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Echocardiography guidelines note that a flail leaflet is a specific criterion for severe mitral regurgitation (MR) and that regurgitant severity is underestimated in wall-impinging jets (Coandă effect). Both findings are often considered to be pathognomonic of severe MR.In this study, the authors sought to determine the association of flail leaflet and Coandă effect with MR severity quantified by means of cardiac magnetic resonance (CMR).The authors enrolled 158 consecutive patients with primary MR according to echocardiography and CMR. The presence of a flail leaflet or Coandă was determined for each patient. CMR regurgitant volume (RV) and regurgitant fraction (RF) were quantified for all patients.There were 55 patients (35%) with a flail leaflet, 52 (33%) with Coandă, and 22 (14%) with a flail leaflet and Coandă. The mean CMR mitral RV and RF progressively increased in patients without a Coandă or flail, a Coandă, a flail, or a Coandă and a flail (RV: 28 ± 21 mL vs 43 ± 23 mL vs 58 ± 29 mL vs 64 ± 25 mL [P 0.001]; RF: 25% ± 16% vs 34% ± 14% vs 41% ± 12% vs 45% ± 12% [P 0.001]). With the use of CMR RV, 35%, 46%, and 59% of patients had severe MR with the presence of a Coandă, flail leaflet, or both, respectively. With the use of CMR RF, 25%, 31%, and 40% of patients had severe MR with the presence of a Coandă, flail leaflet, or both, respectively.While the presence of a flail leaflet and Coandă effect on echocardiography are associated with higher regurgitant volumes and fractions, they are frequently not associated with severe MR as assessed by means of CMR. (Comparison Study of Echocardiography and Cardiovascular Magnetic Resonance Imaging in the Assessment of Mitral and Aortic Regurgitation; NCT04038879).

Background Many factors affect outcomes after congenital cardiac surgery. Objectives The RLS (Residual Lesion Score) study explored the impact of severity of residual lesions on post-operative outcomes across operations of varying complexity. Methods In a prospective, multicenter, observational study, 17 sites enrolled 1,149 infants undergoing 5 common operations: tetralogy of Fallot repair (n = 250), complete atrioventricular septal defect repair (n = 249), arterial switch operation (n = 251), coarctation or interrupted arch with ventricular septal defect (VSD) repair (n = 150), and Norwood operation (n = 249). The RLS was assigned based on post-operative echocardiography and clinical events: RLS 1 (trivial or no residual lesions), RLS 2 (minor residual lesions), or RLS 3 (reintervention for or major residual lesions before discharge). The primary outcome was days alive and out of hospital within 30 post-operative days (60 for Norwood). Secondary outcomes assessed post-operative course, including major medical events and days in hospital. Results RLS 3 (vs. RLS 1) was an independent risk factor for fewer days alive and out of hospital (p ≤ 0.008) and longer post-operative hospital stay (p ≤ 0.02) for all 5 operations, and for all secondary outcomes after coarctation or interrupted arch with VSD repair and Norwood (p ≤ 0.03). Outcomes for RLS 1 versus 2 did not differ consistently. RLS alone explained 5% (tetralogy of Fallot repair) to 20% (Norwood) of variation in the primary outcome. Conclusions Adjusting for pre-operative factors, residual lesions after congenital cardiac surgery impacted in-hospital outcomes across operative complexity with greatest impact following complex operations. Minor residual lesions had minimal impact. These findings may provide guidance for surgeons when considering short-term risks and benefits of returning to bypass to repair residual lesions.

Accurate quantification of regurgitant volume is a central component to the management of mitral regurgitation. Cardiac magnetic resonance imaging (CMR) accurately quantifies mitral regurgitation as the difference between left ventricular stroke volume and forward stroke volume using steady state free precession and phase contrast imaging. The CMR measurement of mitral regurgitant volume is reproducible and can quantify mitral regurgitation in patients without regard to regurgitant jet morphology, such as patients with multiple and eccentric jets. It can be used to quantify regurgitant volume in patients with multiple valve lesions and concomitant intracardiac shunts without the use of intravenous contrast. Studies have highlighted the accuracy and reproducibility of CMR in quantifying mitral regurgitation and have begun to link CMR to clinical outcomes.

Background: Before the introduction of highly active antiretroviral therapy (HAART), cardiac mortality and morbidity were common in HIV-infected children.Objectives: This study sought to id...

Biykem Bozkurt, MD, PhD, FACC, FAHA, Chair H. Vernon Anderson, MD, FACC, FAHA Garth N. Graham, MD, FACC Hani Jneid, MD, FACC, FAHA Gail K. Jones, MD David Kao, MD, FAHA Michael Kutcher, MD, FACC Leo Lopez, MD, FACC Gregory Marcus, MD, FACC, FAHA Jennifer Rymer, MD James E. Tcheng, MD

The ASE recommends integrating multiple parameters for assessing mitral regurgitation (MR) severity by echo. The ASE guidelines include an algorithm that highlights criteria considered indicative of definitely mild or definitely severe MR. The purpose of this study was to compare this algorithm to

Background: Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear.Objectives: This study sought to d...

Background The decision to undergo mitral valve surgery is often made on the basis of echocardiographic criteria and clinical assessment. Recent changes in treatment guidelines recommending surgery in asymptomatic patients make the accurate assessment of mitral regurgitation (MR) severity even more important. Objectives The purpose of this study was to compare echocardiography and magnetic resonance imaging (MRI) in the assessment of MR severity using the degree of left ventricular (LV) remodeling after surgery as the reference standard. Methods In this prospective multicenter trial, MR severity was assessed in 103 patients using both echocardiography and MRI. Thirty-eight patients subsequently had isolated mitral valve surgery, and 26 of these had an additional MRI performed 5 to 7 months after surgery. The pre-surgical estimate of regurgitant severity was correlated with the postoperative decrease in LV end-diastolic volume. Results Agreement between MRI and echocardiographic estimates of MR severity was modest in the overall cohort (r = 0.6; p Conclusions The data suggest that MRI is more accurate than echocardiography in assessing the severity of MR. MRI should be considered in those patients when MR severity as assessed by echocardiography is influencing important clinical decisions, such as the decision to undergo MR surgery.

Accurate quantification of regurgitant volume is a central component to the management of mitral regurgitation. Cardiac magnetic resonance imaging (CMR) accurately quantifies mitral regurgitation as the difference between left ventricular stroke volume and forward stroke volume using steady state free precession and phase contrast imaging. The CMR measurement of mitral regurgitant volume is reproducible and can quantify mitral regurgitation in patients without regard to regurgitant jet morphology, such as patients with multiple and eccentric jets. It can be used to quantify regurgitant volume in patients with multiple valve lesions and concomitant intracardiac shunts without the use of intravenous contrast. Studies have highlighted the accuracy and reproducibility of CMR in quantifying mitral regurgitation and have begun to link CMR to clinical outcomes.

Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation.This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death.Children 18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death.Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death.Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391).

Before the introduction of highly active antiretroviral therapy (HAART), cardiac mortality and morbidity were common in HIV-infected children.This study sought to identify long-term cardiovascular effects of HAART in HIV-infected children.The CHAART-2 (HAART-Associated Cardiotoxicity in HIV-Infected Children) study prospectively compared 148 echocardiograms from 74 HAART-exposed children to 860 echocardiograms from 140 HAART-unexposed but HIV-infected children from the Pulmonary and Cardiac Complications of Vertically Transmitted HIV Infection (PComparing the HAART-exposed and HAART-unexposed groups, any HAART exposure was positively associated with left ventricular (LV) fractional shortening (z-score for difference = 1.07; p = 0.02) and HAART exposure duration (z-score difference per year = 0.17; p = 0.003. LV mass was negatively associated with any HAART exposure (z-score difference = -0.64; p = 0.01) as was septal thickness (z-score difference = -0.93; p = 0.001). Duration of HAART exposure was negatively associated with LV end-systolic dimension and heart rate (z-score difference per year= -0.11; p = 0.05; and z-score difference per year = -0.10; p = 0.002, respectively). During 11 years of follow-up, in the HAART-exposed group, LV mass and LV end-diastolic septal thickness were lower whereas LV contractility and LV fractional shortening were higher when compared to the HAART-unexposed group.Cardiac structure and function were better in perinatally HIV-infected children exposed to HAART than in those of similar children from the pre-HAART era but did decline over time. Evidence-based strategies for cardiovascular monitoring are needed to inform treatment decisions to improve long-term cardiovascular health.

Objectives This study sought to analyze cardiac magnetic resonance (CMR) measurements of myocardial extracellular volume fraction (ECV) and late gadolinium enhancement (LGE) in children and young adults with congenital aortic stenosis (AS) to determine the extent of fibrosis and examine their association with aortic valve and ventricular function. Background Patients with congenital AS frequently have impaired diastolic ventricular function and exercise capacity that may be related to myocardial fibrosis. Methods A total of 35 patients with congenital AS (median age 16 years) and 27 normal control subjects (median age 16 years) were evaluated by CMR. ECV was calculated from pre- and post-gadolinium contrast T1 measurements of blood and myocardium, and the hematocrit. Results ECV was significantly higher in AS patients than in normal subjects (median 0.27 [range 0.22 to 0.42] vs. 0.25 [range 0.18 to 0.27], p = 0.001). LGE was present in 8 (24%) of the AS patients. A higher ECV was correlated with echocardiographic indexes of diastolic dysfunction including a higher mitral E-wave z-score (r = 0.58, p = 0.002), E/septal E′ z-score (r = 0.56, p = 0.003), E/mean E′ z-score (r = 0.55, p = 0.003), and indexed left atrial volume (r = 0.56, p = 0.001). Other factors associated with an elevated ECV (>0.28) included a greater number of aortic valve interventions (p = 0.004) and a greater number of aortic valve balloon valvuloplasties (p = 0.003). ECV was not significantly associated with AS gradient, left ventricular mass, mass/volume ratio, or ejection fraction. Conclusions In young patients with AS, myocardial ECV is significantly elevated compared with control subjects and is associated with echocardiographic indexes of diastolic dysfunction. ECV measured by CMR may be a useful method for risk stratification and monitoring therapies targeting fibrosis.

Objectives This study sought to determine the incidence and predictors of recovery of normal echocardiographic function among children with idiopathic dilated cardiomyopathy (DCM). Background Most children with idiopathic DCM have poor outcomes; however, some improve. Methods We studied children 2) at diagnosis and who had at least 1 follow-up echocardiogram 30 days to 2 years from the initial echocardiogram. We estimated the cumulative incidence and predictors of normalization. Results Among 868 children who met the inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplantation (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio [HR]: 0.92; 95% confidence interval [CI]: 0.88 to 0.97) and lower LVEDD z-score (HR: 0.78; 95% CI: 0.70 to 0.87) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplantation or died. Conclusions Despite marked LV dilation and depressed function initially, children with idiopathic DCM can recover normal LV size and function, particularly those younger and with less LV dilation at diagnosis. Investigations related to predictors of recovery, such as genetic associations, serum markers, and the impact of medical therapy or ventricular unloading with assist devices are important next steps. Longer follow-up after normalization is warranted as cardiac failure can recur. (Pediatric Cardiomyopathy Registry; NCT00005391 )

ObjectivesThe objective of our study was to compare the indices of vascular health in Kawasaki disease (KD) patients to those of control subjects.BackgroundThe literature on peripheral vascular health after KD is conflicting.MethodsSubjects were patients 11 to 29 years of age with the onset of KD >12 months before the study visit (n = 203) and healthy control subjects (n = 50). We measured endothelial function (using the Endothelial Pulse Amplitude Testing index), intima-media thickness (IMT) of the right common carotid artery (RCCA) and the left common carotid artery (LCCA), and fasting lipid profile and C-reactive protein (CRP). KD patients were classified according to their worst-ever coronary artery (CA) status: group I, always normal CAs (n = 136, 67%); group II, CA z-scores ≥2 but

Objectives The purpose of this study was to establish the incidence of and risk factors for sudden cardiac death (SCD) in pediatric dilated cardiomyopathy (DCM). Background The incidence of SCD in children with DCM is unknown. The ability to predict patients at high risk of SCD will help to define who may benefit most from implantable cardioverter-defibrillators. Methods The cohort was 1,803 children in the PCMR (Pediatric Cardiomyopathy Registry) with a diagnosis of DCM from 1990 to 2009. Cumulative incidence competing-risks event rates were estimated. We achieved risk stratification using Classification and Regression Tree methodology. Results The 5-year incidence rates were 29% for heart transplantation, 12.1% non-SCD, 4.0% death from unknown cause, and 2.4% for SCD. Of 280 deaths, 35 were SCD, and the cause was unknown for 56. The 5-year incidence rate for SCD incorporating a subset of the unknown deaths is 3%. Patients receiving antiarrhythmic medication were at higher risk of SCD (hazard ratio: 3.0, 95% confidence interval: 1.1 to 8.3, p = 0.025). A risk stratification model based on most recent echocardiographic values had 86% sensitivity and 57% specificity. Thirty of 35 SCDs occurred in patients who met all these criteria: left ventricular (LV) end-systolic dimension z-score >2.6, age at diagnosis younger than 14.3 years, and the LV posterior wall thickness to end-diastolic dimension ratio Conclusions The 5-year incidence rate of SCD in children with DCM is 3%. A risk stratification rule (86% sensitivity) included age at diagnosis younger than 14.3 years, LV dilation, and LV posterior wall thinning. Patients who consistently meet these criteria should be considered for implantable cardioverter-defibrillator placement. (Pediatric Cardiomyopathy Registry; NCT00005391 )

Objectives: The aim of this study was to investigate the possible effects of antiretroviral therapy (ART) in utero on cardiac development and function in human immunodeficiency virus (HIV)–negative...

Objectives We explored the association of noncoronary cardiac abnormalities with coronary artery dilation and with laboratory inflammatory markers early after Kawasaki disease (KD) diagnosis. Background Left ventricular (LV) dysfunction, mitral regurgitation (MR), and aortic root dilation occur early after diagnosis; their associations with coronary artery dilation and inflammatory markers have not been well-described. Methods Centrally interpreted echocardiograms were obtained at KD diagnosis and 1 and 5 weeks after diagnosis on 198 subjects in the National Institutes of Health-sponsored Pediatric Heart Network KD pulsed steroid trial. Regression models were constructed to investigate the relationships among early LV dysfunction, MR, and aortic root dilation with coronary artery dilation and laboratory inflammatory markers. Results At diagnosis, LV systolic dysfunction was present in 20% of subjects and was associated with coronary artery dilation, seen in 29% (p = 0.004). Although LV dysfunction improved rapidly, LV dysfunction at diagnosis predicted greater odds of coronary artery dilation at 1 and 5 weeks after diagnosis (5-week odds ratio: 2.7, 95% confidence interval: 1.2 to 6.3). At diagnosis, MR was present in 27% of subjects and aortic root dilation was present in 8%; each was associated with larger coronary artery size at diagnosis. Left ventricular dysfunction was associated with higher erythrocyte sedimentation rate and, at diagnosis only, lower serum albumin; MR was associated with higher erythrocyte sedimentation rate and lower albumin at all times. Aortic root size had little association with inflammatory markers. Conclusions Noncoronary cardiac abnormalities are associated with coronary artery dilation and laboratory evidence of inflammation in the first 5 weeks after KD, suggesting a shared inflammatory mechanism. (Trial of Pulse Steroid Therapy in Kawasaki Disease [A Trial Conducted by the Pediatric Heart Network]; NCT00132080 )