Data from the Global Burden of Disease study indicate that there were 319,400 deaths due to rheumatic heart disease and 33.4 million cases of rheumatic heart disease in 2015. The highest death and ...prevalence rates were found in Oceania, South Asia, and central sub-Saharan Africa.
With the advent of three-dimensional (3D) printers and high-resolution cardiac imaging, rapid prototype constructions of congenital cardiac defects are now possible. Typically, source images for ...these models derive from higher resolution, cross-sectional cardiac imaging, such as cardiac magnetic resonance imaging or computed tomography. These imaging methods may involve intravenous contrast, sedation, and ionizing radiation. New echocardiographic transducers and advanced software and hardware have optimized 3D echocardiographic images for this purpose. Thus, the objectives of this study were to confirm the feasibility of creating cardiac models from 3D echocardiographic data and to assess accuracy by comparing 3D model measurements with conventional two-dimensional (2D) echocardiographic measurements of cardiac defects.
Nine patients undergoing 3D echocardiography were identified (eight with ventricular septal defects, one with three periprosthetic aortic valve leaks). Raw echocardiographic image data were exported anonymously and converted to Digital Imaging and Communications in Medicine format. The image data were filtered for noise reduction, imported into segmentation software to create a 3D digital model, and printed. Measurements of the defects from the 3D model were compared with defect measurements from conventional 2D echocardiographic data. Meticulous care was taken to ensure identical measurement planes.
Long- and short-axis measurements of eight ventricular septal defects and three perivalvar leaks were obtained. Mean ± SD values for the 3D model measurements and conventional 2D echocardiographic measurements were 7.5 ± 6.3 and 7.1 ± 6.2 mm respectively (P = .20), indicating no significant differences between the standard 2D and 3D model measurements. The two groups were highly correlated, with a Pearson correlation coefficient of 0.988. The mean absolute error (2D - 3D) for each measurement was 0.4 ± 0.9 mm, indicating accuracy of the 3D model of <1 mm.
Three-dimensional printed models of echocardiographic data are technically feasible and may accurately reflect ventricular septal defect anatomy. Three-dimensional models derived from 3D echocardiographic data sets represent a new tool in procedural planning for children with congenital heart disease.
Acute rheumatic fever remains a serious healthcare concern for the majority of the world's population despite its decline in incidence in Europe and North America. The goal of this statement was to ...review the historic Jones criteria used to diagnose acute rheumatic fever in the context of the current epidemiology of the disease and to update those criteria to also take into account recent evidence supporting the use of Doppler echocardiography in the diagnosis of carditis as a major manifestation of acute rheumatic fever.
To achieve this goal, the American Heart Association's Council on Cardiovascular Disease in the Young and its Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee organized a writing group to comprehensively review and evaluate the impact of population-specific differences in acute rheumatic fever presentation and changes in presentation that can result from the now worldwide availability of nonsteroidal anti-inflammatory drugs. In addition, a methodological assessment of the numerous published studies that support the use of Doppler echocardiography as a means to diagnose cardiac involvement in acute rheumatic fever, even when overt clinical findings are not apparent, was undertaken to determine the evidence basis for defining subclinical carditis and including it as a major criterion of the Jones criteria. This effort has resulted in the first substantial revision to the Jones criteria by the American Heart Association since 1992 and the first application of the Classification of Recommendations and Levels of Evidence categories developed by the American College of Cardiology/American Heart Association to the Jones criteria.
This revision of the Jones criteria now brings them into closer alignment with other international guidelines for the diagnosis of acute rheumatic fever by defining high-risk populations, recognizing variability in clinical presentation in these high-risk populations, and including Doppler echocardiography as a tool to diagnose cardiac involvement.
In a randomized trial of secondary antibiotic prophylaxis in Ugandan children and adolescents with latent rheumatic heart disease, penicillin G benzathine given every 4 weeks for 2 years reduced the ...risk of disease progression. Among 458 participants in the prophylaxis group, 2 had serious adverse events that were attributable to prophylaxis, including one episode of anaphylaxis.
To evaluate the long-term clinical outcomes of children with rheumatic heart disease (RHD) in Uganda, and determine characteristics that predict adverse outcomes.
This retrospective cohort study ...evaluated the risk of death in Ugandan children with clinical RHD from 2010 to 2018; enrolling children aged 5-18 years old from an existing registry. Demographic data and clinical data (baseline complications, RHD severity, cardiac interventions) were collected. The primary outcome was survival. Univariable and multivariable hazard ratios (HR) were obtained from Cox proportional hazards regression. Survival probabilities were developed using Kaplan-Meier curves; log-rank tests compared survival based on cardiac interventions, disease severity and time of enrolment.
612 cases met inclusion criteria; median age 12.8 years (IQR 5.3), 37% were male. Thirty-one per cent (187 of 612) died during the study period; median time to death 7.8 months (IQR 18.3). In univariable analysis, older age (HR 1.26, 95% CI=1.0 to 1.58), presence of baseline complications (HR 2.06, 95% CI=1.53 to 2.78) and severe RHD (HR 5.21, 95% CI=2.15 to 12.65) were associated with mortality. Cardiac intervention was associated with a lower risk of mortality (HR 0.06, 95% CI=0.02 to 0.24). In multivariable models, baseline complications (HR 1.78, 95% CI=1.31 to 2.41), severe RHD (HR 4.58, 95% CI=1.87 to 11.23) and having an intervention (HR 0.05, 95% CI=0.01 to 0.21) remained statistically significant. Kaplan-Meier survival curves demonstrated >25% mortality in the first 30 months, with significant differences in mortality based on intervention status and severity of disease.
The mortality rate of children with clinical RHD in Uganda exceeds 30%, over an 8-year time frame, despite in-country access to cardiac interventions. Children at highest risk were those with cardiac complications at baseline and severe RHD.
Advances in fetal echocardiography have improved recognition of congenital heart disease (CHD). Imaging protocols have been developed that predict delivery room (DR) risk and anticipated postnatal ...level of care (LOC). The aim of this study was to determine the utility of fetal echocardiography in the perinatal management of CHD.
A retrospective analysis of fetal and postnatal records was conducted. The anticipated LOC was assigned by fetal echocardiography (LOC 1, nursery consult/outpatient follow-up; LOC 2, stable in DR with transfer to cardiac hospital; LOC 3 or 4, DR instability/urgent intervention needed). Prenatal diagnoses and LOC assignment were compared with postnatal diagnoses, treatment, and short-term outcomes.
From 2004 to 2012, 8,101 fetuses were evaluated; 7,405 were normal. Of 696 with CHD, 101 terminated, 40 died in utero, and 37 received palliative care. LOC was assigned in the remaining 518. Of 219 LOC 1, 195 (89%) had postnatal follow-up. Only two required transfer for intervention (LOC 1 sensitivity, 0.9; LOC 1 positive predictive value, 0.99). Of 260 assigned LOC 2, 229 (88%) had follow-up. Of these, 200 (87%) were transferred for surgery or intervention. The median time to admission was 195 min. Twenty-two patients (10%) assigned LOC 2 did not require intervention; however, seven (all with D-transposition of the great arteries) required catheter intervention before surgery. Hospital survival was 86% (LOC 2 sensitivity, 0.97; LOC 2 positive predictive value, 0.87). All LOC 3 and 4 patients had follow-up. Thirty-four (87%) needed urgent intervention, with 100% DR and 87% hospital survival (LOC 3 and 4 sensitivity, 0.83; LOC 3 and 4 positive predictive value, 0.87).
Fetal echocardiography enables accurate postnatal risk stratification in CHD, with the exception of D-transposition of the great arteries. LOC 1 assignment facilitated outpatient follow-up; LOC 2 assignment facilitated transfer for intervention. LOC 3 and 4 patients underwent stabilizing intervention or surgery with good short-term outcomes. Given the inability to predict need for intervention in D-transposition of the great arteries, all such patients should be assigned as LOC 3 or 4. Fetal echocardiography with LOC assignment should be used in the planning of postnatal care in CHD.
Identification of children with latent rheumatic heart disease (RHD) by echocardiography, before onset of symptoms, provides an opportunity to initiate secondary prophylaxis and prevent disease ...progression. There have been limited artificial intelligence studies published assessing the potential of machine learning to detect and analyze mitral regurgitation or to detect the presence of RHD on standard portable echocardiograms.
We used 511 echocardiograms in children, focusing on color Doppler images of the mitral valve. Echocardiograms were independently reviewed by an expert adjudication panel. Among 511 cases, 229 were normal, and 282 had RHD. Our automated method included harmonization of echocardiograms to localize the left atrium during systole using convolutional neural networks and RHD detection using mitral regurgitation jet analysis and deep learning models with an attention mechanism. We identified the correct view with an average accuracy of 0.99 and the correct systolic frame with an average accuracy of 0.94 (apical) and 0.93 (parasternal long axis). It localized the left atrium with an average Dice coefficient of 0.88 (apical) and 0.9 (parasternal long axis). Maximum mitral regurgitation jet measurements were similar to expert manual measurements (
value=0.83) and a 9-feature mitral regurgitation analysis showed an area under the receiver operating characteristics curve of 0.93, precision of 0.83, recall of 0.92, and F1 score of 0.87. Our deep learning model showed an area under the receiver operating characteristics curve of 0.84, precision of 0.78, recall of 0.98, and F1 score of 0.87.
Artificial intelligence has the potential to detect RHD as accurately as expert cardiologists and to improve with more data. These innovative approaches hold promise to scale echocardiography screening for RHD.
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