213 results on '"Meryl S. Cohen"'
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2. Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult
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Wyman W. Lai, Luc L. Mertens, Meryl S. Cohen, Tal Geva, Wyman W. Lai, Luc L. Mertens, Meryl S. Cohen, Tal Geva
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- 2021
3. Imaging of Left Ventricular Hypoplasia
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Meryl S, Cohen
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Heart Defects, Congenital ,Echocardiography ,Heart Ventricles ,Hypoplastic Left Heart Syndrome ,Pediatrics, Perinatology and Child Health ,Infant, Newborn ,Humans ,Mitral Valve ,Surgery ,General Medicine ,Cardiology and Cardiovascular Medicine - Abstract
Left ventricular hypoplasia is a common finding in various forms of congenital heart disease. Echocardiography in the setting of left ventricular hypoplasia must comprehensively assess the size and function of all left-sided structures including the mitral valve, left ventricular outflow tract, aortic valve and aortic arch. Of most importance in any variation of left ventricular hypoplasia is the left ventricular inlet. In neonates, the left ventricular inlet often determines the adequacy of the left ventricle and is the most difficult component to treat surgically.
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- 2022
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4. Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries
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Meryl S Cohen and Luc L Mertens
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congenital heart defects ,congenitally corrected transposition of great arteries ,transposition of the great arteries ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
Echocardiographic assessment of patients with transposition of the great arteries and congenitally corrected transposition requires awareness of the morphology and commonly associated lesions. The pre-operative echocardiography should include a full segmental and sequential analysis. Post-operative assessment is not possible without awareness of the type of surgical procedure performed and consists of assessing surgical connections and residual lesions.
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- 2019
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5. Dynamic Annular Modeling of the Unrepaired Complete Atrioventricular Canal Annulus
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John M. Simpson, Jing Huang, Christian Herz, Hannah H. Nam, Gabor Fichtinger, Patrick V. Dinh, Alison M. Pouch, Meryl S. Cohen, Andras Lasso, Ahmed H. Aly, Saleha Kabir, Andrew C. Glatz, Adriana Posada, David M. Harrild, Matthew A. Jolley, and Gerald R. Marx
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Male ,Pulmonary and Respiratory Medicine ,Echocardiography, Three-Dimensional ,Regurgitation (circulation) ,Article ,Mitral valve ,Humans ,Medicine ,Mitral Valve Annulus ,cardiovascular diseases ,Cardiac Surgical Procedures ,Retrospective Studies ,Annulus (mycology) ,Atrioventricular valve ,Cardiac cycle ,business.industry ,Heart Septal Defects ,Infant ,Anatomy ,equipment and supplies ,medicine.disease ,medicine.anatomical_structure ,Child, Preschool ,cardiovascular system ,Atrioventricular canal ,Female ,Surgery ,Cardiology and Cardiovascular Medicine ,business ,Mitral valve regurgitation ,Echocardiography, Transesophageal - Abstract
BACKGROUND: Repair of complete atrioventricular canal (CAVC) is often complicated by atrioventricular valve regurgitation, particularly of the left-sided valve. Understanding the three-dimensional (3D) structure of the atrioventricular canal annulus prior to repair may help to inform optimized repair. However, the 3D shape and movement of the CAVC annulus has yet to be quantified nor has it been rigorously compared to a normal mitral valve annulus. METHODS: The complete annuli of 43 patients with CAVC were modeled in 4 cardiac phases using transthoracic 3D echocardiograms and custom code. The annular structure was compared to the annuli of 20 normal pediatric mitral valves using 3D metrics and statistical shape analysis (Procrustes analysis). RESULTS: The unrepaired CAVC annulus varied in shape significantly throughout the cardiac cycle. Procrustes analysis visually demonstrated that the average normalized CAVC annular shape is more planar than the normal mitral annulus. Quantitatively, the annular height to valve width ratio of the native left CAVC atrioventricular valve was significantly lower than that of a normal mitral valve in all systolic phases(p
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- 2022
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6. Modeling of the Tricuspid Valve and Right Ventricle in Hypoplastic Left Heart Syndrome With a Fontan Circulation
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Hannah H. Nam, Maura Flynn, Andras Lasso, Christian Herz, Patricia Sabin, Yan Wang, Alana Cianciulli, Chad Vigil, Jing Huang, Jared Vicory, Beatriz Paniagua, David Allemang, David J. Goldberg, Mohammed Nuri, Meryl S. Cohen, Gabor Fichtinger, and Matthew A. Jolley
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Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine - Abstract
Background: In hypoplastic left heart syndrome, tricuspid regurgitation (TR) is associated with circulatory failure and death. We hypothesized that the tricuspid valve (TV) structure of patients with hypoplastic left heart syndrome with a Fontan circulation and moderate or greater TR differs from those with mild or less TR, and that right ventricle volume is associated with TV structure and dysfunction. Methods: TV of 100 patients with hypoplastic left heart syndrome and a Fontan circulation were modeled using transthoracic 3-dimensional echocardiograms and custom software in SlicerHeart. Associations of TV structure to TR grade and right ventricle function and volume were investigated. Shape parameterization and analysis was used to calculate the mean shape of the TV leaflets, their principal modes of variation, and to characterize associations of TV leaflet shape to TR. Results: In univariate modeling, patients with moderate or greater TR had larger TV annular diameters and area, greater annular distance between the anteroseptal commissure and anteroposterior commissure, greater leaflet billow volume, and more laterally directed anterior papillary muscle angles compared to valves with mild or less TR (all P P P Conclusions: Moderate or greater TR in patients with hypoplastic left heart syndrome with a Fontan circulation is associated with greater leaflet billow volume, a more laterally directed anterior papillary muscle angle, and greater annular distance between the anteroseptal commissure and anteroposterior commissure. However, there is significant heterogeneity of structure in the TV leaflets in regurgitant valves. Given this variability, an image-informed patient-specific approach to surgical planning may be needed to achieve optimal outcomes in this vulnerable and challenging population.
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- 2023
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7. Transposition of the Great Arteries
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Xavier Iriart, Meryl S. Cohen, and Luc L. Mertens
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- 2021
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8. Ventricular Septal Defects
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Shobha Natarajan and Meryl S. Cohen
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medicine.medical_specialty ,medicine.anatomical_structure ,business.industry ,Internal medicine ,Cardiology ,medicine ,Vascular resistance ,business - Published
- 2021
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9. Cardiac Malposition and Heterotaxy Syndrome
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Lindsay S. Rogers and Meryl S. Cohen
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medicine.medical_specialty ,Heterotaxy Syndrome ,business.industry ,Internal medicine ,Cardiology ,Medicine ,business - Published
- 2021
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10. Left Ventricular Measurements and Strain in Pediatric Patients Evaluated for Systemic Hypertension and the Effect of Adequate Anti-hypertensive Treatment
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Nina Laney, Craig Tinker, Meryl S. Cohen, Heather Griffis, Michelle Kaplinski, Melodee Mendoza, Fang Liu, Kevin E.C. Meyers, and Shobha Natarajan
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Heart transplantation ,medicine.medical_specialty ,Univariate analysis ,business.industry ,medicine.medical_treatment ,Diastole ,Cardiac surgery ,Interquartile range ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Cardiology ,Medicine ,Mass index ,cardiovascular diseases ,Risk factor ,Cardiology and Cardiovascular Medicine ,business ,Subclinical infection - Abstract
Pediatric hypertension (HTN) is an epidemic that is associated with HTN in adulthood and adverse cardiovascular outcomes. We hypothesized that children with HTN would have left ventricular (LV) hypertrophy and abnormal LV global longitudinal strain (GLS) on echocardiogram and that these values would differ by weight, race, and HTN treatment. Data were collected from first visits to the HTN Program from 12/2011 to 9/2018, excluding patients with cardiac disease or heart transplantation. LV measurements including LV mass index (LVMI), LV GLS, and diastolic indices were compared between groups. Multivariable logistic regression was used to identify risk factors for an abnormal LVMI. There were 212 patients with an interquartile age range of 13–18 years. On univariate analysis, LVMI was higher in hypertensive, obese, and African American patients. LV strain was less negative in obese and African American patients. Adequately treated patients with HTN had a higher LVMI and a higher E/e′ ratio compared to patients with no HTN. On multivariate analysis, only obesity was associated with an LVMI ≥ 95th percentile (OR 2.9, 95% CI 1.4, 5.8). LVMI is higher in hypertensive, obese, and African American patients; however, in the multivariate analysis, obesity was the only independent risk factor for an abnormal LVMI. LVMI was still higher in those adequately treated for HTN compared to patients without HTN, possibly due to concomitant obesity. Future studies should focus on subclinical changes in LV performance seen in obese and hypertensive patients and the impact on long-term health.
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- 2021
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11. Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature – The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11)
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Stephen P. Sanders, Rodney C. G. Franklin, James D. St. Louis, Jeffrey P. Jacobs, Andrew C. Cook, Lindsay S. Rogers, Amy L. Juraszek, Kristine J. Guleserian, Shubhika Srivastava, Martin J. Elliott, Henry L. Walters, Hiromi Kurosawa, Jeffrey R. Boris, Charles W. Shepard, Lianyi Wang, Elif Seda Selamet Tierney, Rohit Loomba, Christo I. Tchervenkov, Marina L. Hughes, Diane E. Spicer, Bohdan Maruszewski, Marshall L. Jacobs, Jill J. Savla, Constantine Mavroudis, Steven D. Colan, Jorge M. Giroud, Meryl S. Cohen, Marie J. Béland, Vera Demarchi Aiello, Adrian Crucean, Stephen P. Seslar, Allen D. Everett, Lazaro E. Hernandez, Justin T. Tretter, O. N. Krogmann, Giovanni Stellin, Leo Lopez, J. William Gaynor, Frédérique Bailliard, Paul M. Weinberg, and Lucile Houyel
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Heart Defects, Congenital ,medicine.medical_specialty ,Standardization ,Unification ,030204 cardiovascular system & hematology ,World Health Organization ,Eleventh ,World health ,Code (semiotics) ,Terminology ,03 medical and health sciences ,0302 clinical medicine ,International Classification of Diseases ,medicine ,Humans ,Medical physics ,Registries ,Intensive care medicine ,Child ,Nomenclature ,Societies, Medical ,Global system ,business.industry ,General Medicine ,030228 respiratory system ,Pediatrics, Perinatology and Child Health ,Female ,Surgery ,Cardiology and Cardiovascular Medicine ,business - Abstract
Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.
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- 2021
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12. Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult
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Wyman W. Lai, Luc L. Mertens, Meryl S. Cohen, Tal Geva
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- 2015
13. Physician Wellness in Academic Cardiovascular Medicine: A Scientific Statement From the American Heart Association
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Elisa A, Bradley, David, Winchester, Carlos E, Alfonso, Andrea J, Carpenter, Meryl S, Cohen, Dawn M, Coleman, Miriam, Jacob, Hani, Jneid, Miguel A, Leal, Zainab, Mahmoud, Laxmi S, Mehta, and Chittur A, Sivaram
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Physicians ,Humans ,Medicine ,American Heart Association ,Burnout, Professional ,Delivery of Health Care ,United States - Abstract
Academic medicine as a practice model provides unique benefits to society. Clinical care remains an important part of the academic mission; however, equally important are the educational and research missions. More specifically, the sustainability of health care in the United States relies on an educated and expertly trained physician workforce directly provided by academic medicine models. Similarly, the research charge to deliver innovation and discovery to improve health care and to cure disease is key to academic missions. Therefore, to support and promote the growth and sustainability of academic medicine, attracting and engaging top talent from fellows in training and early career faculty is of vital importance. However, as the health care needs of the nation have risen, clinicians have experienced unprecedented demand, and individual wellness and burnout have been examined more closely. Here, we provide a close look at the unique drivers of burnout in academic cardiovascular medicine and propose system-level and personal interventions to support individual wellness in this model.
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- 2022
14. Congenital polyvalvular disease expands the cardiac phenotype of the <scp>RASopathies</scp>
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Meryl S. Cohen, David A. Stevenson, Sarah E Sheppard, Dena R. Matalon, Beth Keena, Angela E. Lin, Elizabeth J. Bhoj, Elaine H. Zackai, and Avni Santani
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Heart Defects, Congenital ,Male ,Proto-Oncogene Proteins B-raf ,0301 basic medicine ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Cardiovascular Abnormalities ,Dwarfism ,Protein Tyrosine Phosphatase, Non-Receptor Type 11 ,Disease ,030105 genetics & heredity ,RASopathy ,Short stature ,Article ,03 medical and health sciences ,Genetics ,medicine ,Humans ,Child ,Genetics (clinical) ,business.industry ,Noonan Syndrome ,Infant, Newborn ,Hypertrophic cardiomyopathy ,Facies ,Infant ,Cardiomyopathy, Hypertrophic ,medicine.disease ,Connective tissue disease ,Musculoskeletal Abnormalities ,Pulmonary Valve Stenosis ,PTPN11 ,Phenotype ,030104 developmental biology ,Aortic Valve ,Child, Preschool ,Cohort ,Skin Abnormalities ,ras Proteins ,Noonan syndrome ,Female ,medicine.symptom ,business - Abstract
The RASopathies are a group of similar genetic syndromes with cardiovascular abnormalities, characteristic facial features, short stature, abnormalities of the skin and musculoskeletal system, and variable neurodevelopmental challenges. The most common cardiovascular abnormalities include pulmonary valvular stenosis and hypertrophic cardiomyopathy. Congenital polyvalvular disease (CPVD) refers to congenital dysplasia of two or more cardiac valves. We diagnosed a RASopathy in two individuals with CPVD and noted that CPVD in RASopathies has rarely been reported in the literature. Thus, we performed a retrospective chart review and literature review to investigate the association and characterize the phenotype of CPVD in the RASopathies. CPVD was present in 2.5% (n = 6/243) of individuals in our RASopathy cohort. Involvement of two cardiac valves, commonly the aortic and pulmonic valves, was seen in the majority of individuals (6/8; 75%) in our cohort, but only 27% (3/11) of reported CPVD and RASopathy cases in the literature. CPVD should be considered an associated cardiovascular phenotype of the RASopathies, which has implications for diagnosis and management.
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- 2021
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15. Recommendations for Multimodality Assessment of Congenital Coronary Anomalies: A Guide from the American Society of Echocardiography
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Benjamin W. Eidem, Leo Lopez, B. Kelly Han, Shubhika Srivastava, Peter C. Frommelt, Beth F. Printz, Anne Marie Valente, H. Helen Ko, Masaki Nii, Vasiliki V Dimas, Meryl S. Cohen, and Richard Lorber
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medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,medicine ,Psychological intervention ,Radiology, Nuclear Medicine and imaging ,Magnetic resonance imaging ,Cardiology and Cardiovascular Medicine ,Intensive care medicine ,business ,Cardiovascular angiography ,Multimodality - Published
- 2020
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16. Clinical Innovation: A Multidisciplinary Program for the Diagnosis and Treatment of Systemic Hypertension in Children and Adolescents
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Kevin E.C. Meyers, Heather Griffis, Melodee Mendoza, Michelle Kaplinski, Meryl S. Cohen, Craig Tinker, Nina Laney, Fang Liu, and Shobha Natarajan
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Male ,Nephrology ,medicine.medical_specialty ,Ambulatory blood pressure ,Adolescent ,Cardiology ,030204 cardiovascular system & hematology ,Risk Assessment ,03 medical and health sciences ,0302 clinical medicine ,Multidisciplinary approach ,030225 pediatrics ,Internal medicine ,Ambulatory Care ,medicine ,Humans ,Mass Screening ,In patient ,cardiovascular diseases ,Program Development ,Child ,Antihypertensive Agents ,business.industry ,Blood Pressure Determination ,Blood Pressure Monitoring, Ambulatory ,Lipid screening ,medicine.disease ,Obesity ,Organizational Innovation ,Blood pressure ,Hypertension ,Pediatrics, Perinatology and Child Health ,Emergency medicine ,Cohort ,Female ,Interdisciplinary Communication ,business - Abstract
Pediatric systemic hypertension (HTN) is underdiagnosed and undertreated. The Divisions of Cardiology and Nephrology at our institution developed a comprehensive outpatient HTN program to (1) screen children at risk for HTN, (2) assess cardiovascular health, and (3) optimize medical management. We report our findings during all initial visits (n = 304) from December 2011 to September 2018. Of the cohort, 38% were obese and 36% reported little to no exercise. More than half of patients ≥11 years old did not have recommended lipid screening. When evaluating ambulatory blood pressure monitoring results, clinic blood pressure did not accurately diagnose patients with or without HTN and many patients on antihypertensive medications were inadequately treated. Visit recommendations included addition of or changes to antihypertensive medication in 35% of patients. A multidisciplinary program dedicated to pediatric HTN helps screen patients who are at risk. Ambulatory blood pressure monitoring identifies HTN in patients with normal clinic blood pressure and those on antihypertensive medication.
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- 2020
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17. Fetal Doppler Echocardiographic Assessment Predicts Severe Postnatal Obstruction in Total Anomalous Pulmonary Venous Connection
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Matthew J. Campbell, Brian R. White, Jack Rychik, Jarrett Linder, Jennifer A. Faerber, Zhiyun Tian, and Meryl S. Cohen
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Heart Defects, Congenital ,Scimitar Syndrome ,Infant, Newborn ,Infant ,Reproducibility of Results ,Pregnancy ,Pulmonary Veins ,Echocardiography ,Humans ,Radiology, Nuclear Medicine and imaging ,Female ,Cardiology and Cardiovascular Medicine ,Child ,Retrospective Studies - Abstract
Obstructed total anomalous pulmonary venous connection (TAPVC) is a form of critical congenital heart disease that usually requires urgent postnatal intervention. Knowing which patients have severe obstruction can aid delivery planning. The authors previously developed a novel quantitative metric of pulmonary venous flow, the pulmonary venous variability index (PVVI). The aim of this study was to test the hypothesis that fetal PVVI and vertical vein Doppler velocities are associated with severe pulmonary vein obstruction postnatally.A retrospective cohort study of neonates with prenatally diagnosed TAPVC was performed. Patients who underwent fetal echocardiography at the Children's Hospital of Philadelphia with Doppler interrogation of the vertical vein were included for analysis. Twenty-nine patients met criteria (21 with heterotaxy, 18 with supracardiac TAPVC). The latest gestation fetal echocardiogram was used. Severe pulmonary vein obstruction was defined as preoperative death or urgent surgery or catheter-based intervention (first day of life). Measurements of PVVI, defined as (maximum velocity - minimum velocity)/mean velocity, were made offline. Wilcoxon rank sum models were used to assess the associations of severe obstruction and PVVI and maximum, mean, and minimum velocities.The mean gestational age at the latest fetal echocardiographic examination was 35 weeks (range, 30-39 weeks). Twelve of the 29 patients (41%) met criteria for severe pulmonary vein obstruction. Lower PVVI was associated with greater risk for severe pulmonary venous obstruction (P = .008). The maximum, mean, and minimum velocities in the vertical vein were all significantly associated with severe pulmonary venous obstruction (P = .03, P = .03, and P = .007, respectively). Qualitative assessment of obstruction was not significantly associated with the outcome. Interobserver reliability for all vertical vein Doppler metrics was high (intraclass correlation coefficientgt; 0.9).Fetal PVVI and maximum, mean, and minimum velocities are associated with severe postnatal pulmonary vein obstruction in TAPVC. Accurate prediction of obstructed TAPVC could allow safer delivery planning. Further research with larger sample sizes is needed to identify the ideal cutoff values for these Doppler measures.
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- 2022
18. Visualization and Quantification of the Unrepaired Complete Atrioventricular Canal Valve Using Open-Source Software
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Hannah H. Nam, Christian Herz, Andras Lasso, Alana Cianciulli, Maura Flynn, Jing Huang, Zi Wang, Beatriz Paniagua, Jared Vicory, Saleha Kabir, John Simpson, David Harrild, Gerald Marx, Meryl S. Cohen, Andrew C. Glatz, and Matthew A. Jolley
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Heart Septal Defects ,Echocardiography, Three-Dimensional ,Humans ,Mitral Valve ,Mitral Valve Insufficiency ,Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine ,Software - Abstract
Repair of complete atrioventricular canal (CAVC) is often complicated by residual left atrioventricular valve regurgitation. The structure of the mitral and tricuspid valves in biventricular hearts has previously been shown to be associated with valve dysfunction. However, the three-dimensional (3D) structure of the entire unrepaired CAVC valve has not been quantified. Understanding the 3D structure of the CAVC may inform optimized repair.Novel open-source work flows were created in SlicerHeart for the modeling and quantification of CAVC valves on the basis of 3D echocardiographic images. These methods were applied to model the annulus, leaflets, and papillary muscle (PM) structure of 35 patients (29 with trisomy 21) with CAVC using transthoracic 3D echocardiography. The mean leaflet and annular shapes were calculated and visualized using shape analysis. Metrics of the complete native CAVC valve structure were compared with those of normal mitral valves using the Mann-Whitney U test. Associations between CAVC structure and atrioventricular valve regurgitation were analyzed.CAVC leaflet metrics varied throughout systole. Compared with normal mitral valves, the left CAVC PMs were more acutely angled in relation to the annular plane (P .001). In addition, the anterolateral PM was laterally and inferiorly rotated in CAVC, while the posteromedial PM was more superiorly and laterally rotated, relative to normal mitral valves (P .001). Lower native CAVC atrioventricular valve annular height and annular height-to-valve width ratio before repair were both associated with moderate or greater left atrioventricular valve regurgitation after repair (P .01).It is feasible to model and quantify 3D CAVC structure using 3D echocardiographic images. The results demonstrate significant variation in CAVC structure across the cohort and differences in annular, leaflet, and PM structure compared with the mitral valve. These tools may be used in future studies to catalyze future research intended to identify structural associations of valve dysfunction and to optimize repair in this vulnerable and complex population.
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- 2022
19. CHALLENGES WITH USING CORONARY ARTERY Z-SCORES IN CHILDREN WITH KAWASAKI DISEASE OR MULTISYSTEM INFLAMMATORY SYNDROME ASSOCIATED WITH COVID-19
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Dongngan T. Truong, Leo Lopez, Peter C. Frommelt, Steven D. Colan, Jane W. Newburger, Russell Gongwer, Aarti H. Bhat, Kristin Marie Burns, Meryl S. Cohen, Tiffanie R. Johnson, John P. Kovalchin, Joseph Mahgerefteh, Brian W. McCrindle, Sean Lang, Simon Lee, Laura Olivieri, David Parra, Charitha D. Reddy, Ritu Sachdeva, Megan Schoessling, Shubhika Srivastava, Elif Seda Selamet Tierney, Poonam Thankavel, Mary van der Velde, and L. LuAnn Minich
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Cardiology and Cardiovascular Medicine - Published
- 2023
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20. Abstract 9693: Predicting Biventricular Repair in Common Atrioventricular Canal by Cardiac Magnetic Resonance Imaging: The New Normal?
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Andrea L Jones, Brian R White, Reena M Ghosh, Antara Mondal, Steve B Ampah, Deborah Y Ho, David M Biko, Matthew A Harris, Sara Partington, Kevin K Whitehead, Meryl S Cohen, and Mark A Fogel
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Physiology (medical) ,Cardiology and Cardiovascular Medicine - Abstract
Introduction: Patients with malaligned common atrioventricular (AV) canal (CAVC) are difficult to manage when one ventricle is borderline hypoplastic. The decision between the single ventricle pathway or attempting a biventricular repair depends upon pre-operative parameters which have previously been studied only by echocardiography. The purpose of this study was to determine the utility of cardiac magnetic resonance (CMR) in predicting success of a biventricular repair in CAVC. Methods: We performed a single center, retrospective cohort study of children with varying degrees of CAVC malalignment who underwent CMR prior to definitive repair (7/1/10 to 2/28/21). The primary outcome was successful biventricular repair. CMR measurements were performed by the investigators. To account for both dominant right ventricles (RV) and left ventricles (LV), ratios of ventricular and AV valve size were normalized as differences from their expected values. Associations were tested using univariable logistic regression. Multivariable regression models using combinations of two variables with p-values less than 0.2 were tested. Results: We included 28 patients (median age at CMR: 5.2 months, 43% male). Clinical and CMR variables are shown in Table 1. The optimal multivariable predictive model included the normalized LV end diastolic volume (EDV) to total ventricular EDV ratio and the LV-RV angle in diastole. Area under the receiver operator characteristic curve was 0.83 (p = 0.041). An LV EDV to total EDV ratio between 0.30 and 0.66 had a sensitivity of 92% and specificity of 63% for predicting successful biventricular repair. Including an LV-RV angle in diastole of less than 72 degrees resulted in a sensitivity of 83% and specificity of 81%. Conclusions: Multiple CMR measures were associated with surgical outcomes. CMR measures such as LV EDV to total ventricular EDV ratio and LV-RV angle in diastole can accurately predict successful biventricular canal repair.
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- 2021
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21. Abstract 11736: Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries is Associated With Risk of Heart Block and ECMO, but Not Mortality
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Bethan A Lemley, Felina Mille, Katsuhide Maeda, Michael L Obyrne, and Meryl S Cohen
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Physiology (medical) ,Cardiology and Cardiovascular Medicine - Abstract
Introduction: Congenitally corrected transposition of the great arteries (CC-TGA) is characterized by atrioventricular and ventriculoarterial discordance with circulation remaining in series. The double switch operation and atrial switch with Rastelli are anatomic repairs of CC-TGA, achieving a systemic morphologic left ventricle. In contrast, physiologic repair (addressing associated lesions like ventricular septal defects and pulmonary stenosis) or no repair leave the right ventricle in the systemic position. Anatomic repair may confer superior longevity with increased upfront risk. We sought to determine if anatomic repair was associated with increased short-term risk of mortality, heart block, or extracorporeal membrane oxygenation (ECMO). Methods: Single-center retrospective cohort study of CC-TGA patients from 1990-2017. Cox proportional hazard analyses and Fisher’s exact test were performed. Results: Inclusion criteria were met by 54 patients, 21 anatomic repair and 33 physiologic/no repair. Median follow-up was 8.9 years (IQR 1.4-18.0) with no difference between cohorts (p=0.38). The anatomic repair cohort was more likely to have ventricular septal defects, 86% versus 33% (p Conclusions: Anatomic repair of CC-TGA conferred a higher short-term risk of heart block and ECMO compared to the physiologic/no repair cohort, though risk of heart block was linked with anatomy. This upfront risk did not result in higher mortality in the anatomic repair cohort but long-term risk remains unknown.
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- 2021
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22. Longitudinal growth in patients with single ventricle cardiac disease receiving tube‐assisted feeds
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Christopher Teng, Erika Mejia, Arene Butto, Jennifer Faerber, Jonathan B Edelson, Carrie Daymont, Meryl S. Cohen, Laura Mercer-Rosa, and Chitra Ravishankar
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Male ,Time Factors ,medicine.medical_treatment ,Jejunostomy ,Disease ,030204 cardiovascular system & hematology ,Weight Gain ,Body Mass Index ,Child Development ,0302 clinical medicine ,Risk Factors ,Medicine ,Longitudinal Studies ,Child ,Gastrostomy ,Palliative Care ,Age Factors ,General Medicine ,Patient Discharge ,Norwood Operation ,Cardiac surgery ,Treatment Outcome ,medicine.anatomical_structure ,Child, Preschool ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,Heart Defects, Congenital ,medicine.medical_specialty ,Heart Ventricles ,Article ,03 medical and health sciences ,Enteral Nutrition ,030225 pediatrics ,Humans ,Radiology, Nuclear Medicine and imaging ,Cardiac Surgical Procedures ,Retrospective Studies ,business.industry ,Infant ,Retrospective cohort study ,Body Height ,Surgery ,Ventricle ,Pediatrics, Perinatology and Child Health ,business ,Weight gain - Abstract
Objective Children with single ventricle cardiac disease (SVCD) have poor growth in early life. Tube-assisted feeding (TF) is used to improve weight gain, but its impact on long-term growth remains unknown. We sought to compare the longitudinal growth of SVCD patients receiving TF after initial cardiac surgery with those fed entirely by mouth. Design We conducted a retrospective cohort study of SVCD patients who underwent initial surgical palliation between 1999 and 2009. We defined TF as the use of nasogastric, gastrostomy, or jejunostomy TF. We compared maximal attained growth z-scores for each year of life between TF and non-TF patients. A secondary analysis compared surgical and clinical factors between groups. Results A total of 134 patients were included; 64% were male and 68% underwent the Norwood operation. One third of patients (44) received TF. Adjusting for age, TF patients had an average of 0.56 lower weight-for-age z-score (WAZ) than non-TF patients (P = 0.007) through the age of 6 years. Longitudinal height was not affected by TF status (P = 0.15). In a subanalysis of Norwood patients, TF patients had lower WAZ at initial hospital discharge despite longer LOS. TF patients had diminished WAZ after adjusting for complications, interstage hospitalizations, and timing of subsequent operations. Conclusions In this single-center study, patients with SVCD requiring TF at discharge from initial surgical palliation had diminished WAZ at discharge and on long-term follow-up, despite controlling for other identifiable risk factors. Further investigation is needed to understand the mechanisms underlying this phenomenon and to risk stratify infants who go home on TF.
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- 2019
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23. Repair of Total Anomalous Pulmonary Venous Connection: Risk Factors for Postoperative Obstruction
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Hannah Katcoff, Jennifer Faerber, Meryl S. Cohen, Brian R. White, Christopher E. Mascio, Andrew C. Glatz, Deborah Ho, and Paul Stephens
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Male ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Heart disease ,030204 cardiovascular system & hematology ,Doppler echocardiography ,urologic and male genital diseases ,Article ,03 medical and health sciences ,Postoperative Complications ,0302 clinical medicine ,Risk Factors ,medicine ,Humans ,Heart Atria ,Total anomalous pulmonary venous connection ,Risk factor ,Vascular Patency ,Retrospective Studies ,medicine.diagnostic_test ,business.industry ,Cardiovascular Surgical Procedures ,Scimitar Syndrome ,Infant, Newborn ,Infant ,Retrospective cohort study ,medicine.disease ,Venous Obstruction ,Echocardiography, Doppler ,Surgery ,Stenosis ,Stenosis, Pulmonary Vein ,030228 respiratory system ,Pulmonary Veins ,Female ,Cardiology and Cardiovascular Medicine ,business ,Heterotaxy ,Follow-Up Studies - Abstract
Background Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) results in substantial morbidity and mortality. Risk factors for postoperative obstruction remain ambiguous. In addition, the existing literature has no standard definition for preoperative obstruction, making patient counseling difficult. Methods All patients undergoing repair of TAPVC at our institution from January 1, 2006, to October 23, 2017, were identified. The primary outcome was the development of postoperative obstruction, analyzed as a time-to-event outcome. Clinical information was extracted to assess risk factors. Degrees of preoperative obstruction were defined based on echocardiographic, catheterization, and clinical findings. Univariable and multivariable Cox proportional hazard regression methods were used to identify factors associated with the primary outcome. Results During the study interval, 119 patients underwent repair of TAPVC (40% single ventricle), and postoperative obstruction developed in 25 patients (21%). Risk factors associated with obstruction were heterotaxy syndrome, single-ventricle heart disease, additional procedures at the time of vein repair, mixed-type TAPVC, and preoperative obstruction. Having even mild preoperative obstruction (≥1.2 m/s by Doppler echocardiography) was predictive of postoperative obstruction. A multivariable model showed mixed-type TAPVC and the presence of preoperative obstruction were associated with a more than twofold greater hazard of obstruction. Conclusions TAPVC in the setting of heterotaxy and a single ventricle remains challenging, with high rates of postoperative obstruction. Mixed-type TAPVC is an independent risk factor for postoperative obstruction, particularly in patients with isolated TAPVC. Even mild preoperative obstruction is a risk factor for postoperative obstruction. These results may help risk-stratify TAPVC patients.
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- 2019
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24. Preoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect
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Patrick Gray, David J. Goldberg, Meryl S. Cohen, Heather M. Griffis, Danielle S. Burstein, Andrew C. Glatz, and J. William Gaynor
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Male ,Reoperation ,medicine.medical_specialty ,Down syndrome ,030204 cardiovascular system & hematology ,Time-to-Treatment ,03 medical and health sciences ,0302 clinical medicine ,Double outlet right ventricle ,medicine ,Humans ,Atrioventricular canal defect ,Retrospective Studies ,Tetralogy of Fallot ,Surgical repair ,business.industry ,Heart Septal Defects ,Infant ,Retrospective cohort study ,Vascular surgery ,medicine.disease ,Cardiac surgery ,Surgery ,Treatment Outcome ,030228 respiratory system ,Echocardiography ,Pediatrics, Perinatology and Child Health ,Female ,Down Syndrome ,Cardiology and Cardiovascular Medicine ,business ,Infant, Premature - Abstract
In complete atrioventricular canal defect (CAVC), there are limited data on preoperative clinical and echocardiographic predictors of operative timing and postoperative outcomes. A retrospective, single-center analysis of all patients who underwent primary biventricular repair of CAVC between 2006 and 2015 was performed. Associated cardiac anomalies (tetralogy of Fallot, double outlet right ventricle) and arch operation were excluded. Echocardiographic findings on first postnatal echocardiogram were correlated with surgical timing and postoperative outcomes using bivariate descriptive statistics and multivariable logistic regression. 153 subjects (40% male, 84% Down syndrome) underwent primary CAVC repair at a median age of 3.3 (IQR 2.5–4.2) months. Median postoperative length of stay (LOS) was 7 (IQR 5–15) days. Eight patients (5%) died postoperatively and 24 (16%) required reoperation within 1 year. On multivariable analysis, small aortic isthmus (z score
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- 2019
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25. Ventricular contribution and the impact on outcome of the Fontan operation: less is more or more is less?
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Meryl S. Cohen
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Pulmonary and Respiratory Medicine ,Heart Defects, Congenital ,medicine.medical_specialty ,business.industry ,Heart Ventricles ,General Medicine ,Fontan Procedure ,Outcome (game theory) ,Ventricular morphology ,Internal medicine ,Cardiology ,Medicine ,Humans ,Surgery ,Cardiology and Cardiovascular Medicine ,business - Published
- 2021
26. Left Ventricular Measurements and Strain in Pediatric Patients Evaluated for Systemic Hypertension and the Effect of Adequate Anti-hypertensive Treatment
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Michelle, Kaplinski, Heather, Griffis, Fang, Liu, Craig, Tinker, Nina C, Laney, Melodee, Mendoza, Meryl S, Cohen, Kevin, Meyers, and Shobha S, Natarajan
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Adult ,Ventricular Dysfunction, Left ,Adolescent ,Heart Ventricles ,Hypertension ,Humans ,Hypertrophy, Left Ventricular ,Child ,Antihypertensive Agents - Abstract
Pediatric hypertension (HTN) is an epidemic that is associated with HTN in adulthood and adverse cardiovascular outcomes. We hypothesized that children with HTN would have left ventricular (LV) hypertrophy and abnormal LV global longitudinal strain (GLS) on echocardiogram and that these values would differ by weight, race, and HTN treatment. Data were collected from first visits to the HTN Program from 12/2011 to 9/2018, excluding patients with cardiac disease or heart transplantation. LV measurements including LV mass index (LVMI), LV GLS, and diastolic indices were compared between groups. Multivariable logistic regression was used to identify risk factors for an abnormal LVMI. There were 212 patients with an interquartile age range of 13-18 years. On univariate analysis, LVMI was higher in hypertensive, obese, and African American patients. LV strain was less negative in obese and African American patients. Adequately treated patients with HTN had a higher LVMI and a higher E/e' ratio compared to patients with no HTN. On multivariate analysis, only obesity was associated with an LVMI ≥ 95th percentile (OR 2.9, 95% CI 1.4, 5.8). LVMI is higher in hypertensive, obese, and African American patients; however, in the multivariate analysis, obesity was the only independent risk factor for an abnormal LVMI. LVMI was still higher in those adequately treated for HTN compared to patients without HTN, possibly due to concomitant obesity. Future studies should focus on subclinical changes in LV performance seen in obese and hypertensive patients and the impact on long-term health.
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- 2021
27. ASE Statement on Adapting Pediatric, Fetal, and Congenital Heart Disease Echocardiographic Services to the Evolving COVID-19 Pandemic
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Madhav Swaminathan, Melissa Wasserman, Meryl S. Cohen, Bhawna Arya, Piers Barker, Mark B. Lewin, Carolyn A. Altman, Mary T. Donofrio, and Gregory J. Ensing
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Heart Defects, Congenital ,medicine.medical_specialty ,2019-20 coronavirus outbreak ,Heart disease ,Coronavirus disease 2019 (COVID-19) ,viruses ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,Disease ,030204 cardiovascular system & hematology ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Special Article ,0302 clinical medicine ,Pregnancy ,Pandemic ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Intensive care medicine ,Child ,Societies, Medical ,business.industry ,Public health ,Recem nascido ,Infant, Newborn ,virus diseases ,COVID-19 ,medicine.disease ,United States ,Echocardiography ,Radiology Nuclear Medicine and imaging ,Female ,business ,Cardiology and Cardiovascular Medicine ,Delivery of Health Care - Abstract
Over the 12 months since the start of the coronavirus disease 2019 pandemic, an explosion of investigation and an increase in experience have led to vast improvement in our knowledge about this disease. However, coronavirus disease 2019 remains a huge public health threat.
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- 2021
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28. Infected Pseudoaneurysm in Congenital Heart Disease
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Meryl S. Cohen
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medicine.medical_specialty ,Heart disease ,business.industry ,Case Report ,medicine.disease ,Pseudoaneurysm ,Double outlet right ventricle ,Internal medicine ,endocarditis ,medicine ,Cardiology ,echocardiography ,Endocarditis ,Cardiology and Cardiovascular Medicine ,business ,Editorial Comment ,double outlet right ventricle - Abstract
Corresponding Author
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- 2021
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29. In Memoriam: Paul Morris Weinberg, MD
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Jeffrey P. Jacobs and Meryl S. Cohen
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Philadelphia ,Psychoanalysis ,business.industry ,Mentors ,Pillar ,Medical school ,General Medicine ,030204 cardiovascular system & hematology ,History, 20th Century ,Paediatric cardiology ,03 medical and health sciences ,0302 clinical medicine ,030228 respiratory system ,Pediatrics, Perinatology and Child Health ,Medicine ,Humans ,Cardiology and Cardiovascular Medicine ,business ,Child ,Students - Abstract
On 15 October, 2020, Dr. Paul M. Weinberg, a true giant in the field of paediatric cardiology, succumbed to a prolonged illness. Dr. Weinberg had a 43-year career and was described as a pillar of The Children’s Hospital of Philadelphia and the spirit of the Division of Cardiology, a cherished and beloved teacher, and an outstanding clinician. His impact on the field and on the careers of his students will be remembered for generations to come.In 2019, Dr. Weinberg wrote for Jefferson Medical School’s 50th year reunion memory book: “In the true spirit of Hippocrates, I seek to mentor the next generation as I was mentored by the last, without expectation of reward. I am forever indebted to these educators for all the knowledge they imparted to me and for the wisdom that I acquired under their tutelage.” These words are a true reflection of his unassuming dedication to teaching the next generation of paediatric cardiologists. His legacy will continue to live on through these trainees and impact the field for generations to come.
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- 2021
30. Venous flow variation predicts preoperative pulmonary venous obstruction in children with total anomalous pulmonary venous connection
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Brian R. White, Meryl S. Cohen, Christopher E. Mascio, Hannah Katcoff, Jennifer Faerber, and Andrew C. Glatz
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Heart Defects, Congenital ,medicine.medical_specialty ,Cardiac Catheterization ,Pulmonary Circulation ,medicine.medical_treatment ,030204 cardiovascular system & hematology ,Doppler echocardiography ,Article ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Intubation ,Humans ,Radiology, Nuclear Medicine and imaging ,Total anomalous pulmonary venous connection ,Child ,Cardiac catheterization ,Retrospective Studies ,medicine.diagnostic_test ,business.industry ,Infant ,Pulmonary edema ,medicine.disease ,Venous Obstruction ,Catheter ,Echocardiography ,Pulmonary Veins ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Venous return curve - Abstract
OBJECTIVE: Identifying preoperative pulmonary venous obstruction in total anomalous pulmonary venous connection (TAPVC) is important to guide treatment-planning and risk prognostication. No standardized echocardiographic definition of obstruction exists in the literature. Definitions based on absolute velocities are affected by technical limitations and variations in pulmonary venous return. We developed a metric to quantify pulmonary venous blood flow variation: pulmonary venous variability index (PVVI). We aimed to demonstrate its accuracy in defining obstruction. METHODS: All patients cared for with TAPVC at our institution were identified. Echocardiograms were reviewed, and maximum (V(max)), mean (V(mean)), and minimum velocities (V(min)) along the pulmonary venous pathway were measured. PVVI was defined as (V(max)–V(min))/V(mean). These metrics were compared to pressures measured by cardiac catheterization. Echocardiographic measures were then compared between the patients with and without clinical preoperative obstruction (defined as a need for preoperative intubation, catheter-based intervention, or surgery within one day of diagnosis), as well as pulmonary edema by chest X-ray and markers of lactic acidosis. 137 patients were included with 22 having catheterization pressure recordings. RESULTS: Maximum and mean velocity were not different between patients with catheter gradients ≥4 mmHg and
- Published
- 2021
31. Conotruncal Anomalies
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Laura M. Mercer-Rosa and Meryl S. Cohen
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- 2021
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32. Pediatric Heart Network Echocardiographic Z Scores: Comparison with Other Published Models
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Andreea Dragulescu, L. LuAnn Minich, Leo Lopez, Carolyn L. Taylor, Ricardo H. Pignatelli, Christopher F. Spurney, Brian W. McCrindle, Luciana T. Young, Ritu Sachdeva, Justin T. Tretter, Lindsay R. Freud, Peter C. Frommelt, Jonathan H. Soslow, Ashwin Prakash, Russell Gongwer, Aarti Bhat, Michele A. Frommelt, Kristin M. Burns, Mario Stylianou, Felicia L. Trachtenberg, Thor Thorsson, Meryl S. Cohen, Joseph Mahgerefteh, Poonam P. Thankavel, Irene D. Lytrivi, and Steven D. Colan
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Body surface area ,business.industry ,Body Surface Area ,Racial Groups ,Heart ,030204 cardiovascular system & hematology ,Standard score ,urologic and male genital diseases ,CARDIOVASCULAR MEASUREMENTS ,Article ,030218 nuclear medicine & medical imaging ,Large sample ,Correlation ,03 medical and health sciences ,0302 clinical medicine ,Echocardiography ,Statistics ,Medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Clinical care ,Cardiology and Cardiovascular Medicine ,business ,Child ,Boston - Abstract
Background Different methods have resulted in variable Z scores for echocardiographic measurements. Using the measurements from 3,215 healthy North American children in the Pediatric Heart Network (PHN) echocardiographic Z score database, the authors compared the PHN model with previously published Z score models. Methods Z scores were derived for cardiovascular measurements using four models (PHN, Boston, Italy, and Detroit). Model comparisons were performed by evaluating (1) overlaid graphs of measurement versus body surface area with curves at Z = −2, 0, and +2; (2) scatterplots of PHN versus other Z scores with correlation coefficients; (3) Bland-Altman plots of PHN versus other Z scores; and (4) comparison of median Z scores for each model. Results For most measurements, PHN Z score curves were similar to Boston and Italian curves but diverged from Detroit curves at high body surface areas. Correlation coefficients were high when comparing the PHN model with the others, highest with Boston (mean, 0.99) and lowest with Detroit (mean, 0.90). Scatterplots suggested systematic differences despite high correlations. Bland-Altman plots also revealed poor agreement at both extremes of size and a systematic bias for most when comparing PHN against Italian and Detroit Z scores. There were statistically significant differences when comparing median Z scores between the PHN and other models. Conclusions Z scores from the multicenter PHN model correlated well with previous single-center models, especially the Boston model, which also had a large sample size and similar methodology. The Detroit Z scores diverged from the PHN Z scores at high body surface area, possibly because there were more subjects in this category in the PHN database. Despite excellent correlation, significant differences in Z scores between the PHN model and others were seen for many measurements. This is important when comparing publications using different models and for clinical care, particularly when Z score thresholds are used to guide diagnosis and management.
- Published
- 2020
33. Diretrizes de Multimodalidade de Imagem em Pacientes com Transposição das Grandes Artérias: Relatório da Sociedade Americana de Ecocardiografia Desenvolvido em Colaboração com a Sociedade de Ressonância Magnética Cardiovascular e Sociedade de Tomografia Computadorizada Cardiovascular
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B. Kelly Han, Peter C. Frommelt, Luc Mertens, Mark A. Fogel, RebeJatene K. Johnson, Benjamin W. Eidem, Frank Cetta, Andrew J. Powell, Leo Lopez, SPEThen M. Paridon, Javier Ganame, Thomas R. Kimball, and Meryl S. Cohen
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General Medicine - Published
- 2020
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34. A new, expanded approach to pediatric cardiology fellowship orientation
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Felina K. Mille, Meryl S. Cohen, Chitra Ravishankar, and Danielle S. Burstein
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medicine.medical_specialty ,Palliative care ,business.industry ,Cardiac anatomy ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,Orientation (mental) ,Preparedness ,Pediatrics, Perinatology and Child Health ,medicine ,Medical physics ,030212 general & internal medicine ,Technical skills ,Cardiology and Cardiovascular Medicine ,business ,Pediatric cardiology - Abstract
Background Because of limited and varying resident exposure to pediatric cardiology, rapidly acquiring the necessary knowledge, clinical and technical skills can be overwhelming for incoming pediatric cardiology fellows. To address this, we developed a pediatric cardiology fellowship orientation at the beginning of fellowship using an extended orientation model. Methods We designed and implemented a three-week extended orientation for all rising first year pediatric cardiology fellows at our institution to take place the first month of their training. The extended orientation consisted of: 1) daily lunchtime didactic sessions focused on general pediatric cardiology topics; 2) hands-on echocardiography experience (total 4.5 days) and high fidelity simulations focused on cardiac emergencies, extracorporeal membrane oxygenation, and cardiac palliative care; and 3) day-long precepted experiences in core clinical rotations. Pre- and post-extended orientation knowledge self-assessment results were compared using paired t-tests. Results Due to protection from clinical duties during the three-week extended orientation period, all seven first-year pediatric cardiology fellows were able to participate in 100% of these sessions. After the extended orientation, participants demonstrated significant improvement in self-assessed knowledge in all clinical areas, with mean scores increasing from 45 ± 15% pre- to 74 ± 11% post-orientation (p ≤ 0.001). The greatest improvements occurred in echocardiography, catheterization, and cardiac anatomy. Based on a post-orientation feedback, additional education dedicated to electrophysiology, including EKG interpretation, arrhythmia management, and pacemaker basics, was recommended. Conclusion A three-week extended orientation model for pediatric cardiology fellowship orientation at the beginning of fellowship effectively introduces incoming fellows to key clinical topics unique to cardiology. In the current educational era, this represents an alternative approach to the traditional orientation model. Facilitating full participation in these sessions without competing clinical duties may enable all fellows to start their training with the necessary clinical foundation and may improve their preparedness for clinical responsibilities.
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- 2018
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35. Postoperative Obstruction of the Pulmonary Veins in Mixed Total Anomalous Pulmonary Venous Connection
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Brian R. White, Deborah Ho, Andrew C. Glatz, Meryl S. Cohen, Paul Stephens, and Christopher E. Mascio
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Male ,Reoperation ,medicine.medical_specialty ,Heart disease ,030204 cardiovascular system & hematology ,Pulmonary vein ,03 medical and health sciences ,Postoperative Complications ,0302 clinical medicine ,medicine ,Mixed total anomalous pulmonary venous connection ,Humans ,030212 general & internal medicine ,Cardiac Surgical Procedures ,Total anomalous pulmonary venous connection ,Child ,Coronary sinus ,Retrospective Studies ,Surgical repair ,business.industry ,Scimitar Syndrome ,Angiography ,Infant, Newborn ,Infant ,Vascular surgery ,medicine.disease ,Cardiac surgery ,Surgery ,Pulmonary Veins ,Pediatrics, Perinatology and Child Health ,cardiovascular system ,Cardiology and Cardiovascular Medicine ,business - Abstract
Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease in which the pulmonary veins drain by various pathways to the right atrium instead of the left atrium. Postoperative obstruction of the pulmonary veins is a known complication. Identifying risk factors for morbidity and mortality is important for counseling and monitoring. We describe a pattern of postoperative obstruction in a specific arrangement of mixed TAPVC. Five patients with a type of mixed TAPVC, namely, three pulmonary veins connecting to the coronary sinus and the left upper pulmonary vein (LUPV) connecting to the innominate vein, were identified over an 11-year period at our institution. Two additional patients with this TAPVC arrangement were cared for at our institution after having surgery at other institutions. Of these, one patient received only comfort care at birth due to other clinical issues. The six other patients underwent surgical unroofing of the coronary sinus. The anomalous LUPV was not addressed during the initial surgery in any of these cases. Following repair, one patient died from non-cardiac reasons. The remaining five patients all developed obstruction of the repaired pulmonary veins with decompression through the unrepaired LUPV, requiring surgical revision. Three patients underwent a second reoperation as well. Three of the six repaired patients also developed refractory atrial arrhythmias. This cohort suggests that this mixed TAPVC pattern predisposes patients to obstruction after surgical repair. Further investigation may aid pediatric cardiologists in risk-stratifying and counseling these patients. Alternative surgical approaches may need to be considered.
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- 2018
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36. Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome
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Jami C. Levine, David A. Parra, Julie De Backer, Elif Seda Selamet Tierney, Arni Nutting, Reed E Pyeritz, Meryl S. Cohen, Gail D. Pearson, Aimee Liou, Shubhika Srivastava, Timothy J. Bradley, Steven D. Colan, Angela M. Sharkey, Aaron K Olson, Shan Chen, Arvind Hoskoppal, M. Jay Campbell, Edward Marcus, Haleh Heydarian, Mary Ella M Pierpont, William Ravekes, Lynn A. Sleeper, Luciana Young, Ronald V. Lacro, Mary J. Roman, Wyman W. Lai, and Beth F. Printz
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0301 basic medicine ,Marfan syndrome ,medicine.medical_specialty ,Adolescent ,Aortic Diseases ,Kaplan-Meier Estimate ,030204 cardiovascular system & hematology ,Risk Assessment ,Severity of Illness Index ,Drug Administration Schedule ,Losartan ,Article ,Marfan Syndrome ,03 medical and health sciences ,Vascular Stiffness ,0302 clinical medicine ,Internal medicine ,medicine.artery ,Ascending aorta ,medicine ,Humans ,Child ,Survival rate ,Aorta ,Proportional Hazards Models ,Dose-Response Relationship, Drug ,business.industry ,Hazard ratio ,Prognosis ,medicine.disease ,Atenolol ,Confidence interval ,Survival Rate ,Cardiac Imaging Techniques ,Treatment Outcome ,030104 developmental biology ,Child, Preschool ,Linear Models ,cardiovascular system ,Cardiology ,Aortic stiffness ,Cardiology and Cardiovascular Medicine ,business ,medicine.drug - Abstract
The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed. Stiffness index (SI) and elastic modulus (EM) were calculated for aortic root and ascending aorta. Data were analyzed using multivariable mixed effects modeling and Cox regression. Heart rate–corrected aortic-root SI over 3 years decreased with atenolol but did not change with losartan (−0.298 ± 0.139 vs 0.141 ± 0.139/year, p = 0.01). In the entire cohort, above-median aortic-root SI (>9.1) and EM (>618 mm Hg) predicted a smaller annual decrease in ARz (p ≤0.001). Upper-quartile aortic-root EM (>914 mm Hg) predicted the composite outcome of aortic-root surgery, dissection, or death (hazard ratio 2.17, 95% confidence interval 1.02 to 4.63, p = 0.04). Crude 3-year event rates were 10.4% versus 3.2% for higher versus lower EM groups. In conclusion, atenolol was associated with a decrease in aortic-root SI, whereas losartan was not. Higher baseline aortic-root SI and EM were associated with a smaller decrease in ARz and increased risk for clinical outcomes. These data suggest that noninvasive aortic stiffness measures may identify patients at higher risk of progressive aortic enlargement and adverse clinical outcomes, potentially allowing for closer monitoring and more aggressive therapy.
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- 2018
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37. Total Anomalous Pulmonary Venous Return with No Connection
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Meryl S. Cohen, Valerie DeMatteo, Michael D. Quartermain, Michael Liu, Michael P. DiLorenzo, and Craig Tinker
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medicine.medical_specialty ,business.industry ,Misconnection Consequence ,Pulmonary vein atresia ,General Medicine ,030204 cardiovascular system & hematology ,Total anomalous pulmonary venous connection ,medicine.disease ,Total anomalous pulmonary venous return ,Connection (mathematics) ,03 medical and health sciences ,0302 clinical medicine ,Echocardiography ,030225 pediatrics ,Internal medicine ,Cardiology ,Medicine ,business ,ComputingMethodologies_COMPUTERGRAPHICS - Abstract
Graphical abstract, Highlights • Total anomalous pulmonary venous return with no connection has a poor prognosis. • Echocardiography aids in diagnosing unique features associated with this disease. • Additional diagnostic imaging modalities may be needed to confirm diagnosis.
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- 2018
38. Intraoperative transesophageal echocardiographic predictors of recurrent left ventricular outflow tract obstruction in children undergoing subaortic stenosis resection
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Meryl S. Cohen, Laura Mercer-Rosa, Alexandra Channing, and Hythem Nawaytou
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Adult ,Male ,Aortic valve ,medicine.medical_specialty ,Adolescent ,Ventricular outflow tract obstruction ,Ventricular Outflow Obstruction ,030204 cardiovascular system & hematology ,Doppler echocardiography ,Cardiac Valve Annuloplasty ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Predictive Value of Tests ,Risk Factors ,Monitoring, Intraoperative ,Internal medicine ,medicine ,Humans ,Ventricular outflow tract ,Radiology, Nuclear Medicine and imaging ,030212 general & internal medicine ,Child ,Retrospective Studies ,medicine.diagnostic_test ,business.industry ,Infant ,Retrospective cohort study ,Aortic Stenosis, Subvalvular ,Echocardiography, Doppler ,medicine.anatomical_structure ,Aortic Valve ,Child, Preschool ,Predictive value of tests ,cardiovascular system ,Cardiology ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Echocardiography, Transesophageal ,Follow-Up Studies - Abstract
Background Intraoperative transesophageal echocardiography (iTEE) is used to assess for residual left ventricular outflow tract obstruction (LVOTO) after surgical resection of subaortic membrane causing subaortic stenosis (sub-AS). We aimed to identify the iTEE features associated with recurrence of LVOTO. Methods We conducted a retrospective study of children undergoing sub-AS resection from June 2006 to June 2014. Doppler assessment of the flow velocity and the anatomical features of the left ventricular outflow tract were analyzed from stored echocardiograms. Recurrent LVOTO was defined as an increase in the mean pressure gradient across the left ventricular outflow tract of > 15 mm Hg on the most recent follow-up echocardiogram from the mean pressure gradient on the predischarge echocardiogram or as doubling of the mean pressure gradient to a value ≥20 mm Hg. Results Thirty-five patients were included, with median age at surgery was 8.1 years (range: 0.7-29 years) and median follow-up was 47 months (2-91 months). Ten patients (29%) had recurrent LVOTO, which was associated with a shorter distance between the narrowest diameter of the outflow tract and the aortic valve on iTEE [median 0.59 cm (range 0.39-0.74) vs 0.98 cm (0.75-1.5), P = .03]. No patients with more than mild residual LVOTO on iTEE regressed to mild or no LVOTO on follow-up echocardiograms. Conclusions LVOTO recurrence after sub-AS resection is common, and residual LVOTO remains the same or increases over time. Proximity of the LVOTO to the aortic valve is a risk factor for recurrent LVOTO. These findings may be useful in counseling patients and to guide the frequency of postoperative follow-up.
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- 2018
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39. Assessing the borderline ventricle in a term infant
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Meryl S. Cohen
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Heart Defects, Congenital ,medicine.medical_specialty ,Surgical strategy ,Heart Ventricles ,Clinical Decision-Making ,Heart Valve Diseases ,Physiology ,Aorta, Thoracic ,030204 cardiovascular system & hematology ,Fontan Procedure ,Ventricular Outflow Obstruction ,03 medical and health sciences ,0302 clinical medicine ,Quality of life ,medicine ,Humans ,Pulmonary blood flow ,business.industry ,Heart Septal Defects ,Infant, Newborn ,Infant ,Aortic Valve Stenosis ,medicine.disease ,Pulmonary hypertension ,Cardiac surgery ,Ebstein Anomaly ,medicine.anatomical_structure ,030228 respiratory system ,Term Infant ,Pulmonary Atresia ,Ventricle ,Mitral Valve ,Cardiology and Cardiovascular Medicine ,business ,Pediatric cardiology - Abstract
PURPOSE OF REVIEW The purpose of this review is to describe the challenges associated with the diagnosis and treatment of children with borderline ventricles. A borderline ventricle is one in which there is concern that it will not be able to support its circulation. If a biventricular repair is attempted and fails, outcome is often poor. Thus, this early decision is important. RECENT FINDINGS For the borderline right ventricle, options to add an additional source of pulmonary blood flow make the surgical strategy a bit more flexible than for patients with a borderline left ventricle. In general, outcome for a so-called one and one-half ventricle repair are generally good, though the long-term outcome and the effects of this physiology on lifelong exercise performance and quality of life remain to be seen. For the small left ventricle, often multiple surgeries are required to 'force' blood into the left ventricle and potentially help it grow. Though this strategy is successful in some, in others it results in significant residual cardiac issues including pulmonary hypertension. SUMMARY Determining whether a patient will be better off in the long term with a marginal biventricular repair versus a Fontan circulation remains one of the most difficult problems in the field of pediatric cardiology and cardiac surgery.
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- 2018
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40. Parents Request Withdrawing Feeding From Neurologically Impaired Newborn
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Meryl S. Cohen, Carlen G. Fifer, and David M. Kwiatkowski
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Male ,Parents ,Pulmonary and Respiratory Medicine ,Brain Diseases ,Pediatrics ,medicine.medical_specialty ,business.industry ,Clinical Decision-Making ,Infant, Newborn ,MEDLINE ,Enteral Nutrition ,Parenteral nutrition ,Withholding Treatment ,Humans ,Medicine ,Surgery ,Bioethical Issues ,Cardiology and Cardiovascular Medicine ,business ,Attitude to Health ,Decision Making, Shared ,Neurologically impaired - Published
- 2019
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41. Trisomy 13, Large Ventricular Septal Defect With Failure to Thrive: Family Wishes to Have Complete Repair
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Ashraf S Harahsheh and Meryl S. Cohen
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Heart Septal Defects, Ventricular ,Male ,Pulmonary and Respiratory Medicine ,Pediatrics ,medicine.medical_specialty ,Trisomy 13 Syndrome ,Clinical Decision-Making ,MEDLINE ,Clinical decision making ,Humans ,Medicine ,Family ,Bioethical Issues ,Cardiac Surgical Procedures ,Heart septal defect ,business.industry ,Infant ,medicine.disease ,Failure to Thrive ,Failure to thrive ,Surgery ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Trisomy ,Attitude to Health ,Decision Making, Shared - Published
- 2019
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42. Echocardiography in Pediatric and Congenital Heart Disease : From Fetus to Adult
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Wyman W. Lai, Luc L. Mertens, Meryl S. Cohen, Tal Geva, Wyman W. Lai, Luc L. Mertens, Meryl S. Cohen, and Tal Geva
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- Echocardiography, Congenital heart disease--Imaging
- Abstract
The new edition of the acclaimed reference text on the most critical tool in pediatric cardiology practice Echocardiography in Pediatric and Congenital Heart Disease provides comprehensive guidance on the use of non-invasive ultrasound imaging in the diagnosis and treatment of pediatric cardiac conditions. Written by a team of experts from the world's leading pediatric cardiology centers, this highly-illustrated, full-color reference covers anatomy, pathophysiology, ultrasound physics, laboratory setup, patient preparation and safety, pediatric echocardiogram protocols, quantitative methods of echocardiographic evaluation, and more. Offering a wealth of additional material on state-of-the-art techniques and technologies in echocardiography, the thoroughly revised third edition features entirely new chapters on examination guidelines and standards, quality improvement in the laboratory, perioperative echocardiography, hemodynamic assessment of the neonate, early fetal echocardiography, and multimodality imaging. This edition offers updated and expanded discussion of the latest advances in echocardiography, particularly those related to speckle tracking and 3D echocardiography. An essential resource for all practitioners, instructors, and trainees in the field, Echocardiography in Pediatric and Congenital Heart Disease: Provides up-to-date reference to ultrasound imaging of the hearts of fetuses, children, and adults with both acquired and congenital heart disease Covers the echocardiographic examination of congenital cardiovascular abnormalities before, during, and after treatment Describes quantitative methods of echocardiographic evaluation, including assessment of diastolic function, right ventricular function and assessment of the post-Fontan patient Discusses intraoperative echocardiography, heart disease in pregnancy, and other special techniques and topics Includes more than 1200 high-quality color images as well as a companion website with over 600 video clips Echocardiography in Pediatric and Congenital Heart Disease, Third Edition, remains an essential textbook for cardiac sonographers, pediatric and adult cardiologists, echocardiography nurses and technicians, and adult cardiologists with interest in congenital heart disease.
- Published
- 2022
43. A vascular endothelial growth factor A genetic variant is associated with improved ventricular function and transplant-free survival after surgery for non-syndromic CHD
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Jerry H. Kim, Donna M. McDonald-McGinn, Daniel Seung Kim, J. William Gaynor, Deborah A. Nickerson, David R. Crosslin, Mark W. Russell, Nancy Burnham, Meryl S. Cohen, Thomas L. Spray, Constantine D. Mavroudis, Ian B. Stanaway, Alexandra H Morss, Hakon Hakonarson, Gail P. Jarvik, Susan C. Nicolson, Amber A. Burt, and Elaine H. Zackai
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Heart Defects, Congenital ,Male ,Vascular Endothelial Growth Factor A ,0301 basic medicine ,medicine.medical_specialty ,Adolescent ,SOD2 ,030204 cardiovascular system & hematology ,Polymorphism, Single Nucleotide ,law.invention ,03 medical and health sciences ,0302 clinical medicine ,law ,medicine ,Cardiopulmonary bypass ,Humans ,Ventricular Function ,Cardiac Surgical Procedures ,Allele ,Child ,Alleles ,Philadelphia ,business.industry ,Infant, Newborn ,Infant ,General Medicine ,Stepwise regression ,Cardiac surgery ,Surgery ,Minor allele frequency ,Vascular endothelial growth factor A ,030104 developmental biology ,Echocardiography ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Linear Models ,Heart Transplantation ,Female ,Cardiology and Cardiovascular Medicine ,business ,Vascular endothelial growth factor production ,Follow-Up Studies - Abstract
BackgroundWe have previously shown that the minor alleles of vascular endothelial growth factor A (VEGFA) single-nucleotide polymorphism rs833069 and superoxide dismutase 2 (SOD2) single-nucleotide polymorphism rs2758331 are both associated with improved transplant-free survival after surgery for CHD in infants, but the underlying mechanisms are unknown. We hypothesised that one or both of these minor alleles are associated with better systemic ventricular function, resulting in improved survival.MethodsThis study is a follow-up analysis of 422 non-syndromic CHD patients who underwent neonatal cardiac surgery with cardiopulmonary bypass. Echocardiographic reports were reviewed. Systemic ventricular function was subjectively categorised as normal, or as mildly, moderately, or severely depressed. The change in function was calculated as the change from the preoperative study to the last available study. Stepwise linear regression, adjusting for covariates, was performed for the outcome of change in ventricular function. Model comparison was performed using Akaike’s information criterion. Only variables that improved the model prediction of change in systemic ventricular function were retained in the final model.ResultsGenetic and echocardiographic data were available for 335/422 subjects (79%). Of them, 33 (9.9%) developed worse systemic ventricular function during a mean follow-up period of 13.5 years. After covariate adjustment, the presence of the VEGFA minor allele was associated with preserved ventricular function (p=0.011).ConclusionsThese data support the hypothesis that the mechanism by which the VEGFA single-nucleotide polymorphism rs833069 minor allele improves survival may be the preservation of ventricular function. Further studies are needed to validate this genotype–phenotype association and to determine whether this mechanism is related to increased vascular endothelial growth factor production.
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- 2017
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44. Clinical findings in right ventricular noncompaction in hypoplastic left heart syndrome
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Monique M. Gardner and Meryl S. Cohen
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medicine.medical_specialty ,Noncompaction cardiomyopathy ,medicine.medical_treatment ,Cardiomyopathy ,030204 cardiovascular system & hematology ,Pericardial effusion ,Hypoplastic left heart syndrome ,03 medical and health sciences ,Pseudoaneurysm ,0302 clinical medicine ,Internal medicine ,medicine ,Radiology, Nuclear Medicine and imaging ,business.industry ,General Medicine ,medicine.disease ,Norwood Operation ,Surgery ,030228 respiratory system ,Pericardiocentesis ,Pediatrics, Perinatology and Child Health ,Cardiology ,Norwood procedure ,Cardiology and Cardiovascular Medicine ,business - Abstract
Background Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution. Methods We performed a retrospective review of patients diagnosed with RVNC over a 12-month period at our institution and reviewed their imaging and clinical course. Results Three patients were identified. All had diagnosis of RVNC by echocardiography (echo) made on postnatal imaging which reviewed degree of trabeculation, and noncompaction-to-compaction ratio of the myocardium. Patient A was a neonate with hypoplastic left heart syndrome (HLHS) who underwent a Norwood operation with Sano modification. Her postoperative course was notable for low-normal RV function. She returned with a pericardial effusion warranting immediate pericardiocentesis. She continued to have effusions, which were medically managed. She was subsequently found to have an RV apical pseudoaneurysm, which required surgical resection. Patient B was a neonate with HLHS who had a Norwood operation with Sano modification. She had low-normal RV function on echo. She required medical management for pericardial effusion. Patient C was a neonate with HLHS who also underwent a Norwood operation with Sano modification. His postoperative course was notable for elevated serum brain natriuretic peptide, which was treated with digoxin. Conclusion RVNC is a rare diagnosis with limited known clinical impact. One of these patients had a very rare complication after pericardiocentesis (pseudoaneurysm) that may have been related to the RVNC. Our understanding of this disease process is limited and requires additional investigation, but emphasizes the importance of appropriate diagnosis to allow for timely follow-up and counseling for this unique population.
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- 2017
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45. Milrinone Pharmacokinetics and Pharmacodynamics in Neonates with Persistent Pulmonary Hypertension of the Newborn
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Michael L. O'Byrne, Athena F. Zuppa, Ganesh S. Moorthy, Amit M. Mathur, Beena G. Sood, Meryl S. Cohen, Haresh Kirpalani, and Annie Giaccone
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Male ,Cardiotonic Agents ,Metabolic Clearance Rate ,Gestational Age ,Pilot Projects ,030204 cardiovascular system & hematology ,Nitric Oxide ,Persistent Fetal Circulation Syndrome ,Article ,Hypoxemia ,03 medical and health sciences ,Oxygen Consumption ,0302 clinical medicine ,Bolus (medicine) ,Pharmacokinetics ,Intensive Care Units, Neonatal ,030225 pediatrics ,Intensive care ,Humans ,Medicine ,Endothelium-Dependent Relaxing Factors ,Dose-Response Relationship, Drug ,business.industry ,Infant, Newborn ,Milrinone Lactate ,Obstetrics and Gynecology ,Gestational age ,Treatment Outcome ,Anesthesia ,Pharmacodynamics ,Pediatrics, Perinatology and Child Health ,Milrinone ,Administration, Intravenous ,Female ,medicine.symptom ,business ,medicine.drug - Abstract
Objective To describe the pharmacokinetics and pharmacodynamics of milrinone in infants with persistent pulmonary hypertension of the newborn (PPHN) and to explore the impact of age on milrinone disposition. Design Randomized, open label pilot study. Setting Multicenter; level 3 and level 4 neonatal intensive care units. Patients Six infants ≥34 weeks' gestational age and Intervention Intravenous milrinone lactate in one of two dosing regimens: (1) low dose, 20 mcg/kg bolus followed by 0.2 mcg/kg/minute, and (2) standard dose, 50 mcg/kg bolus followed by 0.5 mcg/kg/minute. Measurements and Main Results The final structural model was a two-compartment disposition model with interindividual variability estimated on clearance (CL). The estimated value of CL is 7.65 mL/minute/3.4 kg (3.05 mL/minute/kg). The addition of age improved the precision of the CL estimate, and CL increased with chronological age in days. The oxygenation index was highly variable within each participant and improved with time. There were no observed safety concerns in either dosing group. Conclusion The CL of milrinone in newborns with PPHN is reduced and increases with age. In this pilot study, we did not see significant pharmacodynamic or safety effects associated with drug exposure.
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- 2017
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46. EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries
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Meryl S, Cohen and Luc L, Mertens
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transposition of the great arteries ,Review ,congenitally corrected transposition of great arteries ,congenital heart defects - Abstract
Echocardiographic assessment of patients with transposition of the great arteries and congenitally corrected transposition requires awareness of the morphology and commonly associated lesions. The pre-operative echocardiography should include a full segmental and sequential analysis. Post-operative assessment is not possible without awareness of the type of surgical procedure performed and consists of assessing surgical connections and residual lesions.
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- 2019
47. Left Valvar Morphology Is Associated With Late Regurgitation in Atrioventricular Canal Defect
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Meryl S. Cohen, Heather Griffis, Hannah Katcoff, Stephanie Fuller, Deborah Ho, and Laura Mercer-Rosa
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Pulmonary and Respiratory Medicine ,Male ,medicine.medical_specialty ,Time Factors ,Genetic syndromes ,Regurgitation (circulation) ,030204 cardiovascular system & hematology ,03 medical and health sciences ,symbols.namesake ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,Atrioventricular canal defect ,Cardiac Surgical Procedures ,Papillary muscle ,Fisher's exact test ,Retrospective Studies ,business.industry ,Heart Septal Defects ,Infant ,Mitral Valve Insufficiency ,Odds ratio ,Papillary Muscles ,medicine.disease ,medicine.anatomical_structure ,030228 respiratory system ,Echocardiography ,cardiovascular system ,symbols ,Cardiology ,Atrioventricular canal ,Surgery ,Female ,Cardiology and Cardiovascular Medicine ,business ,Left atrioventricular valve regurgitation ,Follow-Up Studies - Abstract
Background Left atrioventricular valve regurgitation (LAVVR) after atrioventricular canal (AVC) repair remains a significant cause of morbidity. Papillary muscle arrangement may be important. To investigate the implications of left mural leaflet morphology, we examined anatomic characteristics of the LAVV to determine possible associations with postoperative LAVVR. Methods All patients with biventricular AVC repair at our institution between January 1, 2011, and December 31, 2016, with necessary imaging were retrospectively reviewed. We assessed papillary muscle structure and novel measures of the left mural leaflet from preoperative echocardiograms and the degree of LAVVR from the first and last available follow-up echocardiograms. Associations with degree of early and late postoperative LAVVR were assessed with t tests, analysis of variance, or χ2 or Fisher exact tests, and multivariable logistic regression. Results There were 58 of 156 patients (37%) with significant (moderate or severe) early postoperative LAVVR, and 30 of 93 (32%) had significant LAVVR after 6 or more months. Fewer patients with closely spaced or asymmetric papillary muscles had moderate or severe late LAVVR vs those with widely spaced papillary muscles (17% vs 40%, P = .019). Controlling for weight at operation, genetic syndromes, and bypass time, widely spaced papillary muscles increased the odds ratio for late LAVVR to 3.6 (P = .026). Larger mural leaflet area was also associated with late LAVVR on univariable (P = .019) and multivariable (P = .023) analyses. One-third of patients with significant late LAVVR had no significant early postoperative regurgitation. Conclusions Mural leaflet and papillary muscle anatomy are associated with late LAVVR after AVC repair. Late regurgitation can develop in the absence of early LAVVR, suggesting different mechanisms.
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- 2019
48. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair
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Meryl S. Cohen, Thomas L. Spray, Anusha Jegatheeswaran, Christopher E. Mascio, Stephanie Fuller, and J. William Gaynor
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Pulmonary and Respiratory Medicine ,Heart Septal Defects, Ventricular ,Male ,medicine.medical_specialty ,Defect repair ,medicine.medical_treatment ,Hemodynamics ,Ventricular Septum ,030204 cardiovascular system & hematology ,03 medical and health sciences ,0302 clinical medicine ,Hematoma ,Extracorporeal Membrane Oxygenation ,Postoperative Complications ,medicine ,Extracorporeal membrane oxygenation ,Humans ,Decompensation ,cardiovascular diseases ,Interventricular septum ,Tetralogy of Fallot ,Retrospective Studies ,business.industry ,Infant, Newborn ,Infant ,medicine.disease ,Surgery ,medicine.anatomical_structure ,030228 respiratory system ,cardiovascular system ,Female ,Cardiology and Cardiovascular Medicine ,business ,Complication ,Echocardiography, Transesophageal - Abstract
Objectives Interventricular septal hematoma (IVSH) is a rare complication, which may result from ventricular septal defect (VSD) repair. IVSH can result in conduction and/or hemodynamic abnormalities related to impaired ventricular filling or outflow tract obstruction. We report the novel use of extracorporeal membrane oxygenation (ECMO) for management. Methods Echocardiography reports (January 1980 to December 2016) were searched for the term "hematoma" in our institutional database and reviewed to determine appropriate cases. Charts and imaging (reports) data were abstracted. All intraoperative and select postoperative echocardiograms were reread by a pediatric cardiologist. Results N = 12 patients with IVSH. Mean age and weight at surgery were 59 ± 41 days and 3.4 (2.9-5.1) kg, respectively, while the most frequent diagnosis was tetralogy of Fallot. Although all patients had intraoperative transesophageal echocardiography (TEE), only 55% (6 of 11, missing [m] = 1) of IVSH were discovered intraoperatively. Of the 5 patients not discovered intraoperatively (m = 1), IVSH was postoperatively detected secondary to arrhythmia/decompensation by echocardiogram 10.1 ± 7.9 hours postoperatively. Five patients (42%) were managed with ECMO (1 unable to separate from bypass). Overall mortality was 33%. For patients in whom ECMO was used, 2 of 5 (40%) survived. Mean time to IVSH resolution in all survivors was 20 ± 185 days. Conclusions IVSH from VSD repair can result in clinical decompensation and mortality. This may relate to the high proportion missed intraoperatively. ECMO should be considered an important modality, which can allow for IVSH resolution. However, considerations must be made to allow for appropriate anticoagulation to avoid hematoma expansion and repeat imaging during ECMO to continually assess the interventricular septum.
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- 2019
49. A Noncompliant Teen Needs a Second Heart Transplant
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Meryl S. Cohen, David M. Peng, and Carlen G. Fifer
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,business.industry ,MEDLINE ,Medication adherence ,Medication Adherence ,Family medicine ,Retreatment ,medicine ,Heart Transplantation ,Humans ,Surgery ,Female ,Bioethical Issues ,Cardiology and Cardiovascular Medicine ,business ,Child - Published
- 2019
50. Pulmonary artery banding in complete atrioventricular septal defect
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Craig E. Fleishman, Tara Karamlou, Paul J. Devlin, Brian W. McCrindle, William G. Williams, Jeanne M. Baffa, Christian Pizarro, Luc Mertens, Ali Dodge-Khatami, Kamal Pourmoghadam, Eugene H. Blackstone, Pirooz Eghtesady, Anusha Jegatheeswaran, Jeffrey P. Jacobs, Meryl S. Cohen, David B. Meyer, William M. DeCampli, Cheryl Fackoury, and David M. Overman
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Pulmonary and Respiratory Medicine ,Male ,Reoperation ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Complete atrioventricular septal defect ,Regurgitation (circulation) ,Pulmonary Artery ,Pulmonary artery banding ,Cohort Studies ,Internal medicine ,Medicine ,Humans ,cardiovascular diseases ,Atrioventricular Septal Defect ,Atrioventricular valve ,business.industry ,Heart Septal Defects ,Infant ,Survival Rate ,Treatment Outcome ,Echocardiography ,cardiovascular system ,Cardiology ,Surgery ,Female ,Cardiology and Cardiovascular Medicine ,business - Abstract
Objectives To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. Methods PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. Results At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). Conclusions PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.
- Published
- 2019
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