Title : Rare Cardiovascular Diseases Encountered of Pediatric Cardiac Surgery And Cardiology.
Abstract:
In domestic cardiac surgery, there is currently a trend towards an increasing incidence of complex, combined, and rare pathologies of the heart and blood vessels in children.
The purpose of the report : to study rare congenital heart anomalies encountered in pediatric cardiac surgery and cardiology.
Material and methods: 80 children with congenital heart disease (CHD) . All children underwent standard examinations: ultrasound examination of the heart and blood vessels, ECG, CT, laboratory and histological studies, and examination of macropreparations.
Results: Among the patients were: newborns - 22; up to 1 year - 35; up to 5 years - 23 patients. We diagnosed: congenital dilated cardiomyopathy with low ejection fraction up to 31% in 2 newborn; multiple rhabdomyomas of the heart - in 4, in one of whom congenital tuberous sclerosis (Pringle-Bourneville disease) was
confirmed; mitral valve ?nd left ventricle fibroma in a newborn- in 4 ; right atrial cyst - 1, mitral and aortic valve defects with the development of severe myxomatous deformation and insufficiency were observed in 46 children with mucopolysaccharidosis;; transposition of the great vessels with total anomalous drainage of the pulmonary veins - 1; syndrome of anomalous origin of the left coronary artery from the pulmonary trunk (Bland-White-Garland syndrome)-4; double origin of the great vessels from the right ventricle with dextrocardia - 1; congenital complete atrioventricular block - in 2 newborns; Abbernethy syndrome - in 2 ; arteriovenous malformation - 2, 1- chylous pericarditis; cystic fibrosis - 3; parasitic infection with Echinococcosis - 3 children; idiopathic dilatation of the pulmonary artery trunk-2; Congenital pulmonary lymphangiectasia with total anomalous pulmonary venous drainage-4.
26 children underwent surgery : radical excision of cardiac lesions in 2, pacemaker implantation in 2; radical correction of congenital heart disease on open heart – 8. 4 children died after surgery, 1 – before surgery. 44 patients received conservative therapy.
Thus, there are difficulties in diagnosing and treating rare cardiac anomalies and diseases . These patients are more likely to develop complications in the postoperative period, and their clinical course is unique. Diagnosing rare and complex congenital heart defects requires a more thorough examination of the patient's medical history and manifestations, as well as a broad range of tests, necessitating referral of these children to specialized cardiac surgery and cardiology centers.
