Comparison of echocardiography with tissue Doppler imaging and magnetic resonance imaging with delayed enhancement in the assessment of children with hypertrophic cardiomyopathy.

INTRODUCTION: In children with hypertrophic cardiomyopathy (HCM) there often occurs a non-ischemic pattern of myocardial fibrosis, which could be the cause of impaired left ventricular (LV) diastolic function assessed by tissue Doppler imaging (TDI). The aim of the study was to determine the prevalence of myocardial fibrosis in children with HCM, and to evaluate its relationship with echocardiographic parameters including LV diastolic dysfunction. MATERIAL AND METHODS: Sixty-three children with HCM, mean age 12.2 ±4.5 years, underwent magnetic resonance imaging (MRI) and echocardiographic study from January 2010 to April 2014. The results of MRI, echocardiography, and TDI velocities were analyzed and compared between children with and without myocardial fibrosis. Moreover, correlations between the results of echocardiography and MRI were assessed. RESULTS: Our results showed a significant correlation between magnetic resonance and echocardiographic measurements of septal wall thickness, posterior wall thickness, LV mass and left atrial dimension. Children with myocardial fibrosis (60%) had a significantly thicker interventricular septum (21.3 vs. 1.8 mm; p < 0.0001) and larger left atrial dimension (36.7 vs. 27.8 mm; p = 0.0004) and volume index (42.0 vs. 26.6 ml/m²; p = 0.0011). Tissue Doppler imaging demonstrated significantly decreased lateral E' (9.02 vs. 13.53 cm/s; p < 0.0001) and septal E' (7.05 vs. 9.36 cm/s; p = 0.0082) velocities and a significantly increased transmitral lateral (10.34 vs. 6.68; p = 0.0091) and septal (13.1 vs. 9.8; p = 0.046) E/E' ratio in children with myocardial fibrosis. CONCLUSIONS: Myocardial fibrosis in children with hypertrophic cardiomyopathy was associated with markers for disease severity such as larger septum thickness, enlargement of the left atrium as well as impairment of left ventricular diastolic function. Tissue Doppler imaging is a helpful tool to detect the presence of left ventricular diastolic dysfunction in children with hypertrophic cardiomyopathy and myocardial fibrosis.

Predictors of Long-Term Outcome in Children with Hypertrophic Cardiomyopathy.

To date limited data are available to predict the progression to end-stage heart failure (HF) with subsequent death (non-SCD), need for heart transplantation, or sudden cardiac death (SCD) in children with hypertrophic cardiomyopathy (HCM). We aimed to determine predictors of long-term outcome in children with HCM. A total of 112 children (median 14.1, IQR 7.8-16.6 years) were followed up for the median of 6.5 years for the development of morbidity and mortality, including arrhythmic and HF-related secondary end points. HF end point included HF-related death or heart transplant, and arrhythmic end point included resuscitated cardiac arrest, appropriate ICD discharge, or SCD. Overall, 23 (21 %) patients reached the pre-defined composite primary end point. At 10-year follow-up, the event-free survival rate was 76 %. Thirteen patients (12 %) reached the secondary arrhythmic end point, and 10 patients (9 %) reached the secondary HF end point. In multivariate model, prior cardiac arrest (r = 0.658), QTc dispersion (r = 0.262), and NSVT (r = 0.217) were independent predictors of the arrhythmic secondary end point, while HF (r = 0.440), LV posterior wall thickness (r = 0.258), LA size (r = 0.389), and decreased early transmitral flow velocity (r = 0.202) were all independent predictors of the secondary HF end point. There are differences in the risk factors for SCD and for HF-related death in childhood HCM. Only prior cardiac arrest, QTc dispersion, and NSVT predicted arrhythmic outcome in patients aged <18 years. LA size, LV posterior wall thickness, and decreased early transmitral flow velocity were strong independent predictors of HF-related events.

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.

[Paediatric echocardiography in clinical practice. 2012 Recommendations of the Echocardiography Working Group of the Polish Cardiac Society]. / Echokardiografia dziecieca w praktyce klinicznej.Rekomendacje 2012 Sekcji Echokardiografii Polskiego Towarzystwa Kardiologicznego.

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.

Early screening for critical congenital heart defects in asymptomatic newborns in Mazovia province: experience of the POLKARD pulse oximetry programme 2006-2008 in Poland.

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.

Assessment of the role of copy-number variants in 150 patients with congenital heart defects.

BACKGROUND: Congenital heart defects are the most common group of major birth anomalies and one of the leading causes of infant deaths. Mendelian and chromosomal syndromes account for about 20% of congenital heart defects and in some cases are associated with other malformations, intellectual disability, and/or dysmorphic features. The remarkable conservation of genetic pathways regulating heart development in animals suggests that genetic factors can be responsible for a significantly higher percentage of cases. THE AIM: Assessment of the role of CNVs in the etiology of congenital heart defects using microarray studies. MATERIAL AND METHODS: Genome-wide array comparative genomic hybridization, targeting genes known to play an important role in heart development or responsible for abnormal cardiac phenotype was used in the study on 150 patients. In addition, we have used multiplex ligation-dependent probe amplification specific for chromosome 22q11.2 region. RESULTS: We have identified 21 copy-number variants, including 13 known causative recurrent rearrangements (12 deletions 22q11.2 and one deletion 7q11.23), three potentially pathogenic duplications (5q14.2, 15q13.3, and 22q11.2), and five variants likely benign for cardiac anomalies. We suggest that abnormal copy-number of the ARRDC3 and KLF13 genes can be responsible for heart defects. CONCLUSIONS: Our study demonstrates that array comparative genomic hybridization enables detection of clinically significant chromosomal imbalances in patients with congenital heart defects.

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.

[Symptoms, diagnosis and characteristic abnormalities in the coronary arteries in Kawasaki disease in children]. / Symptomatologia a ustalenie rozpoznania i wystepowanie zmian w tetnicach wiencowych w chorobie Kawasaki u dzieci.

INTRODUCTION: Kawasaki disease (KD) is a vasculitis of unknown etiology, the diagnosis is based upon symptoms, characteristic abnormalities in the coronary arteries are the most important complications. AIM: Establishment of a data base of the patients with KD, assignation of symptoms, and frequency of abnormalities in the coronary arteries according to the clinical manifestations and time of the diagnosis. MATERIALS AND METHODS: The patients'data from selected centres of paediatric cardiology were put into a questionnaire form in the Internet. The data of 120 children were placed into data base in the year 2007, of which we analyzed 112 cases. RESULTS: The most frequent symptoms were fever and abnormalities of oral mucosa. Complete KD was diagnosed in 73%, incomplete in 27% (median of age 28 and 19 months respectively). The disease was diagnosed before its 10th day in 60% with similar frequency of both clinical manifestations, abnormalities in the coronary arteries were observed in 59% with complete and in 44% with incomplete KD clinical sings. The disease lasting longer than 70 days was diagnosed in 40% with similar frequency in both groups of clinical manifestations, abnormalities in the coronary arteries were in 79% with complete and in 75% with incomplete KD. In all cases, abnormalities in coronary arteries were in 67% with complete and in 57% with incomplete KD. CONCLUSIONS: The most frequent symptoms of Kawasaki disease are fever and abnormalities in the oral mucosa. Incomplete KD is more frequent in younger children. Despite complete KD in 40% of cases diagnosis was delayed. Abnormalities in the coronary arteries were more frequent in patients in whom KD was diagnosed only after the 10th day of the illness, but with the same frequency in both clinical manifestations. Diagnosis of incomplete KD before the 10th day of the illness was associated with lower risk of incidence of abnormalities in the coronary arteries.

[Role of the AV node and adrenergic stimulation in initiation of fascicular and atrioventricular nodal tachycardias--a case report]. / Rola wezla przedsionkowo-komorowego oraz stymulacji adrenergicznej w zawiazaniu czestoskurczu peczkowego i wezlowego--opis przypadku.

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.

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.

Reversal of tacrolimus-related hypertrophic cardiomyopathy after conversion to rapamycin in a pediatric liver transplant recipient.

Tacrolimus (Tac)-related hypertrophic cardiomyopathy (HCM) has been reported to be an unusual but serious complication affecting pediatric patients after solid organ transplantation. Herein, we present a case of young liver transplant recipient with Tac-induced HCM, treated by discontinuation of Tac followed by conversion to rapamycin (Rap). Our case report points out the potential but rather low risk of HCM during Tac immunosuppression in pediatric liver transplants and demonstrates that replacement of calcineurin inhibitors with mammalian target of Rap (mTOR) inhibitors may be an efficacious therapeutic tool to effect regression of established cardiac hypertrophy.

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.

[Symptomatic recurrent bradycardia--always an indication for pacemaker implantation? The role of RF ablation for incessant focal tachycardia from AV node region and advanced AV nodal block]. / Objawowa bradykardia--czy zawsze jest wskazaniem do wszczepienia ukladu stymulujacego? Rola ablacji.

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.

[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.