Diseases Commons^™

Association Of Ireb2 And Chrna3 Polymorphisms With Airflow Obstruction In Severe Alpha-1 Antitrypsin Deficiency., Woo Jin Kim, Alice M. Wood, Alan F. Barker, Mark L. Brantly, Edward J. Campbell, Edward Eden, Gerard Mcelvaney, Stephen I. Rennard, Robert A. Sandhaus, James M. Stocks, James K. Stoller, Charlie Strange, Gerard Turino, Edwin K. Silverman, Robert A. Stockley, Dawn L. Demeo

Journal Articles: Pulmonary & Critical Care Med

BACKGROUND: The development of COPD in subjects with alpha-1 antitrypsin (AAT) deficiency is likely to be influenced by modifier genes. Genome-wide association studies and integrative genomics approaches in COPD have demonstrated significant associations with SNPs in the chromosome 15q region that includes CHRNA3 (cholinergic nicotine receptor alpha3) and IREB2 (iron regulatory binding protein 2).We investigated whether SNPs in the chromosome 15q region would be modifiers for lung function and COPD in AAT deficiency.

METHODS: The current analysis included 378 PIZZ subjects in the AAT Genetic Modifiers Study and a replication cohort of 458 subjects from the UK AAT Deficiency National …

Bioabsorbable Atrial Septal Occluder For Percutaneous Closure Of Atrial Septal Defect In Children, Osman Baspinar, Mehmet Kervancioglu, Metin Kilinc, Ahmet Irdem

The Texas Heart Institute Journal

The BioSTAR bioabsorbable septal repair implant is a new transcatheter secundum atrial septal defect occlusion device that is absorbed and replaced by healthy native tissue. This retrospective analysis was designed to determine the most significant factors for its successful use in children. From October 2009 through December 2010, 33 children underwent catheterization to close secundum atrial septal defects by means of the BioSTAR. The mean age of the patients was 6.8 ± 3.4 years (range, 2.5-13 yr), and the mean body weight was 22.6 ± 11 kg (range, 11-55 kg). The device was successfully implanted in 91% of patients (30/33). …

False Scents, False Sense, And False Cents: Why Physicians Should Read Sherlock Holmes, Ira Martin Grais

The Texas Heart Institute Journal

No abstract provided.

Culprit-Vessel Percutaneous Coronary Intervention Followed By Contralateral Angiography Versus Complete Angiography In Patients With St-Elevation Myocardial Infarction, Chadi Dib, Elias B Hanna, Muhammad A Chaudhry, Thomas A Hennebry, Stavros Stavrakis, Mazen S Abu-Fadel

The Texas Heart Institute Journal

In patients with ST-elevation myocardial infarction, delay in door-to-balloon time strongly increases mortality rates. To our knowledge, no randomized studies to date have focused on reducing delays within the catheterization laboratory.

We performed a retrospective analysis of all patients who presented with ST-elevation myocardial infarction at our institution from July 2006 through June 2010, looking primarily at time differences between percutaneous coronary intervention in the culprit vessel on the basis of ECG criteria, followed by contralateral angiography (Group 1), versus complete coronary angiography followed by culprit-vessel percutaneous intervention (Group 2).

There were 49 patients in Group 1 and 57 patients …

Nitinol Thermoreactive Clips For Secondary Sternal Closure In Cases Of Noninfective Sternal Dehiscence, Arif Gucu, Faruk Toktas, Cuneyt Eris, Yusuf Ata, Tamer Turk

The Texas Heart Institute Journal

Postoperative sternal dehiscence is a potentially catastrophic sequela to median sternotomy that can cause not only chest-wall discomfort and pulmonary dysfunction but infection, both superficial and mediastinal. Nitinol thermoreactive clips use a novel material in the treatment of sternal dehiscence. We sought to determine whether the use of these clips is an effective remedy for noninfective sternal dehiscence.

From January 2008 through December 2011, we retrospectively studied the data on 10 patients whose sternums had been closed with nitinol thermoreactive clips after the development of noninfective sternal dehiscence. Diagnosis was made on the bases of clinical criteria, chest radiography, and …

Does The Expression Of Transforming Growth Factor Β-1: Affect The Outcome Of The Radiofrequency Modified Maze Procedure In Patients With Rheumatic Atrial Fibrillation?, Wei Wang, Lei Liu, Yuan Li, Sheng-Shou Hu, Yun-Hu Song, Xin Wang

The Texas Heart Institute Journal

We investigated whether the expression of transforming growth factor β-1 in the left atrial appendage affected the outcome of the radiofrequency modified maze procedure in patients with rheumatic valve disease and long-standing persistent atrial fibrillation.

Messenger RNA and protein expression of transforming growth factor β-1 and volume fractions of collagen types I and III were measured in 80 patients with rheumatic valve atrial fibrillation who underwent valve surgery with the radiofrequency modified maze procedure; the same was done in a control group of 20 patients with rheumatic valve disease and sinus rhythm who underwent valve surgery alone.

At 6 months' …

Extreme Thrombocytosis And Cardiovascular Surgery: Risks And Management, Ethan A Natelson

The Texas Heart Institute Journal

Extreme thrombocytosis is a major risk factor for excessive bleeding and for thrombosis, either of which can complicate cardiovascular surgical and interventional procedures. Extreme thrombocytosis can also cause an unusual syndrome, erythromelalgia, that results in a type of chronic microvascular occlusive arterial disease. We present the differential diagnosis of conditions that may lead to extreme thrombocytosis, 3 cases (each of which illustrates a different potential complication), and a review of the pertinent medical literature. Correcting excessive thrombocytosis is typically not difficult, whether electively or acutely, and effective therapy usually controls thrombosis and excessive hemorrhage post-procedurally.

Long-Term Results Of Aortic Banding For Complex Infrarenal Neck Anatomy And Type I Endoleak After Endovascular Abdominal Aortic Aneurysm Repair, Zvonimir Krajcer, Kathryn G Dougherty, Igor D Gregoric

The Texas Heart Institute Journal

For many patients with abdominal aortic aneurysm, unsuitable anatomy of the infrarenal aortic neck precludes endovascular aortic aneurysm repair or causes type I endoleak after the procedure. In an attempt to overcome these challenges, we retrospectively examined the usefulness of aortic banding as an adjunctive procedure to endovascular repair in 8 patients who had an abdominal aortic aneurysm with a complex infrarenal aortic neck. The procedures were performed with the patients under general anesthesia and involved making an 8-cm upper-midline laparotomy incision to expose the aneurysmal aorta. Three patients underwent aortic banding before endovascular repair; the other 5 underwent banding …

Potential Role Of Plasma Myeloperoxidase Level In Predicting Long-Term Outcome Of Acute Myocardial Infarction, Mehmet Gungor Kaya, Ridvan Yalcin, Kaan Okyay, Fatih Poyraz, Nilufer Bayraktar, Hatice Pasaoglu, Bulent Boyaci, Atiye Cengel

The Texas Heart Institute Journal

We investigated the prognostic importance of plasma myeloperoxidase levels in patients with ST-elevation myocardial infarction (STEMI) at long-term follow-up, and we analyzed the correlations between plasma myeloperoxidase levels and other biochemical values.

We evaluated 73 consecutive patients (56 men; mean age, 56 ±11 yr) diagnosed with acute STEMI and 46 age- and sex-matched healthy control participants. Patients were divided into 2 groups according to the median myeloperoxidase level (Group 1: plasma myeloperoxidase ≤68 ng/mL; and Group 2: plasma myeloperoxidase >68 ng/mL). Patients were monitored for the occurrence of major adverse cardiovascular events (MACE), which were defined as cardiac death; reinfarction; …

Eptifibatide-Induced Thrombocytopenia: With Thrombosis And Disseminated Intravascular Coagulation Immediately After Left Main Coronary Artery Percutaneous Coronary Angioplasty, Michael W Tempelhof, Keith H Benzuly, Dan Fintel, Marc Z Krichavsky

The Texas Heart Institute Journal

Early clinical trials of eptifibatide did not show a significant association between eptifibatide and the development of thrombocytopenia, thrombosis, or disseminated intravascular coagulation. However, more recent literature has suggested a significant association between eptifibatide and the development of thrombocytopenia and thrombosis. Although the true incidence and the pathophysiology of these associations are unknown, the development of these events can be life-threatening. Herein, we describe the case of a patient who experienced acute onset of profound thrombocytopenia, developing thrombosis, pulmonary emboli, and disseminated intravascular coagulation. This paper adds to the few previous reports of cases that suggested an association between thrombocytopenia, …

Accuracy Of Automated Attenuation-Based 3-Dimensional Segmentation: In The Analysis Of Left Ventricular Function Compared With Magnetic Resonance Imaging, Harald Brodoefel, Ilias Tsiflikas, Ulrich Kramer, Nina Lang, Anja Reimann, Christoph Burgstahler, Claus D Claussen, Martin Heuschmid

The Texas Heart Institute Journal

We evaluated attenuation-based 3-dimensional segmentation for the analysis of left ventricular function, using as our standard of reference magnetic resonance imaging and dual-source computed tomography with traditional short-axis planimetry.

Twenty patients with known or suspected coronary artery disease were examined prospectively. In all magnetic resonance and computed tomographic datasets, global functional values were determined by 2-dimensional planimetry. Computed tomographic scans were further evaluated by automated 3-dimensional segmentation, and the results were compared by Pearson correlation and Bland-Altman analysis.

Agreement between magnetic resonance imaging and dual-source computed tomographic 2-dimensional planimetry was good for all values (end-diastolic volume, bias= −4.2, r=0.99; end-systolic …

Constrictive Pericarditis Treated By Surgery, Alireza A Ghavidel, Maziar Gholampour, Majid Kyavar, Yalda Mirmesdagh, Mohammad-Bagher Tabatabaie

The Texas Heart Institute Journal

We reviewed the records of 45 patients (mean age, 46.6 ± 14.9 yr; range, 21–84 yr) with a diagnosis of constrictive pericarditis who had undergone pericardiectomy from 1994 through 2006. Preoperatively, 2 of the patients (4.4%) were in New York Heart Association (NYHA) functional class I, 20 (44.4%) in class II, 22 (48.9%) in class III, and 1 (2.2%) in class IV. Pericardial calcification was detected in 20% of plain chest radiographs. Constrictive pericarditis was caused by tuberculosis in 22.2%, chronic renal failure in 8.9%, a history of sternotomy in 4.4%, and malignancy in 4.4%. The cause was idiopathic in …

Acute Promyelocytic Leukemia As A Cause Of Intracoronary Drug-Eluting-Stent Thrombosis, Zaven Sargsyan, Christopher Higgins, Sanda Alexandrescu, David A Ott, Surendra K Jain

The Texas Heart Institute Journal

Stent thrombosis is a potentially lethal complication of percutaneous coronary intervention. We describe the case of a 51-year-old man who presented with acute anterior ST-segment-elevation myocardial infarction and underwent successful percutaneous transluminal coronary angioplasty and placement of 3 drug-eluting stents in the left anterior descending coronary artery. Despite receiving dual antiplatelet therapy, the patient presented a week later with a non-ST-segment-elevation myocardial infarction and was found to have nonocclusive thrombosis of the left anterior descending coronary artery stents and his ostial left main and left circumflex coronary arteries. Subsequently, bone marrow biopsy analysis indicated that the patient had acute myelogenous …

Restoration Of Microcirculatory Patency After Myocardial Infarction: Results Of Current Coronary Interventional Strategies And Techniques, Marlos R Fernandes, R David Fish, John Canales, Jonathan Aliota, Guilherme V Silva, Emerson C Perin, Macarthur A Elayda, James M Wilson

The Texas Heart Institute Journal

We sought to evaluate the restoration of microcirculatory patency after primary percutaneous coronary intervention (PCI) in an unselected cohort of patients at a tertiary center.

We retrospectively evaluated distributions of the Thrombolysis in Myocardial Infarction (TIMI) myocardial perfusion grade (TMPG) and the myocardial blush grade (MBG) in all primary PCI procedures performed at our institution during 2008. We defined optimal microvascular perfusion as simultaneous TMPG 3 and MBG 3 at procedure's end.

Ninety-nine patients (mean age, 61.5 ± 12.7 yr; 64 men) underwent primary PCI. Microvascular perfusion was optimal in 69 patients (69.7%) and was associated with lower peaks of …

Cellular Retrograde Cardiomyoplasty And Relaxin Therapy For Postischemic Myocardial Repair In A Rat Model, Gabriella Di Lascio, Guy Harmelin, Mattia Targetti, Cristina Nanni, Giacomo Bianchi, Tommaso Gasbarri, Sandro Gelsomino, Daniele Bani, Sandra Zecchi Orlandini, Massimo Bonacchi

The Texas Heart Institute Journal

We sought to determine whether skeletal myoblasts, wild-type or engineered to express relaxin, might improve myocardial viability and performance in a rat model of chronic myocardial infarction. Our purpose was to investigate a potential new therapy for heart failure.

From October 2005 through September 2009, we surgically induced acute myocardial infarction in 80 male Wistar rats. Thirty days after surgery, the rats underwent reoperation for the retrograde coronary venous infusion of skeletal myoblasts, relaxin, or both. The animals were randomly assigned to 4 experimental groups: R1 (the control group, which underwent saline-solution infusion), R2 (systemic relaxin therapy), R3 (myoblast infusion), …

Intramyocardial Hematoma From Blunt Trauma Mimicking Apical Hypertrophic Cardiomyopathy, Meta Linda Mobula, Sammy Zakaria, Glenn A Hirsch

The Texas Heart Institute Journal

No abstract provided.

Transcatheter Retrieval And Repositioning Of Embolized Stent From The Right Ventricle In An Infant, Daisuke Kobayashi, Harinder R Singh, Daniel R Turner, Thomas J Forbes, Srinath T Gowda

The Texas Heart Institute Journal

Intracardiac stent embolization is a challenging complication in a small infant. A Palmaz stent was placed across the atrial septum in a 3-month-old boy to relieve symptoms of right-side heart failure. On routine chest radiography one week later, the stent was found to have embolized into the right ventricle. The stent was retrieved and repositioned by means of transcatheter technique, without subsequent complications. We found this method to be a viable alternative to surgery in a high-risk infant. To our knowledge, this is the first report of the successful transcatheter retrieval and repositioning of an expandable intravascular stent from an …

Management Of Intra-Aortic Balloon Pump Entrapment: A Case Report And Review Of The Literature, Gerard J Fitzmaurice, Anton Collins, Haralambos Parissis

The Texas Heart Institute Journal

An intra-aortic balloon pump is one of the most valuable tools in the cardiac surgeon's armament to assist in the management of the failing heart. Despite its widespread use, there are associated risks and complications, one of which is balloon rupture with associated entrapment. Numerous approaches for dealing with this complication have been described; here we review the previous experience with intra-aortic balloon pump entrapment and discuss potential management, with particular reference to a recent case of our own.

Chronic Kidney Disease Is Associated With Increased Coronary Artery Atherosclerosis As Revealed By Multidetector Computed Tomographic Angiography, Sion K Roy, Albert Cespedes, Dong Li, Tae-Young Choi, Matthew J Budoff

The Texas Heart Institute Journal

Chronic kidney disease (CKD) is an independent risk factor for cardiovascular events. We evaluated whether multidetector computed tomographic angiography (MDCTA) revealed a link between pre-dialysis CKD and coronary artery atherosclerosis.

We retrospectively analyzed 549 patients who underwent MDCTA. Patients were divided into 3 groups: normal glomerular filtration rate (GFR) (GFR >90 mL/min/1.73 m2 body surface area), mild CKD (>60GFR≤90 mL/min/1.73 m2), and moderate CKD (>30GFR≤60 mL/min/1.73 m2). Normality testing was performed to determine if continuous variables were modeled in Gaussian distribution before analysis of variance was applied. The χ2 test was used to compare GFR subgroups. Multiple linear …

Triterpenoid Modulation Of Il-17 And Nrf-2 Expression Ameliorates Neuroinflammation And Promotes Remyelination In Autoimmune Encephalomyelitis, Tej K. Pareek, Abdelmadjid Belkadi, Sashi Kesavapany, Anita Zaremba, Sook L. Loh, Lianhua Bai, Mark L. Cohen, Colin Meyer, Karen T. Liby, Robert H. Miller, Michael B. Sporn, John J. Letterio

Dartmouth Scholarship

Inflammatory cytokines and endogenous anti-oxidants are variables affecting disease progression in multiple sclerosis (MS). Here we demonstrate the dual capacity of triterpenoids to simultaneously repress production of IL-17 and other pro-inflammatory mediators while exerting neuroprotective effects directly through Nrf2-dependent induction of anti-oxidant genes. Derivatives of the natural triterpene oleanolic acid, namely CDDO-trifluoroethyl-amide (CDDO-TFEA), completely suppressed disease in a murine model of MS, experimental autoimmune encephalomyelitis (EAE), by inhibiting Th1 and Th17 mRNA and cytokine production. Encephalitogenic T cells recovered from treated mice were hypo-responsive to myelin antigen and failed to adoptively transfer the disease. Microarray analyses showed significant suppression of …

Factors Affecting Growth In Infants With Single Ventricle Physiology: A Report From The Pediatric Heart Network Infant Single Ventricle Trial., Richard V. Williams, Victor Zak, Chitra Ravishankar, Karen Altmann, Jeffrey Anderson, Andrew M. Atz, Carolyn Dunbar-Masterson, Nancy Ghanayem, Linda Lambert, Karen Lurito, Barbara Medoff-Cooper, Renee Margossian, Victoria L. Pemberton, Jennifer Russell, Mario Stylianou, Daphne Hsu, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To describe growth patterns in infants with single ventricle physiology and determine factors influencing growth.

STUDY DESIGN: Data from 230 subjects enrolled in the Pediatric Heart Network Infant Single Ventricle Enalapril Trial were used to assess factors influencing change in weight-for-age z-score (z) from study enrollment (0.7 ± 0.4 months) to pre-superior cavopulmonary connection (SCPC; 5.1 ± 1.8 months, period 1) and pre-SCPC to final study visit (14.1 ± 0.9 months, period 2). Predictor variables included patient characteristics, feeding regimen, clinical center, and medical factors during neonatal (period 1) and SCPC hospitalizations (period 2). Univariate regression analysis was performed, …

Percutaneous Implantation Of The Edwards Sapien Transcatheter Heart Valve For Conduit Failure In The Pulmonary Position: Early Phase 1 Results From An International Multicenter Clinical Trial., Damien Kenny, Ziyad M. Hijazi, Saibal Kar, John Rhodes, Michael Mullen, Raj Makkar, Girish S. Shirali, Mark Fogel, John Fahey, Mary G. Heitschmidt, Christopher Cain

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: The purpose of this study was to evaluate the safety and effectiveness of the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position in patients with moderate to severe pulmonary regurgitation with or without stenosis.

BACKGROUND: Transcatheter pulmonary valve replacement is evolving, but to date, experience has been limited to the Melody valve (Medtronic Inc., Minneapolis, Minnesota).

METHODS: Eligible patients with dysfunctional right ventricle-to-pulmonary artery conduits were screened if body weight was ≥35 kg and the in situ conduit diameter was ≥16 mm and ≤24 mm. Standardized implantation and follow-up protocols were used.

RESULTS: …

Non-Geometric Echocardiographic Indices Of Ventricular Function In Patients With A Fontan Circulation., Jonathan Rhodes, Renee Margossian, Lynn A. Sleeper, Piers Barker, Timothy J. Bradley, Minmin Lu, Mark Fogel, Matthew A. Harris, Wyman W. Lai, Andrew J. Powell, Michael D. Puchalski, Girish S. Shirali, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Complex anatomy and limited windows complicate echocardiographic assessments of ventricular function in Fontan patients. For the Pediatric Heart Network Fontan Cross-Sectional Study, data were acquired from which mean ventricular pressure change during isovolumetric contraction (dP/dt(ic)), Tei index, and maximal systolic annular velocity (S') could be measured. The purpose of this study was to compare these nongeometric indices of ventricular function to cardiac magnetic resonance (CMR) measurements of ventricular ejection fraction (EF).

METHODS: Echocardiographic and CMR studies were performed prospectively using standardized protocols; measurements were completed by core laboratories. Data from both modalities were available from 137 patients.

RESULTS: A …

Characterization Of Street Food Consumption In Palermo: Possible Effects On Health, Silvio Buscemi, Annamaria Barile, Vincenza Maniaci, John A. Batsis

Dartmouth Scholarship

Background: Street Food (SF) consists of out-of-home food consumption and has old, historical roots with complex social-economic and cultural implications. Despite the emergence of modern fast food, traditional SF persists worldwide, but the relationship of SF consumption with overall health, well-being, and obesity is unknown.

Methods: This is an observational, cross-sectional study. The study was performed in Palermo, the largest town of Sicily, Italy. Two groups were identified: consumers of SF (n = 687) and conventional restaurant food (RES) consumers (n = 315). Study subjects answered a questionnaire concerning their health conditions, nutritional preferences, frequency of consumption of SF and …

Functional Plasticity Of Central Trpv1 Receptors In Brainstem Dorsal Vagal Complex Circuits Of Streptozotocin-Treated Hyperglycemic Mice, Andrea Zsombok, Muthu D. Bhaskaran, Hong Gao, Andrei V. Derbenev, Bret N. Smith

Physiology Faculty Publications

Emerging data indicate that central neurons participate in diabetic processes by modulating autonomic output from neurons in the dorsal motor nucleus of the vagus (DMV). We tested the hypothesis that synaptic modulation by transient receptor potential vanilloid type 1 (TRPV1) receptors is reduced in the DMV in slices from a murine model of type 1 diabetes. The TRPV1 agonist capsaicin robustly enhanced glutamate release onto DMV neurons by acting at preterminal receptors in slices from intact mice, but failed to do so in slices from diabetic mice. TRPV1 receptor protein expression in the vagal complex was unaltered. Brief insulin preapplication …

Decision Quality Instrument For Treatment Of Hip And Knee Osteoarthritis: A Psychometric Evaluation, Karen R. Sepucha, Dawn Stacey, Catharine F. Clay, Yuchiao Chang

Dartmouth Scholarship

A high quality decision requires that patients who meet clinical criteria for surgery are informed about the options (including non-surgical alternatives) and receive treatments that match their goals. The aim of this study was to evaluate the psychometric properties and clinical sensibility of a patient self report instrument, to measure the quality of decisions about total joint replacement for knee or hip osteoarthritis.

Factors Associated With Serum Brain Natriuretic Peptide Levels After The Fontan Procedure., Andrew M. Atz, Victor Zak, Roger E. Breitbart, Steven D. Colan, Sara K. Pasquali, Daphne T. Hsu, Minmin Lu, Lynn Mahony, Stephen M. Paridon, Michael D. Puchalski, Tal Geva, Brian W. Mccrindle, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Although a useful marker of heart failure in adults, the utility of brain natriuretic peptide concentration (BNP) for children after the Fontan procedure is not well studied.

DESIGN: BNP was measured in 510 patients who were 6-18 years old in the Pediatric Heart Network Fontan cross-sectional study at a median of 8.2 years after Fontan. Patients underwent echocardiography, exercise testing, magnetic resonance imaging (MRI) and functional health status questionnaires. Associations of BNP with baseline patient characteristics, medical history and cross-sectional assessment were examined with multivariable linear regression analyses.

RESULTS: The distribution of BNP was highly skewed, median 13.0 pg/mL …

Emotional Climate, Feeding Practices, And Feeding Styles: An Observational Analysis Of The Dinner Meal In Head Start Families, Sheryl O Hughes, Thomas G Power, Maria A Papaioannou, Matthew B Cross, Theresa A Nicklas, Sharon K Hall, Richard M Shewchuk

Faculty, Staff and Students Publications

BACKGROUND: A number of studies conducted with ethnically diverse, low-income samples have found that parents with indulgent feeding styles had children with a higher weight status. Indulgent parents are those who are responsive to their child's emotional states but have problems setting appropriate boundaries with their child. Because the processes through which styles impact child weight are poorly understood, the aim of this study was to observe differences in the emotional climate created by parents (including affect, tone of voice, and gestures) and behavioral feeding practices among those reporting different feeding styles on the Caregiver's Feeding Styles Questionnaire. A secondary …

Surgical Management Of Complete Atrioventricular Septal Defect: Associations With Surgical Technique, Age, And Trisomy 21., Andrew M. Atz, John A. Hawkins, Minmin Lu, Meryl S. Cohen, Steven D. Colan, James Jaggers, Ronald V. Lacro, Brian W. Mccrindle, Renee Margossian, Ralph S. Mosca, Lynn A. Sleeper, L Luann Minich, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes.

METHODS: The demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006.

RESULTS: The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect …

Renin-Angiotensin-Aldosterone Genotype Influences Ventricular Remodeling In Infants With Single Ventricle., Seema Mital, Wendy K. Chung, Steven D. Colan, Lynn A. Sleeper, Cedric Manlhiot, Cammon B. Arrington, James F. Cnota, Eric M. Graham, Michael E. Mitchell, Elizabeth Goldmuntz, Jennifer S. Li, Jami C. Levine, Teresa M. Lee, Renee Margossian, Daphne T. Hsu, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

Background: We investigated the effect of polymorphisms in the renin-angiotensin-aldosterone system (RAAS) genes on ventricular remodeling, growth, renal function, and response to enalapril in infants with single ventricle.

Methods and results: Single ventricle infants enrolled in a randomized trial of enalapril were genotyped for polymorphisms in 5 genes: angiotensinogen, angiotensin-converting enzyme, angiotensin II type 1 receptor, aldosterone synthase, and chymase. Alleles associated with renin-angiotensin-aldosterone system upregulation were classified as risk alleles. Ventricular mass, volume, somatic growth, renal function using estimated glomerular filtration rate, and response to enalapril were compared between patients with ≥2 homozygous risk genotypes (high risk), and those …