Kevin G Friedman

Kevin G Friedman
  • MD
  • Pediatric Cardiology at Boston Children's Hospital

About

159
Publications
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7,036
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Introduction
Skills and Expertise
Current institution
Boston Children's Hospital
Current position
  • Pediatric Cardiology
Additional affiliations
June 2008 - present
Boston Children's Hospital
Position
  • Pediatric Cardiology

Publications

Publications (159)
Article
Importance Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited. Objective To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms ( z score ≥2.5), and noncardiac i...
Article
Kawasaki disease (KD), an acute self-limited febrile illness that primarily affects children <5 years old, is the leading cause of acquired heart disease in developed countries, with the potential of leading to coronary artery dilation and coronary artery aneurysms in 25% of untreated patients. This update summarizes relevant clinical data publishe...
Article
Introduction: Fetal “brain sparing” autoregulatory mechanisms respond to reduced cardiac output or substrate delivery by decreasing cerebrovascular resistance to maintain cerebral blood flow. In patients with congenital heart disease (CHD), data are conflicting on the association between fetal cerebral Doppler measures and postnatal neurodevelopmen...
Article
Full-text available
Objective To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease. Methods An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Pat...
Article
OBJECTIVES Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes. METHODS We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012 to 2021. The primary outcome was a comp...
Article
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Background FAV is offered to fetuses with severe aortic valve stenosis and evolving hypoplastic left heart syndrome. An inferential analysis of TS and SAE in a large series has not been reported. Objectives The purpose of this study was to determine factors associated with fetal aortic valvuloplasty (FAV) technical success (TS) and serious adverse...
Article
OBJECTIVES Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population. METHODS Single center, retrospective analysis of patients who underwent surgical repair with diagnosis of ToF-CAVC from 1979 to 2022, divided in two different periods an...
Article
Coarctation of the aorta (CoA) is a ductus arteriosus (DA)-dependent form of congenital heart disease (CHD) characterized by narrowing in the region of the aortic isthmus. CoA is a challenging diagnosis to make prenatally and is the critical cardiac lesion most likely to go undetected on the pulse oximetry-based newborn critical CHD screen. When un...
Article
Background: Congenital atrioventricular block (AVB) is a rare disease with poor outcomes. Heart rate (HR) is a major prognostic factor. To date, no study has evaluated the contribution of cardiac output (CO), stroke volume (SV), and HR on outcomes. Methods: In this retrospective study we evaluated associations between fetal echocardiographic parame...
Article
Introduction: Cardiovascular (CV) involvement in multisystem inflammatory syndrome in children (MISC) is frequent (80-85%). Data on 2D speckle tracking echocardiography and its value in predicting CV outcomes are limited. Hypothesis: We hypothesized that an association exists between 2D speckle tracking strain/strain rate parameters at admission an...
Article
Objective: Kawasaki disease (KD) is a systemic vasculitis of young children that can lead to development of coronary artery aneurysms. We aimed to identify diagnostic markers to distinguish KD from other pediatric inflammatory diseases. Methods: We used the proximity extension assay (PEA) to profile proinflammatory mediators in plasma samples fr...
Article
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Objective The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experienc...
Article
Objective: Acute outcomes after atrioventricular canal defects (AVCD) repair in the current era are excellent, yet despite surgical advances, ∼15% of patients require future left atrioventricular valve (LAVV) repair. Among AVC patients who undergo LAVV repair after primary AVC repair, we sought to characterize the durability of these repairs. Spec...
Article
As new variants of SARS-Co-V 2 have emerged over time and Omicron sub-variants have become dominant, the severity of illness from COVID-19 has declined despite greater transmissibility. There are fewer data on how the history, diagnosis, and clinical characteristics of multisystem inflammatory syndrome in children (MIS-C) have changed with evolutio...
Article
Full-text available
To determine clinical differences for children with complete Kawasaki disease (KD) with and without evidence of preceding SARS-CoV-2 infection. From January 2020, contemporaneous patients with complete KD criteria were classified as either SARS-CoV-2 positive (KDCOVID+; confirmed household exposure, positive PCR and/or serology) or SARS-CoV-2 negat...
Article
Introduction: SARS CoV2 has been associated with acute and subacute myocarditis. Screening after COVID-19 has been recommended prior to returning to physical activity. The yield of such investigations, particularly in youth, remains unknown. Methods: Retrospective chart review was performed of patients evaluated in a cardiology clinic for return to...
Article
Introduction: Obesity is a pro-inflammatory state and may influence presentation and outcomes of both Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19. Methods: From January 2020 to January 2022, n=2566 contemporaneous KD, MIS-C and acute COVID-19 pediatric patients from 39 sites in 8 countrie...
Article
Full-text available
Background Cardiac complications related to COVID‐19 in children and adolescents include ventricular dysfunction, myocarditis, coronary artery aneurysm, and bradyarrhythmias, but tachyarrhythmias are less understood. The goal of this study was to evaluate the frequency, characteristics, and outcomes of children and adolescents experiencing tachyarr...
Article
Objective Aortic valve bicuspidization has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported. Methods We performed a retrospective review was performed of patients who...
Article
Key points What is known? Fetal aortic valvuloplasty (FAV) may avert the progression of mid‐gestation aortic stenosis (AS) to Hypoplastic left heart syndrome (HLHS) in a subset of fetus. What this study adds? Novel assessment of flow through the mitral valve (MV) to estimate left heart output in fetus with AS after a FAV.
Article
Background: There are minimal data characterizing the trajectory of left heart growth and hemodynamics following fetal aortic valvuloplasty (FAV). Methods: This retrospective study included patients who underwent FAV between 2000 and 2019 with echocardiograms performed pre-FAV, immediately post-FAV, and in late gestation. Results: Of 118 fetus...
Article
Background The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki Disease (KD) and coronary artery aneurysms (CAAs) is unknown. Methods Using data from the International KD Registry in patients with ≥ medium CAA we evaluate associations of treatment with outcomes and major adverse cardiac events (MACE). Result...
Article
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Objective To identify risk factors associated with high-frequency ventilation (HFV) following definitive closure of the patent ductus arteriosus (PDA). Methods We performed a retrospective study of premature infants (<37 weeks) who were mechanically ventilated before and after surgical or transcatheter PDA closure. Primary outcome was HFV requirem...
Article
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Myocarditis is a rare complication of the COVID-19 mRNA vaccine. We previously reported a case series of 15 adolescents with vaccine-associated myocarditis, 87% of whom had abnormalities on initial cardiac magnetic resonance (CMR), including late gadolinium enhancement (LGE) in 80%. We performed follow-up CMRs to determine the trajectory of myocard...
Article
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Objective To provide evidence‐based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists. Methods Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comp...
Article
Full-text available
Objective To provide evidence‐based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists. Methods Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comp...
Article
Full-text available
Ultrasound 'Echocardiogram (echo)' images generated by commercial scanners are non-invasive, economical, and have therefore become an important clinical and research tool for cardiovascular disease. The Boston Children's Hospital (BCH, USA) cardiology department has developed echo image handling processes and software tools to simplify and manage t...
Article
OBJECTIVES Among patients with hypoplastic left heart syndrome (HLHS), tricuspid valve regurgitation (TR) portends a poor prognosis. Our goal was to describe the outcomes of tricuspid valve reconstruction (TVR) concomitant with the Norwood operation and using two-dimensional echocardiography and evaluate the structural factors associated with succe...
Article
Background: Neurodevelopmental impairment is common in children with congenital heart disease (CHD), yet postnatal variables explain only 30% of the variance in outcomes. To explore whether the antecedents for neurodevelopmental disabilities might begin in utero , we analyzed whether fetal brain volume predicted subsequent neurodevelopmental outcom...
Article
Full-text available
Objective To provide guidance on the management of Multisystem Inflammatory Syndrome in Children (MIS‐C), a condition characterized by fever, inflammation, and multiorgan dysfunction that manifests late in the course of SARS–CoV‐2 infection. Recommendations are also provided for children with hyperinflammation during COVID‐19, the acute, infectious...
Article
Evaluate outcomes of single leaflet aortic valve reconstruction using Ozaki sizer and template. Single institute retrospective analysis between August 2015 and August 2019. Thirty-three patients, median age 9.3 years and weight 29.2 kg underwent single leaflet Ozaki repair. Preoperative indications were: AR (n=17), AS (n=3) or AS/AR (n=13). Baselin...
Article
Full-text available
Background Health care transition (HCT) is a period of high vulnerability for patients with chronic childhood diseases, particularly when patients shift from a pediatric to an adult care setting. An increasing number of patients with Kawasaki disease (KD) who develop medium and large coronary artery aneurysms (classified by the American Heart Assoc...
Article
Objective To characterize viscoelastic testing profiles of children with multisystem inflammatory syndrome in children (MIS-C). Methods This single-center retrospective review included 30 patients diagnosed with MIS-C from March 1 to September 1, 2020. Thromboelastography (TEG) with platelet mapping was performed in 19 (63%) patients and compared...
Article
Full-text available
Background: American Heart Association (AHA) guidelines for management of Kawasaki disease (KD) rely on coronary artery (CA) z-scores from echocardiograms. Compared with echocardiography, cardiac CT (CCT) offers better visualization of distal segments and evaluation for thrombosis and stenosis. Despite increasing use of CCT in KD, CA z-scores for...
Chapter
Kawasaki disease (KD) is the most common form of acquired pediatric heart disease in the developed world and has a worldwide distribution, affecting children of all races. Three linked processes are responsible for KD vasculopathy: necrotizing arteritis, subacute chronic vasculitis, and luminal myofibroblastic proliferation. KD presents acutely as...
Article
Severe forms of pulmonary embolism (PE) in children are rare but cause significant morbidity and mortality. In this article, we review the pathophysiology of severe (high- and intermediate-risk) PE and suggest novel pediatric-specific risk stratifications and an acute treatment algorithm to expedite emergent decision making. We define pediatric hig...
Article
Full-text available
Background Coronary artery (CA) aneurysms in children are a rare but potentially life-threatening finding and are highly associated with Kawasaki disease (KD). Case presentation We describe a four-year-old female with a vasculitis and CA aneurysms. She had a prolonged course with recurrent fever and systemic inflammation several times upon discont...
Article
Full-text available
Importance The BNT162b2 (Pfizer-BioNTech) messenger RNA COVID-19 vaccine was authorized on May 10, 2021, for emergency use in children aged 12 years and older. Initial reports showed that the vaccine was well tolerated without serious adverse events; however, cases of myocarditis have been reported since approval. Objective To review results of co...
Article
Background The Long-terM OUtcomes after the Multisystem Inflammatory Syndrome In Children (MUSIC) study aims to characterize the frequency and time course of acute and long-term cardiac and non-cardiac sequelae in MIS-C, which are currently poorly understood. Methods This multicenter observational cohort study will enroll at least 600 patients <21...
Article
Objective To evaluate practice variation in pharmacological management in the International Kawasaki Disease Registry (IKDR). Study design Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy, and anticoagulation was described. Results We included 1,627 patients fr...
Article
Full-text available
Background The assessment of real-world effectiveness of immunomodulatory medications for multisystem inflammatory syndrome in children (MIS-C) may guide therapy. Methods We analyzed surveillance data on inpatients younger than 21 years of age who had MIS-C and were admitted to 1 of 58 U.S. hospitals between March 15 and October 31, 2020. The effe...
Article
Management of recurrent symptomatic tracheobronchomalacia and tracheobronchial compression after prior aortopexy and tracheobronchopexy is a surgical challenge. In patients with right aortic arch, the course of the aortic arch over the right mainstem bronchus can result in superior and posterior compression of the airway. We present two cases of re...
Preprint
Objective: To characterize viscoelastic testing profiles of children with multisystem inflammatory syndrome in children (MIS-C). Methods: This single-center retrospective review included 30 patients diagnosed with MIS-C from January 1 to September 1, 2020. Thromboelastography (TEG) with platelet mapping was performed in 19 (63%) patients and compar...
Article
Full-text available
Background Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA). Case presentation We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospec...
Article
Full-text available
In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) for long-term management are defined by both maximal and current coronary artery (CA) dimensions normalized as z-scores. We sought to determine the degree to which current recommended practice differs from past actual practice, highlighting areas for kn...
Article
Importance Refinement of criteria for multisystem inflammatory syndrome in children (MIS-C) may inform efforts to improve health outcomes. Objective To compare clinical characteristics and outcomes of children and adolescents with MIS-C vs those with severe coronavirus disease 2019 (COVID-19). Setting, Design, and Participants Case series of 1116...
Article
Full-text available
Objectives: To characterize the socioeconomic and racial and/or ethnic disparities impacting the diagnosis and outcomes of multisystem inflammatory syndrome in children (MIS-C). Methods: This multicenter retrospective case-control study was conducted at 3 academic centers from January 1 to September 1, 2020. Children with MIS-C were compared wit...
Article
Full-text available
Objective To provide guidance on the management of Multisystem Inflammatory Syndrome in Children (MIS‐C), a condition characterized by fever, inflammation, and multiorgan dysfunction that manifests late in the course of severe acute respiratory syndrome coronavirus 2 (SARS–CoV‐2) infection. Recommendations are also provided for children with hyperi...
Article
Full-text available
Initial reports on COVID-19 described children as largely spared from severe manifestations, with only 2–6% of children requiring intensive care treatment. However, since mid-April 2020, clusters of pediatric cases of severe systemic hyperinflammation and shock epidemiologically linked with COVID-19 have been reported. This condition was named as S...
Article
Full-text available
Purpose of review Prenatal diagnosis of congenital heart disease (CHD) is continuously evolving with each passing decade. Early efforts in fetal cardiology focused on identifying CHD in mid-gestation and understanding of fetal circulation in pathologic conditions. Improving prenatal detection rates for CHD remains an ongoing challenge and increasin...
Article
We thank Dr. Kotnik and colleagues for their commentary on the American College of Rheumatology’s clinical guidance for pediatric patients with multisystem inflammatory syndrome in children (MIS‐C). The use of anticoagulation in this population remains an intensely debated topic with little clinical evidence to guide treatment decisions. For this r...
Article
Full-text available
Background Cardiac manifestations in multisystem inflammatory syndrome in children (MIS-C) occur in ∼80% of patients. Left ventricular (LV) systolic dysfunction is the most frequent cardiac finding. Methods In this single-center, retrospective cohort study we report on detailed assessment of LV function in MIS-C patients using strain and strain ra...
Article
Objectives: Fetal aortic valvuloplasty (FAV) for severe aortic stenosis (AS) has shown promise in averting progression to hypoplastic left heart syndrome. After FAV, predicting which fetuses will achieve a biventricular (BiV) circulation after birth remains challenging. Identifying predictors of postnatal circulation on late gestation echocardiogr...
Article
Full-text available
Background The unpredictable trajectory of pediatric advanced heart disease makes prognostication difficult for physicians and informed decision‐making challenging for families. This study evaluated parent and physician understanding of disease burden and prognosis in hospitalized children with advanced heart disease. Methods and Results A longitu...
Article
Objectives To determine predictors of more than one emergency department (ED) visit for a Kawasaki disease diagnosis in a quaternary care pediatric hospital and compare outcomes between patients with 1 versus > 1 visit for KD diagnosis. Study design Medical records of patients evaluated for KD between January 2006 and August 2018 at Boston Childre...
Article
Introduction: The American Heart Association (AHA) guidelines recommend testing for inducible myocardial ischemia in all patients with a history of coronary artery aneurysm (CAA) after Kawasaki disease (KD). Because the prevalence of clinical coronary complications is exceedingly low in patients whose worst-ever CAA dimension was <large/giant, we e...
Article
Coronavirus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with significant cardiovascular implications. Although infection with SARS-CoV-2 is usually mild in children, some children later develop a severe inflammatory disease that can have manifestation...
Article
Introduction Guidelines recommend trans‐esophageal echocardiography (TEE) for patients with atrial fibrillation (AF) or atrial flutter (AFL) for >48 h, due to risk of intracardiac thrombus formation. With growing evidence that AFL in adults with structurally normal hearts has less thrombogenic potential compared to AF, and the need for TEE question...
Article
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Objective Congenital heart disease (CHD) is associated with abnormal brain development in utero. We applied innovative fetal magnetic resonance imaging (MRI) techniques to determine whether reduced fetal cerebral substrate delivery impacts the brain globally, or in a region‐specific pattern. Our novel design included two control groups, one with an...
Article
Full-text available
Objective To provide guidance on the management of multisystem inflammatory syndrome in children (MIS‐C), a condition characterized by fever, inflammation, and multiorgan dysfunction that manifests late in the course of severe acute respiratory syndrome coronavirus 2 (SARS–CoV‐2) infection, and to provide recommendations for children with hyperinfl...
Article
Objective: Patients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy beyond intravenous immunoglobulin (IVIG). We evaluate the effect of primary adjunctive corticosteroid therapy on outco...
Article
Background: The risks of ascending aortic dilation and the indications for intervention in pediatric patients is unclear. Given the concern for aortic size mismatch with growth, larger ascending aortic diameters have been accepted in pediatric patients. The purpose of this study was to evaluate the effectiveness of ascending aortic reduction at th...
Preprint
Full-text available
Background. Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA). Case presentation. We describe our experience using cyclophosphamide (CYC) for the treatment of such cases as well as a review of the literature on the use of CYC in KD. Through a retrospe...
Article
Background: Children are at risk for multisystem inflammatory syndrome in children (MIS-C) after infection with severe acute respiratory syndrome coronavirus 2. Cardiovascular complications, including ventricular dysfunction and coronary dilation, are frequent, but there are limited data on arrhythmic complications. Methods: Retrospective cohort...
Article
Treatment of Kawasaki disease (KD) with intravenous immunoglobulin (IVIG) administered within the initial 10 days of fever onset decreases the risk of coronary artery aneurysms (CAAs) from ∼ 25% to less than 5%. However, patients with IVIG resistance, young infants, men, highly inflamed patients, and/or those with coronary changes at diagnosis rema...
Article
Full-text available
Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in pati...
Article
Background: Pediatric SARS-CoV-2 infection can be complicated by a dangerous hyperinflammatory condition termed multisystem inflammatory syndrome in children (MIS-C). The clinical and immunologic spectrum of MIS-C and its relationship to other inflammatory conditions of childhood have not been studied in detail. Methods: We retrospectively studi...
Article
Full-text available
Background and purpose: Brain MRI of newborns with congenital heart disease show signs of immaturity relative to healthy controls. Our aim was to determine whether the semiquantitative fetal total maturation score can detect abnormalities in brain maturation in fetuses with congenital heart disease in the second and third trimesters. Materials an...
Article
Background The substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-weight heparin [LMWH] or warfarin) for thromboprophylaxis in large CAAs. Methods Data from 383 p...
Article
The use of the left ventricle as the sub-pulmonary ventricle in order to achieve a 1.5 or biventricular circulation is feasible in heterotaxy patients with complex intra-cardiac anatomy and acceptable right ventricular function. It is an alternative in patients who are not ideal candidates for single ventricle palliation. We highlight two cases whe...
Article
The aortic valve neocuspidization (AVNeo) or Ozaki procedure has been shown to be technically reproducible and have acceptable results in the aortic position in adult and pediatric patients. We report a case in which innovative techniques were utilized to create a three-leaflet pulmonary valve using a modified AVNeo technique.
Article
Background Tracheobronchomalacia and airway obstruction from severely dilated pulmonary arteries in Tetralogy of Fallot with absent pulmonary valve (TOF-APV) has been associated with high rates of respiratory failure and mortality (15-25%). It is not known whether aggressive pulmonary artery and/or direct airway intervention during early definitive...
Article
Background: Fetal aortic valvuloplasty (FAV) may prevent progression of midgestation aortic stenosis to hypoplastic left heart syndrome. However, FAV has well-established risks, and its survival benefit remains unknown. Our primary aim was to determine whether FAV for midgestation aortic stenosis increases survival from fetal diagnosis to age 6 ye...
Article
Apixaban is well studied in adults; paediatric data are extremely limited. We describe 3 children (age 2-6 years, weight 13-17 kg) with congenital heart disease who developed intracardiac thrombosis, and in whom typical treatments had failed (in 1) or were considered impractical (in 2). All were treated with apixaban 2.5 mg orally twice daily with...
Article
Objectives: To describe a single institutional experience managing fetuses with SVT and to identify associations between patient characteristics and fetal and postnatal outcomes. Background: Sustained fetal supraventricular tachycardia (SVT) is associated with significant morbidity and mortality if untreated, yet the optimal management strategy...
Article
Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the rationale, selection criteria, technical features, and current outcomes for the three most commonly performed FCI: fetal aortic stenosis with evolving hypoplastic left heart syndrom...
Article
Full-text available
Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare congenital anomaly with the most common pattern being an anomalous left coronary artery (LCA) from the pulmonary artery (ALCAPA). A very rare pattern is the anomalous origin of a single coronary artery from the pulmonary artery (ASCAPA) wherein the single coronary ostium...
Article
Fetal cardiac intervention was first proposed in the early 1990s to impact cardiac development and survival of fetuses with fetal aortic stenosis and evolving hypoplastic left heart syndrome (HLHS). Although initial attempts of fetal aortic valvuloplasty were unsuccessful and carried a high rate of morbidity and mortality, our collaborative group a...
Article
Full-text available
Purpose of review We review the acute management of patients with Kawasaki disease (KD), including adjunctive primary therapies and the treatment of patients with resistance to intravenous immunoglobulin (IVIG). Recent findings IVIG administered within 10 days of fever in patients with (KD decreases the risk of coronary artery complication from ~...
Article
Full-text available
Background Accurate prediction of coronary artery aneurysms ( CAAs ) in patients with Kawasaki disease remains challenging in North American cohorts. We sought to develop and validate a risk model for CAA prediction. Methods and Results A binary outcome of CAA was defined as left anterior descending or right coronary artery Z score ≥2.5 at 2 to 8...
Article
Objective: To evaluate the association of neighborhood socioeconomic status (SES) with time to intravenous immunoglobulin treatment, length of stay (LOS), and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. Study design: We examined the relationship of SES in 915 patients treated at a large academic center between 2000 and 20...
Article
Background: Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease. Treatment with intravenous immunoglobulin (IVIg) within 10 days of fever onset reduces the risk of CAA from 25% to <5%. Corticosteroids and infliximab are often used in high-risk patients or those with CAA at diagnosis, but there are no data on their longer...
Article
Full-text available
Background In neonates with single ventricle, smaller ascending aorta diameter is associated with cerebral white matter (WM) microstructural abnormalities. We sought to determine whether this association persists into adolescence. Methods and Results Ascending aorta Z scores were obtained from first postnatal echocardiogram. Brain magnetic resonan...
Data
Table S1. Participant, Echocardiogram, and Medical History Characteristics of Fontan Participants With Diffusion‐Tensor Imaging Data Table S2. White Matter Region of Interest Measures (n=42)
Article
Full-text available
Fetuses with congenital heart disease (CHD) have third trimester alterations in cortical development on brain magnetic resonance imaging (MRI). However, the intersulcal relationships contributing to global sulcal pattern remain unknown. This study applied a novel method for examining the geometric and topological relationships between sulci to feta...
Article
Importance American Heart Association guidelines recommend echocardiography in Kawasaki disease at baseline, 1 to 2 weeks, and 4 to 6 weeks after treatment to detect coronary artery abnormalities. However, these examinations are expensive and may require sedation in young children, which is burdensome and carries some risk. Objective To assess the...
Article
Little information exists on left atrial (LA) function in healthy children and in patients with congenital aortic valve stenosis (AS). We sought to determine whether patients with significant congenital AS have detectable abnormalities in LA size and function compared with matched normal controls. Retrospective chart review and analysis of 2-dimens...
Article
Interrupted right aortic arch (IRAA) is a rare congenital cardiovascular anomaly typically associated with other forms of congenital heart disease. We report 2 cases of IRAA associated with isolated left pulmonary artery and bilateral ductus arteriosus in the first case and with truncus arteriosus (TA) in the second. Computed tomography was complim...

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