Katarzyna Leszczyńska

Hybrid cardiovascular procedures in the treatment of selected congenital heart disease in children: a single-centre experience

BACKGROUND Paediatric hybrid cardiovascular procedures are becoming increasingly popular due to the wide spectrum of cardiovascular abnormalities and a large population of patients who could benefit from hybrid therapy. Contemporary hybrid procedures are introduced in the settings where routine cardiac surgery or cardiac interventions would not allow satisfactory results. Combining cardiac surgery and interventional cardiology techniques appears more beneficial and less invasive for the patient. AIM To present results of hybrid procedures performed in the treatment of selected congenital heart disease in our centre in 2008-2013. METHODS We retrospectively analysed consecutive 80 patients referred for hybrid procedures. RESULTS Overall, 73 patients survived, with 4 early and 3 late deaths. CONCLUSIONS Hybrid procedures in children with congenital heart disease combine the experience of cardiac surgery and interventional cardiology. Hybrid treatment is an alternative option for selected borderline patients. Initial results of hybrid treatment encourage further development of these methods and strategies to provide optimal benefits for the patients.

Pregnancy and Delivery in Patients with Mastocytosis Treated at the Polish Center of the European Competence Network on Mastocytosis (ECNM)

Objective To present current guidelines regarding treatment of mastocytosis in pregnancy on the example of observed patients. Design Case control national study. Setting Polish Center of the European Competence Network on Mastocytosis (ECNM). Population or Sample 23 singleton spontaneous pregnancies in 17 women diagnosed with mastocytosis in years 1999–2014, before becoming pregnant. Methods Prospective analysis outcomes of pregnancies and deliveries. Main Outcome Measures Survey developed in cooperation with the Spanish Instituto de Estudios de Mastocitosis de Castilla-La Mancha (CLMast), Hospital Virgen del Valle, Toledo, Red Espańola de Mastocitosis (REMA), Spain. Results All 23 pregnancies resulted from natural conception. Obstetrical complications recorded in the first trimester included spontaneous miscarriage (5 pregnancies). Four patients delivered preterm, including one delivery due to preeclampsia at 26 weeks which resulted with neonate death due to extreme prematurity. Five women delivered via cesarean: four due to obstetrical indications and one due to mastocytosis, during which no anesthesia related complications were recorded. Of patients delivering vaginally, two received extradural anesthesia, three required oxytocin infusion due to uterine hypotonia. No labor complications were recorded. In one woman with pregnancy-induced hypertension, early puerperium was complicated by the presence of persistent arterial hypertension. One neonate was born with the signs of cutaneous mastocytosis. Another neonate was diagnosed with Patau syndrome. Four women were treated for mastocytosis prior to conception and continued therapy after becoming pregnant. One patient was put on medications in the first trimester due to worsening of her symptoms. Pregnancy exerted only a slight effect on the intensity and frequency of mastocytosis-related symptoms observed. Worsening of the disease-related symptoms was documented in only four patients (23%). None of the patients showed the signs of anaphylaxis, either before becoming pregnant, or during pregnancy and puerperium. Conclusions There is no contraindication to pregnancy when mastocystosis-related pathologies are under appropriate medical control.

Inflammatory marker levels after hybrid treatment of selected congenital heart disease in children

BACKGROUND Hybrid treatment of congenital heart disease is indicated in a selected group of borderline patients who do not have clear indications for either surgery or interventional treatment. Hybrid procedures take the best from cardiac surgery and interventional cardiology to reduce patient risk and trauma. Hybrid closure of ventricular septal defect (VSD) and atrial septal defect (ASD) without the use of extracorporeal circulation (ECC) might be less traumatic for patient. AIM To compare levels of inflammatory markers and clinical symptoms of systemic inflammatory response syndrome during early postoperative care after conventional cardiac surgery and hybrid treatment. METHODS Our study group of 36 paediatric cardiac surgical patients in Gdansk included 22 children with perimembraneous VSD and/or ASD who underwent cardiac surgery using with ECC and 12 children with muscular VSD and 2 children with ASD who underwent hybrid treatment. We retrospectively evaluated inflammatory markers including C-reactive protein (CRP) level, white blood cell (WBC) count and mean platelet volume (MPV), and clinical variables including total duration of stay in the ward, blood transfusions, prolonged respiratory support, fever, arrhythmias, catecholamine support and wound infections. The Mann-Whitney U test was used to compare CRP levels, WBC count and MPV between the study groups. RESULTS Surgical outcomes were good in all children, with no residual leakage. A similar, predictable increase in inflammatory markers (CRP, WBS, MPV) was found in all groups: conventional cardiac surgery with ECC, hybrid treatment without ECC, and hybrid treatment with ECC. Comparison of these groups showed no statistically significant differences between levels of inflammatory markers as well as other clinical variables. CONCLUSIONS Hybrid septal defect closure induces systemic inflammatory response syndrome activation as measured by CRP level, WBC count, and MPV, which is similar to that observed after cardiac surgery using ECC. Elevations of CRP level, WBC count, and MPV were similar after hybrid treatment with or without ECC. No significant differences were noted between trends of postoperative changes in the levels of inflammatory markers after treatment.

Alternative hybrid and staged interventional treatment of congenital heart defects in critically ill children with complex and non-cardiac problems

Introduction An individually designed strategy of comprehensive alternative hybrid and staged interventional treatment (AHASIT) can be a reasonable alternative to conventional treatment of congenital heart defects, reduce the risk of cardiac surgery or interventions performed separately, and give an additional chance for critically ill children. Aim To present our experience and the results of AHASIT of severely ill or borderline children referred for surgery with the diagnosis of congenital heart defects. Material and methods A group of 22 patients with complex cardiac and non-cardiac pathologies was retrospectively selected and analyzed. An individual preoperative severity scale was established for AHASIT patients, with one point for each of the following preoperative complications: prematurity, low body weight, cyanosis, intolerance to drug therapy, failed interventional treatment prior to admission, mechanical ventilation prior to the procedure, chronic respiratory failure and non-cardiac, mainly congenital malformations (congenital diaphragmatic hernia, lower extremity agenesia, duodenal atresia) and acquired problems (newborn edema, necrotic enterocolitis, intracranial hemorrhage, liver and renal failure, anemia and thrombocytopenia, infections or colonization with drug-resistant pathogens). Results The analysis of the postoperative course showed that the patients with 5 AHASIT points or more had a more complicated postoperative course than the patients with 1 to 4 AHASIT points. Conclusions The AHASIT of pediatric congenital heart defects with complex and non-cardiac problems appeared to be an attractive option for selected severely ill patients. The strategy was found to be effective in selected neonates suffering from complex and accompanying non-cardiac pathologies, with positive final results of both cardiological intervention and planned surgery.

Prenatalnie zdiagnozowana złożona wrodzona wada serca z zespołem Cri du chat i wieloma przeciwwskazaniami do leczenia kardiochirurgicznego — opis przypadku

It was presented a case of prenatally diagnosed Cri du chat syndrome, complicated by multiple congenital heart disease, with many additional problems that eventually may become the basis for disqualification the child from complex cardiac surgery. Due to the complexity of congenital heart disease, the potential for multistage treatment, the severe form of the genetic syndrome confirmed by detailed cytogenetic studies, severe clinical course, and multiple organ dysfunction, patient has not been qualified for cardiac surgery. Knowledge based on prenatal diagnosis allows to predict severe consequences in the newborn. Detailed genetic counseling makes it possible to prepare parents for a genetic birth defect and a congenital heart defect not eligible for cardiac surgery.

Recommendations for Fetal Echocardiography in Twin Pregnancy in 2016

Abstract Progress in the fields of fetal cardiology and fetal surgery have been seen not only in singleton pregnancies but also in multiple pregnancies. Proper interpretation of prenatal echocardiography is critical to clinical decision making, family counseling and perinatal management for obstetricians, maternal fetal medicine specialists, neonatologists and pediatric cardiologists. Fetal echocardiography is one of the most challenging and time-consuming prenatal examinations to perform, especially in multiple gestations. Performing just the basic fetal exam in twin gestations may take an hour or more. Thus, it is not practical to perform this exam in all cases of multiple gestations. Therefore our review and recommendations are related to fetal echocardiography in twin gestation.

Minimally invasive transxiphoid approach for management of pediatric cardiac tamponade – one center’s experience

Introduction Cardiac tamponade is excessive collection of fluid in the pericardial sac surrounding the heart that leads to restriction of cardiac function and causes critical cardiogenic shock and rapid circulatory depression. Despite the potential variety of different etiologies in the face of a dangerous decrease of cardiac output, the emergency life-saving procedure is surgical pericardial fluid evacuation. Aim To perform a retrospective analysis of clinical data and the results of minimally invasive transxiphoid pediatric cardiac tamponade evacuation procedures performed in a cardiac surgery center. Material and methods We performed a retrospective analysis of all consecutive patients referred for treatment in our department in a period of 6 years (15 patients) who underwent emergency pericardial drainage after an echocardiographically proven diagnosis. The procedure of choice was minimally invasive transxiphoid fluid evacuation with routine pericardial drainage. Retrospective operative data analysis was performed: clinical symptoms, pre-admission and initial emergency diagnostics and interventions, the morphology and total amount of drained pericardial fluid, length of stay, final results, and overall survival rate. We introduced an original pediatric tamponade index (PTI). The PTI was analyzed according to catecholamine support before the drainage and the length of hospital stay after the procedure. Results All patients survived the procedure. No early complications of the presented minimally invasive subxiphoid approach were noted. Mean PTI in patients with intensive catecholamine support before the operation was significantly higher than in patients without it. Conclusions Minimally invasive surgical transxiphoid interventions appear to be a safe and effective method to provide life-saving support with retrieval of the fluid for further laboratory investigations.

Cranial ultrasound through posterolateral fontanelle in visualization of posterior fossa abnormalities in preterm and term neonates.

OBJECTIVES The aim of the study was to evaluate the usefulness of US in the diagnosis of posterior fossa abnormalities in neonates by posterolateral fontanelle as compared with the anterior fontanelle approach and MRI. MATERIAL AND METHODS US studies were performed on 1337 neonates, including 512 preterm infants, through the anterior and posterolateral fontanelles. Abnormalities were detected in 134 patients. Among them, abnormalities in posterior fossa were visualized with the posterolateral approach in 14 neonates. MR images were obtained in that subgrqup. RESULTS The lesions consisted of cerebellar hemorrhage and congenital cerebellar malformations. Foci of hemorrhage were visualized by US in preterm neonates (n = 5), only through the posterolateral approach and on MRI. Dandy-Walker malformations (n = 2) were detected by US with both approaches and confirmed on MRI. In pontocerebellar hypoplasia (n =2), US with both approaches, showed hypoplastic cerebellar hemispheres and fluid in the posterior fossa. MRI, additionally visualized pontine hypoplasia. Fluid collection in the posterior fossa and translocation of cerebellar hemispheres were observed in the other 6 neonates by US with both approaches. MRI revealed arachnoid cysts (n = 2), mega cisterna magna (n = 3) and Blakes pouch (n = 1). CONCLUSIONS US using posterolateral fontanelle is the method of choice for the diagnosis of cerebellar hemorrhage. These lesions are not visualized through anterior fontanelle. US visualization of the abnormal structures in some cerebellar malformations has similar effectiveness for both approaches. MRI plays the crucial role in identification and differential diagnosis of these malformations.

[Analysis of pregnancy, labor and neonatal course in babies with prenatally-diagnosed complete atrioventricular heart block].

INTRODUCTION Complete heart block (third-degree atrioventricular block) is a defect of the conduction system of the heart, in which the impulse generated in the sinoatrial node does not propagate to the ventricles, and thus the latter contract independently of the atria. A third-degree atrioventricular block can be either congenital or acquired. In 60-70% of the cases, the congenital heart block results from destruction of the conduction system of the fetal heart by anti-Ro/SSA and anti-La/SSB antibodies present in maternal serum. The antibodies are synthesized in the course of autoimmune maternal conditions, most often systemic lupus erythematosus or rarer rheumatoid arthritis, dermatomyositis or Sjögrens syndrome. The complete block can occur as an isolated defect or be associated with structural anomalies of the fetal heart. MATERIAL AND METHODS A total of five patients whose fetuses were diagnosed with the third-degree atrioventricular block have been hospitalized at the Department of Obstetrics, Medical University of Gdansk between 2012 and 2014. RESULTS We present the data of the five patients, hospitalized at the Department of Obstetrics, Medical University of Gdansk, whose fetuses were diagnosed prenatally with the complete heart block. The cases differ in terms of etiology clinical outcome, and postnatal treatment. All data are presented in Table I. CONCLUSIONS We emphasize the role of appropriate pregnancy management and careful monitoring of the fetal condition. From obstetrical perspective, it is important to monitor the condition of fetuses with the third-degree atrioventricular block ultrasonographically and echocardiographically; in turn, cardiotocography is less useful in this setting. Therefore, a non-reactive cardiotocographic tracing should not constitute an indication for a preterm delivery. An affected fetus should be delivered in a tertiary center for perinatal care that cooperates with a pediatric cardiology center. An efficient program for cardologic prenatal care and close cooperation between obstetricians, neonatologists, pediatric cardiologists, and cardiac surgeons constitute the key to a successful outcome.

Kinetics of common inflammatory biomarkers in postoperative course after congenital heart defects procedures with extracorporeal circulation in children

BACKGROUND Extracorporeal circulation is associated with systemic inflammatory response syndrome. Therefore, the diagnosis of infection should be differentiated from a typical postoperative course. AIM The aim of the study was to evaluate the kinetics of inflammatory biomarkers in children in the first days after cardiac surgery with extracorporeal circulation. METHODS Prospective data were collected from 51 consecutive children referred for surgical treatment in Department of Paediatric Cardiac Surgery, St. Adalbertus Hospital in Gdańsk, between February and August 2015. Blood samples were collected on the first, second, and third postoperative days and sent to the institutional laboratory for routine investigations: white blood cell count, serum C-reactive protein (CRP) and procalcitonin concentrations. RESULTS The highest levels of procalcitonin were on the first postoperative day (median 3.53 ng/mL), although the peak values of CRP concentration and white blood cell count were on the second postoperative day (96 mg/L and 17.3 G/L). In the group of patients with foreign material implantation (Contegra® or Gore-Tex®), the higher values of procalcitonin concentration and white blood cell count were measured in the subsequent postoperative days. CONCLUSIONS The kinetics of analysed inflammatory biomarkers on the first days after cardiac surgery for congenital heart disease in children have different characteristics. The knowledge about the kinetics of inflammatory biomarkers could be useful in determining the possibility of evolving infections in the early postoperative period.