Efficacy of a Standardized Computer-Based Training Curriculum to Teach Echocardiographic Identification of Rheumatic Heart Disease to Nonexpert Users.

The ability to integrate echocardiographic for rheumatic heart disease (RHD) into RHD prevention programs is limited because of lack of financial and expert human resources in endemic areas. Task shifting to nonexperts is promising; but investigations into workforce composition and training schemes are needed. The objective of this study was to test nonexperts' ability to interpret RHD screening echocardiograms after a brief, standardized, computer-based training course. Six nonexperts completed a 3-week curriculum on image interpretation. Participant performance was tested in a school-screening environment in comparison to the reference approach (cardiologists, standard portable echocardiography machines, and 2012 World Heart Federation criteria). All participants successfully completed the curriculum, and feedback was universally positive. Screening was performed in 1,381 children (5 to 18 years, 60% female), with 397 (47 borderline RHD, 6 definite RHD, 336 normal, and 8 other) referred for handheld echo. Overall sensitivity of the simplified approach was 83% (95% CI 76% to 89%), with an overall specificity of 85% (95% CI 82% to 87%). The most common reasons for false-negative screens (n = 16) were missed mitral regurgitation (MR; 44%) and MR ≤1.5 cm (29%). The most common reasons for false-positive screens (n = 179) included identification of erroneous color jets (25%), incorrect MR measurement (24%), and appropriate application of simplified guidelines (39.4%). In conclusion, a short, independent computer-based curriculum can be successfully used to train a heterogeneous group of nonexperts to interpret RHD screening echocardiograms. This approach helps address prohibitive financial and workforce barriers to widespread RHD screening.

Handheld echocardiography versus auscultation for detection of rheumatic heart disease.

BACKGROUND: Rheumatic heart disease (RHD) remains a major public health concern in developing countries, and routine screening has the potential to improve outcomes. Standard portable echocardiography (STAND) is far more sensitive than auscultation for the detection of RHD but remains cost-prohibitive in resource-limited settings. Handheld echocardiography (HAND) is a lower-cost alternative. The purpose of this study was to assess the incremental value of HAND over auscultation to identify RHD. METHODS: RHD screening was completed for schoolchildren in Gulu, Uganda, by using STAND performed by experienced echocardiographers. Any child with mitral or aortic regurgitation or stenosis plus a randomly selected group of children with normal STAND findings underwent HAND and auscultation. STAND and HAND studies were interpreted by 6 experienced cardiologists using the 2012 World Heart Federation criteria. Sensitivity and specificity of HAND and auscultation for the detection of RHD and pathologic mitral or aortic regurgitation were calculated by using STAND as the gold standard. RESULTS: Of 4773 children who underwent screening with STAND, a subgroup of 1317 children underwent HAND and auscultation. Auscultation had uniformly poor sensitivity for the detection of RHD or valve disease. Sensitivity was significantly improved by using HAND compared with auscultation for the detection of definite RHD (97.8% vs 22.2%), borderline or definite RHD (78.4% vs 16.4%), and pathologic aortic insufficiency (81.8% vs 13.6%). CONCLUSIONS: Auscultation alone is a poor screening test for RHD. HAND significantly improves detection of RHD and may be a cost-effective screening strategy for RHD in resource-limited settings.

Impact of telemedicine on hospital transport, length of stay, and medical outcomes in infants with suspected heart disease: a multicenter study.

BACKGROUND: Previous single-center studies have shown that telemedicine improves care in newborns with suspected heart disease. The aim of this study was to test the hypothesis that telemedicine would shorten time to diagnosis, prevent unnecessary transports, reduce length of stay, and decrease exposure to invasive treatments. METHODS: Nine pediatric cardiology centers entered data prospectively on patients aged <6 weeks, matched by gestational age, weight, and diagnosis. Subjects born at hospitals with and without access to telemedicine constituted the study group and control groups, respectively. Data from patients with mild or no heart disease were analyzed. RESULTS: Data were obtained for 337 matched pairs with mild or no heart disease. Transport to a tertiary care center (4% [n = 15] vs 10% [n = 32], P = .01), mean time to diagnosis (100 vs 147 min, P < .001), mean length of stay (1.0 vs 26 days, P = .005) and length of intensive care unit stay (0.96 vs 2.5 days, P = .024) were significantly less in the telemedicine group. Telemedicine patients were significantly farther from tertiary care hospitals than control subjects. The use of inotropic support and indomethacin was significantly less in the telemedicine group. By multivariate analysis, telemedicine patients were less likely to be transported (odds ratio, 0.44; 95% confidence interval, 0.23-0.83) and less likely to be placed on inotropic support (odds ratio, 0.16; 95% confidence interval, 0.10-0.28). CONCLUSIONS: Telemedicine shortened the time to diagnosis and significantly decreased the need for transport of infants with mild or no heart disease. The length of hospitalization and intensive care stay and use of indomethacin and inotropic support were less in telemedicine patients.

Echocardiography in pediatric infective endocarditis.

Transesophageal echocardiography is more sensitive than transthoracic echocardiography (TTE) in detecting endocarditis in adults; however, pediatric data are limited. Retrospectively, 39 subjects with endocarditis were divided into adult-sized (≥60 kg) and pediatric-sized (<60 kg). TTE was 97% sensitive in pediatric-sized subjects but 70% in adult-sized subjects (P < 0.05). TTE is usually sufficient for detection of endocarditis in children <60 kg.

B-type natriuretic peptide and heart failure in patients with ventricular septal defect: a pilot study.

In adults without congenital heart disease, B-type natriuretic peptide (BNP) has been shown to be a very sensitive and specific marker of heart failure. The utility of BNP as a marker of clinical heart failure in children with a ventricular septal defect (VSD) has yet to be determined. A prospective, observational study evaluated BNP levels and other measures of heart failure. Eligible patients were <2 years old, scheduled to undergo surgical repair of a VSD, and without other significant structural heart disease. Data collected before and after surgical repair included echocardiographic measurements, electrocardiographic (ECG) findings, Ross score, BNP measurements, and weight gain. A total of 21 patients were enrolled and 14 patients had complete postoperative follow-up data. For patients with complete data, mean BNP decreased by 94 pg/ml (118 pre vs. 24 post; paired t-test, p = 0.041), mean left ventricular end-diastolic dimension z-score decreased by 1.75 (+0.86 vs. -0.89; paired t-test, p = 0.013), mean weight z-score change per month increased by 0.35 (-0.25 vs. +0.10; Wilcoxon test, p = 0.013), and the incidence of biventricular hypertrophy on ECG decreased (46% vs. 0%; McNemar test, p = 0.031). The change in BNP showed a trend toward a negative correlation with weight z-score change per month (r = -0.531, p = 0.075). In conclusion, BNP, along with other measures of heart failure, decreased following VSD repair, and the change in BNP was most closely correlated with improved weight gain.

Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease--the perspective of cardiology.

This review includes a brief discussion, from the perspective of the pediatric cardiologist, of the rationale for creation and maintenance of multi-institutional databases of outcomes of the treatment of patients with congenital and paediatric cardiac disease, together with a history of the evolution of such databases, and a description of the current state of the art. A number of projects designed to have broad-based impact are currently in the design phase, or have already been implemented. Not surprisingly, most of the efforts thus far have focused on catheterization procedures and interventions, although some work examining other aspects of paediatric cardiology practice is also beginning. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiology including the Central Cardiac Audit Database of the United Kingdom, national database initiatives for pediatric cardiology in Switzerland and Germany, various database initiatives under the leadership of the Working Groups of The Association for European Paediatric Cardiology, the IMPACT Registry (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry of The American College of Cardiology Foundation and The Society for Cardiovascular Angiography and Interventions (SCAI), the Mid-Atlantic Group of Interventional Cardiology (MAGIC) Catheterization Outcomes Project, the Congenital Cardiac Catheterization Project on Outcomes (C3PO), the Congenital Cardiovascular Interventional Study Consortium (CCISC), and the Joint Council on Congenital Heart Disease (JCCHD) National Quality Improvement Initiative. These projects, each leveraging multicentre data and collaboration, demonstrate the enormous progress that has occurred over the last several years to improve the quality and consistency of information about nonsurgical treatment for congenital cardiac disease. The paediatric cardiology field is well-poised to move quickly beyond outcome assessment and benchmarking, to collaborative quality improvement.

Complete atrioventricular canal: comparison of modified single-patch technique with two-patch technique.

BACKGROUND: The purpose of this study was to compare the modified single-patch technique to the two-patch technique for infants with complete atrioventricular canal (CAVC) defects. METHODS: Between January 2000 and June 2006, 55 infants underwent CAVC repair. Twenty-six patients had a modified single-patch technique; 29 patients had a two-patch technique. Trisomy 21 was present in 23 of 26 and 26 of 29 patients (p = not significant [ns]). Mean age was 4.4 +/- 1.3 months (single-patch) versus 5.5 +/- 1.9 months (two-patch, p < 0.02). Mean weight was 4.74 +/- 0.92 versus 5.28 +/- 1.67 kilograms (p = ns). RESULTS: There was one death in the modified single-patch group (postoperative day 130, liver failure) and no deaths in the two-patch group. Cross-clamp times and cardiopulmonary bypass times were shorter in the modified single-patch group (97.3 +/- 19.9 vs 123.3 +/- 28.2 minutes, p < 0.0003; 128 +/- 25 vs 157 +/- 37, p < 0.03). Rastelli classification was type A (18 vs 14), B (1 vs 0), and C (7 vs 15). Mean size of the ventricular septal defect as assessed by transesophageal echocardiogram was 9 +/- 2 mm, (single-patch) versus 10 +/- 3 mm (two-patch) (p = ns). Median postoperative length of stay did not differ (10 vs 8 days). There was no difference in the degree of postoperative left or right AV valve insufficiency as assessed by serial echocardiography. One patient (4%) required reoperation for mitral insufficiency in the modified single-patch versus three patients in the two-patch group (10%, p = ns). There were no patients with third degree atrioventricular block or that required reoperation for residual VSD in the modified single-patch group. There was one patient with third-degree AV block that required a pacemaker and one patient who had reoperation for a residual ventricular septal defect in the two-patch group (p = ns). No patient in either group required reoperation for left ventricular outflow tract obstruction. CONCLUSIONS: The modified single-patch technique produced results comparable with the two-patch technique in younger patients with similarly sized ventricular septal defects. Furthermore, the modified single-patch technique was performed with significantly shorter cross-clamp and cardiopulmonary bypass times.

Noninherited risk factors and congenital cardiovascular defects: current knowledge: a scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics.

Prevention of congenital cardiovascular defects has been hampered by a lack of information about modifiable risk factors for abnormalities in cardiac development. Over the past decade, there have been major breakthroughs in the understanding of inherited causes of congenital heart disease, including the identification of specific genetic abnormalities for some types of malformations. Although relatively less information has been available on noninherited modifiable factors that may have an adverse effect on the fetal heart, there is a growing body of epidemiological literature on this topic. This statement summarizes the currently available literature on potential fetal exposures that might alter risk for cardiovascular defects. Information is summarized for periconceptional multivitamin or folic acid intake, which may reduce the risk of cardiac disease in the fetus, and for additional types of potential exposures that may increase the risk, including maternal illnesses, maternal therapeutic and nontherapeutic drug exposures, environmental exposures, and paternal exposures. Information is highlighted regarding definitive risk factors such as maternal rubella; phenylketonuria; pregestational diabetes; exposure to thalidomide, vitamin A cogeners, or retinoids; and indomethacin tocolysis. Caveats regarding interpretation of possible exposure-outcome relationships from case-control studies are given because this type of study has provided most of the available information. Guidelines for prospective parents that could reduce the likelihood that their child will have a major cardiac malformation are given. Issues related to pregnancy monitoring are discussed. Knowledge gaps and future sources of new information on risk factors are described.

Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics.

The intent of this review is to provide the clinician with a summary of what is currently known about the contribution of genetics to the origin of congenital heart disease. Techniques are discussed to evaluate children with heart disease for genetic alterations. Many of these techniques are now available on a clinical basis. Information on the genetic and clinical evaluation of children with cardiac disease is presented, and several tables have been constructed to aid the clinician in the assessment of children with different types of heart disease. Genetic algorithms for cardiac defects have been constructed and are available in an appendix. It is anticipated that this summary will update a wide range of medical personnel, including pediatric cardiologists and pediatricians, adult cardiologists, internists, obstetricians, nurses, and thoracic surgeons, about the genetic aspects of congenital heart disease and will encourage an interdisciplinary approach to the child and adult with congenital heart disease.

Report of the National Heart, Lung, and Blood Institute Working Group on research in adult congenital heart disease.

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives. The Working Group reviewed data on the epidemiology of ACHD, long-term outcomes of complex cardiovascular malformations, issues in assessing morphology and function with current imaging techniques, surgical and catheter-based interventions, management of related conditions including pregnancy and arrhythmias, quality of life, and informatics. After research and training barriers were discussed, the Working Group recommended outreach and educational programs for adults with congenital heart disease, a network of specialized adult congenital heart disease regional centers, technology development to support advances in imaging and modeling of abnormal structure and function, and a consensus on appropriate training for physicians to provide care for adults with congenital heart disease.

A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2.

We report heterozygous mutations in the genes encoding either type I or type II transforming growth factor beta receptor in ten families with a newly described human phenotype that includes widespread perturbations in cardiovascular, craniofacial, neurocognitive and skeletal development. Despite evidence that receptors derived from selected mutated alleles cannot support TGFbeta signal propagation, cells derived from individuals heterozygous with respect to these mutations did not show altered kinetics of the acute phase response to administered ligand. Furthermore, tissues derived from affected individuals showed increased expression of both collagen and connective tissue growth factor, as well as nuclear enrichment of phosphorylated Smad2, indicative of increased TGFbeta signaling. These data definitively implicate perturbation of TGFbeta signaling in many common human phenotypes, including craniosynostosis, cleft palate, arterial aneurysms, congenital heart disease and mental retardation, and suggest that comprehensive mechanistic insight will require consideration of both primary and compensatory events.

Transesophageal Echocardiographic Imaging for Congenital Lesions of the Left Ventricular Outflow Tract and the Aorta.

A comprehensive transthoracic echocardiographic imaging is possible for most pediatric patients. However, for patients in whom accurate anatomical and physiological assessment is not possible, transesophageal echocardiography (TEE) provides a supplemental diagnostic modality. Imaging the left ventricular outflow tract and the aorta involves a complicated technique of rotation, flexion, and changes in the depth of the transesophageal probe because the areas of interrogation involve multiple planes within the thoracic cavity. Furthermore, the relationship between the esophagus and the cardiovascular structures changes at various levels of the thorax. Transesophageal probes having characteristics of frequency agility, all forms of Doppler capability, and a higher number of crystal elements are now available. Abnormalities of the subaortic area, the aortic valve, coronary arteries, and the entire thoracic aorta can be clearly demonstrated. TEE also has played a complementary role in diagnostic and interventional catheterization. It has become vital in the operating room for the preoperative definition of certain aspects of the anatomy and for immediate postoperative evaluation of the result of surgery. (ECHOCARDIOGRAPHY, Volume 13, July 1996)