Clinical Presentation and Therapy of Truncus Arteriosus.

Truncus arteriosus (TA, also known as common arterial trunk) consists of only one great artery ("the truncus") with a semilunar valve (truncus valve) arising from the heart and an additional ventricular septal defect and (Fig. 50.1). This great artery is positioned above the ventricular septal defect and gives rise to the coronary arteries, the pulmonary arteries, and the aortic arch. Historically, TA has been classified by Collet and Edwards in three types, where in type I there was a common pulmonary artery truncus, in type II the left and right PA arise separately but close to each other, in type III both PA arise independently; in addition, there was a type IV that was later characterized as pulmonary atresia with VSD and major aortopulmonary collateral arteries arising from the descending aorta.

Survival After Single-Stage Repair of Truncus Arteriosus and Associated Defects.

BACKGROUND: The goal of this study was to describe in-hospital and long-term mortality after single-stage repair of truncus arteriosus communis (TAC) and explore factors associated with these outcomes. METHODS: This was a cohort study of consecutive patients undergoing single-stage TAC repair between 1982 and 2011 reported to the Pediatric Cardiac Care Consortium registry. In-hospital mortality was obtained for the entire cohort from registry records. Long-term mortality was obtained for patients with available identifiers by matching with the National Death Index through 2020. Kaplan-Meier survival estimates were created for up to 30 years after discharge. Cox regression models estimated hazard ratios for the associations with potential risk factors. RESULTS: A total of 647 patients (51% male) underwent single-stage TAC repair at a median age of 18 days; 53% had type I TAC, 13% had interrupted aortic arch, and 10% underwent concomitant truncal valve surgery. Of these, 486 (75%) patients survived to hospital discharge. After discharge, 215 patients had identifiers for tracking long-term outcomes; 30-year survival was 78%. Concomitant truncal valve surgery at the index procedure was associated with increased in-hospital and 30-year mortality. Concomitant interrupted aortic arch repair was not associated with increased in-hospital or 30-year mortality. CONCLUSIONS: Concomitant truncal valve surgery but not interrupted aortic arch was associated with higher in-hospital and long-term mortality. Careful consideration of the need and timing for truncal valve intervention may improve TAC outcomes.

Right Ventricular Outflow Tract Reconstruction in Truncus Arteriosus: A 30-Year Two-Center Comparison between Homografts and Bovine Jugular Vein.

INTRODUCTION: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. METHODS: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. RESULTS: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). CONCLUSION: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

Outcomes in Primary Repair of Truncus Arteriosus with Significant Truncal Valve Insufficiency: A Systematic Review and Meta-analysis.

Data regarding the effect of significant TVI on outcomes after truncus arteriosus (TA) repair are limited. The aim of this meta-analysis was to summarize outcomes among patients aged ≤ 24 months undergoing TA repair with at least moderate TVI. A systematic literature search was conducted in PubMed, Scopus, and CINAHL Complete from database inception through June 1, 2022. Studies reporting outcomes of TA repair in patients with moderate or greater TVI were included. Studies reporting outcomes only for patients aged > 24 months were excluded. The primary outcome was overall mortality, and secondary outcomes included early mortality and truncal valve reoperation. Random-effects models were used to estimate pooled effects. Assessment for bias was performed using funnel plots and Egger's tests. Twenty-two single-center observational studies were included for analysis, representing 1,172 patients. Of these, 232 (19.8%) had moderate or greater TVI. Meta-analysis demonstrated a pooled overall mortality of 28.0% after TA repair among patients with significant TVI with a relative risk of 1.70 (95% CI [1.27-2.28], p < 0.001) compared to patients without TVI. Significant TVI was also significantly associated with an increased risk for early mortality (RR 2.04; 95% CI [1.36-3.06], p < 0.001) and truncal valve reoperation (RR 3.90; 95% CI [1.40-10.90], p = 0.010). Moderate or greater TVI before TA repair is associated with an increased risk for mortality and truncal valve reoperation. Management of TVI in patients remains a challenging clinical problem. Further investigation is needed to assess the risk of concomitant truncal valve surgery with TA repair in this population.

Transapical Delivery of a Sapien Valve for Transcatheter Aortic Valve Replacement in an 11-Year-Old Patient with Truncus Arteriosus.

Complex congenital heart defects may necessitate repeated surgical interventions throughout a patient's lifetime. Each subsequent procedure exposes patients to a greater cumulative risk, thus adding to the potential morbidity and mortality of the surgery. Transcatheter interventions can help mitigate the surgical risk for many defects and can delay or mitigate the need for surgery. This case report describes the rare use of a transapically delivered transcatheter aortic valve replacement (TAVR) therapy in a high-risk pediatric patient to postpone the need for surgery and potentially reduce the number of lifelong surgical interventions. The case highlights how transcatheter aortic valve therapies can be considered for non-standard, higher risk pediatric patients to postpone the need for surgical valve replacement and may serve as a paradigm shift in the care of complex patients with aortic valve pathology.

Use of a QDOT MICRO catheter to identify a premature ventricular contraction origin in the right ventricular anterior papillary muscle in a repaired truncus arteriosus.

The ventricular papillary muscles (VPMs) can be a source of premature ventricular contractions (PVCs). Catheter ablation of VPM PVCs is challenging because of the anatomical complexity, such as the apical structures in proximity to the ventricular walls. The QDOT MICRO catheter (Biosense Webster, Diamond Bar, CA, USA) has microelectrodes embedded along the circumference of its distal tip and can provide information on which side of its tip myocardial activation is earlier. This repaired truncus arteriosus case demonstrates the usefulness of the microelectrode recording in identifying a PVC origin in a right VPM apex close to the right ventricular anterior wall.

Natural History of Truncal Root Dilatation and Truncal Valve Regurgitation in Truncus Arteriosus.

BACKGROUND: The natural history of the dilated truncal root in repaired truncus arteriosus (TA) is incompletely understood. METHODS: A single-center review of patients who underwent TA repair between January 1984 and December 2018 was performed. Echocardiographically determined root diameters and derived z scores were measured at the annulus, sinus of Valsalva (SoV), and sinutubular junction (STJ) immediately before TA repair and throughout follow-up. Linear mixed-effects models assessed trends in root dimensions over time. RESULTS: Of 193 patients who underwent TA repair at a median age of 12 days (interquartile range, 6-48 days) and survived to discharge, 34 (17.6%), 110 (57.0%), and 49 (25.4%) patients had bicuspid, tricuspid, and quadricuspid truncal valves, respectively. Median postoperative follow-up was 11.6 years (interquartile range, 4.4-22.0 years; range, 0.1-34.8 years). Truncal valve or root intervention was required in 38 patients (19.7%). The mean rates of annular, SoV, and STJ growth were 0.7 ± 0.3 mm/y, 0.8 ± 0.5 mm/y, and 0.9 ± 0.4 mm/y, respectively. Root z scores remained stable with time. At baseline, compared with patients with tricuspid leaflet anatomy, bicuspid patients had larger diameters at the SoV (P = .003) and STJ (P = .029), whereas quadricuspid patients had larger STJ diameters (P = .004). Over time, the bicuspid and quadricuspid cohorts demonstrated comparatively greater annular dilatation (both P < .05). Patients with ≥75th percentile root growth rates had a higher incidence of moderate-severe truncal regurgitation (P = .019) and truncal valve intervention (P = .002). CONCLUSIONS: Root dilatation in TA persisted for up to 30 years after primary repair. Patients with bicuspid and quadricuspid truncal valves demonstrated greater root dilatation over time and required more valve interventions. Continued longitudinal follow-up is warranted in this higher-risk cohort.

Congenital Aortic Valve Repair When the Options aren't Good: Truncus Arteriosus and Transposition of the Great Arteries.

Patients with truncus arteriosus and transposition of great arteries are prone to neo-aortic valve insufficiency. Although presenting at opposite ends of the age spectrum, both conditions tend to be commonly associated with neo aortic root dilatation. In patients with truncus arteriosus there is an additional complexity of quadricuspid valve morphology, which make up the majority of valves requiring repair. A unified approach to all these patients would include reduction and stabilization of the annulus and sinotubular junction, as well as achieving equal and symmetrical coaptation of the valve leaflets. By systematically employing these techniques, valve replacement should be avoidable in most children.

Non-Conduit Repair of Truncus Arteriosus.

INTRODUCTION: The procedure of choice for treatment of truncus arteriosus is one-stage repair within the first few months of life. Establishing right ventricle-pulmonary artery direct continuity without conduit can be a good alternative in the absence of valved conduits in developing centers. METHODS: Between January 2021 and June 2021, a total of five patients (three males, two females) underwent definitive repair of truncus arteriosus without an extracardiac conduit. We used the Barbero-Marcial technique to allow age-related growth, eliminate the risk of conduit-related complications, and to avoid forcing a conduit to place in a very small mediastinal space. RESULTS: The patients' mean age was 31.2 days (11-54 days). Their mean bodyweight was 3.2 kg (2.7-3.8kg). Mean postoperative intensive care unit stay was 39.6 days (7-99 days). There were two mortalities in the intensive care unit on postoperative days 12 and 61 due to lung-related problems. The remaining three cases' mean ventilation time was 15.6 days (8-22 days). CONCLUSION: Having access to a valved conduit is still challenging for some centers, and the non-conduit repair technique defined by Barbero-Marcial can be a successful, life-saving alternative easy for young surgeons to perform in newly based centers.

Contemporary Multicenter Outcomes for Truncus Arteriosus With Interrupted Aortic Arch.

BACKGROUND: Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited. METHODS: A retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed. Major adverse cardiac events (MACE) were defined as postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. TA-IAA patients were compared with TA patients who underwent repair during the study period from the same institutions. RESULTS: We reviewed 35 patients with TA-IAA. MACE occurred in 12 patients (34%). Improvement over time was observed during the study period with 11 events (92%) occurring in the first half of the study period (P = .03). Factors associated with MACE included moderate or severe truncal valve insufficiency (P < .01), concomitant truncal valve repair (P = .04), and longer cardiopulmonary bypass duration (P = .02). In comparison with 216 patients who underwent TA repair, patients with TA-IAA had a higher rate of MACE, but this finding was not statistically significant (34% vs 20%, respectively; P = .07). Additionally no differences between TA-IAA and TA groups were observed for unplanned reoperations (14% vs 22%, respectively; P = .3), hospital length of stay (24 vs 23 days, P = .65), or late deaths (7% vs 7%, P = 1.00). CONCLUSIONS: In this contemporary, multicenter cohort the rate of MACE after repair of TA-IAA was high but improved during the study period. Early childhood outcomes of patients with TA-IAA were similar to those with TA.

Modified Barbero-Marcial repair for type I truncus arteriosus in a 2.7-kg neonate.

Truncus arteriosus is a rare cardiac anomaly, accounting for less than 4% of all congenital lesions. It is the result of failed aorticopulmonary septation during the fifth week of gestation leading to a single arterial trunk overriding the interventricular septum, a single semilunar valve, and typically a large conotruncal ventricular septal defect. Several classifications exist, and it typically requires surgical repair in the neonatal period. We present a 5-day old female neonate who was diagnosed postnatally with type I truncus arteriosus in which the pulmonary arteries arose from a discrete pulmonary trunk that originated from the posterolateral aspect of the common arterial trunk. A successful repair was undertaken using a variant of the Barbero-Marcial technique.

Acyanotic Truncus Arteriosus: Not a Misnomer But a True Rarity.

Truncus arteriosus, also referred to as common arterial trunk (CAT), is generally classified as a cyanotic congenital heart disease characterized by a single arterial trunk arising from the heart and supplying both pulmonary and systemic circulations. Cyanosis exists by virtue of it being an admixture lesion. We report a 13-year-old boy diagnosed to have type 1 CAT who was acyanotic at presentation and had all features of an operable lesion even at this age. He underwent a successful repair with closure of the subtruncal VSD and insertion of a hand-sewn valved right ventricle-to-pulmonary artery conduit made of bovine pericardium and Gore-Tex membrane.

Outcome of truncus arteriosus repair: 20 years of single-center experience comparing early versus late surgical repair.

BACKGROUND: Truncus arteriosus is a rare CHD. Neonatal and early infancy repair is recommended though some cases may present late. The aim of our study is to investigate the current results of truncus arteriosus repair and to analyse the differences in outcome and reintervention need between early versus late truncus arteriosus surgical repair. MATERIAL AND METHODS: In this cohort study, we reviewed all children who underwent truncus arteriosus repair from 2001 till 2021. We divided patients into two groups; early repair group including patients repaired at age less than 3 months and late repair group including patients who had repair at 3 months of age and later. We compared both groups for outcome variables. RESULTS: Sixty-four children had truncus arteriosus repair including 48(75%) patients in early repair and 16(25%) patients in late repair groups. Peri-operative course was comparable between both groups. Post-surgery, we observed pulmonary hypertension in 6(12%) patients in early repair group comparing with 11(69%) patients in late repair group (p = 0.0001). In the last follow-up visit, pulmonary hypertension resolved in all early repair group patients while 6(37.5%) patients in late repair group continued to have pulmonary hypertension (p = 0.0001). Twenty-three(36%) patients required reintervention including 22(48%) in early repair group versus 1(6%) in late repair group (p = 0.007). CONCLUSION: In general, the outcome of early truncus arteriosus repair is excellent with resolution of pulmonary hypertension following early repair. Late repair caries higher risk of persistent pulmonary hypertension (37.5%). About one-third of the patients who had truncus arteriosus repair will require re-intervention within 38±38.4 months after initial surgery.

Contemporary patterns of the management of truncus arteriosus (primary versus staged repair): outcomes from the Japanese National Cardiovascular Database.

OBJECTIVES: Although primary repair in early infancy has for decades been the prevalent strategy for management of truncus arteriosus (TA), recent concerns about the levels of morbidity and mortality have led to consideration of a staged surgical approach. Our goal was to describe recent patterns of management, to characterize patients who underwent primary or staged repair and to evaluate risk factors associated with operative mortality in a contemporary multicentre cohort. METHODS: In the Japanese Cardiovascular Surgery Database, we identified all cases of TA undergoing an initial surgical procedure from 2008 to 2018. Operative mortality was defined as death within 30 days of an operation or in-hospital death regardless of the length of hospital stay. The hospital volume was defined by the average volume of TA repairs per year. RESULTS: The total number of patients undergoing initial surgery for TA was 286. Sixty-eight (24%, 68/286) underwent primary repair (primary repair group). The remaining 218 (76%, 218/286) underwent initial bilateral pulmonary artery banding as part of a planned staged approach (staged repair group). One hundred sixty-two patients out of 218 initially banded patients underwent the repair of TA during this study period. Concomitant diagnoses in the entire cohort included interrupted aortic arch repair in 36 patients and truncal valve regurgitation in 32. No centres handling an average of ≥2 truncus cases/year of the repair of TA were identified in this cohort. A total of 30% (85/286) of the cases were performed at centres that handled an average of ≥1 and <2 cases/year. The remaining 70% were at centres with <1 case/year. Overall, 37 patients (12.9%; 37/286) died. The operative mortality rates in the primary and staged repair groups were similar: that for the primary repair group was 16.2% (11/68) versus 11.9% for the staged repair group (26/218; P = 0.41). With multivariable logistic regression analysis, the factors most strongly associated with operative mortality were preoperative heart failure requiring catecholamine support (odds ratio, 4.18; 95% confidence interval 1.96-8.96) and the repeat bilateral pulmonary artery banding (odds ratio, 3.89; 95% confidence interval 1.08-14.07). CONCLUSIONS: The staged repair of TA has emerged as the preferred option for surgical timing at most of the centres participating in the Japanese Cardiovascular Surgery Database. The management outcomes of the patients with TA were favourable, even for the patients at low-volume centres.

Periocardiophrenic Release to Prevent Conduit Compression After Neonatal Truncus Arteriosus Repair.

Sternal approximation postcardiac surgery in neonates can sometimes be challenging. Neonatal truncus arteriosus repair using a right ventricle-to-pulmonary artery homograft conduit is one such surgical procedure wherein there is a risk of developing conduit compression after sternal closure. We describe our technique of pericardiophrenic release at the time of delayed sternal closure to prevent hemodynamic compromise and conduit compression after sternal approximation in such cases.

Right Ventricle to Pulmonary Artery Conduit Size Is Associated with Conduit and Pulmonary Artery Reinterventions After Truncus Arteriosus Repair.

We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p<.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality.