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Certificación , Humanos , Niño , Cardiopatías/terapia , Competencia Clínica , Pediatría/educación , Pediatría/normas , Cuidados Críticos/normasAsunto(s)
Implantación de Prótesis de Válvulas Cardíacas , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Tetralogía de Fallot , Obstrucción del Flujo Ventricular Externo , Humanos , Válvula Pulmonar/cirugía , Tetralogía de Fallot/cirugía , Insuficiencia de la Válvula Pulmonar/cirugía , Resultado del Tratamiento , Implantación de Prótesis de Válvulas Cardíacas/métodos , Obstrucción del Flujo Ventricular Externo/cirugíaRESUMEN
BACKGROUND: Enteral ibuprofen was first approved as a prescription drug in 1974 for the US market. An intravenous (IV) ibuprofen formulation is approved for use in children older than 6 months of age, but there are limited studies specifically evaluating the pharmacokinetics and safety in children 1-6 months of age. AIMS: The primary purpose of this study was to evaluate the pharmacokinetics of IV ibuprofen in infants younger than 6 months of age. The secondary objective was to evaluate the safety of single and repeated doses of IV ibuprofen in infants younger than 6 months of age. METHODS: This was an industry-sponsored multi-center study. Institutional Review Board approval and informed parental consent were obtained prior to enrollment. Hospitalized neonates and infants younger than 6 months of age with fever or expected postoperative pain were eligible. Enrolled patients received 10 mg/kg of IV ibuprofen every 6 h, with up to four doses per day. Patients were randomized to two sparse sampling technique pharmacokinetic sample time groups. Group 1 samples were drawn at 0, 30 min, and 2 h, while group 2 samples were drawn at 0 min, 1, and 4 h after administration. RESULTS: A total of 24 children were enrolled in the study, with 15 male patients and 9 female patients. The median age of the cohort was 4.4 months (range 1.1-5.9 months), and the median weight was 5.9 kg (range 2.3-8.8 kg). The arithmetic mean and standard error for peak plasma ibuprofen concentration was 56.28 ± 2.77 µg/mL. Plasma levels declined rapidly with a mean elimination half-life of 1.30 h. Time to peak ibuprofen effect and concentration were similar when compared with older pediatric patients. Clearance and volume of distribution were also similar to those reported in older pediatric patients. No drug-related adverse events were reported. CONCLUSIONS: The pharmacokinetic and short-term safety profiles of IV ibuprofen in pediatric patients 1-6 months of age are comparable to those in children older than 6 months of age. TRIAL REGISTRATION: Clinicaltrials.gov Trial Registration number and date: NCT02583399-Registered July 2017.
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Fiebre , Ibuprofeno , Recién Nacido , Humanos , Masculino , Lactante , Femenino , Niño , Anciano , Ibuprofeno/efectos adversos , Dolor Postoperatorio/tratamiento farmacológico , Administración Intravenosa , Infusiones IntravenosasRESUMEN
We aimed to study the disparity in the clinical profile and outcomes of hospitalized Multisystem Inflammatory Syndrome in Children (MIS-C) patients at our center. The second goal was to examine the temporal association with preceding SARS-CoV-2 infection by race/ethnicity in our community in Mississippi. We found the racial disparity in the prevalence of MIS-C exceeded its temporal association with SARS-CoV-2 infections. We included 51 consecutive MIS-C patients hospitalized, whose median age was 9 (interquartile range [IQR] 5-12) years, 58% were male, 71% were black, 25% were white, and 4% belonged to other groups. We found a delay between onset of symptoms and hospitalization in black patients compared with white patients with a median of 2 (IQR 0-7) vs median of 0 (0-5) urgent care visits (P = .022), respectively. Black patients were hospitalized longer (median 8, IQR 2-39 days) than whites (median 5, IQR 3-14 days), P = .047. A total of 38.9% of blacks and 23.1% of whites were admitted to intensive care unit (P = .498); 36.1% of blacks had severe cardiac involvement vs 23.1% of white patients, P = .531. Future studies of MIS-C are required to improve health equity for children.
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COVID-19 , SARS-CoV-2 , Niño , Preescolar , Femenino , Humanos , Masculino , COVID-19/complicaciones , COVID-19/epidemiología , Etnicidad , Mississippi/epidemiologíaRESUMEN
Whole exome sequencing has identified an infant girl with fulminant dilated cardiomyopathy (DCM), leading to severe acute heart failure associated with ribosomal protein large 3-like (RPL3L) gene pathologic variants. Other genetic tests for mitochondrial disorders by sequence analysis and deletion testing of the mitochondrial genome were negative. Secondary causes for DCM due to metabolic and infectious etiologies were ruled out. She required a Berlin-Excor left ventricular assist device due to worsening of her heart failure as a bridge to orthotopic heart transplantation. At three months follow-up after heart transplantation, she has been doing well. We reviewed the literature on published RPL3L-related DCM cases and their outcomes. Bi-allelic variants in RPL3L have been reported in only seven patients from four unrelated families in the literature. RPL3L is a newer and likely pathogenic gene associated with a severe form of early-onset dilated cardiomyopathy with poor prognosis necessitating heart transplantation.
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Neonatal dilated cardiomyopathy (DCM) is rare with high etiologic heterogeneity. Recently, biallelic, autosomal recessive, pathogenic variants in RPL3L (ribosomal protein L3-like) have been reported in the literature with severe early-onset DCM. In the present brief report, we identified two pathogenic RPL3L variants, each harbored in unaffected heterozygous parents: mother (RPL3L c.1076_1080delCCGTG (p.Ala359Glyfs*4)) and father (RPL3L c.80G > A (p.Gly27Asp)). Pathogenic variants were segregated as autosomal recessive to two offspring born with compound heterozygous RPL3L variants and affected by neonatal DCM. This is the second report in the literature to the best of our knowledge and our findings support the pathogenicity of biallelic RPL3L pathologic variants associated with rapidly progressive neonatal DCM and heart failure with a poor prognosis.
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This is a cross-sectional study of 29 published cases of acute myopericarditis following COVID-19 mRNA vaccination. The most common presentation was chest pain within 1-5 days after the second dose of mRNA COVID-19 vaccination. All patients had an elevated troponin. Cardiac magnetic resonance imaging revealed late gadolinium enhancement consistent with myocarditis in 69% of cases. All patients recovered clinically rapidly within 1-3 weeks. Most patients were treated with non-steroidal anti-inflammatory drugs for symptomatic relief, and 4 received intravenous immune globulin and corticosteroids. We speculate a possible causal relationship between vaccine administration and myocarditis. The data from our analysis confirms that all myocarditis and pericarditis cases are mild and resolve within a few days to few weeks. The bottom line is that the risk of cardiac complications among children and adults due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection far exceeds the minimal and rare risks of vaccination-related transient myocardial or pericardial inflammation.
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BACKGROUND: Traditional palliation for biventricular cyanotic congenital heart lesions often involves staging with systemic-to-pulmonary arterial shunts to secure pulmonary blood flow (PBF) in the newborn period prior to complete repair. However, shunts may lead to life-threatening events secondary to shunt occlusion or acute coronary steal. They may be associated with morbidity secondary to diastolic runoff, systemic steal and volume loading, and do not provide pulsatile flow which has the potential to promote pulmonary artery (PA) growth. We have alternatively performed modified right ventricular outflow (mRVO) procedures by establishing antegrade right ventricle-to-PA flow. METHODS: Retrospective review of data on all patients who underwent the mRVO procedure from 2013 to 2016, including anatomy, number of interstage catheterizations, reoperations, intensive care unit admissions, hypercyanotic episodes, interval to complete repair, and mortality. RESULTS: Seventeen nonconsecutive patients included tetralogy of Fallot (n = 14), pulmonary valve stenosis (n = 2), and 1 with pulmonary atresia-intact septum; 14 had significant branch PA stenosis. Median age of first mRVO procedure was 14 days (range 5-193), and median duration of follow-up was 15.3 months (range 4-47 months). No patients had post-palliation acute hypercyanotic episodes. Nine were admitted to the ICU for persistent interstage hypoxemia, 7 of whom required reintervention prior to complete repair, which was achieved in 11 patients. Two late deaths unrelated to mRVO occurred. CONCLUSIONS: The mRVO procedure is a potential option with satisfactory results. It avoids potential shunt-related sudden death. The physiology of the mRVO palliation may provide unique benefits by providing antegrade pulsatile PBF, facilitates catheter interventions, and avoids branch PA distortion and stenosis.
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Tetralogía de Fallot/cirugía , Cianosis/cirugía , Femenino , Estudios de Seguimiento , Ventrículos Cardíacos/cirugía , Humanos , Recién Nacido , Masculino , Cuidados Paliativos , Arteria Pulmonar/cirugía , Atresia Pulmonar/cirugía , Estenosis de la Válvula Pulmonar/cirugía , Reoperación , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
The primary extracardiac inferior cavopulmonary connection is an unusual novel palliation for single-ventricle physiology, which we first performed in the setting of unfavourable upper-body systemic venous anatomy for a standard bi-directional Glenn, and in lieu of leaving our patient with shunt-dependent physiology. After an initial 16-month satisfactory follow-up, increasing cyanosis led to the discovery of a veno-venous collateral that was coiled, but, more importantly, to impressive growth of a previously diminutive superior caval vein, which allowed us to perform completion Fontan with a good outcome. Performing the single-ventricle staging in a reverse manner, first from below with a primary inferior cavopulmonary connection, followed by Fontan completion from above with a standard superior caval vein bi-directional Glenn, is also possible when deemed necessary.
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Procedimiento de Fontan/métodos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Angiografía Coronaria , Ventrículos Cardíacos/cirugía , Humanos , Lactante , Recién Nacido , Masculino , Arteria Pulmonar/cirugía , Resultado del Tratamiento , Vena Cava Superior/cirugíaRESUMEN
BACKGROUND: Antegrade cerebral perfusion (ACP) typically is used with deep hypothermia for cerebral protection during aortic arch reconstructions. The impact of ACP on cerebral oxygenation and serum creatinine at a more tepid 25 °C was studied in newborns and children. METHODS: Between 2010 and 2014, 61 newborns and children (<5 years old) underwent aortic arch reconstruction using moderate hypothermia (25.0±0.9 °C) with ACP and a pH-stat blood gas management strategy. These included 44% Norwood-type operations, 30% isolated arch reconstructions, and 26% arch reconstructions with other major procedures. Median patient age at surgery was 9 days (range, 3 days-4.7 years). Cerebral oxygenation (NIRS) was monitored continuously perioperatively for 120 hours. Serum creatinine was monitored daily. RESULTS: Median cardiopulmonary bypass (CPB) and cross clamp times were 181 minutes (range, 82-652 minutes) and 72 minutes (range, 10-364 minutes), respectively. ACP was performed at a mean flow rate of 46±6 mL/min/kg for a median of 48 minutes (range, 10-123 minutes). Cerebral and somatic NIRS were preserved intraoperatively and remained at baseline postoperatively during the first 120 hours. Peak postoperative serum creatinine levels averaged 0.7±0.3 mg/dL for all patients. There were 4 (6.6%) discharge mortalities. Six patients (9.8%) required ECMO support. Median postoperative length of hospital and intensive care unit (ICU) stay were 16 days(range, 4-104 days) and 9 days (range, 1-104 days), respectively. Two patients (3.3%) received short-term peritoneal dialysis for fluid removal, and none required hemodialysis. Three patients (4.9%) had an isolated seizure which resolved with medical therapy, and none had a neurologic deficit or stroke. CONCLUSIONS: ACP at 25 °C preserved perioperative cerebral oxygenation and serum creatinine for newborns and children undergoing arch reconstruction. Early outcomes are encouraging, and additional study is warranted to assess the impact on late outcomes.
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Cardiopatías Congénitas/diagnóstico , Tamizaje Neonatal , Algoritmos , Humanos , Recién Nacido , OximetríaRESUMEN
The superior cavopulmonary anastomosis - bi-directional Glenn - is the standard palliation for single ventricle physiology. When upper body systemic venous anatomic concerns such as superior caval vein stenosis, hypoplasia, or inadequate collateral tributaries are present, a Glenn may be precluded or have a high risk of poor outcome. A primary inferior cavopulmonary connection with an extracardiac conduit is an alternative palliation that provides a generous pathway for pulmonary blood flow, with the additional benefit of including hepatic venous return. We report a case of primary extracardiac inferior cavopulmonary connection in a patient unsuitable for Glenn, with successful post-operative outcome and early follow-up.
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Procedimiento de Fontan , Puente Cardíaco Derecho/métodos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Arteria Pulmonar/cirugía , Vena Cava Superior/cirugía , Angiografía por Tomografía Computarizada , Hemodinámica , Humanos , Lactante , Masculino , Periodo PosoperatorioRESUMEN
Earlier attempts at percutaneous closure of perimembranous ventricular septal defects (Pm VSDs) were abandoned because of incidence of heart block likely as a result of device rigidity and/or oversizing. This is retrospective review and data reporting of patients who underwent percutaneous closure using the softer second-generation Amplatzer vascular occluders; namely the Amplatzer vascular plug, second generation, (AVP II) and the Amplatzer duct occluder, second generation (ADO II) in our institution. A total of 20 patients were identified; AVP II was used in 9 patients and ADO II in 11 patients. Median weight was 13.45 kg (range 6.5 to 76); age 28.5 months (range 11 to 352). After procedure, 4 were noted to have aortic insufficiency; trivial in 3 and mild in 1 (unrelated to the device). Mild tricuspid regurgitation possibly device or procedure related was seen in 4. Residual flow through the device was common after procedure and disappeared in all but 3, graded as trivial in 1, small in 2. Average follow-up period was 7.54 months ± 7.5 (1 day to 25 months). There was no incidence of heart block, bacterial endocarditis, hemolysis, device embolization, or fracture. The aortic insufficiency resolved in 1 patient and was estimated to be trivial in the remaining 3 patients. In conclusion, percutaneous closure of Pm VSDs using the softer new generation devices as the AVP II and the ADO II is feasible and safe. Longer follow-up and larger series are needed.
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Cateterismo Cardíaco/métodos , Procedimientos Quirúrgicos Cardíacos/métodos , Defectos del Tabique Interventricular/cirugía , Dispositivo Oclusor Septal , Adolescente , Adulto , Niño , Preescolar , Ecocardiografía , Diseño de Equipo , Femenino , Estudios de Seguimiento , Defectos del Tabique Interventricular/diagnóstico , Humanos , Lactante , Masculino , Radiografía Torácica , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
INTRODUCTION: Debilitating patient-related non-cardiac co-morbidity cumulatively increases risk for congenital heart surgery. At our emerging programme, flexible surgical strategies were used in high-risk neonates and infants generally considered in-operable, in an attempt to make them surgical candidates and achieve excellent outcomes. MATERIALS AND METHODS: Between April, 2010 and November, 2013, all referred neonates (142) and infants (300) (average scores: RACHS 2.8 and STAT 3.0) underwent 442 primary cardiac operations: patients with bi-ventricular lesions underwent standard (n=294) or alternative (n=19) repair/staging strategies, such as pulmonary artery banding(s), ductal stenting, right outflow patching, etc. Patients with uni-ventricular hearts followed standard (n=96) or alternative hybrid (n=34) staging. The impact of major pre-operative risk factors (37%), standard or alternative surgical strategy, prematurity (50%), gestational age, low birth weight, genetic syndromes (23%), and major non-cardiac co-morbidity requiring same admission surgery (27%) was analysed on the need for extracorporeal membrane oxygenation, mortality, length of intubation, as well as ICU and hospital length of stays. RESULTS: The need for extracorporeal membrane oxygenation (8%) and hospital survival (94%) varied significantly between surgical strategy groups (p=0.0083 and 0.028, respectively). In high-risk patients, alternative bi- and uni-ventricular strategies minimised mortality, but were associated with prolonged intubation and ICU stay. Major pre-operative risk factors and lower weight at surgery significantly correlated with prolonged intubation, hospital length of stay, and mortality. DISCUSSION: In our emerging programme, flexible surgical strategies were offered to 53/442 high-risk neonates and infants with complex CHDs and significant non-cardiac co-morbidity, in order to buffer risk and achieve patient survival, although at the cost of increased resource utilisation.
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Cardiopatías Congénitas/cirugía , Mortalidad Hospitalaria , Recién Nacido de Bajo Peso , Recien Nacido Prematuro , Procedimientos Quirúrgicos Vasculares/efectos adversos , Comorbilidad , Femenino , Edad Gestacional , Humanos , Lactante , Recién Nacido , Unidades de Cuidados Intensivos , Tiempo de Internación , Modelos Lineales , Masculino , Mississippi , Factores de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: Management of systemic semilunar valve disease in growing, young patients is challenging. When replacement is necessary, use of a pulmonary autograft is sometimes not possible for anatomic, pathologic, or technical reasons or due to parental or patient preference. We employed a stentless, porcine, full-root bioprosthesis in this setting and report our outcomes. METHODS: Over 9 years (2005 to 2013), 24 patients of mean age 13.1 years (range, 3 months to 20.3 years) underwent operation for mixed stenosis and insufficiency in 16 of 24 (67%), pure insufficiency in 7 of 24 (29%), and pure stenosis in 1 of 24 (4%). Twenty patients had previous interventions of repair or replacement, valvuloplasty, or multiple operations. Survival, follow-up echocardiographic findings, and outcomes were documented. All patients were maintained on daily aspirin. RESULTS: There were no hospital deaths and no early or late deaths over a mean follow-up for 23 patients of 46.1 months (range, 14 months to 9.2 years). One patient moved abroad and was lost to follow-up. Echocardiographic follow-up (mean 34.0 months) demonstrated that no patient developed more than mild insufficiency or moderate stenosis. In total, 20 of 24 (83%) showed no insufficiency and 11 of 24 patients (46%) showed no stenosis. Near or complete normalization of left ventricular mass and dimension was demonstrated. There were no explants and no thromboembolic or bleeding events. CONCLUSIONS: When use of a pulmonary autograft is not an option, the porcine full-root bioprosthesis appears favorable for systemic semilunar valve replacement in the pediatric and young adult population. Of note, when prosthetic degeneration does occur, stenosis predominates rather than insufficiency. Longer term studies are warranted.
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Válvula Aórtica/cirugía , Bioprótesis , Enfermedades de las Válvulas Cardíacas/cirugía , Prótesis Valvulares Cardíacas , Válvula Pulmonar/cirugía , Adolescente , Animales , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Diseño de Prótesis , Reimplantación , Estudios Retrospectivos , Porcinos , Adulto JovenRESUMEN
BACKGROUND: Results of surgical management of hypoplastic left heart syndrome (HLHS) and related anomalies are often compared to published benchmark data which reflect the use of a variety of surgical and hybrid protocols. We report encouraging results achieved in an emerging program, despite a learning curve at all care levels. Rather than relying on a single preferred protocol, surgical management was based on matching surgical strategy to individual patient factors. METHODS: From 2010 to 2014, a total of 47 consecutive patients with HLHS or related anomalies with ductal-dependent systemic circulation underwent initial surgical palliation, including 30 Norwood stage I, 8 hybrid stage I, and 9 salvage-to-Norwood procedures. True hybrid procedures entailed bilateral pulmonary artery banding and ductal stenting. In the salvage-to-Norwood strategy, ductal stenting was withheld in favor of continued prostaglandin infusion in anticipation of a deferred Norwood procedure. Cardiac comorbidities (obstructed pulmonary venous return, poor ventricular function, and atrioventricular valve regurgitation) and noncardiac comorbidities influenced the choice of treatment strategies and were analyzed as potential risk factors for extracorporeal membrane oxygenation (ECMO) support or in-hospital mortality. RESULTS: Overall hospital survival was 81% (Norwood 83.3%, hybrid 88%, "salvage" 67%; P = .4942). Extracorporeal membrane oxygenation support was used for eight (17%) patients with two survivors. For cases with obstructed pulmonary venous return (n = 10, 21%), management choices favored a hybrid or salvage strategy (P = .0026). Aortic atresia (n = 22, 47%) was treated by a Norwood or salvage-to-Norwood. No cardiac, noncardiac, or genetic comorbidities were identified as independent risk factors for ECMO or discharge mortality in a multivariable analysis. CONCLUSIONS: Our emerging program achieved outcomes that compare favorably to published benchmark data with respect to hospital survival. These results reflect rigorous interdisciplinary teamwork and a flexible approach to surgical palliation based on matching surgical strategy to patient factors. With major associated cardiac/noncardiac comorbidity and antegrade coronary flow, a true hybrid with ductal stenting was our preferred strategy. For high-risk situations such as aortic atresia with obstructed pulmonary venous return, the salvage hybrid-bridge-to-Norwood strategy may help achieve survival albeit with increased resource utilization.
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Benchmarking , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Cuidados Paliativos/métodos , Válvula Aórtica/cirugía , Enfermedad de la Válvula Aórtica Bicúspide , Femenino , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/cirugía , Enfermedades de las Válvulas Cardíacas/mortalidad , Enfermedades de las Válvulas Cardíacas/cirugía , Mortalidad Hospitalaria , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/mortalidad , Lactante , Tiempo de Internación , Masculino , Procedimientos de Norwood/métodos , Procedimientos de Norwood/mortalidad , Complicaciones Posoperatorias/etiología , Arteria Pulmonar/anomalías , Arteria Pulmonar/cirugía , Factores de Riesgo , Stents , Procedimientos Quirúrgicos VascularesAsunto(s)
Centros Médicos Académicos/estadística & datos numéricos , Procedimientos Quirúrgicos Cardíacos/estadística & datos numéricos , Cardiopatías Congénitas/cirugía , Calidad de la Atención de Salud/estadística & datos numéricos , Humanos , Tiempo de Internación , Mississippi , Complicaciones Posoperatorias , Indicadores de Calidad de la Atención de SaludRESUMEN
Interindividual variation in the ability of aspirin to inhibit platelet cyclooxygenase-1 (COX-1) could account for some on-treatment cardiovascular events. Here, we sought to determine whether there are clinical phenotypes that are associated with a suboptimal pharmacological effect of aspirin. In a prospective, 2-week study, we evaluated the effect of aspirin (81 mg) on platelet COX-1 in 135 patients with stable coronary artery disease by measuring serum thromboxane B(2) (sTxB(2)) as an indicator of inhibition of platelet COX-1. A nested randomized study compared enteric-coated with immediate-release formulations of aspirin. We found that sTxB(2) was systematically higher among the 83 patients with metabolic syndrome than among the 52 patients without (median: 4.0 versus 3.02 ng/mL; P=0.013). Twelve patients (14%) with metabolic syndrome, but none without metabolic syndrome, had sTxB(2) levels consistent with inadequate inhibition of COX (sTxB(2) ≥13 ng/mL). In linear regression models, metabolic syndrome (but none of its individual components) significantly associated with higher levels of log-transformed sTxB(2) (P=0.006). Higher levels of sTxB(2) associated with greater residual platelet function measured by aggregometry-based methods. Among the randomized subset, sTxB(2) levels were systematically higher among patients receiving enteric-coated aspirin. Last, urinary 11-dehydro thromboxane B(2) did not correlate with sTxB(2), suggesting that the former should not be used to quantitate aspirin's pharmacological effect on platelets. In conclusion, metabolic syndrome, which places patients at high risk for thrombotic cardiovascular events, strongly and uniquely associates with less effective inhibition of platelet COX-1 by aspirin.