Williams-Beuren syndrome is a multisystem genetic disorder caused by hemizygous deletion on chromosome 7q11.23, encompassing about 28 genes including the elastin gene, ELN. Cardiovascular abnormalities are frequent and are related to elastin insufficiency. These abnormalities include supravalvular aortic stenosis (SVAS) in 70% of case, pulmonic valve stenosis, and renal artery stenosis. Definitive therapy for supravalvar aortic stenosis consists of surgical correction of the arteriopathies. Outcomes after surgical correction of SVAS depend on the extent of the arteriopathy and the presence of other associated lesions. We present a case of a 4-year-old boy, with Williams - Beuren syndrome with an SVAS. The patient was assessed with computerized tomography angiography to determine the extent of the aortopathy before surgical intervention.

Background Congenital heart defects (CHDs) are shown to have an association with maternal diabetes mellitus. The Bahraini population has a high prevalence of diabetes 16.3% thus putting it at increased risk of developing CHDs in infants of diabetic mothers (IDMs). Objective Describing the prevalence of CHDs in IDM in the Kingdom of Bahrain. Design A retrospective clinical study. Setting Bahrain Defense Force Hospital, Kingdom of Bahrain. Methods The study took place from January 1998 to January 2020. A history was recorded for all patients who were referred to the only tertiary cardiac center in Bahrain for echocardiography. Data was recorded on an Excel Sheet for analysis. A cardiac anatomy survey was conducted by an experienced pediatric cardiologist for each patient and the defects were categorized into acyanotic and cyanotic lesions. Results Five thousand five hundred sixty‐nine patients were referred for cardiac echocardiography. Three thousand two hundred fifty‐six patients were diagnosed with CHDs, 2,987 were non‐IDM whereas 269 were IDM. Patients diagnosed with non‐structural defects were excluded. Atrial septal defect (ASD) was identified in 744 patients and was more likely to occur in non‐IDM (p‐value = .005). Hypertrophic obstructive cardiomyopathy (HOCM) was identified in 35 patients and was more likely to occur in IDM (p‐value < .001). Transposition of the great arteries (TGAs) was identified in 80 patients and was more likely to occur in IDM (p‐value .002). Double inlet left ventricle (DILV), Hypoplastic Left Heart Syndrome (HPLHS), and Other Uni‐Ventricular Hearts were all more likely to occur in IDM with p‐values < .05. Conclusion This study showed significant association between fetal exposure to diabetes and the development of ASD, HOCM, TGA, DILV, HPLHS, and Other Uni‐Ventricular Hearts.

Congenital Birth Defects (CBD) are the leading causes of infant mortality in developed countries. Congenital Heart Defects (CHD) form the major proportion of the CBD. It is estimated that they contribute almost one-half of the deaths due to CBD, both as isolated lesions and as part of other multiple CBD 1. This prevalence of CHD however, remains static despite modern techniques and technology to improve the evaluation of these cases 2. New referrals to tertiary cardiac centers had and continued to increase dramatically despite the static prevalence of CHD 3. Reports reveal that between 25% to 100% of referrals have normal hearts 3,4,5. The continued increase in referrals is due to numerous factors, which include primary care physician's reluctance to diagnose normality without echocardiography and parental demands of echocardiography to confirm normality. This results in extensive usage of societal and personal resources. The cost involved to society is immense and continue to rise.

We report a case with recoarctation within a tubed graft. Covered stent placed inside the tube graft safely and effectively treated the recoarctation of the aorta.

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