Prognosis in children with pulmonary arterial hypertension: 10-year single-centre experience.

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 2004­2013. 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.

The usefulness of cardiovascular magnetic resonance imaging in children with myocardial diseases.

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.

The usefulness of scintigraphy with (99m)Tc-anti-granulocyte antibody for diagnosis and follow-up in children with myocarditis.

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.

Antiepileptic treatment before the onset of seizures reduces epilepsy severity and risk of mental retardation in infants with tuberous sclerosis complex.

BACKGROUND: Epilepsy appears in 70-80% of patients with tuberous sclerosis complex, most commonly in the first year of age. Early manifestation of epilepsy is associated with drug-resistant epilepsy and mental retardation in more than 80% of patients. Clinical epileptic seizures are preceded by deterioration of EEG recording thus infants with high risk of epilepsy can be identified. AIMS: We hypothesized that preventative antiepileptic treatment of infants with multifocal activity on EEG might lower the incidence of drug-resistant epilepsy and mental retardation. METHODS: Forty-five infants with early diagnosis of tuberous sclerosis complex were included in the open-label study. They were divided in two groups: standard (n=31) and preventative one (n=14). In standard group the antiepileptic treatment was launched early, but after the onset of seizures. In preventative group medication was commenced when active epileptic discharges were seen on EEG, but before the onset of clinical seizures. Children were followed till the end of 2 years of age. RESULTS: At 24 months of age mental retardation was significantly more frequent and severe in "standard" vs "preventative" group (48% vs 14%; p=0.031; mean IQ score 68.7 vs 92.3; p<0.05). The "preventative" group was characterized by higher ratio of seizure-free patients (93% vs 35%; p=0.004), lower incidence of drug-resistant epilepsy (7% vs 42%; p=0.021) and lower number of patients requiring polytherapy (21% vs 55%; 0.039) than the "standard group. CONCLUSIONS: Preventative antiepileptic treatment of infants with tuberous sclerosis complex and high risk of epilepsy markedly improves their neurodevelopmental outcome and reduces the incidence of drug-resistant seizures.

Chromosome 22q11.2 microdeletion in children with conotruncal heart defects: frequency, associated cardiovascular anomalies, and outcome following cardiac surgery.

The association of conotruncal heart defects with 22q11.2 chromosomal microdeletions is well established. However, it is not clear whether particular types of conotruncal malformations or additional cardiovascular anomalies are associated with microdeletions. In addition, cardiac surgery outcome in children with conotruncal defects and del22q11.2 is not well described. We prospectively enrolled 214 children with conotruncal defects: 126 with tetralogy of Fallot (TOF), 18 with pulmonary atresia-ventricular septal defect (PA-VSD), 15 with truncus arteriosus communis (TAC) type I, one with interrupted aortic arch (IAA) type B, and 54 with the transposition of great arteries, who were consecutively hospitalized at the Pediatric Cardiology Department between 2003 and 2005. 22q11.2 microdeletion was identified by fluorescence in situ hybridization. The postoperative course following cardiac surgery was compared in patients with TOF and its more severe form, PA-VSD, with/without del22q11.2 (groups A and B) and TAC with/without del22q11.2 (groups C and D). In 15 of 214 patients, 22q11.2 microdeletion was diagnosed (in 11 with TOF/PA-VSD, in three with TAC, in one with IAA type B). In patients with TOF/PA-VSD and microdeletion anatomic features that were significantly associated with 22q11.2, deletion included right aortic arch (p = 0.018), aberrant right subclavian artery (p < 0.001), and major aortopulmonary collateral arteries (p = 0.016). A complicated postoperative course was more frequent and mortality was higher in patients with conotruncal defects and with/without microdeletion. We conclude that additional cardiovascular anomalies are significantly more frequent in children with 22q11.2 microdeletion and TOF/PA-VSD. Children with conotruncal heart defects and 22q11.2 microdeletion more frequently experienced complicated postoperative course after cardiac surgery.

[Atrioventricular nodal and atrioventricular reentrant tachycardia in a patient with Ebsteinanomaly--the role of percutaneous RF ablation, surgery and Amplatzer septal occluder]. / Dwie postacie czestoskurczu nawrotnego u chorej z zespolem Ebsteina przyczyna ekwiwalentów zespolu MAS.Rola przezskórnej ablacji, operacji i zapinki Amplatza.

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.

Risk factors for cardiac arrhythmias in children with congenital heart disease after surgical intervention in the early postoperative period.

OBJECTIVE: Early postoperative arrhythmias are a recognized complication of pediatric cardiac surgery. METHODS: Diagnosis and treatment of early postoperative arrhythmias were prospectively analyzed in 402 consecutive patients aged 1 day to 18 years (mean 29.5 months) who underwent operation between January and December 2005 at our institute. All children were admitted to the intensive care unit, and continuous electrocardiogram monitoring was performed. Risk factors, such as age, weight, Aristotle Basic Score, cardiopulmonary bypass time, aortic crossclamp time, and use of deep hypothermia and circulatory arrest, were compared. Statistical analysis using the Student t test, Mann-Whitney U test, or Fisher exact test was performed. Multivariate stepwise logistic regression was used to assess the risk factors of postoperative arrhythmias. RESULTS: Arrhythmias occurred in 57 of 402 patients (14.2%). The most common types of arrhythmia were junctional ectopic tachycardia (21), supraventricular tachycardia (15), and arteriovenous block (6). Risk factors for arrhythmias, such as lower age (P = .0041*), lower body weight (P = .000001*), higher Aristotle Basic Score (P = .000001*), longer cardiopulmonary bypass time (P = .000001*), aortic crossclamp time (P = .000001*), and use of deep hypothermia and circulatory arrest (P = .0188*), were identified in a univariate analysis. In the multivariate stepwise logistic regression, only higher Aristotle Basic Score was statistically significant (P = .000003*) compared with weight (P = .62) and age (P = .40); in the cardiopulmonary bypass group, only longer aortic crossclamp time was statistically significant (P = .007*). CONCLUSION: Lower age, lower body weight, higher Aristotle Basic Score, longer cardiopulmonary bypass time, aortic crossclamp time, and use of deep hypothermia and circulatory arrest are the risk factors for postoperative arrhythmias. Junctional ectopic tachycardia and supraventricular tachycardia were the most common postoperative arrhythmias.

[Morgagni-Adams-Stokes after adenosine injection in a patient with WPW syndrome--a case report]. / Zespól Morgagni-Adams-Stockesa po wstrzyknieciu adenozyny u pacjentki z zespolem WPW.

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.