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.

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.

[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.

[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.

[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.

[Electrocardiographic changes in children with surgery for defects of the atrial septum]. / Elektrokardiografske promjene u djece operirane zbog defekta septuma atrija.

Electrocardiograms obtained prior to surgery, after surgery and in the period between 6 months and 2 years following the surgery were analyzed in 44 children operated because of atrial septal defect. The following electrocardiographic parameters were selected: the P wave, PQ interval, QRS complex and right bundle branch block The P wave narrowing developed gradually, i.e. not earlier than the follow-up period, and it was statistically significant (p = 0.001). The duration of the PQ interval decreased significantly right after the operation (p = 0.001). No changes were seen in the width of the QRS complex neither following the surgical procedure nor in the later postoperative period. The number of children with right bundle branch block decreased significantly immediately after the surgery (p = 0.0108). Willson's right bundle branch block disappeared gradually after operation (p = 0.0282).

The influence of pulmonary artery banding on outcome after the Fontan operation.

Thirty-eight patients were selected from a total of 120 patients who underwent the Fontan operation between 1974 and 1988. They were classified into two groups. Group 1 consisted of 18 patients with previous pulmonary artery banding at a mean age of 7 months (2 days to 59 months), and group 2 comprised 20 patients with native pulmonary stenosis. In group 1, 10 children had tricuspid atresia (seven with normally connected and three with transposed great arteries), six had double-inlet ventricle, and two had complex heart malformations. Group 2 consisted of 12 patients with tricuspid atresia and normally connected great arteries, six with double-inlet ventricle, and two with complex malformations. The following clinical and hemodynamic parameters at cardiac catheterization and cineangiocardiography were determined in both groups before the Fontan operation: age and body surface area, hemoglobin concentration and hematocrit value, atrial and pulmonary artery pressures, end-diastolic pressure of the systemic ventricle, arterial oxygen saturation, pulmonary/systemic flow ratio, end-diastolic volume, ejection fraction and mass of the systemic ventricle, cardiac index, and Nakata index. After the Fontan operation in all patients, the presence or absence of pericardial and pleural effusions, ascites, protein-losing enteropathy, and liver and kidney dysfunction was assessed and the clinical status was classified according to New York Heart Association criteria. All preoperative and postoperative parameters were tested for differences between the two groups, and they were compared with normal values. Hematocrit value was higher in group 2 than in group 1 (57.8% versus 53.1%; p less than 0.05). Ventricular mass index was increased in group 1 when compared with group 2 (125.8 gm/m2 versus 87 gm/m2; p less than 0.05). Severe pericardial effusions in the early postoperative period were significantly more frequent in group 1 and were particularly prevalent in the subgroup with long-standing pulmonary artery banding (p less than 0.01). Subaortic stenosis was observed more frequently in group 1. The remaining parameters were not statistically different between the two groups. We conclude that the significant increment in ventricular mass after pulmonary artery banding may represent a risk for unfavorable outcome after the Fontan operation, which increases with time. Therefore, long-standing pulmonary artery banding as a palliative procedure for candidates for the Fontan operation should be avoided.