Over the past 5 years, the advent of echocardiographic screening for rheumatic heart disease (RHD) has revealed a higher RHD burden than previously thought. In light of this global experience, the development of new international echocardiographic guidelines that address the full spectrum of the rheumatic disease process is opportune. Systematic differences in the reporting of and diagnostic approach to RHD exist, reflecting differences in local experience and disease patterns. The World Heart Federation echocardiographic criteria for RHD have, therefore, been developed and are formulated on the basis of the best available evidence. Three categories are defined on the basis of assessment by 2D, continuous-wave, and color-Doppler echocardiography: ‘definite RHD’, ‘borderline RHD’, and ‘normal’. Four subcategories of ‘definite RHD’ and three subcategories of ‘borderline RHD’ exist, to reflect the various disease patterns. The morphological features of RHD and the criteria for pathological mitral and aortic regurgitation are also defined. The criteria are modified for those aged over 20 years on the basis of the available evidence. The standardized criteria aim to permit rapid and consistent identification of individuals with RHD without a clear history of acute rheumatic fever and hence allow enrollment into secondary prophylaxis programs. However, important unanswered questions remain about the importance of subclinical disease (borderline or definite RHD on echocardiography without a clinical pathological murmur), and about the practicalities of implementing screening programs. These standardized criteria will help enable new studies to be designed to evaluate the role of echocardiographic screening in RHD control.

We retrospectively reviewed the surgical treatment of 12 patients (nine female, mean age 16.1+/-8.7 years) with sub-mitral aneurysms managed in our institution between 1991 and 2002. We identified three groups of patients in accordance with the degree of posterior mitral annular involvement by the aneurysm. A single aneurysm neck was found in seven patients, multiple necks in two and involvement of the entire posterior mitral annulus in three patients. Involvement of the entire posterior annulus by the aneurysmal process has not been previously described. The mean age of this latter group 29+/-5.1 years was significantly older than the former (P=0.001), suggesting a possible progressive nature of sub-mitral aneurysms. An intracardiac surgical approach was used in six patients and a combined intra and extracardiac approach in the remainder. There was no operative mortality. The mitral valve was initially repaired in eight patients. Failure of closure of the aneurysm necessitating reoperation occurred in four patients (33.3%). An understanding of the inter-relationship between the aneurysm and mitral valve is essential for successful surgical repair. Histology of the aneurysm tissue showed rheumatic heart disease in two patients and tuberculosis in two patients. Hence, although sub-valvar aneurysms are thought to be congenital, a third of our patients had evidence of co-existent rheumatic heart disease or tuberculosis.

The most consistent finding was stenosis of the aorta. Marginal irregularity/undulation of the aorta was also a useful angiographic diagnostic feature in subtle disease. The incidence of aneurysms was high compared to other studies and both fusiform and saccular aneurysms were encountered. Percutaneous transluminal angioplasty (PTA) was successful in all eight patients in whom it was performed. MRI, CT angiography and US are discussed as less invasive imaging alternatives. TA is a significant cause of renovascular hypertension in children in South Africa where there is a high incidence of tuberculous infection. Knowledge of the angiographic features and pattern of aortic involvement is essential for diagnosis and initiation of early and appropriate treatment, including PTA.