Differences in Cardiovascular Manifestation of Marfan Syndrome Between Children and Adults.

Marfan syndrome (MFS) is a connective tissue disorder characterized by a broad range of clinical manifestations. Cardiovascular involvement is the most life-threatening aspect of the syndrome. Although abnormalities within the cardiovascular system in adults are well documented, there is still a paucity of data regarding manifestation of MFS in childhood. The aim of the study was to compare cardiovascular manifestation of MFS between children and adults. The study population consisted of 236 patients (144 children and 92 adults), who were referred to our department with suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 101 (44 children and 57 adults) out of the 236 patients. The other patients were diagnosed with Ehlers-Danlos syndrome, Loeys-Dietz syndrome, MASS phenotype, ectopia lentis syndrome, marfanoid habitus and other rare syndromes. The most common cardiovascular abnormality was aortic root dilatation (81.19% of patients). It was found that both adults and children had similar high rates of aortic root dilatation. Similarly, there was no significant difference with regard to the prevalence of aortic valve regurgitation and mitral valve prolapse among children and adults. These findings equivocally indicate that the aforementioned abnormalities develop in early childhood, therefore, they may be used in the early identification of patients with MFS. Other assessed abnormalities, which included mitral valve regurgitation, pulmonary artery dilation, aneurysms of aortic arch, descending thoracic aorta and abdominal aorta were found mostly in adults, and thus, are of less use in the early detection of MFS.

Early and long-term prognosis of patients with coronary artery disease treated with percutaneous coronary interventions in 2005. Experience of single large-volume PCI center.

PURPOSE: The progress which has been made in interventional cardiology contributes to the gradual improvement of the results of CHD (coronary heart disease) therapy. The aim of the study was the assessment of early and long-term prognosis in all the patients with CHD treated invasively in one large-volume PCI center in 2005. MATERIAL AND METHODS: 1390 consecutive patients with CHD treated with PCI in 2005 were included in the analysis. Patients with ST-elevation myocardial infarction (STEMI) accounted for 50% of cases, patients with stable angina (SA) amounted to 25%, and patients with non-ST elevation acute coronary syndromes (NSTE-ACS) constituted 25%. Mean follow-up was 738 (±237) days. RESULTS: The highest mortality during the hospitalization was noted within the STEMI group(SA vs. NSTE-ACS vs. STEMI; 0% vs. 0.3% vs. 4.1%, respectively; p<0.001). The highest mortality during a 2-year follow-up was also observed in the STEMI group (SA vs. NSTE-ACS vs. STEMI, 6.3% vs. 8.5% vs. 13.8%, respectively; p<0.001). Multiple regression model showed that independent risk factors for death during the follow-up were: age, glycaemia at admission, heart rate, blood pressure, ejection fraction, STEMI, ineffective PCI (R=0.3613; F(10.131)=19.672; p<0.0001 for the model). CONCLUSIONS: The highest relative increase of mortality after the discharge of patients with CHD undergoing PCI referred to the patients with NSTE-ACS. However, in the real life PCI practice STEMI patients have the worst hospital and long-term prognosis. Well recognized risk factors for death in patients with CHD are still of great importance in negative prognosis of patients undergoing PCI.

[Contrast echocardiography in children]. / Echokardiografia kontrastowa u dzieci.

The purpose of this study was to present the use of contrast echocardiography in congenital heart disease such as atrial septum defect or in complex congenital heart disease for the identification of valve atresia. In spite of better and better methods of heart structure imaging with high resolution picture and the flow direction with the help of colour-Doppler the contrast echocardiography is a very useful method for the identification of pathological intracardiac flows. The analysis of contrast flow with the use of freeze frame allows the recognition of valve atresia accompanied by backwards inflow into the vessel.

[Reproduction problems in women with cardiovascular diseases]. / Problemy prokreacyjne u kobiet z patologia ukladu krazenia.

53 female patients who informed the obstetrician about their cardiological problems were examined. In 44 cases the heart defects were diagnosed, in 39 cases of the congenital origin. In 20 patients the shunt congenital heart disease were recognized, in 13 cases the valvular defects, in 6--Fallot Syndrome and in the single cases tricuspid atresia, pulmonary atresia, Ebstein anomaly were observed. The next 9 patients were diagnosed as: in 4 cases hyperthrophy cardiomyopathy, in 2 cases the post myocarditis status, in 2 cases complete atrioventricular block and in 1 case WPW syndrome. The analysed women were pregnancy together 98 times, finished the delivery in 86 times. The physiological delivery were observed in 53 cases. 3 neonates died in the first day of live, 6 children were born prematured. Among 83 newborns who alived 7 required intensive care. The congenital heart diseases was diagnosed in 4 children--the atrial septal defect, pulmonary stenosis, coarctation of the aorta and mitral valve malformation were seen. Two children of the mothers with hyperthrophy cardiomyopathy have the same cardiological problems. The child of mother with congenital aortic stenosis suffered from the anal atresia and agenesis of the kidney.

[The closure of persistent ductus arteriosus using coils in a child with Wilm's tumor]. / Zamkniecie droznego przewodu tetniczego za pomoca sprezynki wewnatrznaczyniowej u dziecka z guzem Wilmsa.

In Pediatric Institute of Medical University of Gdansk in all children with neoplasmatic diseases the echo examination is performed, due to exclude coexisting abnormalities of cardiovascular system. During this kind of procedure in 4.5-year-old girl with Wilms tumour the persistent ductus arteriosus was described. Because of the chemotherapy design and its potential cardiotoxicity the closure of PDA using coils was performed immediately. The successful closure of PDA gave the chance to apply the whole therapeutic protocol.

[Recanalization of the ductus arteriosus in a child with non-Hodgkin's lymphoma]. / Rekanalizacja przewodu tetniczego u dziecka z non-Hodgkin lymphoma.

Dissertation describes the case of 7.5-year-old boy with recanalization of the ductus arteriosus. In this patient treated because of the non-Hodgkin lymphoma during the chemotherapy the respiratory failure occurred and mechanical ventilation was required. After the respiratorotherapy in the controlled echocardiography examination the patency of previously closed ductus arteriosus, was described. There was no description of the similar case in available references.