Right Ventricle Mass Removal from Tricuspid Valve Apparatus: An Unusual Thromboembolic Complication of Severe Ketoacidosis.

The presence of a pathologic mass in the right ventricle (RV) may lead to hemodynamic consequences and to a life-threatening incident of pulmonary embolism. The diagnosis of an unstable thrombus in the right heart chamber usually necessitates intensive treatment to dissolve or remove the pathology. We present a report of an unusual complication of severe ketoacidosis: thrombus in the right ventricle, removed from the tricuspid valve (TV) apparatus. A four-year-old boy was diagnosed with diabetes mellitus (DM) type I de novo. During hospitalization, a 13.9 × 8.4 mm tumor in the RV was found in a routine cardiac ultrasound. The patient was referred for surgical removal of the floating lesion from the RV. The procedure was performed via midline sternotomy with extracorporeal circulation (ECC) and mild hypothermia. Control echocardiography showed complete tumor excision with normal atrioventricular valves and heart function. Surgical removal of the thrombus from the tricuspid valve apparatus was effective, safe, and a definitive therapy for thromboembolic complication of pediatric severe ketoacidosis.

Hybrid approach for closure of muscular ventricular septal defects.

BACKGROUND: The complexity of ventricular septal defects in early infancy led to development of new mini-invasive techniques based on collaboration of cardiac surgeons with interventional cardiologists, called hybrid procedures. Hybrid therapies aim to combine the advantages of surgical and interventional techniques in an effort to reduce the invasiveness. The aim of this study was to present our approach with mVSD patients and initial results in the development of a mini-invasive hybrid procedure in the Gdansk Hybrid Heartlink Programme (GHHP) at the Department of Pediatric Cardiac Surgery, Pomeranian Centre of Traumatology in Gdansk, Poland. MATERIAL AND METHODS: The group of 11 children with mVSDs was enrolled in GHHP and 6 were finally qualified to hybrid trans-ventricular mVSD device closure. Mean age at time of hybrid procedure was 8.22 months (range: from 2.7 to 17.8 months, SD=5.1) and mean body weight was 6.3 kg (range: from 3.4 to 7.5 kg, SD=1.5). RESULTS: The implants of choice were Amplatzer VSD Occluder and Amplatzer Duct Occluder II (AGA Med. Corp, USA). The position of the implants was checked carefully before releasing the device with both transesophageal echocardiography and epicardial echocardiography. All patients survived and their general condition improved. No complications occurred. The closure of mVSD was complete in all children. CONCLUSIONS: Hybrid procedures of periventricular muscular VSD closure appear feasible and effective for patients with septal defects with morphology unsuitable for classic surgical or interventional procedures. The modern strategy of joint cardiac surgical and interventional techniques provides the benefits of close cooperation between cardiac surgeon and interventional cardiologist for selected patients in difficult clinical settings.

Candidemia in children after complex congenital heart defects surgery treated with caspofungin--our own experience and a review of literature.

BACKGROUND: Invasive fungal infections due to Candida species constitute an increasing clinical problem. There are no guidelines for the management of candidemia in children undergoing surgical procedures for congenital heart defects (CHD). The aim of the study was to draw attention to the problem of candidemia in children who are operated on due to congenital heart defects. MATERIAL/METHODS: We retrospectively analyzed medical documentation of 307 children with congenital heart defects treated in 1 clinical centre in Poland, from whom we selected those diagnosed with candidemia during the postoperative period. Next, we analyzed in detail the clinical course of invasive candidiasis in each individual and we performed an analysis of risk factors that lead to candidemia. RESULTS: In the analyzed period, among 307 children who underwent surgical procedures, we observed 2 cases of candidemia (0.65%) which were effectively cured with caspofungin. No adverse effects were observed after treatment with the drug. CONCLUSIONS: Candidemia in children who undergo surgical treatment of congenital heart defects is an important factor that can influence final clinical results. Caspofungin may be an effective therapeutic option when treating candidemia in children after extensive cardiosurgical procedures.

Postoperative kinetics of common inflammatory biomarkers after congenital heart defect 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 Gdansk, 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.

Paediatric Melody® mitral valve replacement in acute endocarditis - alternative surgical-hybrid technique.

BACKGROUND AND AIM: Acute endocarditis (AE) is still rare disease in the paediatric population; nevertheless, the children suffering from AE usually need heart valve repair or replacement in emergency settings. METHODS: We present a case of emergency mitral valve replacement with the use of Melody balloon expandable stented bioprosthesis in a two-year-old patient with AE and subsequent mitral (bicuspid) valve incompetence after aggressive infective destruction with the symptoms of critical multi-organ failure. RESULTS: The patient, with a history of rapid deterioration after two-week-long septicaemia in the course of AE, was operated urgently after initial antibiotic treatment because of huge vegetations into the mitral valve orifice. A Melody TVP 22 valve was expanded over a 16-mm TyShak balloon and implanted into a mitral position (Melody-MVR) with good result. CONCLUSIONS: Based on current knowledge concerning heart valve reconstructions and institutional experience, we conclude that infected mitral valve in children should be primarily repaired; nevertheless, the Melody valve could be reasonably con-sidered as a mitral prosthesis in such conditions.

Department of Pediatric Cardiac Surgery in Gdansk in its new location - previous activity and perspectives for development.

The Department of Pediatric Cardiac Surgery in Gdansk is the only pediatric cardiac surgery center in northern Poland providing comprehensive treatment to children with congenital heart defects. The Department of Pediatric Cardiac Surgery in Gdansk currently offers a full spectrum of advanced procedures of modern cardiac surgery and interventional cardiology dedicated to patients from infancy to adolescence. January 19, 2016 marked the official opening of its new location.

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.

Hybrid stent implantation to the pulmonary artery from peripheral access via recruited systemic-pulmonary shunt.

The implantation of vascular stents in patients with low body weight and difficult anatomy of the stenosis needs individual cannulation strategy or a hybrid approach. We present a successful balloon angioplasty with direct stent implantation to severe ostial stenosis of the left pulmonary artery to xenograft anastomosis (LPA) in a 6-year-old boy late after surgical correction of pulmonary atresia with ventricular septal defect. Peripheral approach to LPA was possible after surgical rethoracotomy and the recruitment of a left Blalock-Taussig (BT) shunt stump. The cooperation of cardiovascular intervention with surgical approach appears a safe strategy for borderline patients referred for staged treatment of complex congenital heart defects.

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.

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.

Kinetics of C-reactive protein in children with congenital heart diseases in the early period after cardiosurgical treatment with extracorporeal circulation.

PURPOSE: The aim of the study was to assess postoperative C-reactive protein (CRP) serum kinetics in children without clinical signs of infection after atrial and ventricular septal defects closure in terms of extracorporeal circulation (ECC). MATERIAL/METHODS: Fifty-two patients met inclusion criteria and were divided into 2 groups: group A (antibiotic prophylaxis with cefazolin given up to 48 h postoperatively) and group B (antibiotic prophylaxis with amoxicillin and clavunic acid given more than 48 h postoperatively). The CRP was measured perioperatively in both groups. The CRP evaluation was the part of routine lab-tests during perioperative period, without any modification of the typical perioperative strategy. In the postoperative period CRP was measured after 24h, 48 h, 72 h and 96 h in both groups. RESULTS: There were no differences between CRP levels between both groups of patients. The peak CRP values were observed after 48 h after the operation in ECC in both groups and decreased in the next postoperative days. CONCLUSIONS: In children with congenital heart defects undergoing cardiosurgical treatment with the use of ECC the assessing CRP values in the first postoperative day remains questionable. The maximum peak CRP value after operation with ECC can be much higher than the reference values without infection complications. Single CRP assessment in early postoperative period in these groups of children can lead to over-diagnosis of infections and antibiotics abuse.

"Planned" permanent pacemaker implantation in one-day-old newborn after prenatal diagnosis of congenital complete atrioventricular heart block.

We present a case of a severely ill newborn with congenital atrioventricular heart block (CAVB) diagnosed prenatally. The initial drug therapy just after birth was ineffective, the heart rhythm remained 54 beats per minute, and control echocardiographies showed forthcoming decrease of the left ventricular function with mitral insufficiency. A permanent pacing system with bipolar electrodes eluting two steroids (Medtronic Capsule, Medtronic Inc. Minneapolis, USA) and Medtronic ADAPTA(®) pulse generator (Medtronic Inc, Minneapolis, USA) was implanted on the first day of life. The pace control as well as wound healing were uncomplicated, and the baby was discharged home without additional medication. The procedure of permanent pacemaker implantation on the first day of life was safe and effective, as a benefit from a successfully performed prenatal program in our cooperating institutions.

Operative treatment of tetralogy of Fallot with concomitant correction of anomalous origin of the left pulmonary artery from Kommerell's diverticulum.

Anomalies in development of the pulmonary valve, pulmonary trunk and peripheral pulmonary arteries are typical accompanying pathologies in patients with tetralogy of Fallot (ToF). Demanding for diagnostics and borderline for treatment is a condition colloquially called "discontinuous pulmonary arteries", while the main branches are supplied with systemic blood from the ascending aorta, aortic arch or descending thoracic aorta. We present a case of a one-year-old girl with ToF and anomalous origin of the left pulmonary artery (LPA) from Kommerell's diverticulum who underwent two-stage surgical therapy with the support of interventional cardiology. We conclude that early diagnosis of discontinuous pulmonary artery is crucial for choosing the optimal operative strategy. In our opinion, simultaneous anatomic intracardiac correction with direct pulmonary reconstruction seems reasonable and effective, particularly when the result is achieved after joint efforts of cardiac surgery and interventional cardiology.

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.

Gdansk Hybrid Heartlink Programme (GHHP) - we need to share the experience with miniinvasive hybrid procedures in borderline babies.

The Gdansk Hybrid Heartlink Programme follows the concept of the hybrid procedures that combine the advantages of classic surgical operations with intraoperative cardiology interventions. With time and our growing experience, we found that hybrid methods were especially beneficial in cardiac emergencies in borderline babies, who would not meet separate criteria either for surgery or for cardiac interventions. Hybrid strategies appeared safe and effective for patients with anatomical variants unsuitable either for classic surgical or interventional procedures introduced alone. Therefore hybrid treatment seems to be a reasonable alternative mainly because of diminished risk of fatal complications. The success of a hybrid programme is possible in institutions that improve the spirit of cooperation between cardiac surgeons and interventional cardiologists, aimed at the common benefit of borderline patients.

Proximal ductus arteriosus stenosis after the hybrid stage I procedure in a newborn with hypoplastic left heart syndrome.

We present a case of a severely ill newborn with hypoplastic left heart syndrome after the hybrid stage I procedure, with right systemic ventricle insufficiency due to proximal ductus arteriosus stenosis. The child was successfully treated with percutaneous second PDA stent implantation.

Miniinvasive interventional bridge to major surgical repair of critical aortic coarctation in a newborn with severe multiorgan failure.

We present a case of a severely ill newborn with complex coarctation, multiorgan failure and massive oedema, who was treated with emergency stenting of the isthmus on the second day of life, which was followed by surgical stent removal and repair of the arch on the 29(th) day, after stabilization of his general status. Interventional percutaneous direct stent implantation was performed, using a coronary stent (Abbott Multi-Link Vision Coronary Stent 3.5 mm/15 mm, USA) to cover the area of the aortic isthmus in the newborn. The area from the origin of the left subclavian artery to the beginning of the descending thoracic aorta beneath the isthmus was widely expanded. Control angiography showed normal size of the isthmus without a systolic gradient in the area. In the next 3 weeks the boy improved his general status, with normalization of liver and renal parameters, as well as resolution of the oedema, and underwent surgery on his 29(th) day of life. The procedure of stent removal with aortic extended end-to-end anastomosis was performed without complications, and the infant was transferred to general paediatrics for further treatment. The strategy of miniinvasive interventional bridge to postpone major surgical repair was effective in the presented infant, with positive final results of both cardiological intervention and subsequent surgical repair.

Reconstruction of the left-sided brachiocephalic trunk after vascular ring operation in left-handed child with Kommerell's diverticulum.

We report a case of a 2-year-old girl with prenatal diagnosis of vascular ring composed of a right-sided aortic arch, a left ligamentum arteriosum, and a left subclavian artery (arteria lusoria sin) originating from a retroesophageal Kommerell's diverticulum, who was referred for surgery with symptoms related to local compression - severe dysphagia and respiratory disorders. Through the left posterolateral thoracotomy, a ligamentum arteriosum between the Kommerell's diverticulum and the left pulmonary artery was divided and because of left-handedness of the child a brachiocephalic trunk was reconstructed in the fashion of anastomosis of the left subclavian artery and the left common carotid artery, originating from the ascending aorta.

Miniinvasive hybrid procedure for device migration after percutaneous closure of persistent arterial duct: a case report.

We report a case of an 8-month-old girl admitted to the Department of Paediatric Cardiac Surgery, Pomeranian Centre of Traumatology in Gdansk with migration of an Amplatzer Duct Occluder II device (AGA Med. Corp., USA) to the left pulmonary artery after interventional patent arterial duct (PDA) closure. Using a hybrid strategy, we performed a classical surgical closure of the PDA with simultaneous intraoperative miniinvasive catheter removal of the displaced implant from the left pulmonary artery using a muscle bioptome (Cook, EU). The procedure was successful, without any further complications. Percutaneous procedures of PDA closure in small children, although safe and effective, are associated with a risk of accompanying complications, especially in patients with inconvenient anatomy. Our strategy demonstrates that a miniinvasive hybrid strategy could be beneficial for the patient with implant PDA migration after a failed interventional procedure. We strictly maintain the practice of qualifying these borderline patients with great care to avoid predictable complications, and to provide immediate surgical support in any emergency, following modern models of cooperation between cardiologists and cardiac surgeons in hybrid heart teams. A modern strategy that combines miniinvasive cardiac surgery with interventional techniques provides new, effective algorithms for selective difficult clinical settings.