Surgical Repair Of Truncus Arteriosus Research Articles

English

Surgical repair of Truncus arteriosus aims to improve long term survival. A clear understanding of the natural history and achievable surgical outcomes informs surgical decision making with respect to the timing of surgery. We sought to determine if it is ever too late to repair T. arteriosus. Pubmed and Google scholar were searched between January 1st 1966 and July 31st 2021. The proximal similarly model was used to determine the most externally valid natural history and surgical outcome data. The most externally valid data were used to develop a composite survival graph to help clinicians optimize surgical decision making. Four hundred and twenty eight results were retrieved. Five studies representing 938 patients who had repair of T. arteriosus were included for external validity assessment. The resultant composite graph suggests that repair after 5 month of age, may not improve 15 year survival when compared to non-operative care. T. arteriosus repair after 5 months of age may not improve survival. Key words: Pediatric, truncus artriosus, operative, outcomes.

Hospital Survival After Surgical Repair of Truncus Arteriosus with Interrupted Aortic Arch: Results from a Multi-institutional Database.

Truncus arteriosus (TA) is a major congenital cardiac malformation that requires surgical repair in the first few weeks of life. Interrupted aortic arch (IAA) is an associated malformation that significantly impacts the complexity of the TA operation. The aim of this study was to (1) define the comorbid conditions associated with TA and (2) determine the hospital survival and morbidity of patients with TA with and without an IAA. Data was collected from the Vizient Clinical Database/Resource Manager, formerly University HealthSystem Consortium, which encompasses more than 160 academic medical centers in the United States. The database was queried for patients admitted from 2002 to 2016 who were ≤ 4months of age at initial admission, diagnosed with TA, and underwent complete surgical repair during that hospitalization. Of the 645 patients with TA who underwent surgery, 98 (15%) had TA with an interrupted aortic arch (TA-IAA). Both TA and TA-IAA were associated with a high prevalence of comorbidities, including DiGeorge syndrome, prematurity, and other congenital malformations. There was no difference in mortality between TA and TA-IAA (13.7-18.4%, p value = 0.227). No comorbid conditions were associated with an increased mortality in either group. However, patients with TA-IAA had a longer post-operative length of stay (LOS) compared to those without IAA (30 versus 40.3days, p value = 0.001) and this effect was additive with each additional comorbid condition. In conclusion, the addition of IAA to TA is associated with an increased post-operative LOS, but does not increase in-hospital mortality.

Delayed surgical repair of truncus arteriosus with interrupted aortic arch following bilateral banding of branch pulmonary arteries: a case report

BackgroundBanding of the bilateral branch pulmonary arteries (PA) has evolved as a palliative method to limit the pulmonary blood flow in various complex congenital heart diseases. Although beneficial in small patients and in those with multiple co-morbidities, the duration of pulmonary artery banding along with growth and status of branch pulmonary arteries following de-banding are of concern in these patients.Case presentationWe present a case of truncus arteriosus with interrupted aortic arch in whom bilateral PA banding was done in neonatal life. The corrective surgery was delayed by 13 months due to multiple infections and underlying co-morbid conditions. Although the surgery was delayed, the branch pulmonary arteries were of adequate size following the debanding procedure.ConclusionBranch PA augmentation may not always be necessary even in delayed corrective surgery following bilateral PA banding.

Alternative techniques of right ventricular outflow tract reconstruction for surgical repair of truncus arteriosus.

This study aimed to evaluate the outcomes and feasibility of different techniques of reconstruction of the right ventricular outflow tract (RVOT) in surgical repair of truncus arteriosus. We retrospectively reviewed all consecutive patients with truncus arteriosus who underwent successful surgical repair in our centre between 1994 and 2017. We analysed late results according to the type of RVOT repair. We collected data from 29 survivors after truncus arteriosus repair. Six (20%) of them were affected by DiGeorge syndrome. The RVOT reconstruction was achieved using a valved conduit in 58.6%, while a direct right ventricle-pulmonary artery (RV-PA) anastomosis, with or without the interposition of the left atrial appendage, was performed in the remaining. At a median follow-up time of 7.9 years (interquartile range 1.8-13.1), 6 patients (3 affected by DiGeorge syndrome) died. Between the 2 groups, there were no differences in terms of the late mortality and onset of adverse events. However, the use of a conduit seemed more prone to reintervention and onset of adverse events. Different RVOT reconstruction techniques are safe and have similar late outcomes. However, use of a direct RV-PA anastomosis and left atrial appendage interposition may reduce the need for reoperation in the long term.

Utilization of inhaled nitric oxide after surgical repair of truncus arteriosus: A multicenter analysis.

Elevated pulmonary vascular resistance (PVR) is common following repair of truncus arteriosus. Inhaled nitric oxide (iNO) is an effective yet costly therapy that is frequently implemented postoperatively to manage elevated PVR. We aimed to describe practice patterns of iNO use in a multicenter cohort of patients who underwent repair of truncus arteriosus, a lesion in which recovery is often complicated by elevated PVR. We also sought to identify patient and center factors that were more commonly associated with the use of iNO in the postoperative period. Retrospective cohort study. 15 tertiary care pediatric referral centers. All infants who underwent definitive repair of truncus arteriosus without aortic arch obstruction between 2009 and 2016. Descriptive statistics were used to demonstrate practice patterns of iNO use. Bivariate comparisons of characteristics of patients who did and did not receive iNO were performed, followed by multivariable mixed logistic regression analysis using backward elimination to identify independent predictors of iNO use. We reviewed 216 patients who met inclusion criteria, of which 102 (46%) received iNO in the postoperative period: 69 (68%) had iNO started in the operating room and 33 (32%) had iNO initiated in the ICU. Median duration of iNO use was 4 days (range: 1-21 days). In multivariable mixed logistic regression analysis, use of deep hypothermic circulatory arrest (odds ratio: 3.2; 95% confidence interval: 1.2, 8.4) and center (analyzed as a random effect, p=.02) were independently associated with iNO use. In this contemporary multicenter study, nearly half of patients who underwent repair of truncus arteriosus received iNO postoperatively. Use of iNO was more dependent on individual center practice rather than patient characteristics. The study suggests a need for collaborative quality initiatives to determine optimal criteria for utilization of this important but expensive therapy.

RF37 LATE OUTCOMES OF SURGICAL REPAIR OF TRUNCUS ARTERIOSUS. A 2 DECADES EXPERIENCE

Background and aim: To determine whether the right ventricle outflow tract (RVOT) type of reconstruction is correlated to different outcomes in long term results in patients who survived after surgical repair of truncus arteriosus (TA). Methods: We retrospectively reviewed the outcomes of 30 patients with TA who underwent successful surgical repair in our center between 1994–2017. We analyzed late results according to the type of RVOT repair. Results: Among 30 survivors after TA repair, 6 (20%) were affected by DiGeorge syndrome. The RVOT reconstruction was achieved by means of a conduit (Contegra or Homograft) in 53.6%. In the remaining group of patients, a direct right ventricle-pulmonary artery anastomosis ± interposition of left atrial appendage (LAA) was performed. At a median follow-up time of 12 years (range 4 months-23 years, 93% complete), 6 patients (3 affected by DiGeorge syndrome) died late. Between RVOT groups, there was no difference in terms of late mortality; however, the use of a conduit was significantly associated with a higher risk for reoperation (p 0.021; OR 8.80, 1.35–57.4, Figure 1). Conclusions: Post surgical history of patients with TA is characterized by a higher incidence of late adverse events when RVOT is reconstructed by means of a conduit. Reconstruction of the RVOT with a direct anastomosis and/or left atrial appendage interposition reduces effectively the need for reoperation in long term.

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

A successful surgical repair of truncus arteriosus in a three-year-old boy

A successful surgical repair of truncus arteriosus in a three-year-old boy

Surgical repair of truncus arteriosus in children: early results and long-term outcomes

To recite early results and long-term outcomes after surgical repair of persistent truncus arteriosus (PTA). The clinic data of 54 patients underwent surgical repair for PTA from January 1999 to December 2009 was analyzed retrospectively. There were 36 male and 18 female patients, with a mean age of (9 ± 10) months (range, 1 to 38 months; median, 5 months). Preoperative mechanical ventilation was required in 5 patients. The surgical procedures were closure of ventricular septal defect and re-establishment of continuity between right ventricle and pulmonary artery. The right ventricular outflow tract (RVOT) was reconstructed by direct anastomosis pulmonary artery to right ventriculotomy with anterior wall patch enlargement (28 cases), or by inserting conduits (26 cases). Valvuloplasty were performed in 4 patients with truncal valves moderate to severe insufficiency and aortoplasty in 3 patients with interrupted aortic arch (IAA). There were 3 patients (5.6%) died of pulmonary hypertensive crisis in hospital. The mean duration of ventilation was 6.8 days in 5 patients who were intubated before operation, while the others were 3.6 days. Forty-seven (92.2%) patients were followed-up for mean (6.8 ± 2.5) years (from 2.5 to 11.0 years). There were 2 patients with mild to moderate aortic regurgitation. One patient with aortic arch obstruction underwent balloon dilatation 2 years postoperatively. Among those patients who underwent direct anastomoses, 8 (32.0%) patients had pulmonary branch stenosis at 7 months to 1.5 years postoperatively, 12 (48.0%) patients were freedom from surgical reintervention 5.0 to 11.0 years postoperatively. Among those inserting conduits, 7 patients (31.8%) had conduit stenosis at 2.8 to 7.0 years after operation. Reoperations were performed for RVOT in 15 patients and there was no mortality. It is difficult to treat the PTA patients with IAA, intra-mural coronary artery or mechanical ventilation support before operation. The technique of direct anastomosis between pulmonary artery and right ventricle offers the potential growth for RVOT, but bilateral pulmonary branch stenosis may be occurred at earlier period of postoperation in some patients.

Our experiences with the surgical repair of truncus arteriosus: early and mid-term results

Our experiences with the surgical repair of truncus arteriosus: early and mid-term results

Invited Commentary

Invited Commentary

Surgical Repair of Truncus Arteriosus Associated With Interrupted Aortic Arch: Long-Term Outcomes

Truncus arteriosus (TA) with interrupted aortic arch (IAA) is a rare combination of complex anomalies. We report long-term follow-up after one-stage repair of TA associated with IAA. From April 1985 to August 2007, 16 patients with TA associated with IAA underwent one-stage repair. There were 2 (12.5%) early deaths and no late deaths. Follow-up was 92.9% complete (13 of 14 patients), with 1 patient lost to follow-up. Median duration of follow-up was 18.2 years (range, 2.1 to 21.9 years). Functional status in all patients was good. Thirteen patients underwent 25 surgical reoperations and 5 interventional procedures (3 aortic arch balloon angioplasties and 2 pulmonary artery balloon angioplasties). Overall freedom from any reoperation was 69.2% at 1 month, 54.5% at 3 years, 30% at 5 years, 11.1% at 10 years, and 0% at 15 years after the initial operation. Freedom from aortic reoperation was 76.9% at 1 month, 72.7% at 3 years, 70% at 5 years, 66.7% at 10 years, and 57.1% at 15 years; while freedom from right ventricular-to-pulmonary artery conduit replacement was 84.6% at 1 month, 63.6% at 3 years, 40% at 5 years, 11.1% at 10 years, and 0% at 15 years. Finally, freedom from truncal valve reoperation was 100% at 5 years, 88.9% at 10 years, and 85.7% at 15 years. One-stage repair of TA-IAA can be undertaken with good long-term results. Despite a significant reoperation rate, patients' functional status remains good at the long-term follow-up.

Surgical repair of truncus arteriosus associated with interrupted aortic arch

The surgical repair of truncus arteriosus associated with an interrupted aortic arch (TAC-IAA) requires performing two major procedures at the same time. Due to the small number of patients, there is nearly no surgical learning curve. The surgical technique has greatly improved since the introduction of a homograft patch enlargement of the small ascending aorta. The association with a severe truncal regurgitation is a major risk factor as well as the presence of preoperative multiple organs failure. The series published by single centers are ≪10 patients, which make statistical analysis troublesome. The mortality varies from 0% to 50%. The multicentric study published in 2006 by the Congenital Heart Surgeons Society (CHSS) reports a 68% mortality (34/50). Nevertheless, the results can be excellent in experienced centers using a modern one stage surgical technique, undertaken in the first week of life.

Presentation of Congenital Heart Disease in the Neonate and Young Infant

Presentation of Congenital Heart Disease in the Neonate and Young Infant

Prenatal sonographic diagnosis of truncus arteriosus associated with holoprosencephaly

We present the case of a truncus arteriosus associated with holoprosencephaly detected in a fetus in the first trimester and the sonographic findings that established this diagnosis. A physical exam was performed on a 35-year-old pregnant woman, gravida 2, para 1. At 20 weeks, sonography showed large-for-date uterine size. Fetal biometry was consistent with menstrual age. Amniotic fluid volume was elevated to 25 AFI cm. Fetal echocardiography revealed a single common artery arising from the heart, a ventricular septal defect, and other cardiac defects. Abnormalities of the fetal brain and face were also shown via sonography, including the presence of a common lateral ventricle without falx cerebri. Alobar holoprosencephaly, the most severe form of holoprosencephaly, was diagnosed. Prenatal chromosomal analysis was offered and the patient elected to have cordocentesis. The chromosome study was normal. Success in surgical repair of truncus arteriosus is established, but alobar holoprosencephaly is associated with neonatal death. Continuation of the pregnancy carried risks for the mother. After proper counseling, the parents of this fetus were offered and accepted termination of pregnancy. A male abortus weighing 320 grams was delivered. Postnatal findings and autopsy confirmed the diagnosis.

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)

Successful surgical repair of truncus arteriosus with interrupted aortic arch in infancy by an anterior approach

A 98-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type B) using a one-stage anterior approach. The interrupted aortic arch was reconstructed by direct anastomosis between the ascending and descending aorta. The ventricular septal defect was closed, and a 12-mm porcine valved conduit was placed on the right side of the ascending aorta to establish continuity between the right ventricle and pulmonary artery. Surgical procedures for the repair of this malformation are the focus of discussion.

Fifteen-year experience with surgical repair of truncus arteriosus: R.M. DiDonato, D.A. Fyfe, F.J. Puga, et al. J Thorac Cardiovasc Surg 89:414–422, (March), 1985

Fifteen-year experience with surgical repair of truncus arteriosus: R.M. DiDonato, D.A. Fyfe, F.J. Puga, et al. J Thorac Cardiovasc Surg 89:414–422, (March), 1985

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.

Early and late results of surgical repair of truncus arteriosus.

Ninety-two patients had corrective operation for truncus arteriosus between 1967 and 1975. During the first 30 days after surgery, 23 patients died. No significant differences appear between early mortality and sex, type of truncus, variations in pulmonary arterial anatomy, truncal valve regurgitation, associated anomalies, previous operation, or duration of extracorporeal circulation. Greater risk is probably encountered with the higher but still operable levels of pulmonary resistance. Reoperation has been required in three patients. Fifty-nine percent of survivors are symptom-free, with all but two of the remainder (38%) being in NYHA functional class II. The late result is suggestively less satisfactory in patients with significant preoperative truncal valve regurgitation. Some late complications related to deterioration of the earlier aortic homograft conduit may be avoided by use of a porcine valve Dacron conduit. The current operative mortality of 9%, and the well-being of 97% of surviving patients suggest the continued advisability of recommending operation for appropriate patients.