncal valve regurgitation was identified clinically and confirmed with cardiac ultrasound in 3 neonates with a mean age of 7 days (range, 4 to 12 days) all with quadracusp truncal valves. Successful truncal valve repair was accomplished in 2 infants, with a third neonate requiring homograft replacement with coronary reimplantation for failure in achieving valvular competence after attempted valvuloplasty. Postoperative echocardiograms in those neonates who underwent truncal valve repair confirmed a functional “tricuspid” valve with only mild to mild-plus regurgitation.

Conclusions. We give further credence to the hypothesis that primary neonatal truncal valve repair is feasible and may be successful in the avoidance and delay of serial truncal valve replacements using either mechanical or allograft substitutes.     

Surgical management of severe truncal insufficiency: experience with truncal valve remodeling techniques

BACKGROUND: Truncal valve insufficiency has been a significant short- and long-term risk factor for repair of truncus arteriosus. Recent reports have documented the virtues of truncal valve repair. The purpose of this report is to review our experience with truncal valve repair and illustrate our techniques. METHODS: Between 1995 and 2000, 8 patients had interventions for severe truncal valve insufficiency at primary repair (3 patients) or in conjunction with conduit replacement (5 patients). One neonate had truncal valve replacement at initial repair early in the experience. The other 7 patients had truncal valve repair, 3 by valvar suture techniques. The remaining 4 patients had leaflet excision and annular remodeling in 3 (coronary reimplantation was required in 2) and commissure resuspension in 1 patient. RESULTS: Trivial to mild truncal valve insufficiency is present in the patients who had leaflet excision and annular remodeling (n = 3) and commissure resuspension (n = 1). Of the 3 patients who had valvar suture truncal valve repair, there was one death and 2 patients required acute valve replacement. The 7 survivors are doing well 1 month to 6 years postoperatively. CONCLUSIONS: Truncal valve repair by valvar suture techniques has not been successful in our practice. Truncal valve remodeling by leaflet excision and reduction annuloplasty is an effective method for truncal valve repair. When leaflet excision of a coronary sinus of Valsalva is required, coronary artery translocation can be accomplished.     

Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus

OBJECTIVE: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair. METHODS: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus. Their ages ranged from 2 days to 6 months (median, 2 weeks). Nine patients had associated interrupted aortic arch. Of the 14 patients (28%) in whom truncal valve regurgitation was diagnosed preoperatively, 5 had mild regurgitation, 5 had moderate regurgitation, and 4 had severe regurgitation. Five underwent truncal valve repair and 1 underwent homograft replacement of the truncal valve with coronary reimplantation. RESULTS: The actuarial survival was 96% at 30 days, 1 year, and 3 years. There were no deaths in patients with associated interrupted aortic arch. The 2 deaths in the series occurred in patients with truncal valve regurgitation, neither of whom underwent repair. Postoperative transthoracic echocardiography in patients who underwent valve repair showed minimal residual valvular regurgitation. None of the patients has required reoperation because of truncal valve problems or aortic arch stenosis at a median follow-up of 23 months (range, 1-60 months). Conduit replacement has been done in 17 patients (34%) after a mean duration of 2 years. The freedom from reoperation for those who had an aortic homograft was 4 years and for those who had a pulmonary homograft was 3 years. CONCLUSION: Despite the magnitude of the operation, excellent results can be achieved in complex forms of truncus arteriosus. In the current era interrupted aortic arch is no longer a risk factor for repair of truncus. Aggressive application of truncal valvuloplasty methods should neutralize the traditional risk factor of truncal valve regurgitation.     

Coronary arterial and sinusal anatomy in hearts with a common arterial trunk

Eighty-four specimens of common arterial trunk were studied with special reference to the arrangement of the leaflets in relation to the atrioventricular valves, the origin of the coronary arteries in relation to the arterial sinuses, and the epicardial course of the coronary arteries. Fourteen normal hearts were used for comparison. In the hearts with common arterial trunk, the location and level of the coronary artery orifices (as well as the relationship of the truncal root to the area of fibrous continuity with the mitral valve) are different from those in normal hearts. In none of the hearts with common arterial trunk (particularly the 53 hearts with three leaflets in the truncal valve) did the appearance of the truncal valve approximate that of a normal aortic valve. Among the 22 hearts with four leaflets, there was a high incidence of coronary artery orifices in opposite sinuses (17/22 or 77.3%) and a low incidence of coronary artery orifices in adjacent sinuses (2/22 or 9.1%). These results suggest that the formation of the truncal valve is independent of the formation of the coronary orifices. Its leaflets are not predestined to become part of either the aortic valve or the pulmonary valve.     

Transplantation in truncus arteriosus combined with interrupted aortic arch

We present the case of an infant with truncus arteriosus combined with a dysplastic truncal valve not amenable to repair and interrupted aortic arch. Due to the disappointing results of truncal valve replacement we decided to perform cardiac transplantation. The infant survived transplantation and was discharged 4 weeks after surgery.     

Successful correction of truncal valve regurgitation and conduit obstruction after previous Rastelli operation for truncus arteriosus (type I)

A 9-year-old boy had a successful surgical correction of truncal valve regurgitation and obstruction of extracardiac conduit which ensued previous Rastelli operation. He underwent Rastelli operation at the age of 10 months with the diagnosis of truncus arteriosus (Collet & Edwards Type I). At the age of 8 years, he was admitted because of slow weight gain and cardiomegaly (CTR 64%). Cardiac catheterization showed elevated RV pressure, increased pressure gradient across the conduit and severe truncal valve regurgitation. The extracardiac conduit was replaced with a 22 mm Hancock valved conduit and the truncal valve with 23 mm St. Jude Medical aortic valve. Postoperative catheterization revealed a normal RV pressure, only slight pressure gradient across the conduit and no truncal valve regurgitation. He is doing quite well 2 years after the operation.     

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.     

Half-turned truncal switch operation for transposition of great arteries with ventricular septal defect and pulmonary regurgitation

A three-month-old girl weighing 4.2 kg, diagnosed with transposition of the great arteries (TGA) and ventricular septal defect (VSD) was referred to us. She had normal-sized pulmonary annulus and moderate pulmonary regurgitation. Because her pulmonary valve was not suitable for systemic circulation due to valvular incompetence, the half-turned truncal switch operation was selected. The postoperative course was uneventful without left or right ventricular outflow obstructions over a year of follow-up. Our report demonstrated that the TGA and VSD with normal pulmonary annulus is not contraindicated for half-turned truncal switch operation.     

Management of Truncal Valvular Regurgitation

The presence of significant truncal valvular regurgitation presents a technically difficult and life-threatening challenge during operations for correction of the truncus arteriosus anomaly. The problem was encountered in 19 of 71 patients (26%) who underwent such operations. A simple technique for managing this problem, namely, temporary cusp approximation, has evolved and has proved successful in all 11 patients in which it was used. The indications for replacement of the regurgitant truncal valve have not yet been defined. The suggestion is made that in all but the most severe instances of truncal valvular regurgitation, persistence of the regurgitation postoperatively may currently be preferable to replacement of the valve.     

Improving early and intermediate results of truncus arteriosus repair: a new technique of truncal valve repair.

BACKGROUND: Despite improved surgical results for truncus arteriosus, overall mortality rates, remain higher than those reported for other complex congenital heart diseases, especially with truncal valve regurgitation or an interrupted aortic arch. METHODS: Seventeen patients had complete repair of the truncus arteriosus at the Cleveland Clinic Foundation between August 1993 and June 1997. The age at operation ranged from 2 days to 4.5 years. Associated abnormalities included interrupted aortic arch in 3 patients and abnormal coronary artery anatomy in 3. Four patients had more than moderate truncal valve insufficiency requiring concomitant truncal valve repair. RESULTS: There were no early deaths and only one late death at a mean follow-up of 24 months. The death occurred 3 months postoperatively and resulted from refractory pulmonary vascular obstructive disease in a patient who was referred at 1 year of age. Reoperation was required in 4 patients. CONCLUSIONS: Even in the presence of associated anomalies complete repair was performed with a low mortality rate. Truncal valve repair can be performed safely in the neonate with good results.     

Effect of proximal gastric vagotomy and truncal vagotomy and pyloroplasty on gastric functions and growth in puppies after massive small bowel resection.

The effects of truncal vagotomy and pyloroplasty and proximal gastric vagotomy on gastric acid hypersecretion, hypergastrinemia, and growth after massive bowel resection were studied in beagle puppies. In puppies with 80% enterectomy, neither type of vagotomy alters significantly the postprandial hypersecretion of acid from the Heidenhain pouch or the concentration of serum gastrin. Proximal gastric vagotomy tended to decrease the hypersecretion more than did truncal vagotomy. In beagle puppies undergoing 70% small bowel resection, growth was significantly decreased but survival was not impaired. Neither proximal gastric vagotomy nor truncal vagotomy and pyloroplasty reversed completely the impaired growth produced by massive resection. Proximal gastric vagotomy caused a small improvements in growth, while truncal vagotomy and pyloroplasty resulted in a slight decrease in growth. It remains possible that proximal gastric vagotomy could be of value in the management of growing infants with hypersecretion of acid due to short bowel syndrome.     

Staged biventricular repair for persistent truncus arteriosus with aortic arch obstruction following bilateral pulmonary artery banding

Persistent truncus arteriosus associated with aortic arch obstruction (Van Praagh type A4) is a rare combination with an extremely high rate of mortality. Our current approach is staged biventricular repair following bilateral pulmonary artery banding (bPAB). The patient in case 1 underwent bPAB at five days old, then biventricular repair was performed 10?days later because duct became restrictive despite prostaglandin administration. In case 2, the patient underwent bPAB via a left side thoracotomy at eight days old concomitant with an aortic arch repair using an extended direct anastomosis. Complete biventricular repair was delayed until eight months after the bPAB due to respiratory issues, then both banded branch pulmonary arteries were augmented by auto-pericardial patches at that time. A catheter examination three years after the complete biventricular repair showed that both branch pulmonary arteries were widely open. The patient in case 3 underwent emergency bPAB and truncal valve repair with a cardiopulmonary bypass at three days old for severe truncal regurgitation. Banding tapes on both sides were tightened two days after the operation. Although the hemodynamic condition was stable and truncal regurgitation was reduced, the patient died one month later from sudden septic shock.     

Congenital aortic and truncal valve reconstruction using the Ozaki technique: Short-term clinical results

ObjectivesAortic valve reconstruction (AVRec) with neocuspidization or the Ozaki procedure with complete cusp replacement for aortic valve disease has excellent mid-term results in adults. Limited results of AVRec in pediatric patients have been reported. We report our early outcomes of the Ozaki procedure for congenital aortic and truncal valve disease.MethodsA retrospective analysis was performed on all 57 patients with congenital aortic and truncal valve disease who had a 3-leaflet Ozaki procedure at a single institution from August 2015 to February 2019. Outcome measures included mortality, surgical or catheter-based reinterventions, and echocardiographic measurements.ResultsTwenty-four patients had aortic regurgitation (AR), 6 had aortic stenosis (AS), and 27 patients had AS/AR. Two patients had quadricuspid valves, 26 had tricuspid, 20 had bicuspid, and 9 had unicusp aortic valves. Four patients had truncus arteriosus. Thirty-four patients had previous aortic valve repairs and 5 had replacements. Preoperative echocardiography mean annular diameter was 20.90 ± 4.98 cm and peak gradient for patients with AS/AR was 53.62 ± 22.20 mm Hg. Autologous, Photofix, and CardioCel bovine pericardia were used in 20, 35, and 2 patients. Eight patients required aortic root enlargement and 20 had sinus enlargement. Fifty-one patients had concomitant procedures. Median intensive care unit and hospital length of stay were 1.87 and 6.38 days. There were no hospital mortalities or early conversions to valve replacement. At discharge, 98% of patients had mild or less regurgitation and peak aortic gradient was 16.9 ± 9.5 mm Hg. Two patients underwent aortic valve replacement. At median follow-up of 8.1 months, 96% and 91% of patients had less than moderate regurgitation and stenosis, respectively.ConclusionsThe AVRec procedure has acceptable short-term results and should be considered for valve reconstruction in pediatric patients with congenital aortic and truncal valve disease. Longer-term follow-up is necessary to determine the optimal patch material and late valve function and continued annular growth.     

Laparoscopic management of acid peptic disease

BACKGROUND: Despite successful medical treatment to reduce acid hypersecretion and eradicate Helicobacter pylori, surgery still plays an important role in the management of complicated peptic ulcer disease. Almost all types of conventional operations available for ulcer disease have been successfully performed by the laparoscopic approach and this has become the preferred approach in tertiary centers for operative management of acid peptic disease. METHOD: Between 1995 and 2004, laparoscopic management was offered to refractory or obstructive acid peptic disease patients. For intractable disease, we performed either posterior truncal vagotomy with anterior fundal seromyotomy or posterior truncal vagotomy with anterior proximal gastric vagotomy. For peptic ulcer disease complicated with gastric outlet obstruction, we carried out bilateral truncal vagotomy with gastrojejunostomy. RESULTS: Two hundred sixty three patients were operated of whom 236 (89.7%) were men and the average age of the patients was 48.4 years. Thirty-two (12.2%) patients underwent posterior truncal vagotomy with anterior seromyotomy, 89 (33.8%) underwent posterior truncal vagotomy with anterior proximal gastric vagotomy, 120 (45.6%) underwent bilateral truncal vagotomy with stapled gastrojejunostomy whereas 22 (8.4%) underwent bilateral truncal vagotomy with hand-sewn gastrojejunostomy. The average operating times were 142, 110, 98, and 72 minutes, respectively. The average postoperative stay was 5.4 days. CONCLUSIONS: Laparoscopic posterior truncal vagotomy with anterior proximal gastric vagotomy for refractory disease and laparoscopic bilateral truncal vagotomy with stapled gastrojejunotstomy for obstructive disease have become the standard at our institution. Regardless of the preference of individual surgeon, our results have shown that laparoscopic surgery may become the gold standard for surgical management of peptic ulcer disease.     

Fifteen-year experience with surgical repair of truncus arteriosus

From 1965 until March, 1982, 167 patients underwent surgical repair of truncus arteriosus. The age at operation ranged from 18 days to 33 years (mean 6 years). There were 48 hospital deaths (28.7%). The following factors had a positive correlation with the possibility of a surgical death: age at operation less than 2 years (p less than 0.001), a postrepair pulmonary arterial/left ventricular pressure ratio greater than 0.5 for patients with two pulmonary arteries (p less than 0.001) and greater than 0.6 for patients with unilateral absence of a pulmonary artery (p less than 0.02), and a postrepair right ventricular/left ventricular pressure ratio greater than 0.8 (p less than 0.008). The 119 hospital survivors were followed up for a total of 829 person-years. Late survival rate at 5 years was 84.4% and at 10 years, 68.8%. Preoperative factors that correlated with a reduced long-term survival rate were as follows: increasing age at operation (p = 0.004), the presence of moderate or severe truncal valve insufficiency (p = 0.008), lower pulmonary/systemic flow ratio (p = 0.04), and unilateral absence of a pulmonary artery (p less than 0.001). Thirty-six patients required reoperation during the follow-up period (30%) primarily for replacement of the right ventricular-pulmonary arterial conduit and/or for truncal valve replacement. The long-term results obtained in these patients support the need for early repair of the anomaly, improvement in the methods for control or repair of the truncal valve insufficiency, and the continued search for better extracardiac valved conduits.     

Semilunar Valve Replacement with a Cylindrical Valve

A bstract A cylindrical valve was designed to prevent regurgitation of the semilunar valve. The valve is made of a sheet of polytetrafluoroethylene (PTFE) or porcine pericardium, and has three cusps and three commissures. The diameter of the valve is equal to the height of the cusps. We have used these valves in pulmonary stenosis after Jatene's operation and total correction of tetralogy of Fallot, and for truncal valve regurgitation. Regurgitation was trivial on color Doppler echocardiography in all cases. Advantages in comparison with the implantation of commercially available artificial valves include the ability to insert a larger size and no compression of the valve ring when closing the sternum. Outflow tract obstruction does not occur even when the valve is implanted in a small infant. In the present report, we describe this simple technique.     

Repair of truncus arteriosus in the neonate and young infant

Infants with truncus arteriosus present a difficult management issue. Because of the high operative mortality, repair is often delayed beyond the first 3 to 6 months of age. We reviewed our experience with 11 neonates and young infants with truncus arteriosus undergoing repair (median age, 21 days). Five patients also had major truncal valve insufficiency, and 2 required valve replacement. Right ventricle-pulmonary artery continuity was established with a porcine valved conduit in 3 patients and an aortic or pulmonary homograft in 8. There was 1 operative death (9%; 70% confidence limits, 3%-22%) and 1 late death over a mean follow-up of 21 months (range, 4 to 32 months). Eight of the 9 late survivors are growing normally. Echocardiographic examination revealed normal ventricular function in all patients (mean shortening fraction, 39%). Doppler assessment demonstrated trivial prosthetic or homograft valve regurgitation in 7 patients and mild to moderate obstruction in 5 patients. This recent experience with repair of truncus arteriosus indicates that the operative risk is low even in the neonate. Repair in the first month of life should be recommended before the development of critical congestive heart failure or irreversible pulmonary vascular disease.     

Rastelli and Norwood combination for the treatment of type I truncus arteriosus and hypoplastic aortic arch: a case report

Although truncus arteriosus is often treated with low mortality and morbidity rates, truncal valve patency and aortic arch and coronary artery anomalies are factors that can contribute to a worse outcome. In this report, we present our experience with the combination of Rastelli and Norwood procedures for the treatment of Type I truncus arteriosus that was complicated by a hypoplastic aortic arch.     

The anesthetic management of a patient with Cohen syndrome

Cohen syndrome is a rare genetic disorder caused by autosomal recessive inheritance and is characterized by the following features: mental retardation, infantile hypotonia, micrognathia, narrow and high-arched palate, microcephaly, prominent upper central incisors, poor dentition, short stature, and truncal obesity. Some patients have strabismus, myopia, optic atrophy, and total blindness. A small number of cases present with heart defects or mitral valve prolapse. Only approximately 100 cases have been reported in the world literature. The administration of general anesthesia in patients with Cohen syndrome can be a challenge because most of these patients are mentally retarded and uncooperative and have facial malformations that may make intubation difficult. We present our experience with the anesthetic management of a patient with Cohen syndrome.