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.

Long-term Outcomes of Tetralogy of Fallot in the Kingdom of Bahrain.

Introduction: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Surgical correction has improved survival but re-intervention is often required. Objectives: The objective is to assess outcomes after surgical repair of TOF, long-term follow-up, and factors that influence these results. Materials and Methods: This is a retrospective study conducted in a tertiary care center. Records of patients diagnosed with TOF from 1992 to 2019 (37 years) were retrieved from a detailed database. Patients who underwent complete correction were grouped according to diagnosis, the technique utilized in surgical repair, need for staged repair, and syndromic association. Univariate actuarial and event-free survival analysis was performed. The endpoint for an event was death or re-intervention. Results: A total of 230 patients were diagnosed with TOF and 174 patients underwent complete surgical repair. At 40 years postoperatively, survival was 96%. Actuarial survival was independent of syndromic associations, anatomical diagnosis, type of surgery, or previous shunt. Event-free survival (EFS) survival was 8.12%. EFS was significantly worse for patients with pulmonary atresia (PA) (Hazard ratio, 4.1125; 95% confidence interval [CI], 1.2654-13.3657; P < 0.0001) and for those that required homograft/conduit. The median duration for EFS was 22.73 years, 19.58 years, and 9.12 years for transannular patch (TAP), pulmonary valve-sparing (PVS), and homograft group, respectively. The survival curve for the PVS group merged with that of TAP 20 years postoperatively. Similarly, it merged at 22 years for staged versus primary repair and at 22.73 years for syndromic versus nonsyndromic patients. A weak correlation was found between age at surgery and event-free duration (cc, 0.309; P < 0.0001). The need for TAP was not influenced by the previous palliation, χ2(1, n = 154) = 3.36, P = 0.0667, or with interval to complete correction after the shunt procedure (P = 0.9672). Conclusions: Total correction of TOF has low perioperative mortality and good long-term survival, but the need for re-interventions is high. This study demonstrated that patients requiring homograft/conduit and those with a diagnosis of PA had worse outcomes. Comparison between different surgical groups showed merging of survival curves in follow-up that signifies gradual loss of survival advantage over time.

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.