RESUMO
To explore the association of congenital heart disease and malformations of the gastrointestinal tract/abdominal wall. Over seven years, 105 neonates presenting with congenital gastrointestinal/ abdominal wall anomalies were explored for associated congenital heart disease. Sex, consanguinity, type of cardiac anomaly, type of gastrointestinal/abdominal wall anomaly, and chromosomal anomalies were all analyzed in order to find predisposing factors for this association. Both anomalies were found in 38% of the patients, predominantly in males. Esophageal atresia and esotracheal fistulas were the most frequent anomalies [36%], followed by anal atresia [30.5%]. The most common associated cardiac anomaly was the ventricular septal defect [37.5%]. Chromosomal anomalies were correlated to the presence of congenital malformations, mainly atrioventricular septal defects [86%, p < 0.05] and intestinal atresia [54.4%, p < 0.05]. First-cousin consanguinity was found in 13.3% of the children with gastrointestinal/abdominal wall anomalies. This percentage rises to 25% with the association of a cardiac anomaly and to 50% with VACTERL syndrome. Congenital anomalies of the gastrointestinal tract/abdominal wall are associated with congenital heart disease in 38% of the cases. Consanguinity is a risk factor for the association of both these anomalies and for VACTERL syndrome. In case of gastrointestinal/abdominal wall anomalies, an associated cardiac anomaly must be actively sought, even if the clinical exam seems normal
RESUMO
To study the frequency, prevalence, clinical presentation, management and out come of inflammatory heart diseases [IHD] in Lebanese children. Prospective survey of a group of children carriers [or at high risk] of an IHD [n: 156] recorded over a period of six years, between May 1[st], 1999, and April 30[th], 2005, at the National Register of Paediatric and Congential Heart Disease, Lebanese Society of Cardiology. The diagnosis was confirmed in all cases by echocardiography. Cases related to cardiac surgery were excluded. Acute rheumatic fever [ARF] is the most frequent pathology: 35.9%, followed by Kawasaki disease [KD]: 24.4%, dilated cardiomyopathy [DCM]: 22.4% pericardial effusion: 10.9% and finally infective endocarditis [IE]: 6.4%. There is a seasonal predominance for the ARF and KD during fall and early spring. Consanguineous marriage of first degree dosen't seem to be a factor predisposing for ARF and KD. ARF with carditis benefited from steroids with partial to total regresstion in 41/45 patients [91%], two patients under went valve repair or replacement, another patient died from severe pancarditis. All children affected with KD [except 2 cases] received IV immunoglobulins [2 g/kg, single dose], and coronary aneurysms were observed in 2 patients [5.3%]. Patients with DCM in whom there was a strong suspicion of viral myocarditis, were treated medically with complete recuperation of the cardiac function in 88% of cases. Among the patients with pericardial effusion, 3 needed urgent drainage because of a tamponnade. A child with IE was operated of his mitral valve and another one with cerebral palsy had a fatal outcome. No case of cardiac disease associated with HIV infection was found. In Lebanon, ARF remains the main cause of IHD during childhood, before KD. Currently, non-surgical treatments are highly efficient. Delayed diagnosis increased morbidity. Efforts are necessary for early recongnition and primary prevention