Maternal diabetes as a teratogenic factor for congenital heart defects in infants of diabetic mothers.

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.

Dacron Tube Graft-Covered Stenting of Recoarctation of the Aorta.

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.

Computerized Tomography Use in Williams-Beuren Syndrome Aortopathy.

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.