Anna Mazurek-Kula

Interventions Complementing Surgery as Part of Multistage Treatment for Hypoplastic Left Heart Syndrome: One Center's Experience

Background Interventional procedures often are used as part of multistage treatment for hypoplastic left heart syndrome (HLHS). This study aimed to evaluate the efficacy of interventions in multistage treatment of HLHS. Methods and Results Between 1999 and 2007, 78 interventions were performed for 58 children with HLHS at different stages of surgical treatment. Group 1, involving 30 interventions for 22 patients with postoperative pulmonary arterial stenosis, showed reduced pulmonary artery pressure and increased vessel diameter. Group 2, involving 15 interventions after the Norwood operation for 12 patients with aortic arch obstruction, showed success for 11 patients. Group 3, involving eight balloon angioplasties of narrowed Glenn anastomosis for seven patients, showed abolition of the pressure gradient between the superior vena cava and the right pulmonary artery. Group 4, involving eight interventional closures of a fenestration for eight patients after a Fontan operation, showed increased oxygen saturation for all eight patients. Group 5, involving seven interventions for six patients in whom venovenous collaterals were closed percutaneously, showed improved oxygen saturation. For Group 6, uncommon miscellaneous interventions were used to stabilize the patients’ condition before the next surgical treatment. Conclusions Interventional procedures play an important role during multistage treatment of HLHS. They allow for a reduction in the number of operations or stabilization of the patients’ condition before the next surgical treatment.

Bronchial compression as adverse effect of left pulmonary artery stenting in a patient with hypoplastic left heart syndrome.

A 5-year-old child with hypoplastic left heart syndrome (HLHS) was noted to have left pulmonary artery stenosis after Glenn shunt placement. Therefore, a stent was implanted into the left pulmonary artery to relieve stenosis. After redilatation of the pulmonary artery stent, wheezing, fatigue, asymmetric bronchial sound, and desaturation was noted. Computed tomography, bronchoscopy, and bronchography confirmed critical left main bronchus compression as a consequence of the mass effect of the dilated stent. Balloon plasty of the left bronchus successfully relieved bronchial stenosis. Tracheobronchial obstruction is a possible adverse effect of vascular stenting in children with congenital heart defects. Balloon plasty seems to be a safe and efficient therapeutic option in those cases.

A 10-year single-centre experience in percutaneous interventions for multi-stage treatment of hypoplastic left heart syndrome

OBJECTIVES The purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications. BACKGROUND Constant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment. METHODS Between January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation. RESULTS Percutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2 saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries. CONCLUSIONS Percutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2 saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.

Fenestration closure with Amplatzer Duct Occluder II in patients after total cavo-pulmonary connection

Introduction Creation of a fenestration during completion of a total cavopulmonary connection (TCPC) has been associated with a reduction in early mortality and morbidity. However, the long-term benefits are negated by an associated limitation in exercise tolerance and the potential risks of thrombo-embolic complications. We sought to describe the safety and efficacy of an Amplatzer Duct Occluder II (ADO II) for transcatheter fenestration closure following TCPC. Material and methods Between January 2000 and July 2014, 102 patients underwent percutaneous closure of extra-cardiac TCPC fenestrations with a range of devices. Patients in whom fenestration closure was performed with an ADO II and who had at least 6 months of follow-up were included in this study. Results Forty-seven patients had successful fenestration occlusion with an ADO II. The mean oxygen saturation and mean systemic venous pressures increased from 84.8 ±6.1% before to 97.6 ±2.9% (p < 0.001) after and from 14.2 ±2.15 mm Hg before to 15.6 ±2.2 mm Hg after closure (p < 0.001). Eight patients developed heart failure symptoms, managed by optimization of medical therapy, with 1 patient requiring device removal to reopen the fenestration. Color Doppler transthoracic echocardiography demonstrated residual flow across the device in 18 (38%), 10 (22%), 5 (11%) and 4 (9%) patients before discharge, at 1 and 6 months, and at the latest outpatient visit, respectively. Conclusions The ADO II can be safely and effectively used to close fenestrations in extra-cardiac type Fontan completions. Many of the design features of this device confer potential benefit in this population.

Ocena funkcji systemowej prawej komory z wykorzystaniem tkankowej echokardiografii doplerowskiej u dzieci z zespołem niedorozwoju lewego serca po operacji metodą Fontana — wyniki wstępne

Introduction. Difficulties in the echocardiographic assessment of single ventricle function, particularly with right ventricular morphology, prompt to use different available echocardiographic techniques. Echocardiographic evaluation of the degree of hemodynamic disturbances affecting systemic ventricular function not only allows better investigation of the cardiovascular system but also helps determine prognostic factors to guide further therapeutic decisions. The role of tissue Doppler imaging (TDI) in paediatric patients with univentricular heart has been highlighted but only few studies evaluated the utility of this method in the evaluation of systemic ventricle systolic and diastolic function after the Fontan procedure. The aim of the study was to assess the relationship between conventional echocardiographic and TDI parameters in the evaluation of the performance of a functional single right ventricle in children with hypoplastic left heart syndrome (HLHS) after the Fontan procedure. Material and methods. A total of 34 patients with HLHS after staged surgical treatment culminating in the Fontan procedure underwent evaluation, including medical interview, physical examination, electrocardiography, and transthoracic echocardiography in standard views using M-mode, two-dimensional imaging, continuous and pulsed wave Doppler, colour Doppler, and TDI. The effect of demographic and perioperative factors on selected parameters of the echocardiographic evaluation of systemic ventricular function was analysed. In addition, we evaluated relations between conventional echocardiographic and TDI parameters in the evaluation of single right ventricular function in children after Fontan procedure. Results. Statistical analysis showed no significant correlation between TDI parameters of systolic and diastolic function (E’, E’/A’) and standard echocardiographic indices (E, A, E/A). Likewise, no significant correlation was found between the myocardial performance index (MPI) obtained from TDI and conventional MPI. A significant correlation was found between the E/E’ ratio and MPI. Conclusions. No significant correlation found in children after Fontan procedure between diastolic function parameters and right ventricular MPI measured using TDI and conventional echocardiographic parameters indicates that TDI is a technique complementary to conventional echocardiography in the evaluation of single right ventricle function in children. In addition, TDI does not depend on preload, and thus it can be speculated that this technique may identify ventricular dysfunction (mostly diastolic) at a stage when conventional echocardiographic parameters are still normal. Further studies in a larger number of patients are needed to support these conclusions.