Claudio Ramaciotti

Children's Medical Center Dallas, Dallas, Texas, United States

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Publications (43)156.89 Total impact

  • Journal of Thoracic and Cardiovascular Surgery 09/2014; DOI:10.1016/j.jtcvs.2014.09.006 · 3.99 Impact Factor
  • Poonam P Thankavel, Matthew S Lemler, Claudio Ramaciotti
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    ABSTRACT: Anomalous aortic origin of coronaries from the contralateral sinus (AAOCA) is rare but an important cause of cardiac death in the otherwise healthy young athlete. This necessitates prompt, accurate identification; transthoracic echocardiography (TTE) remains the primary screening tool. Assessment of accuracy is difficult since the true prevalence of the disease is unknown, with estimates at 0.3-1.07 %. The incidence by TTE remains much lower, between 0.09 and 0.17 % even with sophisticated equipment and a high index of suspicion. Our goal was to incorporate two new screening views to our standard TTE protocol and assess improvement in diagnosis of AAOCA by TTE in our laboratory. Recently (2011), we incorporated two new screening methods to standard protocol. The parasternal short axis sweep is extended to visualize the anomalous segment arising superiorly from the ascending aorta before exiting the root at a site close to a 'normal' origin. Secondly, the anomalous, interarterial coronary demonstrates an anterior, steep-angled course visualized in the parasternal long axis between the aorta and pulmonary artery. The echocardiogram database was searched for patients newly diagnosed with AAOCA in 2010 (prior to incorporation of new methods) and 2012. AAOCA incidence in our patient population improved from 0.02 % (2010) to 0.22 % (2012), age range from 4 days to 17 years. Teenagers and symptomatic patients with anomalous right coronary origin (6) underwent additional confirmatory imaging, and three underwent surgery. One patient with anomalous left coronary origin underwent surgical repair. Addition of the screening views can significantly increase the sensitivity of TTE in diagnosing AAOCA in the asymptomatic patients. We propose that these views be incorporated into the standard TTE evaluation of coronary arteries.
    Pediatric Cardiology 08/2014; 36(1). DOI:10.1007/s00246-014-0973-8 · 1.55 Impact Factor
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    ABSTRACT: Mini-Abstract Double-outlet right atrium, also known as straddling tricuspid valve without a ventricular septal defect, is an extremely rare congenital cardiac anomaly, in which the tricuspid valve has 2 ostia. We describe an infant with this finding in association with hypoplastic right heart structures and a dysplastic aortic valve.
    Echocardiography 03/2014; 31(5). DOI:10.1111/echo.12565 · 1.25 Impact Factor
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    ABSTRACT: An unruptured sinus of Valsalva aneurysm is usually asymptomatic, and a rare, often incidental echocardiographic diagnosis. Its presentation in the neonatal period is extremely rare. We describe this finding in a neonate with hypoplastic left heart syndrome, which has not been previously reported in the literature. This constellation of findings may have important clinical implications in regards to medical management and surgical options. Mini-Abstract An unruptured sinus of Valsalva aneurysm is usually asymptomatic, and a rare, often incidental echocardiographic diagnosis. Its presentation in the neonatal period is extremely rare. We describe this finding in a neonate with hypoplastic left heart syndrome, which has not been previously reported in the literature. This constellation of findings may have important clinical implications.
    Echocardiography 12/2013; 31(3). DOI:10.1111/echo.12469 · 1.25 Impact Factor
  • Poonam P Thankavel, Arshid Mir, Claudio Ramaciotti
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    ABSTRACT: Background: Myocardial injury in previously healthy children is rare, with a wide range of aetiologies. It is increasingly being identified on the basis of elevated troponin levels during routine evaluation of cardiorespiratory symptoms. Establishing the aetiology remains challenging because of the lack of an accepted work-up algorithm. Our objective was to delineate the contribution of diagnostic modalities and troponin patterns towards the final diagnosis. Methods: A retrospective chart review of previously healthy patients admitted to the Pediatric Cardiology Service with myocardial injury was carried out. Data analysed included echocardiograms, electrocardiograms, cardiac catheterisations, magnetic resonance imaging, drug screen tests, troponin values, and final diagnosis. Results: A total of 32 patients were identified. The diagnoses were: myocarditis in 16 patients, vasospasm due to drug use in seven, myopericarditis in six, anomalous coronary artery origins in two, and Prinzmetal's angina in one patient. The electrocardiograms were abnormal in 27 of the 32 patients (84%), echocardiograms in 18 of the 32 patients (56%), cardiac magnetic resonance imaging in two of the four patients (50%), urine drug screen in five of the 25 patients (20%), and cardiac catheterisations in two of the 15 patients (13%). Conclusions: Myocarditis is the most common aetiology of myocardial injury in children. Clinical history remains the basic screening tool; drug screens help identify coronary vasospasms secondary to drug use (22% of our cohort). Patients with anomalous coronaries had exertional symptoms. Initial troponin levels and progression were not diagnostic or prognostic. Catheterisation is of limited value and did not change management. Magnetic resonance imaging with gadolinium enhancement is probably the most useful test when initial evaluation is not diagnostic.
    Cardiology in the Young 03/2013; 24(2):1-7. DOI:10.1017/S1047951113000231 · 0.86 Impact Factor
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    ABSTRACT: Anomalous origin of the left main coronary artery from the right sinus (ALMCA) is a rare entity associated with an increased risk for sudden cardiac death. Although transthoracic echocardiography often is the primary screening tool for the diagnosis, its accuracy remains undefined, resulting in the use of more expensive methods such as cardiac catheterization and magnetic resonance imaging when clinical suspicion exists. This report aims to describe a novel echocardiographic screening method for detecting ALMCA and to apply it retrospectively for patients with confirmed findings intraoperatively and/or on autopsy. All patients with a confirmed diagnosis of ALMCA who presented to our institution from 2004 to 2012 were included in the study. Eight patients had adequate images, including two patients with an initial diagnosis of normal coronary origins. A control group consisted of 35 patients with normal coronaries shown by echocardiogram. Five of these patients had normal coronary artery origins confirmed by magnetic resonance imaging (MRI). Echocardiographic images were reviewed, and the left main coronary was identified in the parasternal long-axis view between the aorta and pulmonary trunk. The angle of the left main coronary course was measured. In patients with ALMCA, the proximal course of the vessel was steeper as it coursed posteriorly. In contrast, the course was almost horizontal in patients with normal coronary origins. Based on these findings, a cutoff angle of 28° is proposed. In the parasternal long-axis view, ALMCA can be identified by its anomalous proximal course. This screening method is reliable and increases the accuracy of transthoracic echocardiograms.
    Pediatric Cardiology 10/2012; 34(4). DOI:10.1007/s00246-012-0550-y · 1.55 Impact Factor
  • Holly C DeSena, Claudio Ramaciotti, Alan W Nugent
    Circulation Cardiovascular Interventions 06/2012; 5(3):e37-8. DOI:10.1161/CIRCINTERVENTIONS.112.969899 · 6.98 Impact Factor
  • Circulation Cardiovascular Imaging 05/2012; 5(3):415-8. DOI:10.1161/CIRCIMAGING.111.972034 · 6.75 Impact Factor
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    ABSTRACT: Objective:To determine the accuracy of the patent ductus arteriosus:left pulmonary artery ratio (PDA:LPA) on echocardiogram (ECHO) at 3-day postnatal in predicting spontaneous PDA closure in neonates 30 weeks gestational age (GA).Study Design:ECHOs were performed at 72 h to characterize PDA size as closed-to-small (PDA:LPA <0.5) or moderate-to-large (PDA:LPA 0.5) and at 10 days to determine spontaneous closure (defined as closed-to-small in the absence of medical and/or surgical treatment). Caretakers were blinded to results; treatment was based on standard care. Neonates were prospectively enrolled and stratified: <27 weeks (n=31) and 27 to 30 weeks (n=65).Result:Neonates <27 weeks with closed-to-small PDAs had 60% spontaneous closure vs 9% when moderate-to-large (positive predictive value (PPV) 60%, negative predictive value (NPV) 91%). Neonates 27 to 30 weeks had 95% spontaneous closure vs 27%, respectively (PPV 95%, NPV 73%). Inter-observer variability for the initial ECHO was 0.84.Conclusion:PDA size defined by PDA:LPA at 3 days postnatal in combination with GA predicts spontaneous PDA closure.Journal of Perinatology advance online publication, 12 April 2012; doi:10.1038/jp.2012.41.
    Journal of perinatology: official journal of the California Perinatal Association 04/2012; DOI:10.1038/jp.2012.41 · 2.35 Impact Factor
  • Shelley E Crary, Claudio Ramaciotti, George R Buchanan
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    ABSTRACT: Vascular complications, including pulmonary hypertension (PH), have been reported to occur following splenectomy for various disorders,including hereditary spherocytosis (HS). We performed a prospective cross-sectional study of 36 adults with HS (78% with prior splenectomy)utilizing echocardiography to estimate tricuspid regurgitant jet velocity (TRV) as well as measurement of N-terminal pro-B-type natriuretic peptide (NT-proBNP) to screen for PH. No participant with HS hada significantly elevated TRV or NT-proBNP level, despite a median 25-year interval since splenectomy (95% confidence interval for point prevalence 0, 0.097). Although our study was limited by a small sample size, it appears that persons with HS, following splenectomy, appear unlikely to be at significantly increased risk of developing PH to the degree reported for thalassemia and sickle cell disease
    American Journal of Hematology 12/2011; 86(12):E73-6. DOI:10.1002/ajh.22182 · 3.48 Impact Factor
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    ABSTRACT: Nemaline myopathy is a congenital nonprogressive skeletal muscle disorder with a characteristic rod body formation in the skeletal muscle fibers. Cardiac involvement in nemaline myopathy is rare, although both dilated and hypertrophic cardiomyopathy have been reported. We describe an infant diagnosed with hypertrophic cardiomyopathy and hypotonia on the first day of life. Muscle biopsy confirmed nemaline myopathy at 3 weeks of age. The diagnosis of nemaline myopathy precluded consideration of heart transplantation, thus shifting the focus to comfort care. This is the earliest presentation of hypertrophic cardiomyopathy reported in the literature in the setting of nemaline myopathy. The approach to determining an etiology for hypertrophic cardiomyopathy in an infant is reviewed.
    Congenital Heart Disease 11/2011; 7(4):E37-41. DOI:10.1111/j.1747-0803.2011.00588.x · 1.20 Impact Factor
  • Poonam P Thankavel, Claudio Ramaciotti, Matthew S Lemler
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    ABSTRACT: Retrograde flow in the aortic arch identifies inadequate cardiac output in neonates with congenital heart disease. We present a left congenital diaphragmatic hernia (CDH) infant with retrograde flow in the arch that normalized after CDH repair. We hypothesize transient inadequacy of the left heart; prostaglandins might play a pivotal role.
    Pediatric Cardiology 04/2011; 32(8):1207-8. DOI:10.1007/s00246-011-9997-5 · 1.55 Impact Factor
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    ABSTRACT: transesophageal echocardiography (TEE) is the gold standard for the diagnosis of a patent foramen ovale in adults. In children, acoustic windows on transthoracic echocardiography (TTE) are better than in adults; thus, an invasive TEE may not be necessary. Our goal was to assess the validity of TTE with agitated saline injection for the diagnosis of a patent foramen ovale in children using TEE as the gold standard. fifty consecutive pediatric patients >1 year of age referred for TEE were prospectively enrolled. Imaging included 2-dimensional, color Doppler, and agitated saline contrast injections with and without Valsalva by TTE followed by TEE. Interpreters of the TTE were blinded to TEE results. Studies were categorized as "inconclusive" if the TTE images were inadequate for definitive diagnosis by the blinded interpreter. TTE results were considered conclusive in 43 of 50 (86%) patients. Among the 43 conclusive studies, the 2 modalities disagreed in 1 patient. TTE had a positive predictive value of 100%, negative predictive value 97%, sensitivity of 88%, and specificity of 100% for detecting a patent foramen ovale. TTE with agitated saline injection is diagnostic for the assessment of atrial septal integrity in the majority of children.
    Stroke 01/2011; 42(1):98-101. DOI:10.1161/STROKEAHA.110.595876 · 6.02 Impact Factor
  • Poonam Punjwani, Charles R. Rosenfeld, Claudio Ramaciotti
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    ABSTRACT: Purpose: The prevalence of spontaneous closure of the patent ductus arteriosus (PDA) in neonates with a birth weight of ≤1000gm was examined in a prospective study by Koch et al (2006). The rate of spontaneous closure was 34%, which was further categorized as ~80% in neonates >29wks gestational age (GA), but <20% in neonates <25wks GA. Moreover, there was a correlation between spontaneous closure of the PDA and GA, but not birth weight. These observations suggest that prophylactic closure of the PDA for all neonates ≤1000gm or ≤30wks GA should be re-examined. In a retrospective analysis of echocardiograms (ECHO) obtained in the Koch study (Ramos et al, in press), the PDA:left pulmonary artery (LPA) ratio at 3d postnatal was the only measurement to correlate with the need for subsequent treatment of a PDA in this population. Using this measurement, neonates with a moderate to large PDA at 72h of life had an odds ratio of 15 for requiring subsequent treatment of a PDA (medical or surgical). Although predictive, this has not been examined prospectively or confirmed in another population. In the present study, we sought to determine if the spontaneous closure rate of the PDA in neonates ≤30wks GA could be predicted using the PDA:LPA ratio on ECHOs obtained at 3d postnatal age. Methods: All neonates ≤30wks GA born at our institution were approached for study and informed consent obtained. ECHOs were obtained at 3 and 10d postnatal. A PDA:LPA ratio of ≥1 indicated a large PDA, <1 but ≥0.5 moderate, and <0.5 small. Initial ECHO results were analyzed in two categories: closed-small PDA and moderate-large PDA. Spontaneous closure was defined as a closed-small PDA on the second ECHO in the absence of medical/surgical intervention at any time during the hospitalization. Additionally, neonates were grouped into <27wks and 27–30wks GA. In both groups, spontaneous closure rates based on initial PDA size were analyzed by Fisher t-test. Results: Ninety-two neonates have completed the protocol. The first ECHO was performed at 72±8h postnatal, with the follow-up at 10±1d. In neonates <27wks GA (n=30), the spontaneous closure rate was 73.3% in the closed-small PDA group versus 13.3% in the moderate-large PDA group (p<0.005). The positive predictive value of a moderate-large PDA on initial ECHO was 86.7%. In the 27-30wks GA group (n=62), the closure rate was 93.5% and 25% for closed-small and moderate-large PDA, respectively (p<0.005). The negative predictive value for a closed-small PDA on initial ECHO was 93.5%. Conclusion: These are the first data demonstrating that PDA size, defined by the PDA:LPA ratio on ECHO at 3d postnatal, predicts spontaneous closure of the PDA by 10d postnatal in neonates ≤30wks GA.
    2010 American Academy of Pediatrics National Conference and Exhibition; 10/2010
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    ABSTRACT: The echocardiographic diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) can be challenging. The aim of this study was to assess the hypothesis that diagnosis can be enhanced by using supplemental oxygen, which decreases pulmonary vascular resistance and increases retrograde flow from the coronary artery into the pulmonary artery. Demographic, echocardiographic, and cardiac catheterization data were reviewed in patients presenting with ALCAPA from 1999 to 2007. Twenty-one patients (seven male; median age, 5 months) presented with ALCAPA. Nine underwent imaging with oxygen. Two of these nine (22%) had previous standard echocardiographic studies that missed the diagnosis. Cardiac catheterization was required for diagnosis of ALCAPA in 42% of patients who underwent standard echocardiography compared with 11% of patients who received supplemental oxygen in addition to standard echocardiography. The administration of oxygen caused no significant change in heart rate or cardiorespiratory support. Transient oxygen administration is useful in the noninvasive diagnosis of ALCAPA.
    Journal of the American Society of Echocardiography: official publication of the American Society of Echocardiography 10/2010; 23(10):1099-102. DOI:10.1016/j.echo.2010.07.005 · 3.99 Impact Factor
  • F G Ramos, C R Rosenfeld, L Roy, J Koch, C Ramaciotti
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    ABSTRACT: Identify echocardiographic parameters at <or=4 day postnatal that predict the subsequent need for closure of a clinically significant patent ductus arteriosus (sPDA) in extremely-low-birth-weight neonates (ELBW). Serial echocardiograms obtained in 115 ELBW at <or=10 day postnatal were examined to estimate PDA size using the PDA:left pulmonary artery (LPA) diameter ratio: >or=1 indicated a large PDA, <1 but >or=0.5 moderate, and <0.5 small. Sensitivity, specificity, and positive predictive values (PPV) were determined for ELBW <27 weeks and >or=27 weeks gestational age. Neonates with moderate to large PDA at <or=4 day had 15-times greater likelihood of requiring treatment for sPDA than those with a small PDA (95% confidence interval (CI): 5.6-41). Sensitivity, specificity and PPV of the PDA:LPA at <27 weeks was 80, 86 and 92%, respectively. A moderate to large PDA determined from the PDA:LPA ratio at <or=4 day postnatal identifies neonates <27 week gestation who subsequently require closure of a PDA.
    Journal of perinatology: official journal of the California Perinatal Association 02/2010; 30(8):535-9. DOI:10.1038/jp.2010.14 · 2.35 Impact Factor
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    ABSTRACT: To determine the prevalence of potential intracardiac shunts, including patent foramen ovale (PFO), in children with sickle cell disease (SCD) and stroke. We performed a transthoracic echocardiogram (TTE) on 40 children with SCD (39 with hemoglobin SS and 1 with sickle-beta0 thalassemia) and earlier stroke (overt stroke in 30, silent infarction in 10). We compared 3 TTE techniques: conventional 2-dimensional imaging, color Doppler ultrasound, and intravenous agitated saline contrast injection for the detection of intracardiac shunts. We also evaluated the clinical, laboratory, and radiographic findings of the children with and without shunts. We identified PFO or other potential intracardiac shunts in 10 of 40 children with SCD and earlier stroke (25%; 95% CI, 11.6-38.4). With contrasted TTE, we failed to detect potential shunts in 2 children. In a comparison group of 60 children with stroke but without SCD, retrospective review of clinical echocardiograms identified PFO in 7 of 60 (11.7%; 95% CI, 3.6-19.8). Clinical features significantly associated with the presence of intracardiac shunts were stroke in the setting of vaso-occlusive crisis (P = .026) and headache at stroke onset (P = .014). One-quarter of children with SCD and stroke have potential intracardiac shunts. A combination of echocardiographic techniques is required for optimal shunt detection. Intracardiac shunting could be a risk factor for stroke in children with SCD because they are predisposed to thrombosis and elevations of right heart pressure, which could promote paradoxical embolization across an intracardiac shunt.
    The Journal of pediatrics 12/2009; 156(4):645-50. DOI:10.1016/j.jpeds.2009.10.012 · 3.74 Impact Factor
  • Arpitha Chiruvolu, Poonam Punjwani, Claudio Ramaciotti
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    ABSTRACT: The ductus arteriosus frequently fails to close in premature neonates. Considerable difference in opinion exists around what signifies a hemodynamically significant patent ductus arteriosus (PDA) and how reliable clinical signs are in determining the degree of the left-to-right shunting. Although reliance on clinical signs alone could delay the diagnosis of a PDA, there is insufficient evidence to suggest that early treatment improves outcome. Echocardiography is often used as the gold standard for diagnosing a PDA. A combination of echocardiographic measurements may assist in the early diagnosis of a PDA with a hemodynamically significant degree of left-to-right shunting, especially in extremely premature babies, where closure can be significantly delayed. Decision to treat PDA should be based on a combination of clinical signs and echocardiographic parameters. Monitoring B-type natriuretic peptide may be useful in the diagnosing neonates with symptomatic PDA.
    Early human development 03/2009; 85(3):147-9. DOI:10.1016/j.earlhumdev.2008.12.008 · 1.93 Impact Factor
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    ABSTRACT: Optimal cannula position is essential during extracorporeal membrane oxygenation (ECMO). We hypothesize that echocardiography is superior to chest radiography in diagnosing abnormal cannula position during ECMO. Retrospective. Pediatric hospital. 100 pediatric patients requiring ECMO. We reviewed the medical records of all ECMO patients (n = 100), including reports of all echocardiograms (n = 326), during the years 2002-2004. Of the 91 patients who had echocardiograms while on ECMO, 33 had at least 1 echocardiogram for cannula-position evaluation. Of the remaining 58 patients with echocardiograms for reasons other than cannula-position evaluation, 4 (7%) were found to have abnormal cannula position. These included arterial cannula (AC) within 2-4 mm of the aortic valve (n = 2), AC across the aortic valve into the left ventricle (n = 1), and venous cannula (VC) abutting the atrial septum (n = 1). Of the 33 patients with echocardiograms for evaluation of cannula position, 8 (24%) required intervention. Of those 8 patients, 4 required cannula repositioning due to VC in the coronary sinus (n = 1), VC abutting atrial septum (n = 1), AC in left subclavian artery (n = 1), and AC within 3 mm of aortic valve (n = 1). The remaining 4 with normal cannula position required upsizing of the VC (n = 2), increased circuit flow (n = 1), or intravascular volume administration (n = 1). Overall, 12 of 91 patients (13%) required intervention based on echocardiographic findings. Chest radiography did not detect abnormalities of ECMO cannula position in any of the 8 patients with this problem, nor were any additional patients with abnormal cannula position identified by chest radiography. Echocardiography appears to be superior to chest radiography for assessing ECMO cannula position in our institution. A prospective study, including cost analysis, comparing chest radiography and echocardiography, is needed to definitely determine the preferred diagnostic test or sequence of tests to establish ECMO cannula position.
    Pediatric Critical Care Medicine 01/2009; 10(1):56-9. DOI:10.1097/PCC.0b013e3181937409 · 2.33 Impact Factor
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    ABSTRACT: Aspirin is used to prevent thromboembolism in children with heart disease without evidence supporting its efficacy. Studies in adults report a 5%-51% prevalence of aspirin resistance, yet the mechanisms involved are poorly understood. Our aims were to determine its prevalence in these children and to explore its possible mechanisms. One hundred twenty-three cardiac patients routinely receiving aspirin were prospectively enrolled. Platelet function was measured by Platelet Function Analyzer (PFA)-100 using epinephrine and adenosine diphosphate (ADP) agonists. Aspirin resistance was defined as failure to prolong the epinephrine closure time following aspirin administration. Urine levels of 11-dehydro-thromboxane B(2) (11-dTXB(2)) were measured to determine inhibition of the cyclo-oxygenase pathway. The prevalence of aspirin resistance was 26%. Median ADP closure time was shorter for aspirin-resistant (79.60-115 s) than for aspirin-sensitive (100.60-240 s) patients (p < 0.01). 11-dTXB(2) levels did not correlate with aspirin resistance. Aspirin-resistant patients had higher 11-dTXB(2) levels before (7297 vs. 4160 pg/mg creatinine; p < 0.01) and after (2153 vs. 1412 pg/mg; p = 0.03) aspirin, with a similar percentage decrease in thromboxane (70.5% vs. 66.1%; p = 0.43). Our findings suggest that resistance is not entirely due to lack of inhibition of platelet thromboxane production. Alternative sources of thromboxane and thromboxane-independent mechanisms, such as ADP-induced platelet activation, may contribute to aspirin resistance.
    Pediatric Cardiology 03/2008; 29(2):285-91. DOI:10.1007/s00246-007-9098-7 · 1.55 Impact Factor

Publication Stats

611 Citations
156.89 Total Impact Points

Institutions

  • 2005–2014
    • Children's Medical Center Dallas
      Dallas, Texas, United States
  • 2000–2013
    • University of Texas Southwestern Medical Center
      • • Department of Pediatrics
      • • Division of Pediatric Cardiology
      • • Division of Cardiology
      Dallas, Texas, United States
  • 2010–2012
    • University of North Texas at Dallas
      Dallas, Texas, United States
  • 2001–2007
    • University of Texas at Dallas
      Richardson, Texas, United States