Repair Of Truncus Arteriosus Research Articles

Modified Repair of Type I and II Truncus Arteriosus Limits Early Right Ventricular Outflow Tract Reoperation

Repair of truncus arteriosus often requires early right ventricular outflow tract (RVOT) reoperation. Using a modified repair, the branch pulmonary arteries are left in situ, which may avoid earlier RVOT reoperation. We hypothesized that our modified repair for type I and II truncus arteriosus would extend the time to RVOT reoperation. Infants with truncus arteriosus were divided into 2 groups: (1) traditional technique where the branch pulmonary arteries are excised from the truncal root, or (2) modified repair where the branch pulmonary arteries are left in situ and septated from the truncal root. Regardless of the approach, a bioprosthetic conduit or homograft was used to establish right ventricular to pulmonary artery continuity. Follow-up pulmonary artery angiograms were used to assess for branch pulmonary artery stenosis. From 54 infants (modified repair: 33, traditional technique: 21), there were no significant differences in age at repair, gender, or type of truncus arteriosus. With 100% follow-up, use of the modified repair resulted in a lower rate of branch pulmonary artery stenosis, and greater freedom from surgical branch pulmonary arterioplasty. Five- and 10-year freedom from RVOT reoperation (5 years: modified-81.5% vs traditional-30.5%, P = 0.004; 10 years: modified-53.3% vs traditional-30.5%, P = 0.01) favored the modified repair. Cox regression analysis demonstrated that the modified repair was associated with an independently lower risk for RVOT reoperation (hazard ratio: 0.08, confidence interval: 0.01, 0.75, P = 0.02). Thus, maintaining the branch pulmonary artery architecture resulted in greater freedom from RVOT reoperation.

Transseptal Anchored Vascular Plug Closure of Mitral Valve Perforation

A 15-year-old post-neonatal repair of truncus arteriosus sustained an anterior leaflet mitral valve injury during elective aortic root replacement and aortic valve repair, resulting in severe mitral regurgitation ([Figure 1][1], [Online Video 1][2]). He was deemed not to be a surgical candidate in

Long-Term Outcomes Following Repair of Truncus Arteriosus and Interrupted Aortic Arch

Long-Term Outcomes Following Repair of Truncus Arteriosus and Interrupted Aortic Arch

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

Persistent truncus arteriosus on dual source CT.

Persistent truncus arteriosus is a rare congenital cardiac disease with variable presentation. The exact preoperative diagnosis and delineation of anatomy are very important because the optimal timing and procedure for truncus arteriosus repair are decided on the basis of the morphological characteristics. Moreover, the presence of associated anomalies influences the surgical outcome and mortality in these patients. Dual-source computed tomographic evaluation with three-dimensional post-processing is highly valuable for delineating its precise morphology and to identify and characterize the associated anomalies. Also depiction of the precise aortic arch morphology and simultaneous evaluation of the airway are very useful in treatment planning. This pictorial review provides an overview of the imaging spectrum of truncus arteriosus and various associated anomalies as seen on dual-source computed tomography.

Non-conduit repair of truncus arteriosus

BackgroundPrimary complete repair of truncus arteriosus (TA) is the goal in the current era of cardiac surgery. In the absence of valved conduits, non-conduit repair remains possible in spite of less optimal hemodynamics and could be a solution for this category of patients in the developing countries. Patients and methodsThis is a review of eight patients operated upon in the national heart institute from January 2013 to November 2015. They underwent non-conduit repair of truncus arteriosus by the modifications of the Barbero-Marcial technique through window like separation of the common trunk, approximating the pulmonary artery to right ventricular outflow tract and completed by autologous pericardial patch. ResultsThe mean age was 43.75 days with range 20–80 days. The mean body weight was 3.5 kg with range 2.8–4.5 kg. There were two early mortality cases in the intensive care unit (ICU). The mean ventilation time was 38.6 h with range 18–66 h. The mean ICU stay was 6.3 days with range 3–11 days. Pre-discharge echocardiography revealed mean gradient across the right ventricular outflow tract (RVOT) 24 mmHg with range 14–34 mmHg and mean grade of pulmonary incompetence (PI) was 2.3 with range of 1–3. ConclusionThe non-conduit repair is a good alternative for type I TA, especially when it is the only available solution.

Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years.

This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up. Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1-123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg). The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation. In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.

Clinical and Functional Developmental Outcomes in Neonates Undergoing Truncus Arteriosus Repair: A Cohort Study

Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair. All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis. The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p= 0.0016). Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.

Long-term Outcomes Following Surgical Repair of Persistent Truncus Arteriosus (PTA)

Long-term Outcomes Following Surgical Repair of Persistent Truncus Arteriosus (PTA)

Outcomes of Surgical Repair for Persistent Truncus Arteriosus from Neonates to Adults: A Single Center's Experience.

ObjectiveThis study aimed to report our experiences with surgical repair in patients of all ages with persistent truncus arteriosus.MethodsFrom July 2004 to July 2014, 50 consecutive patients with persistent truncus arteriosus who underwent anatomical repair were included in the retrospective review. Median follow-up time was 3.4 years (range, 3 months to 10 years).ResultsFifty patients underwent anatomical repair at a median age of 19.6 months (range, 20 days to 19.1 years). Thirty patients (60%) were older than one year. The preoperative pulmonary vascular resistance and mean pulmonary artery pressure were 4.1±2.1 (range, 0.1 to 8.9) units.m2 and 64.3±17.9 (range, 38 to 101) mmHg, respectively. Significant truncal valve regurgitation was presented in 14 (28%) patients. Hospital death occurred in 3 patients, two due to pulmonary hypertensive crisis and the other due to pneumonia. Three late deaths occurred at 3, 4 and 11 months after surgery. The actuarial survival rates were 87.7% and 87.7% at 1 year and 5 years, respectively. Multivariate analysis identified significant preoperative truncal valve regurgitation was a risk factor for overall mortality (odds ratio, 7.584; 95%CI: 1.335–43.092; p = 0.022). Two patients required reoperation of truncal valve replacement. One patient underwent reintervention for conduit replacement. Freedom from reoperation at 5 years was 92.9%. At latest examination, there was one patient with moderate-to-severe truncal valve regurgitation and four with moderate. Three patients had residual pulmonary artery hypertension. All survivors were in New York Heart Association class I-II.ConclusionsComplete repair of persistent truncus arteriosus can be achieved with a relatively low mortality and acceptable early- and mid-term results, even in cases with late presentation. Significant preoperative truncal valve regurgitation remains a risk factor for overall mortality. The long-term outcomes warrant further follow-up.

Anatomic Factors Associated With Truncal Valve Insufficiency and the Need for Truncal Valve Repair.

Truncus arteriosus is a complex and heterogeneous form of congenital heart defect. Many of the risk factors from several decades ago, including late repair and interrupted aortic arch, have been mitigated through better understanding of the entity and improved surgical techniques. However, truncal valve dysfunction remains an important cause of morbidity and mortality. The purpose of this study was to evaluate the anatomic factors associated with truncal valve dysfunction and the need for truncal valve surgery. This was a retrospective review of 72 infants who underwent repair of truncus arteriosus at our institution. The median age at surgery was nine days, and the median weight was 3.1 kg. Preoperative assessment of truncal valve insufficiency by echocardiography revealed no or trace insufficiency in 30, mild in 25, moderate in 10, and severe in 7. The need for truncal valve surgery was dictated by the severity of truncal valve insufficiency. Sixteen (22%) of the 72 patients undergoing truncus arteriosus repair had concomitant truncal valve surgery. Anatomic factors associated with the need for truncal valve surgery included an abnormal number of truncal valve cusps (P < .005), presence of valve dysplasia (P < .005), and the presence of an anomalous coronary artery pattern (P < .005). Fifteen (94%) of the sixteen patients who underwent concomitant surgery had two or all three of these anatomic factors (sensitivity = 94%, specificity = 85%). This study demonstrates that the presence of specific anatomic factors was closely associated with the presence of truncal valve insufficiency and the need for concomitant truncal valve surgery. Preoperative evaluation of these anatomic factors may provide a useful tool in determining who should undergo concomitant truncal valve surgery.

Outcomes of Truncus Arteriosus Repair in Children: 35 Years of Experience From a Single Institution

We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0.003), operative weight <2.5kg (P = 0.012), prior surgical intervention (P = 0.018), and coronary artery anomaly (P = 0.037) as risk factors for early mortality. A Cox regression model identified DiGeorge syndrome (P = 0.008) as a risk factor for late mortality. Freedom from right ventricular outflow tract reoperation was 4.6% at 20 years. Concomitant truncal valve (TV) repair or replacement was undertaken in 20 patients. Additionally, 14 patients underwent late TV repair or replacement. The overall survival rate in patients who underwent TV operation was 76.9% at 20 years. A total of 19 patients had concomitant interrupted aortic arch with a survival rate of 89.5% at 20 years. Median follow-up was 19 years (mean = 17 years, range: 1-34 years). All patients were in New York Heart Association Class I/II at last follow-up. Following repair of TA, patients had good long-term functional status but had high reoperation rates. Repair of interrupted aortic arch and TV were not risk factors for mortality. Postoperative extracorporeal membrane oxygenation, operative weight <2.5kg, prior surgical intervention, and coronary artery anomaly were risk factors for early death. DiGeorge syndrome was associated with late death, most commonly from infection.

Use of rotational angiography in assessing relationship of the airway to vasculature during cardiac catheterization.

We are the first to describe the use of three-dimensional rotational angiography (3DRA) in creating multiplanar reconstruction (MPR) and volume rendering, in the catheterization suite, of airways at risk for compression by adjacent cardiac structures. 3DRA has emerged as a promising tool for improved visualization of cardiac and vascular structures in congenital heart disease. This is a retrospective review of all available cases at our institution in which MPR from 3DRA was used to assess airways in relation to surrounding cardiovascular structures. Eight cases were reviewed from January 1, 2011 to November 30, 2013. Seven children had complex biventricular anatomy, including repaired truncus arteriosus, repaired absent pulmonary valve, repaired double outlet right ventricle, and vascular rings. One child had double inlet left ventricle and had undergone a hybrid procedure (stenting of the patent ductus arteriosus and banding of bilateral pulmonary arteries) before the Glenn procedure. Six of these cases involved distortion or stenosis of the pulmonary conduit or branch pulmonary arteries. In all cases, the trachea and the main bronchi were clearly visualized using MPR. Management was affected by the visualization of the airways during the catheterization procedure in seven of eight cases. Four cases had intraprocedural bronchoscopy that confirmed airway findings seen by MPR. In one case, computed tomography confirmed left bronchial compression seen by MPR. 3DRA can visualize airway anatomy and its relationship to the vasculature accurately. This has significant implications for preinterventional planning, intraprocedural management, as well as postprocedural recovery.

Early outcome of window like repair and valveless RV to PA connection for type one truncus arteriosus

Back ground Repair of truncus arteriosus represents a surgical challenge. Many technical modifications were added to improve the surgical outcome, decrease complications and decrease the incidence of re operations especially regarding the RV to PA connections with the consideration of absence or limited number of valved biological conduits in most of our centers in Egypt. Patients and methods Over the last six months 4 patients with TA type 1underwent total surgical repair with window like technique (without separation of the short main pulmonary trunk from the main trunk) and valveless RV to PA connection except one that was done using contegra due to severe PH as the patient was 6 months old. Results Early results of this limited number of patients’ reveals satisfactory results with no in hospital mortality, acceptable ICU and hospital stay and early post-operative echocardiographic study apart from clinically tolerated moderate PR. Conclusion Window like repair of TA and valveless RV to PA connection reveals satisfactory early outcome and considered as good alternatives for the classic techniques utilizing valved biological conduits especially when limited resources are obstacles for repair of this category of complex lesions.

Clinical Neurological, and Functional Outcomes in Patients Having Undergone Truncus Arteriosus Repair: a Cohort Study

Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess clinical, survival and early childhood neurological and functional outcomes in a cohort who underwent TA repair and were followed prospectively.

Truncus Arteriosus Communis With Interrupted Aortic Arch: Successful Repair Using a Novel Technique

We report a successful one-stage biventricular repair of truncus arteriosus communis with interrupted aortic arch in a neonate. This newborn girl was diagnosed with truncus arteriosus communis type III-B (Congenital Heart Surgeons Society classification) by the echocardiography and three-dimensional computed tomography. We present a new reconstructive approach for such heart defects, involving a modified Lecompte maneuver combined with arch reconstruction, by suturing the ascending aorta to the descending aorta in end-to-end fashion called the swing-back technique.

両側肺動脈絞扼術後に根治手術（大動脈弓再建＋Rastelli 手術）を施行した総動脈幹症・大動脈弓離断症の1例

症例は女児．在胎41週1日，体重3,032 gで仮死なく出生し，生直後より心雑音とチアノーゼを認め心エコー検査にて総動脈幹症・大動脈弓離断症と診断された．同日よりプロスタグランディン製剤の持続静注が開始され，当院NICU緊急搬送された．肺血流増多に伴う多呼吸・尿量減少がみられたため，11生日に両側肺動脈絞扼術を施行した．術後に心不全症状は改善し体重増加が得られ，生後2カ月時に根治手術を行った．胸骨再正中切開下に心・大血管周囲を剥離した後に，腕頭動脈および下行大動脈送血を併用した上下半身分離体外循環下に大動脈弓再建（拡大直接吻合）し，心停止下に右室切開を置きVSDを閉鎖した．大動脈遮断解除後にあらかじめ作製しておいた二弁付12 mmGoreTex人工血管を用いて右室流出路再建を行った．人工心肺離脱は特に問題なく，開胸状態で手術を終了し3病日に閉胸し4病日に人工呼吸器を離脱した．術後に右横隔膜の軽度拳上に伴う頻呼吸があったが徐々に改善し，49病日に退院した．

Differential Case Ascertainment in Clinical Registry Versus Administrative Data and Impact on Outcomes Assessment for Pediatric Cardiac Operations

Administrative datasets are often used to assess outcomes and quality of pediatric cardiac programs; however their accuracy regarding case ascertainment is unclear. We linked patient data (2004-2010) from the Society of Thoracic Surgeons Congenital Heart Surgery (STS-CHS) Database (clinical registry) and the Pediatric Health Information Systems (PHIS) database (administrative database) from hospitals participating in both to evaluate differential coding/classification of operations between datasets and subsequent impact on outcomes assessment. Eight individual benchmark operations and the Risk Adjustment in Congenital Heart Surgery, version 1 (RACHS-1) categories were evaluated. The primary outcome was in-hospital mortality. The cohort included 59,820 patients from 33 centers. There was a greater than 10% difference in the number of cases identified between data sources for half of the benchmark operations. The negative predictive value (NPV) of the administrative (versus clinical) data was high (98.8%-99.9%); the positive predictive value (PPV) was lower (56.7%-88.0%). Overall agreement between data sources in RACHS-1 category assignment was 68.4%. These differences translated into significant differences in outcomes assessment, ranging from an underestimation of mortality associated with truncus arteriosus repair by 25.7% in the administrative versus clinical data (7.01% versus 9.43%; p = 0.001) to an overestimation of mortality associated with ventricular septal defect (VSD) repair by 31.0% (0.78% versus 0.60%; p = 0.1). For the RACHS-1 categories, these ranged from an underestimation of category 5 mortality by 40.5% to an overestimation of category 2 mortality by 12.1%; these differences were not statistically significant. This study demonstrates differences in case ascertainment between administrative and clinical registry data for children undergoing cardiac operations, which translated into important differences in outcomes assessment.

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.

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.