Balloon-expandable stents for recoarctation of the aorta in small children. Two centre experience.

BACKGROUND: In young patients with native aortic coarctation (CoA), the management of choice is surgery. However, in re-coarctation (re-CoA) surgery is associated with increased morbidity and even mortality. Some children with native CoA present relative contraindications for surgery. METHODS: From 2006 to 2017, thirty-four patients (male n = 20; 59%) from two centres with re-CoA (31) and native CoA (3) were managed by stent implantation with premounted balloon expandable stents. Inclusion criteria were age < 3 years and >1 month, weight < 16 kg. Median age was 6,5 months (min. 1; max. 34 months), median weight 6,2 kg (min. 3,7; max. 16 kg). Thirteen patients (38%) had Re-CoA and hypoplastic left heart syndrome (HLHS). In three patients (9%) the native CoA was stented due to contraindications for surgical treatment. RESULTS: All procedures were successful. The median peak invasive systolic pressure gradient declined from 31 mm Hg (max. 118; min. 4) to 0 mm Hg (max. 32; min.-7) (p < 0.001). The median minimal diameter of the narrowed segment of aorta increased from 3 mm (max. 6,9; min. 1,0) to 7 mm (max. 11,5; min. 3,5) (p < 0.001). There were no serious complications. The median follow-up time was 12,5 months (max. 88; min. 0 month). During this time ten patients (29%) required re-dilatation and two of them re-stenting. CONCLUSION: Percutaneous stent implantation for Re-CoA and in selected patients for native CoA can be performed successfully in very young patients with a good immediate hemodynamical result. However, repeated stent angioplasties and further on interventional 'opening' of the stent is necessary to augment the aorta to adult size.

Retrospective study of juvenile spondylarthropathies in Croatia over the last 11 years.

OBJECTIVES: To determine the frequency of juvenile spondylarthropathies (JSpA) among other rheumatic diseases in a pediatric clinic population in an 11-year period in Croatia and to review their clinical, epidemiological, radiographic and laboratory. METHODS: Of the 1264 patients with rheumatic diseases seen at a pediatric rheumatology center, 103 (8.2%) were diagnosed as having JSpA (56 boys, mean age 13.1 years, range 4.4-17.8 years), following the strict criteria of the European Spondylarthropathy Study Group. Medical history, clinical laboratory and imaging data of the 103 patients with JSpA were analyzed. RESULTS: Eighty-two (79.6%) patients had undifferentiated spondylarthropathy, 6 (5.8%) patients had reactive arthritis/Reiter's disease, 6 (5.8%) had arthritis associated with inflammatory bowel disease, 5 (4.9%) had psoriatic arthritis, and only 4 (3.9%) patients had ankylosing spondylitis. The most common symptoms at the disease onset in patients with JSpA were peripheral and axial arthritis, followed by enthesitis. A significant increase in the number of patients with axial arthritis, peripheral arthritis, ocular symptoms and enthesitis was found during mean period of follow-up of 6.45 years. HLA-B27 was present in 78 (75.7%) patients. CONCLUSION: In our hospital population the frequency of JSpA among other rheumatic disease was 8.2%. The disease was equally distributed among male and female patients, with onset around the age of 13 years. Most of the patients were diagnosed with undifferentiated spondylarthropathy.

Application of flow cytometry in the study of apoptosis in neonatal rat cardiomyocytes.

Depending on the concentration, catecholamines activate various intracellular signaling pathways and can induce apoptosis in cardiac myocytes. Although 5,5',6,6'-tetrachloro-1,1',3,3'-tetraethylbenzimidazolocarbocyanine iodide (JC-1) has been previously used to study mitochondria in intact cardiomyocytes, there have been no reports on the detection of apoptosis in neonatal cardiomyocytes in combination with flow cytometry and confocal microscopy. In our study, neonatal rat cardiomyocytes were exposed to norepinephrine (NE) and isoproterenol (ISO) in concentrations of 1 and 10 microM for 48 h. NE concentrations of 1 and 10 microM decreased the number of viable cardiomyocytes by 18% (*p < 0.05) and 24% (**p = 0.01), respectively. ISO in a concentration of 1 microM increased the number of viable cardiomyocytes by 13% while 10 microM decreased the number of viable cardiomyocytes by 43% (***p < 0.001). Apoptotic cells were detected by flow cytometry and confocal microscopy. NE in concentrations of 1 and 10 microM increased the percentage of apoptotic cells by 12.2% and 34.3%, respectively, while ISO alone in a concentration of 10 microM increased the percentage of apoptotic cells by 11.3%. The results demonstrated that these two methods are reliable and suitable for the detection and study of apoptosis in cultures of neonatal cardiomyocytes.

Intracardiac juvenile xanthogranuloma in a newborn.

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.

[Juvenile dermatomyositis]. / Juvenilni dermatomiozitis.

Basic aim of this paper is presentation of probable epidemiological characteristics of juvenile dermatomyositis (JDM) in children of Croatia, due to the fact that severity of condition in majority of patients require treatment in our Department of Pediatrics, KBC Zagreb. Our intention is to present guidelines of current diagnostic approach from recent literature, with special accent on therapy. JDM was previously considered as infaust disease, while current therapy has favourable prognosis for complete resolution, as the only one mesenchymopathy with such prognosis. In period between 1988 and 1999 we treated 18 patients with JDM in our Department of Pediatrics. Thirteen cases were male and 5 female (2.6:1). Thirteen patients had clinical course of isolated JDM (72%), while five had JDM inside overlap syndrome (with other mesenchymopathies) (28%). Average age at time of diagnosis for whole group was 10 years (6-14), for girls 10.6 years (6-14) and boys 8.4 years (7-10). At the present time 5 patients (28%) are considered as cured, 7 patients are in remission with low dosis of steroids (39%) and one girl had relaps of JDM after therapy stopage. Three children die (16%), one girl due to respiratory failure and two girls with pulmonary embolia. Two girls have some signs of JDM with dominant clinical signs of sistemic sclerodermia and one girl has signs of generalized morphea. One boy has dominant muscular calcinosis with contractures of large joints, despite treatment and normal laboratory findings. JDM was not discussed in rheumatic literature in Croatia for more then 15 years.

Radioangioscintigraphy and Doppler echocardiography in the quantification of left-to-right shunt.

The magnitude of left-to-right shunt in 55 children with isolated congenital heart disease [atrial septal defect (ASD) or ventricular septal defect (VSD) (muscular and perimembranous)] was estimated by two methods: radionuclide quantification and Doppler echocardiography [flow (L/min) = mean velocity x area x ejection time x heart rate]. We found little difference between the magnitude of left-to-right shunt obtained with Doppler echocardiography and that with radioangioscintigraphy for a whole group of patients (N = 55, -11.42% to 12.04%) and for subgroups of ASD (n = 24, -12.49% to 12.19%) and VSD (n = 31, -10.69% to 12.23%). These results indicate that Doppler echocardiography, in comparison with radioangioscintigraphy, is sufficiently accurate for clinical estimation of the Q(p)/Q(s) ratio in isolated congenital heart disease with left-to-right shunt.

[Myocarditis and dilated cardiomyopathy in children. Old questions-- new answers]. / Miokarditis i dilatacijska kardiomiopatija u djece. Stara pitanja--novi odgovori.

The purpose of this article is to discuss the dilemmas in differential diagnosis of myocarditis and dilated cardiomyopathy of different etiologies. According to the definition of the WHO and the ISFC the dilated cardiomyopathy is a myocardial disease recognized by the severe enlargement of the left and/or the right ventricle causing the decrease in systolic function of the heart (decrease of contractility) with the development of congestive heart failure. In order to distinguish primary cardiomyopathies defined as a muscle heart disease sui generis on the one hand from cardiomyopathies as the outcome of chronic inflammatory myocarditis (chronic persistent myocarditis, chronic immune myocarditis, chronic viral heart disease) on the other hand, it is necessary to follow a complicated diagnostic flow diagram. Methods that are to become routine in diagnostic procedure are described. Etiologic diagnosis of dilated cardiomyopathies is not possible without heart muscle biopsy. The bioptic specimens must be analyzed using light microscope (according to Dallas criteria), electron microscope, and afterwards immunohistologically and immunohistochemically (in situ hybridization). The total result of these investigations leads to the final conclusion about etiological diagnosis of dilated cardiomyopathy. The directions in the treatment of dilated cardiomyopathies and acute myocarditis are stated, as well as the importance of immunosuppressive therapy in these conditions. In the treatment of acute myocarditis digoxin should be avoided and other inotropic substances used instead.

Double patch closure of ventricular septal defect with increased pulmonary vascular resistance.

BACKGROUND: Closure of a large ventricular septal defect (VSD) in children with elevated pulmonary vascular resistance is associated with significant morbidity and mortality. Pulmonary hypertensive episodes continue to be a major cause of postoperative morbidity and mortality. We designed a fenestrated flap valve double VSD patch in an effort to decrease the morbidity and mortality associated with the closure of a large VSD with elevated pulmonary vascular resistance. METHODS: Eighteen children (mean age, 5.7 years) with a large VSD and elevated pulmonary vascular resistance (mean, 11.4 Wood units) underwent double patch VSD closure using moderately hypothermic cardiopulmonary bypass and cardioplegic arrest. The routine VSD patch was fenestrated (4 to 6 mm) and on the left ventricular side of the patch, a second, smaller patch was attached to the fenestration along its superior margin before closure of the VSD. RESULTS: All children survived operation and were weaned from inotropic and ventilator support within 48 hours postoperatively. Postoperative pulmonary artery pressures were significantly lower than preoperative values. One child died 9 months postoperatively. CONCLUSIONS: Closure of a large VSD in children with elevated pulmonary vascular resistance can be performed with low morbidity and mortality when a flap valve double VSD patch is used.

[The mitral valve prolapse syndrome in children and adolescents]. / Sindrom prolapsa mitralne valvule u djece i adolescenata.

The authors studied the prevalence of mitral valve prolapse (MVP) in the group of 656 children and adolescents (329 males and 327 females), who were a representative sample (obtained with the Monte Carlo method of statistical trials) of all newborns in the city of Maribor, Republic of Slovenia, in the period of 18 years (1976-1992). The results were considered positive in children and adolescents who in addition to possible history (chest pain, palpitations, dizziness, loss of consciousness, headaches, perspiration), probable auscultatory finding (mezzosystolic click and late systolic murmur), and suspected phonocardiographic and ECG findings, also had a positive M-mode echocardiographic finding. The criteria for MVP on M-mode echocardiography were taken from the literature: descending of mitral cusp, either anterior or posterior, of at least 3 mm below the line connecting points C and D. Children and adolescents were divided into six age groups (infants, toddlers, preschool children, early school age, children in puberty, adolescents). Assuming MVP as a cause of cardiac arrhythmias, beside standard ECG we also performed holter ECG monitoring in 61 children and adolescents (29 with MVP, 32 without MVP). The results were tested with standard statistical tools (chi 2-test, Student t-test, 2 x 2 Fisher chi 2-test). MVP was found in 71 patients (10.8%, 32 males and 39 females). As regards age and sex we found lower prevalence of MVP in male children (9.7%) compared to female children (11.9%). The highest prevalence was found in early school age, more so in females (14.2 vs 13.7). The differences were not statistically significant (p > 0.05). In both sexes most frequent was endosystolic prolapse (males 59.3%, females 51.3%). Most commonly both cusps are involved in the prolapse (males 78.1%, females 66.7%). Most frequently measured descending of the cusps was 3-4.5 mm (males 56.2%, females 48.7%). Negative auscultatory finding (silent MVP) was detected in 47.8% of the patients with MVP. Most patients with diagnosed MVP had no symptoms (71.8%). The prevalence of asymptomatic MVP declines with age in both sexes. The prevalence of arrhythmias, both in standard ECG and holter ECG, is higher in patients with MVP (6.8:0%--NS and 44.6%:9.3%--p < 0.05). The influence of constitutional changes (dolichostenomelia, asthenic constitution, genua valga) on the appearance of MVP is reflected in statistically significant difference in the Rohr' index in the group of patients with MVP in relation to the healthy group (p < 0.05). The higher prevalence of headache and dizziness in the group with MVP is statistically significant (p < 0.05).

The limitations of decision trees and automatic learning in real world medical decision making.

The decision tree approach is one of the most common approaches in automatic learning and decision making. The automatic learning of decision trees and their use usually show very good results in various "theoretical" environments. But in real life it is often impossible to find the desired number of representative training objects for various reasons. The lack of possibilities to measure attribute values, high cost and complexity of such measurements, and unavailability of all attributes at the same time are the typical representatives. For this reason we decided to use the decision trees not for their primary task--the decision making--but for outlining the most important attributes. This was possible by using a well-known property of the decision trees--their knowledge representation, which can be easily understood by humans. In a delicate field of medical decision making, we cannot allow ourselves to make any inaccurate decisions and the "tips," provided by the decision trees, can be of a great assistance. Our main interest was to discover a predisposition to two forms of acidosis: the metabolic acidosis and respiratory acidosis, which can both have serious effects on child's health. We decided to construct different decision trees from a set of training objects. Instead of using a test set for evaluation of a decision tree, we asked medical experts to take a closer look at the generated trees. They examined and evaluated the decision trees branch by branch. Their comments show that trees generated from the available training set mainly have surprisingly good branches, but on the other hand, for some, no medical explanation could be found.

[Polyarteritis nodosa--cutaneous or systemic form? Possible role of bacterial superantigens in the onset of systemic disease]. / Nodozni poliarteritis--kutani ili sistemni oblik? Moguca uloga bakterijskog superantigena u nastanku sistemnih bolesti.

A boy with diagnostically unclear vasculitis was described, in which development of the disease points out the presence of cutaneous form of poliarteritis nodosa. Nevertheless, there are also signs for systemic form of the disease. His symptoms are marked cutaneous eruptions of the livedo reticularis type, recidives of erythema nodosum on the limbs and trunk, even on the cheeks, sometimes accompanied with fever, arthralgias, myalgias and cutaneous ulcera on the places of mechanical pressure (elbows), but also with monotopic ventricular premature beats and sporadic microhaematuria. Clinical development of the disease and differential diagnosis exclude other autoimmune disorders with great probability. The positive finding of cANCA has pointed out the diagnosis of vasculitis. Microscopical analysis of the skin didn't reveal any pathological changes. In the boy's disease is interposed a streptococcal infection, and maybe, tuberculosis. The paper deals with wide spectrum of diseases in differential diagnosis, and also with a possible role of bacterial superantigens in the genesis of autoimmunity.

[Diagnostic approach in patients with kidney tumors]. / Racionalizacija dijagnostickih postupaka u bolesnika s tumorima bubrega.

The basic reason for the rationalization of diagnostic procedure is the cost benefit relation and making of the diagnostic course algorithms. On the basis of the Receiver Operating Characteristic (ROC) analysis of the diagnostic procedure (excretory urography, ultrasonography, computed tomography, digital subtraction angiography) the significance ranking has been established for certain kidney tumor diagnostic procedures. Angiography has been determined to be the most precise method, but the high degree of sensitivity (98%) and accurate diagnosis probability (98%), as well as noninvasiveness and relative inexpensiveness of ultrasonographic examination procedure, all argue for ultrasonography as the cornerstone, of renal tumor diagnosis. Using conditional probabilities and the Bayes analysis the advantage of the newly suggested over the standard diagnostic course has been calculated. The study has shown a significant decrease in the number of examinations: in 60 patients 16 excretory urographies, 10 computed tomographies, and 22 digital subtraction angiographies can be spared.

[Incipient pericardial tamponade as the first symptom of systemic lupus erythematosus in 2 children]. / Prijeteca perikardijalna tamponada kao prvi simptom sistemnog eritemskog lupusa u dvoje djece.

Two patients (a boy and a girl), with systemic lupus erythematosus (SLE), in which pericarditis with threatening tamponade was an initial symptom of disease, are presented. Pericardial tamponade is very rare initial manifestation, described in only 1-3% of all the cases of SLE. The typical clinical features of tamponade are described (tachycardia, hypotension, venous congestion) and the importance of echocardiography in early diagnosis of pericarditis and pericardial tamponade is pointed out. Therefore, the echocardiography is considered as the complementary method in diagnosis of SLE.

Decision trees based on automatic learning and their use in cardiology.

Computerized information systems, especially decision support systems, have become an increasingly important role in medical applications, particularly in those where important decision must be made effectively and reliably. But the possibility of using computers in medical decision making is limited by many difficulties, including the complexity of conventional computer languages, methodologies and tools. Thus a conceptual simple decision making model with the possibility of automating learning should be used. In this paper we introduce a cardiological knowledge-based system based on the decision tree approach supporting the mitral valve prolapse determination. Prolapse is defined as the displacement of a bodily part from its normal position. The term mitral valve prolaps (PMV), therefore, implies that the mitral leaflets are displaced relative to some structure, generally taken to the mitral annulus. The implications of the PMV are the following: disturbed normal laminar blood flow, turbulence of the blood flow, injury of the chordae tendinae, the possibility of thrombus's composition, bacterial endocarditis, and finally hemodynamic changes defined as mitral insufficiency and mitral regurgitation. Uncertainty persists about how it should be diagnosed and about its clinical importance. It is our deep belief that the echocardiography enables properly trained experts armed with proper criteria to evaluate PMV almost 100%. But unfortunately, there are some problems concerned with the use of echocardiography. In that manner we have decided to start a research project aimed at finding new criteria and enabling the general practitioner to evaluate PMV using conventional methods and to select potential patients from the general population.(ABSTRACT TRUNCATED AT 250 WORDS)

[Modern diagnosis and treatment of pheochromocytoma in children]. / Suvremena dijagnoza i lijecenje feokromocitoma djece.

Pheochromocytoma is a rare tumor of chromaffin tissue of the adrenal gland that synthesizes, stores and releases cathecolamines. In 10% of the patients it arises outside the adrenal gland, and has been called paraganglioma. The following study describes our experience in the diagnostic process and treatment of pheochromocytoma in two children, a 8-year-old boy with the right ureteral paraganglioma, and a 8-year-old girl with pheo-chromocytoma of the leftsuprarenal gland. Characteristic clinical features with predominant signs of hypertension, as well as the diagnostic flow diagram, have been presented. We have analysed recent diagnostic possibilities, sensitivity of diagnostic methods (ultrasonography, CT, selective angiography and J131 MIBG scintigraphy), indications for their use, and the importance of appropriate preoperative management. The major role of the J131-metaiodobenzilguanidine scintigraphy in diagnostic assessment, especially in precise preoperative localization and postoperative verification of tumour removal, has been stressed.

[Relation between the size of the left-right shunt and pulmonary resistance in congenital heart defects]. / Odnos velicne lijevo-desnog shunta i plucne vaskularne rezistencije pri prirodenim greskama srca.

Relation between pulmonary vascular resistance (PVR) and left-to-right (L-D) shunt has been statistically tested in 87 patients with congenital heart diseases. In all patients, PVR and L-D shunt were calculated from the catheterization test results. Among 87 patients, 45 (52%) were female and 42 (48%) male children. The average age at the catheterization was 4.63 (min 6 mo, max 15 yr). Patients were divided into two groups with regard to the level of PVR. The first group consisted of patients with PVR higher than 160 dyn sec cm-5, and the second group with PVR lower than 160 dyn sec cm-5. The whole group was also divided according to VSD, and separate correlation was done for ASD with PVR below 160 dyn sec cm-5. A negative statistically significant correlation was observed between the height of PVR and size of L-D shunt among the entire group with PVR higher than 160 dyn sec cm-5, regardless of congenital heart disease (N = 29, r = -0.4676, P < 0.05). Also, a negative statistically significant correlation was found between PVR and L-D shunt in VSD, with PVR higher than 160 dyn sec cm-5 (N = 17, r = -0.669, P < 0.05). Among patients with low PVR, there was no negative statistically significant correlation between PVR and L-D shunt, neither for the whole group (N = 58, r = 0.1174, P > 0.05), nor among the patients with VSD r = -0.0133, P > 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

[Growth and development in children with congenital heart defects]. / Rast i razvoj djeteta s prirodenom greskom srca.

Growth and development have been analyzed in 222 children with congenital heart disease. Results were compared to a group of 50 control children. There was no significant difference in age and sex between the groups (p < 0.05). Thirty-three children (12.16%) had cynotic congenital heart disease (tetralogy of Fallot and transposition of the great arteries) and 189 (87.84%) acyanotic congenital heart disease (ventricular septal defect, atrial septal defect, patent ductus arteriosus and aortic and pulmonary stenosis). A statistically significant growth retardation was noted in all children with cardiac disease as compared to the control group, and it was evaluated by percentile values of body weight and height (p < 0.01). Weight retardation was more marked than retardation in body height (p < 0.01). Growth retardation was more significant in the cyanotic children that in those with acynotic heart disease (p < 0.01). Among the children with left-to-right intracardial shunt, growth retardation was found to increase proportionally with the size of the shunt and was most significant in patients with large left-to-right shunt (QP/QS > 1.80). No statistically significant difference in growth retardation was established among the children with acyanotic heart disease (p > 0.01).

Mitochondrial cardiomyopathy and scapuloperoneal spinal muscular atrophy in a child.

A 14-year old boy was admitted for signs of heart failure and scapuloperoneal muscle weakness. He fulfilled the clinical, functional and diagnostic criteria for dilated cardiomyopathy. There was also a moderate increase in pulmonary vascular resistance. The immunohistochemical examination of the heart muscle revealed a slightly positive phytohemagglutinin reaction and minimal IgM deposits without complement. The electron microscopy examination disclosed increased numbers of abnormal mitochondria disrupting the usual cell structure; the mitochondria were of various sizes with irregular and abnormal structure of the cristae. The scapuloperoneal spinal muscular atrophy was mild and diagnosed according to clinical and electromyographic findings. Light microscope examination of the skeletal muscle revealed hypotrophic fibers. This patient is presumed to have postinflammatory mitochondriopathy and is currently being managed on low-dose digitalis, diuretics and captopril.