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Background: Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfavourable prognosis., Aim: To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004–2013., Methods: The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH — n = 23 (42%), PAH associated with systemic-to-pulmonary shunts — n = 17 (31%), and PAH after corrective cardiac surgery — n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy., Results: During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03)., Conclusions: Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.

Background: Cardiovascular magnetic resonance (CMR) imaging is a clinically proven and reliable diagnostic method for the assessment of morphology, function, and characteristics of myocardial tissue in patients with myocardial diseases. The use of gadolinium contrast agents has created new diagnostic possibilities for tissue characterisation in patients with suspected or known cardiomyopathy, myocarditis, and cardiac tumours., Aim: To evaluate the usefulness of CMR in the diagnostic process in children with myocardial diseases and to compare the results of CMR and other non-invasive cardiovascular methods, including echocardiography., Methods: The study included 112 children, with an average age of 12 ± 4.64 years, with various forms of myocardial disease: 63 children with hypertrophic cardiomyopathy (HCM), 9 with suspected myocarditis, 5 with history of myocarditis, 4 with dilated cardiomyopathy (DCM), 9 with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC), 6 with left ventricular non-compaction cardiomyopathy (LVNC), 9 with suspected restrictive cardiomyopathy (RCM) to be differentiated with constrictive pericarditis (CP), and 7 with cardiac tumours., Results: CMR confirmed the echocardiographic diagnosis of HCM in 92% of children and ruled it out in 8%, and in three children apical hypertrophy was found. CMR revealed the presence of myocardial fibrosis in 60% of patients with HCM. In 33% of children with clinically suspected myocarditis CMR confirmed this diagnosis, while in 44% of them DCM was recognised. Of the five children with a history of myocarditis, in one patient CMR performed 13 years after myocarditis revealed features of post-inflammatory DCM. In 75% of patients with the echocardiographic diagnosis of post-inflammatory DCM the result of CMR was consistent. CMR ruled out the presence of ARVC in 89% of children. Echocardiographic and CMR diagnosis of LVNC was consistent in 67% of children. CMR confirmed the clinical diagnosis of RCM in 63% of patients, and in one patient CP was recognised. CMR confirmed the presence of cardiac tumour in 57% of children and excluded it in 43% of patients., Conclusions: CMR is increasingly recognised as an important tool in the investigation of myocardial disease and should be part of routine clinical work-up. CMR provides an additional diagnostic technique to assess the presence or exclusion of an active myocarditis. In children with clinical and echocardiographic suspicion of LVNC, ARVC, RCM, CP, and cardiac tumours CMR can conclusively confirm the presence of the disease.

Echocardiography has become the primary imaging tool in the diagnosis and assessment of cardiological disorders in children. The purposes of this paper are to describe indications for paediatric echocardiography, define optimal instrumentation and laboratory setup for paediatric echocardiographic examinations and establish a baseline list of recommended measurements to be performed in a complete pediatric echocardiogram.

Background: Early diagnosis of critical congenital heart defects (CCHD) may be missed both during prenatal echocardiography and the short stay in the neonatal nursery, leading to circulatory collapse or death of the newborn before readmission to hospital., Aim: To assess the usefulness of pulse oximetry as a screening test in early diagnosis of CCHD in newborns., Methods: A prospective screening pulse oximetry test was conducted in 51 neonatal units in the Mazovia province of Poland as part of the POLKARD 2006-2008 programme between 16 January, 2007 and 31 January, 2008. Newborns with no circulatory symptoms or coexisting diseases, and no prenatal diagnosis, were enrolled. The test was performed between the 2(nd) and 24(th) hours of life in stable newborns. A double arterial oxygen saturation (SpO(2)) reading < 95% on a lower extremity led to cardiovascular evaluation and echocardiography., Results: From a population of 52,993 newborns (14.2% of births in Poland), a group of 51,698 asymptomatic infants was isolated. CCHD was diagnosed solely by pulse oximetry in 15 newborns, which constituted 18.3% of all CCHD; 14 (0.026%) false positives were obtained and there were four false negative results. The sensitivity of the test was 78.9% and specificity 99.9%. The positive predictive value was 51.7% and negative 99.9%., Conclusions: Pulse oximetry fulfilling the screening test criteria, performed on a large population of newborns in Poland, proved useful in supporting prenatal diagnostics and postnatal physical examination in the early detection of initially asymptomatic CCHD. Good sensitivity and specificity results of the pulse oximetry test have allowed it to be recommended for use in neonatal units nationwide.

Background and Aim: The aim of this study was to investigate whether scintigraphy with (99m)Tc-Anti-Granulocyte antibody is useful for diagnosis and follow-up in children with myocarditis, and to determine its correlation with endomyocardial biopsy (EMB) and clinical features., Methods: A total of 11 children, mean age 13 years and presenting with symptoms of myocarditis, were evaluated at the time of initial presentation and 6, 12 and 24 months after the first study. In all patients, myocardial scintigraphy was performed with estimation of antigranulocyte antibody uptake. EMB was done in 10 patients at the time of initial presentation and in 8 patients after 6 months., Results: In 10 (91%) patients, positive antigranulocyte uptake was observed, with EMB confirming myocarditis in 8 children. In scintigraphy after 6 months, positive uptake was found in 9 (82%) patients, with EMB performed in 8 patients showing persistent myocarditis; after 12 months, scintigraphy indicated positive uptake in 7 (64%), and after 24 months only in 4 (36%) patients., Conclusions: 1. In 80% of patients with positive scintigraphy results, biopsy-proven myocarditis was observed. 2. The positive antigranulocyte uptake correlated with clinical features at diagnosis and in follow-up. 3. The control scintigraphy performed in follow-up after 6, 12, and 24 months allowed the evaluation of resolved or persistent myocarditis. 4. Myocardial scintigraphy results indicate that the inflammatory process in the myocardium decreases significantly after 12 months from the onset of the disease. 5. Scintigraphy with (99m)Tc-Anti-Granulocyte antibody seems to be a useful diagnostic method in myocarditis, but further studies are needed to establish its sensitivity and specificity.

Fascicular tachycardia is an uncommon form of left ventricular tachycardia in young patients with normal heart. Ventriculo-atrial conduction during VT is usually absent. Retrograde conduction was observed in a 14-year old boy with left posterior fascicular VT (LPF-VT) triggered by exercise. During isoproterenol infusion, atrial stimulation induced a cascade of arrhythmias--echo, pair or runs of AVNRT and fascicular tachycardia triggered by fascicular beats. Also, during infusion LPF-VT was initiated spontaneously. After successful ablation of VT, sustained typical AVNRT was inducible. Finally, ablation of slow pathway of AV node was performed. After ablation,no arrhythmia was inducible following isoproterenol and exercise.

We present a case of a twelve-year-old girl with Ebstein's anomaly, atrial septal defect and two forms of tachycardia. From the first year the patient suffered from episodes of tachycardia. A few months prior to admission, episodes of palpitations became more frequent, periodically incessant and difficult to interrupt, despite amiodarone treatment. During the tachycardia the patient deteriorated with severe cyanosis, hypotension and often with syncope. During sinus rhythm ECG showed RA enlargement and preexcitation. During the documented tachycardia with rate 160-170/min, the ECG showed right bundle branch block (QRS=160 ms) with right axis deviation. An echocardiogram demonstrated dislodgment of septal tricuspid leaflet 30 mm with a high degree of right ventricle atrialisation, moderate tricuspid insufficiency, and ASD with bidirectional shunt. During the EP study two forms of tachycardia were induced: orthodromic atrioventricular reentrant tachycardia, and atrioventricular nodal reentry tachycardia. Both tachycardias were successfully ablated. Four months later the patient underwent TV replacement with aortic homograft, Glenn anastomosis and ASD closure with fenestration. During the next 18 months, the patient was in better general condition, but still was cyanotic especially during exercise (SAT 76%). An Amplatzer occluder was implanted on the 'residual' atrial septal defect. During the 24-month follow-up period, the patients has been in a good general condition, showing good exercise tolerance with no cyanosis or arrhythmia.

A case of a 16-year-old girl with left sided accessory pathway is presented. Following adenosine-induced termination of atrio-ventricular reentrant tachycardia the patient developed polymorphic ventricular tachycardia followed by preexcited atrial fibrillation with very rapid ventricular response and syncope. Arrhythmia was terminated by amiodarone infusion. Potential complications after adenosine injection are discussed.

We present a case of a 17-year-old patient in whom recurrent bradycardia and syncope episodes were present. During the pathological bradycardia incessant runs of irregular tachycardia from the region of atrial extension of AV node were observed. The bradycardia runs were due to advanced functional AV nodal block. The patient was qualified for pacemaker implantation and antiarrhythmic treatment by his GP. Successful ablation of atrial tachycardia revealed that SA and AV node properties were in the normal range; therefore no pacemaker implantation was needed.