Hybrid Stent Implantation Into Right Ventricular Outflow Tract in a Newborn With Tetralogy of Fallot.

A case is presented of a newborn with tetralogy of Fallot and aortic coarctation. Progressive right ventricular outflow tract (RVOT) obstruction required urgent surgical therapy. Coronary artery crossing the outflow tract made ventriculotomy impossible. Hybrid RVOT stent implantation was performed, providing effective pulmonary flow and enabling postponing of corrective jumping graft implantation.

Atrial Septum Perforation Via Levoatriocardinal Vein in a Newborn With Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome with intact atrial septum has high perioperative mortality. An interventional septum perforation from the right atrium may be technically challenging in newborns with high risk of tamponade. However, neonates with a levoatriocardinal vein provide an alternative approach for septostomy from the left atrium side. (Level of Difficulty: Advanced.).

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.

Catheter-Guided Dissolution of a Giant Thrombus in the Left Ventricle in a Newborn.

A newborn with pulmonary hypertension due to the premature fetal arterial duct occlusion was diagnosed with a giant left ventricle thrombus. Cardiopulmonary compromise required multidrug therapy with vasopressors infusions, high-frequency oscillation, and nitric oxide. Alteplase infusion through a guiding catheter into the left atrium dissoluted the clot without sequelae. (Level of Difficulty: Advanced.).

Recoarctation of the aorta after the Norwood procedure may be treated during the second stage of the surgical palliation.

OBJECTIVES: Recoarctation of the aorta (re-CoA) after the Norwood procedure is traditionally treated during catheter-based aortoplasty (CB-A) performed as a separate procedure preceding stage II surgical palliation (S II SP). Our goal was to determine the efficacy of the protocol according to which re-CoA after the Norwood procedure in patients with hypoplastic left heart syndrome is treated during S II SP using hybrid catheter-based aortoplasty. METHODS: We compared 2 groups of infants who developed re-CoA after the Norwood procedure and were treated at the same institution: In group I (n = 18), CB-A was traditionally performed before S II SP; in group II (n = 15), CB-A was performed during S II SP using a hybrid procedure (catheter access was through an aortic cannula routinely used for cardiopulmonary bypass). The right ventricular fractional area change was analysed. RESULTS: The CB-A was performed effectively in both groups. S II SP was performed at a younger age in group II (5.4 ± 0.3 vs 6.0 ± 0.4 months; P = 0.003), with lower body weight (5.6 ± 0.5 vs 6.0 ± 0.4; P = 0.03, respectively). The duration of hospital stay did not differ between the groups (10.6 ± 6.2 vs 11.6 ± 6.4 days; P = 0.91). The right ventricular fractional area change measured before S II SP was higher in group I (39.7 ± 4.2% vs 36.8 ± 3.6%, respectively; P = 0.009), but the difference was not seen 1 month after S II SP (41.0 ± 5.6 vs 39.8 ± 4.1; P > 0.05). The total radiation dose was significantly lower in group II. CONCLUSIONS: re-CoA after the Norwood procedure in patients with hypoplastic left heart syndrome can be treated effectively during S II SP using a hybrid procedure. The strategy allows for reduction of the total radiation dose and of the number of procedures and does not prolong the postoperative course, even in patients with decreased right ventricular systolic function.

[Therapeutic results in children with hypoplastic left heart syndrome after Norwood procedure]. / Wyniki leczenia dzieci z zespolem niedorozwoju lewego serca po operacji metoda Norwooda.

UNLABELLED: Hypoplastic left heart syndrome (HLHS) encompasses hypoplasia or atresia of the left ventricle, stenotic or artretic aortic and mitral valves and hypoplasia of the ascending aorta. The aim of the 1-st stage operation called the modified Norwood procedure is reconstruction of the systemic flow, providing an adequate pulmonary flow and relieving interatrial restriction. The aim of the study was the analysis of treatment results after the Norwood operation in children hospitalized at Cardiology and Cardiac Surgery Departments in the period of 2009-2012. The material consisted of 65 children (42 males, 23 females). The following factors were analyzed: the age on admission, general condition, pre-operative clinical course. RESULTS: The mean age of children on admission was 4.4 ± 4.1 days and mean body weight--3.3 ± 0.5 kg. The general condition was good in the majority of patients (74%). The remaining children were found to have early symptoms of congestive heart failure (CHF). Cardiological and/or surgical interventions were necessary in 33 (50.8%) patients, among them three children required both procedures. In the majority of patients (n = 21, 32%), the main cause of intervention was re-coarctation of the neo-aortic isth- mus treated with balloon plasty and/ or stenting. In the early post-operative period, six children died due to CHF (n = 4) and infections (n = 2), while the remaining six children died due to various complications while waiting for the 2nd stage. CONCLUSIONS: Despite the progress in diagnosis and treatment of children with HLHS the mortality is still high.

Coronary artery abnormalities in Kawasaki disease.

INTRODUCTION: Kawasaki disease is the number one cause of acquired heart disease among children in developed countries. AIM: The aim of the study was a retrospective analysis of the factors that may influence the persistence of coronary artery abnormalities in patients with Kawasaki disease. MATERIALS AND METHODS: Analyzing the medical records of patients hospitalized in the University Children's Hospital of Krakow in the years 2005-2011 we collected the data of 28 patients diagnosed with Kawasaki disease. The group was divided into two subgroups, depending on the duration of the persistence of changes in the coronary arteries - A (n = 17) for up to 6 months, B (n = 11) - for more than 6 months. Both groups were analyzed for the presence of factors that may influence the course of the disease. RESULTS: There were more boys in group A (11 boys (65%), 6 girls (35%)), whereas in group B the distribution was more uniform (6 boys (55%), 5 girls (45%)). The age of onset in group A was 37.9 months (SD 30.8), in group B 39.5 months (SD 16.7). 17.6% of patients in group A and 36.4% in group B were treated with glucocorticoids. CONCLUSIONS: In the group of patients in which coronary artery abnormalities disappeared more quickly, male and slightly older children dominated. The only difference observed between the 2 groups related to the frequency of the use of glucocorticoids, they were used more often in children, in whom coronary artery abnormalities persisted longer.

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.

[Syncope in children and adolescents]. / Omdlenia u dzieci i mlodocianych.

The report presents a definition and causes of syncope in children. Syncope differs from other states with loss of consciousness by causes leading to decreased perfusion and resultant transient cerebral dysfunction with decreased muscle tone. The most common causes of syncope noted in almost 15% of children are neurocardiogenic. This group includes vasovagal, carotid sinus reflexive, situational (coughing, dysphagia, micturation and defecation disturbances) and post-exercise syncope. Another group is represented by orthostatic syncope that may be triggered by primary and secondary dis-autonomy, decreased blood volume (hemorrhage, diarrhea, Addison's disease), some medications and substances of abuse (alcohol). An important group, accounting for 2%-6% of all cases, are cardiogenic syncope, caused mainly by congenital/acquired obstructive cardiac sub- and valvar heart defects, various cardiomyopathies, some heart tumors (e.g. myxoma), exudative pericarditis, pulmonary embolus and hypertension, congenital and acquired coronary anomalies, various significant brady-tachyarrhythmias (sick sinus syndrome, supra- and ventricular tachycardias, congenital and acquired atrio-ventricular blocks). Subclavian steal syndrome as the cause of syncope is exceptional in children. Syncope does not include loss of consciousness due to neurological and metabolic (hypoglycemia) causes, hypoxia, hyperventilation with hypocapnia or CO intoxication. Differential diagnosis should also include pseudo-syncope (hysteria). Preliminary diagnostic management should include a detailed medical history, including family history, on the frequency and circumstances of syncope, sudden deaths, a physical exam with orthostatic assessment of peripheral blood pressure and standard ECG (heart rate, intraventricular and atrioventricular conduction defects, cardiac hypertrophy, arrhythmias, L-QT, changes in ST-T). Further specialist tests depend on preliminary findings.

[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.