Oral health-related quality of life and oral manifestations of Syrian children with congenital heart disease: a case-control study.

BACKGROUND: There was an immense need for studies evaluating the oral health status of Syrian children with congenital heart disease (CHD) and its impact on their quality of life. No contemporary data are available. The objective of this study was to investigate oral manifestations and oral health-related quality of life (OHRQoL) of children with CHD and compare them with healthy controls aged 4-12 years. METHODS: A case-control study was undertaken. A total of 200 patients with CHD and 100 healthy children belonging to the same patient's family were included. Decayed, missed, and filled permanent teeth index (DMFT) and decayed, missed, and filled primary teeth index (dmft), Oral Hygiene Index (OHI), Papillary Marginal Gingivitis Index (PMGI), and dental abnormalities were recorded. The Arabic version of the Child Oral Health-Related Quality of Life Questionnaire (COHRQoL, 36-item) which was divided into 4 different domains (Oral Symptoms, Functional Limitations, Emotional Well-Being, Social Well-Being) were studied. Chi-square test and independent- t-test were used to perform statistical analysis. RESULTS: CHD patients experienced more periodontitis, dental caries, poor oral health, and enamel defects. The dmft mean was significantly higher in CHD patients than in healthy children (5.245 vs. 2.660, P < 0.05). No significant difference was found between patients and controls in the DMFT Mean (P = 0.731). There was a significant difference between CHD patients and healthy children in the mean of the OHI (5.954 vs. 1.871, P < 0.05), and PMGI (1.689 vs. 1.170, P < 0.05). CHD patients have significantly higher enamel opacities (8% vs. 2%) and hypocalcification (10.5% vs. 2%) than controls. Also, the 4 COHRQoL domains, showed significant differences between CHD children and controls. CONCLUSIONS: Evidence about the oral health and COHRQoL of children with CHD was provided. Further preventive measures are still required to improve the health and quality of life of this vulnerable group of children.

Repair of double orifice mitral valve with an atrioventricular septal defect in a girl with Ellis-Van Creveld syndrome.

Ellis-van Creveld syndrome is a rare autosomal recessive disorder caused by mutations in the EVC and EVC2 genes. The four principal manifestations are chondrodysplasia, polydactyly, ectodermal dysplasia, and congenital heart defects. We describe the case of a 7-year-old girl with Ellis-van Creveld Syndrome with the diagnosis of common atrium and partial atrioventricular septal defect. She underwent a successful surgical repair, and intraoperatively, a double orifice mitral valve was diagnosed as well. The correct diagnosis of this disorder in early life is essential in the overall prognosis of the syndrome. Clinical follow-up at regular intervals is very important in these patients to institute proper managements and prevent further complications.

Surgical repair of a giant congenital left atrial aneurysm presenting with respiratory distress in a neonate.

The presentation of congenital left atrial aneurysm is extremely rare in neonates. The neonate may suffer from severe respiratory distress symptoms, and by then, early surgical management is lifesaving.

Coronary artery bypass grafting in a 12-year-old girl with familial hypercholesterolemia.

As CABG operation is indicated in children with familial hypercholesterolemia, it seems that bilateral internal thoracic arteries are the preferred grafts, taking into consideration its superiority over venous graft regarding patency and growth.

Surgical repair of large pulmonary artery to left atrium fistula.

We report a successful surgical closure of a rught pulmonary artery to left atrium fistula (RPALAF) with secondary atrial septal defect (ASD) in a 3.5 years old girl. These fistulae are rare cause of cyanosis and require early diagnosis and treatment to prevent the serious complications of them.

Elevated dimethylglycine in blood of children with congenital heart defects and their mothers.

OBJECTIVE: Congenital Heart Defects (CHD) may be related to nutritional deficiencies affecting the methylation cycle. We aimed to study the metabolic markers of the betaine homocysteine methyl transferase (BHMT) pathway in children with CHD and their mothers compared to children without CHD and their mothers. MATERIALS AND METHODS: Children with CHD (n=105, age < 3 years) and mothers of 80 of the affected children were studied. The controls were non-CHDs children of comparable age as the CHD group (n=52) and their mothers (n=50). We measured serum or plasma concentrations of the metabolites of the methylation cycle homocysteine (HCY), methylmalonic acid (MMA), cystathionine, S-adenosylmethionine (SAM), S-adenosylhomocysteine (SAH), betaine, choline, and dimethylglycine (DMG). RESULTS: Children with CHD had higher plasma SAM (131 vs. 100 nmol/L) and DMG (8.7 vs. 6.0 µmol/L) and lower betaine/DMG ratio (7.5 vs. 10.2) compared to the controls. Mothers of CHD children showed also higher DMG (6.1 vs. 4.1 µmol/L) and lower betaine/DMG ratio compared with the mothers of the controls. Higher SAM levels were related to higher cystathionine, MMA, betaine, choline, and DMG. MMA elevation in the patients was related to higher HCY, SAM, betaine and DMG. CONCLUSIONS: Elevated DMG in CHD children and their mothers compared to the controls can indicate upregulation of the BHMT pathway in this disease group. Nutritional factors are related to metabolic imbalance during pregnancy that may be related to worse birth outcome.