A5110298882- Congenital Heart Disease Studies
- Connective tissue disorders research
- Aortic Disease and Treatment Approaches
- Cardiac Valve Diseases and Treatments
- Cardiovascular Issues in Pregnancy
- Cardiovascular Function and Risk Factors
- Cardiac Structural Anomalies and Repair
- Hemoglobinopathies and Related Disorders
- Coronary Artery Anomalies
- Mechanical Circulatory Support Devices
- Ultrasound in Clinical Applications
- Vascular anomalies and interventions
- Aortic aneurysm repair treatments
- Cardiac Arrhythmias and Treatments
- Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Congenital heart defects research
- Viral Infections and Immunology Research
- Infective Endocarditis Diagnosis and Management
- Cardiovascular Conditions and Treatments
- Cardiovascular and Diving-Related Complications
- Cardiac Imaging and Diagnostics
- Diabetes, Cardiovascular Risks, and Lipoproteins
- Pulmonary Hypertension Research and Treatments
- HIV-related health complications and treatments
- Cardiac tumors and thrombi
Seattle Children's Hospital
2018-2024
University of Washington
2018-2024
University of Washington Medical Center
2022
Washington Center
2022
Lurie Children's Hospital
2007-2020
Northwestern University
2005-2020
Primary Children's Hospital
2019
University of Utah
2001-2019
Children's Hospital of Michigan
2013
University of Miami
2013
Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, current standard therapy most centers.
Thoracic aortic aneurysm and dissection (TAAD) is typically inherited in an autosomal dominant manner, but rare X-linked families have been described. So far, the only known gene FLNA, which associated with periventricular nodular heterotopia type of Ehlers-Danlos syndrome. However, mutations this explain a small number TAAD families.We performed targeted resequencing 368 candidate genes cohort 11 molecularly unexplained Marfan probands. Subsequently, Sanger sequencing BGN 360 male 155...
Blood flow dynamics make it possible to better understand the development of aortopathy and cardiovascular events in patients with Marfan syndrome (MFS). Aortic 3D blood characteristics were investigated relation aortic geometry children adolescents MFS. Twenty-five MFS (age 15.6 ± 4.0 years; 11 females) 21 healthy controls 16.0 2.6 12 underwent magnetic resonance angiography 4D CMR for assessment thoracic size velocities. Data analysis included calculation diameter BSA-indexed dimensions...
Perinatally HIV-infected adolescents may be susceptible to aggregate atherosclerotic cardiovascular disease risk, as measured by the Pathobiological Determinants of Atherosclerosis in Youth (PDAY) coronary arteries and abdominal aorta risk scores, a result prolonged exposure HIV antiretroviral therapy.Coronary PDAY scores were calculated for 165 perinatally adolescents, using weighted combination modifiable factors: dyslipidemia, cigarette smoking, hypertension, obesity, hyperglycemia....
Background: Bicuspid aortic valve (BAV) is the most prevalent congenital heart defect affecting 1% to 2% of population. It associated with ascending aorta dilatation. Valve morphology, stenosis (AS), and insufficiency (AI) have been proposed as potential risk factors; however, evaluating their role difficult, these factors are inherently related. The aim this study was determine whether BAV morphology dysfunction independent determinants for dilatation in pediatric patients. Methods: A...
The so-called Gerbode ventriculo-atrial defect is a rare that permits shunting from the left ventricle to right atrium. It takes 2 forms, either deficiency of atrioventricular membranous septum, or initially through ventricular septal defect, with atrial occurring in leaflet tricuspid valve. In this review, we describe natural history and outcomes surgical repair for variant at site septum.From 1990 2008, identified 6 patients our departmental database who had undergone closure congenital...
Although cardiopulmonary disease is associated with decreased functional capacity among adults sickle cell (SCD), its impact on in children SCD unknown. We evaluated 6-min walk (6MW) distance 77 and young undergoing screening for disease. Of 30 subjects who also underwent exercise testing, we found evidence a significant proportion. Exercise was related to baseline degree of anemia significantly lower history recurrent acute chest syndrome. that 6MW adjusted weight body surface area shorter...
Disturbances of the normal asymmetric placement organs, such as polysplenia or situs inversus, have been defined traditionally laterality defects. However, there is compelling evidence from vertebrate models and human birth defects to hypothesize that midline, isolated congenital heart defects, are etiologically related. We present clinical characteristics three families exhibit a variety midline in addition These observations suggest phenotypic consequences mutations causing include well To...
Abstract Background Prolongation of the QTc interval may be more common than previously believed among individuals with sickle cell disease (SCD). The clinical associations and natural history prolongation remain unclear in this population. Our objectives were to determine prevalence prolonged evaluate its relationship factors children young adults SCD. Procedures We analyzed data from subjects 10 25 years old SCD enrolled our pulmonary hypertension screening protocol. Screening included...
Concerns about the morbidity and mortality associated with tricuspid regurgitant jet velocity (TRJV) elevation, which may indicate pulmonary hypertension (PHT), in adults sickle cell disease (SCD) have prompted growing interest screening pediatric population. The goals of our study were to estimate prevalence TRJV elevation determine its relationship function children young SCD at baseline. Seventy-eight subjects (10-24 years old) underwent prospective by Doppler echocardiogram (ECHO),...
Kawasaki disease (KD) shock syndrome (KDSS) is hypotension with KD. We compared children KDSS and matched control Children more often were female, had a lower platelet count sodium concentration, condition refractory to immunoglobulin, abnormal echocardiography results. unique subset of
Abstract The reproducibility of tricuspid regurgitant jet velocity (TRJV) measurements by Doppler echocardiography has not been subjected to systematic evaluation among individuals with sickle cell disease (SCD) undergoing screening for pulmonary hypertension. We examined sources disagreement associated peak TRJV in children and young adults SCD. Peak was independently measured interpreted a week apart separate sonographers readers, respectively, 30 subjects (mean age, 15.8 ± 3.3 years) who...