Truncal Valve Repair Research Articles

Current results of primary repair of truncus arteriosus in early infancy

Repair of truncus arteriosus in early infant period has become standard practice in many centers. We report the current clinical outcomes of primary repair of truncus arteriosus in early infancy at a single institution. A retrospective analysis of 31 patients with mean age 2.6 ± 1.3 months (range, 15 days–6 months), and mean body weight 3.4 ± 0.6 kg (range, 2.6–4.7 kg) who underwent primary repair of truncus arteriosus. Data were collected regarding demographics, anatomy, operative data, cardiac intensive care unit, and last available follow-up. There were four hospital deaths (12.9%); three of them were due to pulmonary hypertension. At median follow up of 8 months (range, 1–100 months) after surgery, there was no late death. One patient was reoperated on for right ventricle-to-pulmonary artery (RV-to-PA) conduit replacement, residual ventricular septal defect repair, and truncal valve repair. One patient was reoperated on twice for truncal valve repair and later on truncal valve replacement. Three patients required percutaneous balloon dilation and stenting of pulmonary artery branches. Truncus arteriosus can be performed in early infancy with acceptable perioperative mortality and morbidity. Pulmonary hypertension continues to be a risk factor of mortality.

Long-Term Follow-Up of Persistent Truncus Arteriosus: Kuwait Experience

Objective: To evaluate the long-term results of patients in Kuwait who were operated for persistent truncus arteriosus (PTA). Subjects and Methods: The following data were collected for retrospective analysis from 24 medical records of consecutive patients with PTA in Kuwait between August 1993 and August 2009: demographics, morphology, management and outcome. Major associated abnormalities included interrupted aortic arch in 1 patient and abnormal coronary artery anatomy in 2. Results: Of the 24 patients, 16 underwent total intracardiac repair. The age at operation ranged from 15 days to 5 years (mean 166.19 ± 438.63 days) and weight ranged from 2.5 to 15 kg (mean 4.3 ± 3.01 kg). The right ventricle to pulmonary artery continuity was established with aortic homograft in 11, pulmonary homograft in 4 and by implantation of a Contegra conduit in 1 patient. Four patients had moderate truncal valve regurgitation requiring concomitant truncal valve repair. After a mean follow-up period of 81.81 ± 61.58 months (range 3–166) there was no death. Eight of the 16 (50%) patients underwent redo homograft operations. One patient who had concomitant truncal valve repair subsequently underwent aortic valve replacement. Conclusion: The data showed that complete repair of PTA in the neonatal and early infancy period was the treatment with the best potential for survival. The homograft remained one of the conduits of choice to establish continuity between the right ventricle and the pulmonary artery in spite of the high incidence of conduit redo operations.

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 10days 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.

Durability of Truncal Valve Repair

Truncus arteriosus associated with truncal valve dysfunction has been shown to increase the mortality and morbidity associated with repair and the various techniques employed for truncal valve repair continue to evolve. We sought to examine the durability of truncal valve repair in truncus arteriosus. We reviewed all patients who underwent truncal valve repair at our institution from 1995 to 2008. Repair techniques included leaflet delamination, bicuspidization, commissuroplasty, annuloplasty, and leaflet augmentation. We examined long-term valve durability, function and need for valve replacement. A total of 17 patients underwent truncal valve repair, including 3 for predominant stenosis (>70 mm Hg gradient), 2 for mixed moderate stenosis (40 mm Hg gradient) and moderate regurgitation, and 12 for moderate or greater regurgitation. Of these, 14 patients (14 of 17, 82%) underwent valvuloplasty at the time of the initial truncus arteriosus repair. The median age at truncal valve repair was 24 days (range, 4 days to 42 years). Thirteen patients had 1 valvuloplasty, 3 patients had 2 valvuloplasties, and 1 patient had 3 valvuloplasties before undergoing a prosthetic valve replacement at age 13 years. Actuarial freedom from repeat truncal valve operation was 70% at 5 years and 50% at 7 years. Freedom from truncal valve replacement was 100% at 10 years. To date, only 1 patient (6%) has required a prosthetic valve. Echocardiography at 48 ± 33 (mean ± SD) months postoperatively showed a peak gradient of 11 ± 13 mm Hg; 6 patients have moderate regurgitation, and the rest have mild or less regurgitation. Truncal valve repair is a durable option with an acceptable reoperation rate, good function, and a low likelihood of needing truncal valve replacement. This is one of the largest series of truncal valve repairs and emphasizes that valve repair should remain the primary option.

Long-term results after correction of persistent truncus arteriosus in 83 patients

This study aims to analyse long-term results after correction of truncus arteriosus in all patients operated in one institution over 28 years. Between 1981 and 2009, 83 patients, median age 54 days, underwent repair of truncus arteriosus. Interrupted aortic arch was present in 14 (17%), severe truncal valve insufficiency in 10 (12%) and non-confluent pulmonary arteries in five (6%) patients. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 80, and other methods in three patients. At the same time, correction of interrupted aortic arch was done in 14 and truncal valve repair in eight patients. Survivors were repeatedly examined echocardiographically for assessment of residual heart lesions. The early mortality was 19 (23%). Out of 35 patients operated between 1981 and 1996, 17 (46%) died, and out of 48 patients operated between 1997 and 2009, two (4%) died. Operation before 1997 (p=0.001) and aortic cross-clamping time >90min (p=0.009) were found to be risk factors of death. Eight (10%) patients died late, a median of 68 days after surgery. Fifty-seven (69%) patients were followed for 10.9 + or - 6.7 years. Three (4%) patients were lost. Twenty-five (30%) patients are alive with their original conduit 7.5 + or - 5.2 years after correction. Twenty-eight patients required 41 re-operations for conduit dysfunction with first replacement at mean 5.8 + or - 4.1 (range 0.1-14.1 years) years after correction. Nine (11%) patients required 12 truncal valve replacements. Eleven (13%) patients required balloon dilatation or stent for conduit obstruction, pulmonary branch stenosis, aortic arch obstruction or stenosis of vena cava. Recent clinical examination was undertaken in 53 (64%) patients and 49 (59% or 77% of early survivors) are in good/very good condition. Truncus arteriosus remains a challenging heart disease. With growing experience, the early mortality decreased to 4%, but numerous re-interventions for conduit obstruction, pulmonary branch stenosis and truncal valve insufficiency are required. Surgery before 1997 and prolonged cross-clamping were risk factors of death. Pulmonary homografts had the best re-intervention-free survival. Statistically, however, the difference between conduits was not significant. Dysplastic valve and truncal valve insufficiency represent risk factors presenting the need for truncal valve replacement.

Long-term results after repair of truncus arteriosus communis in neonates and infants.

We reviewed our experience of truncus arteriosus communis (TAC) repair. Between 05/90 and 10/01, 16 patients underwent complete repair of TAC (primary repair: group I, 12 patients, secondary repair: group II, 4 patients). Age was 2.4 months [5 days-8.8 months] (median [range]) in group I, and 8.3 [5.6-13.5] years in group II. Continuity from the right ventricle to the pulmonary artery was achieved using a valved conduit. All patients had regular follow-up examinations. There was one early death in each group (12.5%). Follow-up was 9 [1.2-12.7] years. Valved conduit failure occurred in 8 patients (67 %) in group I (group II, 1 patient, 33 %) requiring replacement at 2.5 [0.3-4.3] years (group II, 5.8 years). Severe neo-aortic valve regurgitation after truncal valve repair was observed in one patient, requiring valve replacement at 8.5 years in association with repeat homograft replacement (group I). Actual echocardiographic examination revealed normal ventricular function. Moderate conduit dysfunction was noted in 2 patients (group I). Complete repair of truncus arteriosus communis can be performed with excellent long-term results.

Truncal valve repair in common arterial trunk

Truncus arteriosus communis (common arterial trunk) remains a rare congenital heart defect occurring in approximately 0.04 of 1000 live births (Neonatal Heart Dis. (1992) 428). The initial presentation remains variable but mild cyanosis and tachypnea remain common signs and symptoms due to the obligate pulmonary blood flow via the common arterial trunk and the ventricular septal defect. Severe congestive heart failure develops in time simultaneous with the characteristic decrease in pulmonary vascular resistance. Primary repair of truncus arteriosus during the neonatal period has become the established practice. Neonatal repair minimizes both the possibilities of pulmonary hypertension and the occurrence of late ventricular dysfunction. The presence of moderate to severe truncal valve insufficiency continues to be associated with an increased morbidity and mortality. Traditional strategies for the operative management of these children with neonatal valve replacement either mechanical or homograft has proved to be less than ideal. Truncal valvuloplasty, a natural corollary to semilunar valve repair in the adult population, may hold some promise in the restoration of early, valve competence and/or the delay or avoidance of truncal valve replacement.

Neonatal truncus arteriosus repair: Surgical techniques and clinical management

Truncus arteriosus is now ideally repaired in the neonatal period with low morbidity and mortality. Published reports have documented mortality rates in the range of 4% to 5% with mean age at repair as low as 11 days. The physiologic basis for improved outcomes with earlier repair is the avoidance of damaging sequelae of pulmonary overcirculation and heart failure. Data show that baseline mean pulmonary artery pressure is lower in infants undergoing earlier repair. Improved operative outcomes also have been achieved with aggressive truncal valve repair versus replacement in the presence of truncal valve dysfunction, right ventricular outflow tract reconstructive techniques that are patient anatomy-specific, and use of regional perfusion techniques for repair of associated interrupted aortic arch. In addition, a heightened awareness of anomalies of coronary artery ostial location, number, angle of takeoff, and degree of patency can result in avoidance of inadvertent injury to the artery and associated myocardial insult. Copyright © 2002 by W.B. Saunders Company

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. (J Thorac Cardiovasc Surg 2000;119:508-14)

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.

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.

Long-term follow-up of truncus arteriosus repaired in infancy: A twenty-year experience

Background: There have been few reports of long-term follow-up after truncus arteriosus repair in infancy. Methods: A retrospective review was performed to assess long-term outcomes among 165 patients who survived the initial hospital stay after complete repair of truncus arteriosus since 1975. The median age at truncus repair over this 20-year experience was 3.5 months (range 2 days to 36 years), and 81% of patients were less than 1 year of age. Previous pulmonary artery banding had been performed in 15 patients, and two patients had undergone prior repair of interrupted aortic arch. Significant procedures performed along with truncus repair included truncal valve replacement ( n = 10) or repair ( n = 5) and repair of interrupted aortic arch ( n = 4). Results: Patients were followed up for up to 20.4 years (median 10.5 years). Twenty-five patients were lost at cross-sectional follow-up, with a total of 67 patient-years of follow-up available on these patients. There have been 23 late deaths, eight of which occurred within 6 months of repair and 13 of which occurred within 1 year. Ten of the late deaths were related to reoperations. Actuarial survival among all hospital survivors was 90% at 5 years, 85% at 10 years, and 83% at 15 years and was essentially identical for infants alone. A significant independent risk factor for poorer long-term survival was truncus with moderate to severe truncal valve insufficiency before repair. During the follow-up period, 107 patients underwent 133 conduit reoperations. Median time to conduit reoperation was 5.5 years, and the only factor significantly associated with shorter time to conduit replacement was smaller conduit size at initial repair. In addition, 26 patients underwent 30 truncal valve replacements. Six patients required truncal valve replacement before any conduit-related reintervention, with two associated deaths. Actuarial freedom from truncal valve replacement among patients with no prerepair truncal valve insufficiency was 95% at 10 years. Actuarial freedom from truncal valve replacement was significantly lower among patients with truncal insufficiency before initial repair (63% at 10 years). At follow-up, all patients except three were in New York Heart Association functional class I. Conclusions: Ten- to 20-year survival and functional status are excellent among infants undergoing complete repair of truncus arteriosus. Conduit replacement or revision is almost inevitably necessary in this group of patients. (J Thorac Cardiovasc Surg 1997;113:869-79)

Truncal valve repair: Initial experience with infants and children

The truncal valve regurgitation that frequently arises in patients with truncus arteriosus accounts for increased operative and late mortality. Five patients underwent truncal valve repair at UCLA Medical Center between August 1990 and September 1991. This group consisted of 2 infants who underwent complete repair and 3 who underwent valve repair together with right ventricle-pulmonary artery conduit replacement. The techniques used for repair were individualized according to the specific valve morphology, and consisted of the suturing of partially developed commissures, suspension of the cusps, resection of redundant portions of the cusps, annuloplasty at the commissures, and resection of excrescences on the surface of valve leaflets. In 1 infant who had a severely dysplastic truncal valve, stenosis and regurgitation recurred and progressed, and he died 4 months after truncal valve replacement. The remaining 4 patients, who were followed for from 8 to 21 months after repair, are in New York Heart Association class I, and have minimal or no aortic regurgitation. Except in patients with severely deformed and dysplastic valves, truncal valve repair can be an attractive and successful alternative to valve replacement.

Repair of truncus arteriosus in the neonate

From September 1986 through December 1991, 63 patients with truncus arteriosus underwent surgical repair. The management approach evolved over the period of the study from elective primary repair at 3 months of age to elective primary repair in the early neonatal period. Thirty variables were examined as potential risk factors for the outcome events of death, reoperation, and presence of pulmonary vascular morbidity. According to both univariate and multivariate techniques, severe truncal valve regurgitation, interrupted aortic arch, coronary artery anomalies, and age at repair greater than 100 days were important risk factors for perioperative death. In the 33 patients without these risk factors, early survival was 100%. In the 30 patients with one or more of these risk factors, survival was 63%. Pulmonary hypertensive episodes were fewer, and duration of ventilator dependence and pulmonary artery pressure were significantly less in patients undergoing the operation before 30 days of age. Seven patients required reoperation for right ventricular outflow tract obstruction at a mean follow-up time of 23.4 months, with no deaths. In conclusion, interrupted aortic arch, severe truncal valve insufficiency, coronary anomalies, and repair later than 100 days of age were risk factors for death after repair of truncus arteriosus. In the absence of these associations, truncus arteriosus can be repaired with excellent surgical outcome in the neonatal and early infancy period. Repair in the early neonatal period reduces the prevalence of postoperative pulmonary vascular morbidity.