Stenting aortic coarctation in children: Immediate and midterm results

Introduction. Stent implantation, in patients with different forms of aortic coarctation, has significant theoretical advantages over primary balloon dilatation (BD). It can achieve overdilatation of the coarcted segment with the rigid endoprothesis maintaining the increase in vessel diameter regardless of the intimal injury and, thus, reducing the likelihood of restenosis. Moreover, by preventing vascular recoil, stents can successfully expand long-segment tubular coarctations, hypoplastic isthmus and hypoplastic transverse aortic arch. Finally, by facilitating good apposition of the torn intima to the aortic wall, they can significantly reduce the incidence of aneurysm formation. Objective. Evaluation of the immediate and mid-term results of stent implantation in patients with different forms of aortic coarctation. Methods. Between February 2005 and March 2010 eleven stents were implanted in nine patients (two female and seven male) either with post surgical or post primary BD residual coarctation/recoarctation or with native aortic coarcatation. Mean age of our patients was 14±3 years (9.4-18.1 years) and mean body weight 54±18 kg (29-76 kg). Results. Pressure gradient across the coarctation site was reduced from 24.9±12.4 mm Hg before to 3.9±5.0 mm Hg after stenting (p=0.000). There were no complications. Mean followup was 2.0±1.5 years (range 0.1-5.2 years). In patients with localised aortic arch narrowing no restenosis or aneurysm formation was observed (residual pressure gradient 0-5 mm Hg). Conclusion. In properly selected children (body weight >25 kg), adolescents and young adults stenting is the method of choice for patients with various forms of aortic arch obstruction

The analysis of the risk factors in the arterial switch operation for treatment of D-transposition of the great arteries in one Pediatric Cardiac Center in Serbia

Introduction. The arterial “switch” operation has been the operation of choice for children born with Dtransposition of the great arteries (D-TGA) for more than 30 years in countries with developed pediatric cardiac surgery program. After two decades of successful treatment of these children with the atrial “switch” corrections (Mustard or Senning operative techniques), the arterial “switch” operation (ASO) had been introduced as a routine technique in one pediatric cardiac center in Serbia. Objective. The aim of this study was the analysis of the identified risk factors involved with the ASO in the preoperative, operative and postoperative period and their impact on the survival of the operated children. Methods. A retrospective nonrandomized study of 52 operated patients with D-TGA by the ASO in the period between May 1, 2003 and December 31, 2011, divided into two groups. The data collection consisted of preoperative, operative and postoperative factors during the in-hospital stay and until the discharge from the hospital. Descriptive and differential statistical methods were used for analysis. Results. Ten individual risk factors were identified as significant for the immediate survival of children operated with the ASO technique. Conclusion. The arterial “switch” surgical operative technique is a complex neonatal/young infant procedure in which the preoperative status carried a significant risk as well as the surgical technique itself. These results differ from the results published throughout the world and are a representation of an evolutionary process of one center in Serbia starting the ASO procedure

Levosimendan treatment of severe acute congestive heart failure refractory to dobutamine/milrinone in children

Introduction. Levosimendan is a novel positive inotropic agent which, improves myocardial contractility through its calcium-sensitizing action, without causing an increase in myocardial oxygen demand. Also, by opening ATP-sensitive potassium channels, it causes vasodilatation with the reduction in both afterload and preload. Because of the long halflife, its effects last for up 7 to 9 days after 24-hour infusion. Case report. We presented three patients 2, 15 and 17 years old. All the patients had severe acute deterioration of the previously diagnosed chronic heart failure (dilatative cardiomyopathy; univentricular heart with bidirectional Glenn anastomosis and restrictive bulboventricular foramen; bacterial endocarditis on artificial aortic valve with severe stenosis and regurgitation). Signs and symptoms of severe heart failure, cardiomegaly (cardio-thoracic index 0.65) and left ventricular dilatation (end-diastolic diameter z-score 2.6; 4.1 and 4.0) were confirmed on admission. Also, myocardial contractility was poor with ejection fraction (EF - 27%, 25%, 35%), fractional shortening (FS - 13%, 11%, 15%) and stroke volume (SV - 40, 60, 72 mL/m2). The treatment with standard intravenous inotropic agents resulted in no improvement but in clinical deterioration. Thus, standard intravenous inotropic support was stopped and levosimendan treatment was introduced. All the patients received a continuous 24-h infusion 0.1 μg/kg/min of levosimendan. In a single patient an initial loading dose of 11 μg/kg over 10 min was administrated, too. Levosimendan treatment resulted in both clinical and echocardiography improvement with the improved EF (42%, 34%, 44%), FS (21%, 16%, 22%) and SV (59, 82, 93 mL/m2). Hemodynamic improvement was registered too, with the reduction in heart rate in all the treated patients from 134-138 bpm before, to less than 120 bpm after the treatment. These parameters were followed by the normalization of lactate levels. Nevertheless, left ventricular end-diastolic diameter did not change after the levosimendan treatment. Conclusion. Our initial experience demonstrates that administration of levosimendan in patients with severe chronic heart failure not responsive to standard intravenous inotropic treatment might result in a significant clinical and hemodynamic improvement and that, in selected patients, it might be life saving. According to our best knowledge patients presented are the first pediatric patients treated with levosimendan in our country

Adams-Oliver syndrome: Case report

Introduction. Adams-Oliver syndrome is characterized by congenital aplasia of the vertex skin of the skull in combination with skull and transverse limb defects. Case Outline. We presented a 5-month old female infant with Adams-Oliver syndrome manifested as cutis marmorata, dilated scalp veins and limb reduction defects. Clinical manifestation also included thumb hypoplasia and extreme hypoplasia of other fingers, with agenesis of all toes on both feet. Echocardiogram revealed foramen ovale apertum. Venography showed dilated malformed scalp and neck veins, predominantly on the right side. On the basis of the clinical features and extended investigation we confirmed Adams-Oliver syndrome in the presented patient. Conclusion. We recommended prenatal diagnosis in case of future pregnancies, ultrasound examination, and follow-up of foetal anomalies

Heart Rate Variability in Children with Exercise-Induced Idiopathic Ventricular Arrhythmias

Ventricular arrhythmias (VAs) are common pediatric rhythm disorders requiring comprehensive laboratory evaluation. Although usually idiopathic, implying a benign character and favorable prognosis, the initial clinical approach is still not established in all cases. Considering their prognostic significance, exercise-induced (precipitated or aggravated) VAs usually require additional diagnostics, treatment, and follow-up. A number of reports have presented experimental and clinical evidence that increased sympathetic activity can initiate, or at least facilitate, VAs. Recent data highlight the relationship between exercise-induced idiopathic VAs (IVAs) and the long-term risk of cardiovascular death. The aim of this study was to assess the utility of heart rate variability (HRV) analysis as a noninvasive method for estimating autonomic nervous activity in children with exerciseinduced IVAs. The study included 42 children with IVAs, who were divided into two groups: children with exerciseinduced (precipitated or aggravated) IVAs and children with exercised-suppressed IVAs. Time-domain HRV parameters were analyzed from 24-h ambulatory electrocardiography recordings, and the majority of children underwent an exercise stress test using the McMaster protocol. The results of this study showed no significant changes in parasympathetic index, i.e., the square root of the mean of the sum of the squares of the differences between adjacent NN intervals (the length between two successive heartbeats) between the groups examined. On the other hand, we observed diminished time-domain values for the standard deviation of all adjacent NN intervals, as well as diminished time-domain values for standard deviation of the averages of NN intervals in all 5-min segments in the group of children with exercise-induced IVAs, implicating increased sympathetic activity in such individuals. HRV analysis could be a helpful diagnostic method, giving useful information regarding cardiac autonomic control in some children with exercise-induced IVAs. © Springer Science+Business Media, LLC 2009

European Society of Cardiology Working Group on Adult Congenital Heart Disease and Study Group for Adult Congenital Heart Care in Central and South Eastern European Countries consensus paper : current status, provision gaps and investment required

Aims: To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region. Methods and results: We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for 2017 and/or 2018. Thirteen countries reported at least one tertiary ACHD centre with a median year of centre establishment in 2007 (interquartile range 2002–2013). ACHD centres reported a median of 2114 patients under active follow-up with an annual cardiac catheter and surgical intervention volume of 49 and 40, respectively. The majority (90%) of catheter or surgical interventions were funded by government reimbursement schemes. However, all 19 countries had financial caps on a hospital level, leading to patient waiting lists and restrictions in the number of procedures that can be performed. The median number of ACHD specialists per country was 3. The majority of centres (75%) did not have ACHD specialist nurses. The six countries with no dedicated ACHD centres had lower Gross Domestic Product per capita compared to the remainder (P = 0.005). Conclusion: The majority of countries in CESEE now have established ACHD services with adequate infrastructure and a patient workload comparable to the rest of Europe, but important gaps still exist. ACHD care is challenged or compromised by limited financial resources, insufficient staffing levels, and reimbursement caps on essential procedures compared to Western Europe. Active advocacy and increased resources are required to address the inequalities of care across the continent