Coronary steal phenomenon following right ventricle decompression and revascularization of atretic left coronary ostium: case report.

BACKGROUND: Coronary steal phenomenon and myocardial ischemia is a complication following decompression of a hypertensive right ventricle in patients with left coronary-cameral fistulae. CASE PRESENTATION: We present a 12-year-old girl with a complex heart defect successfully operated on using a hybrid surgical-interventional approach to decompress the ventricle, embolize the fistula and reconstruct the atretic left coronary ostium. CONCLUSIONS: A novel hybrid strategy is the best solution for coronary-cameral fistulas reliant on high ventricular pressure at high risk for coronary steal phenomenon.

[Origin of right pulmonary artery branch from descending aorta. Case report of the successful treatment]. / Nieprawidlowe odejscie galezi prawej tetnicy plucnej od aorty zstepujacej. Opis pomyslnego przebiegu leczenia.

This report describes an extremely rare case involving anomalous origin of part of the right pulmonary branch from the thoracic aorta in a 3 month-old boy and its successful repair.

Percutaneous balloon valvuloplasty for the treatment of pulmonary valve stenosis in children - a single centre experience.

BACKGROUND: Isolated pulmonary valve stenosis (PVS) is a common heart defect (6-9%); the preferred treatment is balloon pulmonary valvuloplasty (BPV). AIM: To assess BPV results in children with isolated PVS treated between 1988 and 2004, with a mean follow-up of 6.1 +/- 3.4 years. METHODS: The studied group consisted of 137 children (76 males and 61 females), aged 5.4 +/- 4.8 years. The diagnosis was based on physical examination, ECG, chest radiograph, echo, haemodynamic and angiocardiographic studies. The patients were divided into three groups, depending on the ratio of right ventricular systolic (RVSP) to systemic pressure (SP): I (n = 58) - RVSP < or = 75% of SP, II (n = 41) - RVSP = 76-100% of SP, III (n = 38) - RVSP > 100% of SP. In 7.3% of patients, dysplastic pulmonary valve (DPV) was seen. The balloon diameter to pulmonary valve annulus ratio was 1.29 +/- 0.1, and 1.42 +/- 0.1 in DPV children. RESULTS: Immediately post-BPV, the patients showed significantly (p < 0.001) decreased pressure gradient across PVS (I: 49.3 +/- 11.1 - 12.5 +/- 7.6, II: 75.6 +/- 12.3 - 17.0 +/- 13.0, III: 117.3 +/- 28 - 17.9 +/- 15.5 mmHg), decreased RVSP (I: 65.3 +/- 10.3 - 28.6 +/- 7.6, II: 91.7 +/- 11.6 - 35.0 +/- 14, III: 133.0 +/- 27.3 - 38.4 +/- 19.2 mmHg) and end-diastolic RV pressure (I: 6.2 +/- 3.0 - 5.6 +/- 7.6, II: 6.3 +/- 3.0 - 5.5 +/- 2.9, III: 8.5 +/- 3.0 - 7.2 +/- 2.3 mmHg), non-significant (p > 0.05) increase in pulmonary artery pressure in group I (15.8 +/- 1.1 - 16.8 +/- 0.9 mmHg) and II (15.8 +/- 1.2 - 17.8 +/- 1.3 mmHg) and a significant (p < 0.003) rise in group III (14.5 +/- 1.3 - 19.4 +/- 2.1 mmHg). The procedure was ineffective only in one (2.4%) child in group II, who required surgery. Complications were seen in five (3.6%) patients, including one case of a balloon being lodged in the iliac vein (surgical repair). Follow-up echo showed similar to immediate post BPV values of pressure gradients across PVS. Pre-BPV subpulmonary stenosis was seen in 5.1%, post-BPV - 15.3%, and end of follow-up - only 3.6% of children, mainly from group III. Pre-BPV tricuspid insufficiency > IIo was noted in 8.8%, significantly more frequently in group III; while in late follow-up, it was seen in 7.2%, e.g. twice as often in group III vs. groups I and II. Pulmonary regurgitation > IIo increased from 2.2% before BPV to 25.5%, i.e. 17.2%, 24.4% and 39.5%, respectively in groups I-III. Restenosis was observed in eight (5.8%) patients (group I - 1, III - 7), of whom five had re-BPV, two were operated on and one was disqualified due to insignificant restenosis. CONCLUSIONS: These long-term follow-up data confirm efficacy and safety of BPV performed in children with isolated PVS.

[Cardiac syncope in children]. / Omdlenia kardiogenne u dzieci.

INTRODUCTION: Syncope are serious problems in paediatrics. Approximately 15% of children and adolescents at the age of 8-18 years experience at least one episode of syncope. Neurocardiogenic syncope are the most common (60-80%). Cardiogenic syncope are rare (6%), although they require special attention due to their background. AIM OF THE STUDY: Evaluation of incidence, causes, methods of treatment and prognosis in cardiogenic syncope in children and adolescents. MATERIAL AND METHODS: Retrospective analysis was made in 18 patients with syncope and different pathology of cardiovascular system. Patients were chosen among 766 children treated in the University Hospital for Children in Cracow from January 2005 to June 2006 due to syncope or pre-syncope. The patients were divided into 2 groups depending on the main cause of syncope: Gr I n = 6 due to arrhythmias without structural heart disorders and Gr II n = 12 due to heart diseases or defects. In all patients physical exam and additional test were analysied carefully (ECG, Holter study, chest X ray, ECHO, angio-TK in 1 patient, and catheterisation with angiography in 3 children). RESULTS: Cardiogenic syncope appeared more frequently in children with structural abnormalities in the heart (Gr II--12 pts/ 66%), rather than in children with arrhythmias (Gr I--6 pts / 33%). Family history was positive in both groups in 17%. Median age at the moment of first episode was 10.3 y +/- 5.0 in Gr I and 12.8 y +/- 3.0 in Gr II. Syncope in Gr II were frequently (33%) initiated by an effort or stress. In Gr I physical examination did not reveal serious symptoms except for irregularity of heart rate, and general condition of children was good (NYHA I). In 5 pts (58%) in Gr II heart murmur and prominent impulse of the left ventricle were found. Symptoms of congestive heart failure were observed in 4 pts (33%) and their condition was poor (III/IV class according to NYHA). In ECG and/or Holter study in Gr I arrhythmias and/or long QTc were found. Pharmacotherapy was sufficient in treatment of serious arrhythmias and prophylaxy of syncope. In Gr II in most cases hypertrorepolarization abnormalities were found. In these children treatment was multidirectional and included both antiarrhythmic drugs, implantation of cardioverter defibrillator (ICD), operation and even heart transplantation. CONCLUSIONS: Cardiogenic syncope are rare causes of loss of consciousness and indicate on serious pathology in the cardiovascular system. Heart structural diseases or defects influence severity on clinical course, complex treatment and serious prognosis while in children with arrhythmias pharmacotherapy is effective and prognosis is usually good.

[Results of cerebral angiography performed after neuroradiological indications in children with CNS diseases]. / Wyniki angiografii mózgowej wykonywanej u dzieci z chorobami OUN ze wskazan neuroradiologicznych.

INTRODUCTION: Despite of the constant progress in the imaging of vessels, cerebral angiography characterises with the best specificity and sensitivity in detection of arterial, arteriovenous malformations and aneurysms. THE AIM: The aim of this study was to compare the results of cerebral angiography and other neuro-radiologic methods in cases suggesting the probability of vascular malformations in children with neurologic syndroms. MATHERIAL AND MEHTODS: In the year 2006 cerebral angiography was performed in 20 children since it was advised after radiological examinations in children hospitalized in the Department of Pediatric Neurology UJ CM in Krakó w. The group of children aged 3-17 years (mean 11 years) consisted of 11 girls and 9 boys. The commonest clinical diagnoses were epilepsy, less common cranial neuropathies, migraine, hemiparesis and agiomatosis of skin. Before cerebral angiography was indicated, other radiological examinations were performed, within CT in 7 cases, CTA in one, MRI in 17 children and MRA in 5. In some children other examinations were also performed, within transcranial doppler evaluation of cerebral blood flow, EEG, video EEG, lumbar puncture and ultrasound eye evaluation. RESULTS: The pathological changes were detected in 5 CT examinations, 1 CTA, 7 MRI and in 4 MRA, suggesting cerebral vascular malformations. Cerebral angiography excluded vascular malformations in 15 children. In 5 children angiography confirmed however the presence of vascular pathology. In 2 of them arterial hypoplasia in the posterior circulation was detected, in 2 cases supratentorial artery hypoplasia and a single venous cerebellar malformation. Children were diagnosed with migraine with aura in two cases, within one with paresis of the right leg and single cases with skin angiomatosis, syncope and sleep myoclonus. CONCLUSIONS: Cerebral angiography allowed to exclude vascular pathology in 75% of children, in whom previous neuroradiological examinations suggested such pathology. It was important for their quality of life. In 25% of patients angiography confirmed results of previous neuroradiological tests detecting vascular pathology. MR with contrast medium is more sensitive than angiography in detection of venous angiomas.

[Echocardiographic assessment of early and midterm results of transcatheter closure of a narrow patent ductus arteriosus in children]. / Echokardiograficzna ocena wczesnych i srednio odleglych wyników interwencyjnego zamykania droznego przewodu tetniczego u dzieci.

This paper presents the echocardiographic assessment of early and midterm results of transcatheter closure of a narrow patent ductus arteriosus (PDA) in children using Jackson and Gianturco coils. The preliminary criterion for qualifying patients for the procedure was the PDA diameter (< or = 4 mm) at the narrowest site, as assessed echocardiographically. The criterion was met by 22 of 116 children with PDA treated at the Department of Pediatric Cardiology, Faculty of Medicine, Jagiellonian University, between January 1, 1996 and December 31, 2000. Angiography revealed, that in one case, the PDA diameter was larger (6.5 mm) and the patient required two Gianturco coils. Of 22 patients (8 males and 14 females) aged 2.7-16 years (x = 6.5 +/- 3.8), the procedure was successful in 21 children (95.5%). One patient with PDA was referred to the surgical department due to complications (the coil was displaced to the periphery of the left pulmonary artery and required removal by an Amplatzer device). The analysis of the follow-up echocardiography performed within 48 hours after the procedure and 1, 6, 12, 24 and 48 months later, revealed immediate total PDA closure in 29% of children. In 33% of patients, the PDA closed within 24 months. One child did not report to follow-up, however, the others are asymptomatic and show residual negligible shunts. The authors determined that the incidence and time of closure of residual shunts is dependent on the magnitude of the left-to-right shunt before the procedure.