Browsing by Subject "truncus arteriosus"

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    Characteristics and Operative Outcomes for Children Undergoing Repair of Truncus Arteriosus: A Contemporary Multicenter Analysis
    (Elsevier, 2019) Mastropietro, Christopher W.; Amula, Venu; Sassalos, Peter; Buckley, Jason R.; Smerling, Arthur J.; Iliopoulos, Illias; Riley, Christine M.; Jennings, Aimee; Cashen, Katherine; Narasimhulu, Sukumar Suguna; Gowda, Keshava Murty Narayana; Bakar, Adnan M.; Wilhelm, Michael; Badheka, Aditya; Moser, Elizabeth A. S.; Costello, John M.; Pediatrics, School of Medicine
    Objective We sought to describe characteristics and operative outcomes of children who underwent repair of truncus arteriosus and identify risk factors for the occurrence of major adverse cardiac events (MACE) in the immediate postoperative period in a contemporary multicenter cohort. Methods We conducted a retrospective review of children who underwent repair of truncus arteriosus between 2009 and 2016 at 15 centers within the United States. Patients with associated interrupted or obstructed aortic arch were excluded. MACE was defined as the need for postoperative extracorporeal membrane oxygenation (ECMO), cardiopulmonary resuscitation (CPR), or operative mortality. Risk factors for MACE were identified using multivariable logistic regression analysis and reported as odds ratios (OR) with 95% confidence intervals (CI). Results We reviewed 216 patients. MACE occurred in 44 patients (20%) and did not vary significantly over time. Twenty-two patients (10%) received postoperative ECMO, 26 (12%) received CPR, and 15 (7%) suffered operative mortality. With multivariable logistic regression analysis (which included adjustment for center effect), factors independently associated with MACE were failure to diagnose truncus arteriosus prior to discharge from the nursery (OR:3.1; 95%CI:1.3,7.4), cardiopulmonary bypass duration greater than 150 minutes (OR:3.5; 95%CI:1.5,8.5), and right ventricle-to-pulmonary artery conduit diameter greater than 50mm/m2 (OR:4.7; 95%CI:2.0,11.1). Conclusions In a contemporary multicenter analysis, 20% of children undergoing repair of truncus arteriosus experienced MACE. Early diagnosis, shorter duration of cardiopulmonary bypass, and use of smaller diameter right ventricle-to-pulmonary artery conduits represent potentially modifiable factors that could decrease morbidity and mortality in this fragile patient population.
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    Molecular and Genetic Insights into Thoracic Aortic Dilation in Conotruncal Heart Defects
    (Frontiers Media SA, 2016) Kay, W. Aaron; Department of Medicine, IU School of Medicine
    Thoracic aortic dilation (AD) has commonly been described in conotruncal defects (CTDs), such as tetralogy of Fallot, double outlet right ventricle and transposition of the great arteries, and truncus arteriosus. Several theories for this have been devised, but fairly recent data indicate that there is likely an underlying histologic abnormality, similar to that seen in Marfan and other connective tissue disease. The majority of aortic dissection in the general population occurs after the age of 45 years, and there have been very few case reports of aortic dissection in CTD. Given advances in cardiac surgery and increasing survival over the past several decades, there has been rising concern that, as patients who have survived surgical correction of these defects age, there may be increased morbidity and mortality due to aortic dissection and aortic regurgitation. This review discusses the most recent developments in research into AD in CTD, including associated genetic mutations.
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    Multicenter Analysis of Early Childhood Outcomes Following Repair of Truncus Arteriosus
    (Elsevier, 2018) Buckley, Jason R.; Amula, Venu; Sassalos, Peter; Costello, John M.; Smerling, Arthur J.; Iliopoulos, lias; Jennings, Aimee; Riley, Christine M.; Cashen, Katherine; Narasimhulu, Sukumar Suguna; Gowda, Keshava Murthy Narayana; Bakar, Adnan M.; Wilhelm, Michael; Badheka, Aditya; Moser, Elizabeth A. S.; Mastropietro, Christopher W.; Biostatistics, School of Public Health
    Background Literature describing morbidity and mortality following truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population. Methods We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after surgery. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modelling. Results We reviewed 216 patients, with median follow-up of 2.9 years (range:0.1-8.8). Operative mortaility occurred in 15 patients (7%). Of the 201 survivors, there were 14 (7%) late deaths. DiGeorge syndrome (HR:5.4; 95%CI:1.6-17.8) and need for postoperative tracheostomy (HR:5.9; 95%CI:1.8-19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention:23 months, range:0.3-93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra® bovine jugular vein conduits (HR:1.9; 95%CI:1.2,3.1) and smaller conduit size (HR per mm/m2:1.05; 95%CI:1.03,1.08). Conclusions In a multicenter dataset, DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, while risk of conduit reintervention was independently influenced by both initial conduit type and size.