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The Residual Lesion Score is a novel tool for assessing the achievement of surgical objectives in congenital heart surgery based on widely available clinical and echocardiographic characteristics. This article describes the methodology used to develop the Residual Lesion Score from the previously developed Technical Performance Score for five common congenital cardiac procedures using the RAND Delphi methodology.
A panel of 11 experts from the field of paediatric and congenital cardiology and cardiac surgery, 2 co-chairs, and a consultant were assembled to review and comment on validity and feasibility of measuring the sub-components of intraoperative and discharge Residual Lesion Score for five congenital cardiac procedures. In the first email round, the panel reviewed and commented on the Residual Lesion Score and provided validity and feasibility scores for sub-components of each of the five procedures. In the second in-person round, email comments and scores were reviewed and the Residual Lesion Score revised. The modified Residual Lesion Score was scored independently by each panellist for validity and feasibility and used to develop the “final” Residual Lesion Score.
The Residual Lesion Score sub-components with a median validity score of ≥7 and median feasibility score of ≥4 that were scored without disagreement and with low absolute deviation from the median were included in the “final” Residual Lesion Score.
Using the RAND Delphi methodology, we were able to develop Residual Lesion Score modules for five important congenital cardiac procedures for the Pediatric Heart Network’s Residual Lesion Score study.
To compare patients treated for incomplete Kawasaki disease whose practitioners followed versus did not follow American Heart Association criteria and to evaluate the association of cardiology consultation with adherence to these guidelines.
Single centre retrospective cohort study of patients <18 years old who received ≥1 dose of intravenous immunoglobulin for Kawasaki disease between 01/2006 and 01/2018. We collected demographics, clinical and laboratory data, coronary artery abnormalities, and cardiology consultation status. Patients treated for incomplete Kawasaki disease were divided into two groups based on adherence versus nonadherence to American Heart Association guidelines and compared by Wilcoxon rank sum test and chi-squared or Fisher’s exact test.
Of the 357 patients treated for Kawasaki disease, 109 (31%) were classified as incomplete Kawasaki disease. The American Heart Association algorithm for identifying patients with incomplete Kawasaki disease was followed in 81/109 (74%). Coronary artery abnormalities were present in 46/109 (42%) of the patients who were treated for incomplete Kawasaki disease. Cardiology consultation was more frequent in those fulfilling American Heart Association criteria for the diagnosis of incomplete Kawasaki disease versus those who did not fulfill criteria (76% versus 48%, p = 0.005).
Over 25% of patients treated for incomplete Kawasaki disease did not meet American Heart Association guidelines. Guidelines were more frequently followed when the paediatric cardiology team was consulted. Consulting physicians with experience and expertise in the evaluation and management of incomplete KD should be strongly considered in the care of these patients.
Significant atrioventricular valve regurgitation at diagnosis in single-ventricle patients has been associated with mortality and morbidity. However, longitudinal data on the effect of valve regurgitation at diagnosis on outcomes in the era of surgical valve interventions are scarce.
Materials and methods
This is a retrospective review of single-ventricle patients admitted to a regional centre from 2005 to 2008. Data were reviewed from birth to 18 months, and association of atrioventricular valve regurgitation at diagnosis with mortality and morbidity was evaluated.
A total of 118 patients were studied, 73% with a single right ventricle. At diagnosis, 37 patients (31%) had mild, 5 (4%) had mild to moderate, and 4 (3%) had ≥ moderate atrioventricular valve regurgitation. Moderate or greater valve regurgitation was associated with mortality (HR 5.51, 95% CI 1.24–24.61, p = 0.025), and all four patients with ≥ moderate valve regurgitation died. However, valve regurgitation was not associated with mortality for left ventricle patients. In all, 12 patients (10%) had surgical atrioventricular valve interventions. There were no independent predictors of valve intervention, and no patient having an intervention had > mild valve regurgitation at diagnosis. There was no association between valve regurgitation and days of hospitalisation or chest tube drainage.
Significant atrioventricular valve regurgitation at diagnosis remains a risk factor for mortality in single-ventricle patients, although it may be less important for single left ventricle patients. However, it is not associated with increased morbidity or surgical atrioventricular valve intervention in survivors. Reliably predicting surgical atrioventricular valve intervention remains a challenge in single-ventricle patients.
Rheumatic heart disease is the only residual morbidity, and the sole cause of mortality, from rheumatic fever. Echocardiography is ideally suited to confirm and follow the course of rheumatic heart disease. Additionally, both minimal valvar pathology in children, and extensive valvar pathology in adults, may not cause a murmur and can be detected only by echocardiography. Whenever possible, echocardiography should be routinely employed for management of patients with rheumatic fever or suspected rheumatic fever.
Obstruction of the left ventricular outflow tract may be associated with hypoplasia of the left heart, which importantly influences the options for treatment. Although the influence of the size of the left heart on the outcome for critical aortic stenosis has been described, less is known about the spectrum of such hypoplasia seen with neonatal aortic coarctation, and how this influences outcome. To determine, first, the spectrum and influence of hypoplasia of the left heart in neonatal coarctation, second, if the previously described critical values for adequacy of the left heart in neonates with critical aortic stenosis are applicable to neonates with coarctation, and, third, if any of the variables or associated abnormalities are risk factors for recoarctation, we studied 63 neonates who underwent repair of coarctation. From the initial echocar diogram, we measured multiple structures in the left heart, and calculated a score for adequacy as has been done for critical aortic stenosis. The sizes were compared to previously reported minimal values. We then analyzed the influence of the variables and the associated anomalies on outcome. There were no deaths. There was a broad spectrum of sizes that did not correlate with the need for re-intervention. The calculated score for adequacy would have predicted survival in only 56% of the patients, and 73% of the neonates had at least one parameter measured in the left heart below the previously reported minimal values. There is, therefore, a broad spectrum of sizes for the left heart in neonates with aortic coarctation that is not predictive of outcome. Minimal sizes, and the score for adequacy used for critical aortic stenosis, are not applicable to neonates with coarctation.
Despite pathologic evidence of myocardial inflammation, the significance of myocarditis in children with acute rheumatic carditis remains controversial. Elevations in cardiac troponin I have been demonstrated in other forms of myocarditis. The purpose of our study was to determine if levels of cardiac troponin I are elevated, suggesting myocardial injury, in patients with acute rheumatic carditis. We identified all those patients with acute rheumatic fever, presenting between July 1998 and December 2000, who had clinical evidence of carditis, such as a new murmur of mitral or aortic regurgitation, and who had an echocardiogram, measurements of levels of cardiac troponin I, erythrocyte sedimentation rate, and/or C-reactive protein performed at the time of presentation. Their charts were reviewed for demographic and clinical data. Echocardiograms were reviewed for severity of aortic and mitral regurgitation, and measurements made of left ventricular ejection fraction, fractional shortening, and end-diastolic dimension. We found 16 patients with acute rheumatic carditis, ranging in age from 2.0 to 16.1 years, with just over one-third having symptoms of congestive heart failure. All patients had evidence of acute inflammation. There was a significant relationship between symptoms and severity of mitral regurgitation. No patient had elevated levels of cardiac troponin I level. The fact that levels of cardiac troponin I are not elevated in the serum of children with acute rheumatic carditis suggests that there is minimal myocytic necrosis in this setting. This supports the concept that acute valvar regurgitation is the major hemodynamic abnormality in these patients.