The diagnosis, clinical course and follow-up of children with cardiac tumours - a single-centre experience.

BACKGROUND: Rhabdomyoma is the most frequent primary cardiac tumour in children (about 50% of all cardiac neoplasms in this population). Fibroma, myxoma, teratoma and haemangioma are less frequent. AIM: To investigate the clinical presentation, diagnosis and follow-up of children diagnosed with cardiac tumours in our department between 1993 and 2008. METHODS: In the 15-year review we found 9 cases of cardiac tumours, confirmed in echo scan in every case and pathomorphologically in 5 out of 9 cases. RESULTS: Cardiac tumours were found in six boys and three girls, usually in the neonatal period. Cardiac murmur was the most common clinical symptom (4 cases). Two children were symptom-free. Out of the remaining children, two had circulatory failure and one had arrhythmia. Five children were operated on: three cases of rhabdomyoma, one fibroma and one teratoma. In 3 children who did not undergo surgery, the most probable diagnosis was rhabdomyoma. The follow-up (possible in 8 out of 9 cases, mean 7 years) showed that six children developed regularly and in two cases neurological abnormalities appeared. CONCLUSIONS: In this series, primary cardiac tumours presented as murmurs or circulatory failure. Most children needed surgery. In most cases, pathomorphology revealed rhabdomyoma. Follow-up showed regular development in six out of nine cases.

N-terminal prohormone brain natriuretic peptide-proBNP levels in ventricular arrhythmias in children.

BACKGROUND: Ventricular arrhythmias are the most common consequences of structural and functional heart diseases, but cases with no evident pathology are also observed. A parameter indicating asymptomatic circulatory failure could support decisions related to possible treatment of ventricular arrhythmias. HYPOTHESIS: The study objective was the evaluation of N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels in children with ventricular arrhythmias and an attempt to determine if this parameter may be used for diagnosis and prognosis of ventricular arrhythmias. MATERIAL AND METHODS: The study population was comprised of 36 children age 5 to 17.5 years old with idiopathic ventricular arrhythmias (Group B) graded mild or potentially malignant; 29 patients with mild ventricular arrhythmias were included into Group B1; and 7 patients with potentially malignant cases into Group B2. In all the patients, NT-proBNP assays were performed. RESULTS: The NT-proBNP levels in Groups B, B1, B2 and the control group (Group K) were as follows: 41.5 +/- 15.1 pg/mL, 35.5 +/- 18.5 pg/mL, 66.3 +/- 24.9 pg/mL and 31.5 +/- 15.1 pg/mL, respectively. Between the groups with and without arrhythmias (Group B vs Group K), no statistically significant differences in NT-proBNP levels were found. However, markedly higher NT-proBNP levels were shown in the children with potentially malignant arrhythmias (Group B2) compared to the patients with mild arrhythmias (B1) and the control group (Group K). CONCLUSIONS: The level of NT-proBNP increases with the severity of ventricular arrhythmia. NT-proBNP assays can be helpful for diagnosing and grading the severity of ventricular arrhythmias.

[Implantation of cardiac pacemaker in children, author's experiences]. / Implantacja ukladu stymulujacego serca u dzieci--doswiadczenia wlasne.

Cardiac pacemaker was implanted as a therapeutic method for children with atrio-ventricular block, sick sinus syndrome, LQT syndrome, cardio-depressive syncope. Twenty six children (aged 1-17) underwent pacemaker implantation or exchange of pacemaker and electrode. In nineteen cases cardiac peacemaker was implanted in the endocardial system; in the next seven cases epicardial system was required. The difference between the implantations depended on patient's age and the character of the disease. The single chamber peacemaker was implanted in twenty children, dual-chamber in six patients. There were no visible complications in follow up observation and constant heart stimulation can be considered as a safe way of children's severe bradycardia treatment.

[Late recognition of Kawasaki disease--difficulties in diagnosis]. / Pózne rozpoznanie choroby Kawasaki--trudnosci diagnostyczne.

Authors describe the case of 4 years old girl with Down syndrome, who was operated due to common atrio-ventricular canal and persistent Botalli's duct. Intermittent total atrio-ventricular block (without significant bradycardia) has been observed one year later and considered as a late postoperative block requiring no treatment. Kawasaki disease was diagnosed because of the presence of 4 out of 6 leading symptoms appearing in typical chronology (fever, mouth and throat inflammation, conjunctivitis, erythema with subsequent desquamation of skin on palms and feet). ECG revealed total atrio-ventricular block, however with significant bradycardia. ECHO showed aneurysms in both coronary arteries. Standard treatment of Kawasaki disease was administered (immunoglobulins, acetylsalicylic acid) and orciprenalin due to described cardiac block. Pacemaker was implanted because of bradycardia. The literature review showed that the treatment with immunoglobulins and aspirin can reduce the risk of coronary aneurysms development. On the other hand, identification of patients at risk coronary aneurysms development is not possible on the ground of biochemical blood analysis and physical signs. Thus, all the patients stricken should be treated with described above costly drugs (immunoglobulins). Finally, the algorithm of procedures in patients with coronary aneurysms was presented.