Symptomatic cardiac hemangioma resected on the first day of life.

Cardiac tumors are extremely rare in neonates. We describe the case of a right atrial hemangioma in a neonate diagnosed prenatally and successfully operated on the first day of life.

Fournier Gangrene Caused by Candida albicans in an Infant After Cardiac Surgery.

Fournier gangrene is a rare, rapidly progressive, life-threatening condition. We report a 23-day-old boy with pulmonary atresia and ventricular septal defect treated surgically, who developed Fournier gangrene. Emergency surgery was performed with tissue sampling for microbiological examination. Candida albicans was confirmed; caspofungin followed by fluconazole was administered with excellent results.

Hybrid Interventional Treatment of Iatrogenic Innominate Artery Aneurysm in a Child.

An iatrogenic aneurysm of an innominate artery is an extremely rare complication, especially in children. Nevertheless, this pathology was diagnosed in a child given palliative care with chronic respiratory insufficiency and a history of encephalitis requiring permanent ventilation at home via a tracheal tube.A nine-year-old girl with colitis ulcerosa and a history of hemorrhagic encephalitis, with chronic home ventilation therapy, was admitted in an emergency setting because of massive bleeding from the upper respiratory tract and the area surrounding the tracheotomy. Repeated tamponade with topically applied thrombin, and administration of tranexamid acid and cyclonamine appeared ineffective Because of a life-threatening condition and unknown origin of massive bleeding, the child was referred for cardiac catheterization with aortography before qualifying for surgery, with the option of alternative interventional treatment. An alternative option with PTFE-coated stent direct implantation into the brachiocephalic trunk from a peripheral vascular approach was performed. The girl was discharged home after a short recovery. Her chronic home ventilation was continued without additional problems.Stenting of a brachiocephalic trunk aneurysm with a PTFE-coated stent appeared to be a safe and effective treatment of massive bleeding from the respiratory tract, with its main advantage of avoiding the risk of a classic surgical approach in a palliatively treated patient.

Damus-Kaye-Stansel Procedure with Extracardiac Fontan: Successful Recruitment of Previously Ligated Pulmonary Valve.

We present a case of a 2.5-year-old-girl with complex congenital heart disease: tricuspid atresia (TA), bulboventricular septal defect (VSD), hypoplastic right ventricle, d-transposition of the great arteries (d-TGA) with aortic outflow from redundant RV. Due to II/III degree atrioventricular block induced after diagnostic cardiac catheterization, an epicardial pacemaker was implanted during the Glenn procedure. Because of severe left ventricle outflow tract obstruction, she was finally referred for extracardiac TCPC (extracardiac Fontan type) with recruitment of PV and Damus-Kaye-Stansel anastomosis. Intraoperatively, the pulmonary trunk stump was opened and a competent pulmonary valve with flaccid leaflets was found. Simple ligation of the pulmonary trunk with a preserved pulmonary valve enabled an effective aorto-pulmonary bridging of systemic outflow tract with the use of natural fully competent ventricle-arterial valves. The relief of single ventricle outflow tract obstruction led to final stabilization of spontaneous sinus rhythm recovery after 2 years of pacemaker stimulation.

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.

[Analysis of pregnancy, labor and neonatal course in babies with prenatally-diagnosed complete atrioventricular heart block]. / Analiza przebiegu ciazy, porodu i losów noworodków z prenatalnie diagnozowanym calkowitym blokiem przedsionkowo--komorowym.

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 Sjögren's 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.

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.

Hybrid technique for muscular ventricular septal defect closure.

Since 2008 we have performed 10 hybrid procedures for perventricular muscular ventricular septal defects (mVSD) closure. The mean age was 8.5 months (range 2.7 to 17.8), the mean diameter of mVSD was 6.3 mm (range 4 to 10 mm). The postoperative course was uncomplicated and complete closure of the mVSD was accomplished in all patients. There was neither in-hospital nor late mortality, and the longest follow-up now exceeds three years (range 2 months to 3.1 years).

Bloody pericardial tamponade in a child treated for pneumonia mimicking a lung tumor and infiltration of the heart.

We present the dramatic course of a female 5-year-old child with pneumonia and symptoms similar to local compression of the pericardium by a tumorous mass originating from the left lung. The child was treated with antibiotics for pneumonia with bilateral pleural effusions that required chest drainage. On the 10(th) day of therapy there was sudden anemia observed with the echocardiographic finding of acute cardiac tamponade. The child was referred for emergency life-saving surgical intervention. The chest was opened via a minimally invasive mini-incision in the area of the xiphoid process and bloody tension pericardial effusion was evacuated. The laboratory and histopathology investigations were not specific for neoplastic disease or tuberculosis infection. In the further observation the girl recovered and was discharged home two weeks after tamponade drainage. Fortunately our initial suspicion of neoplastic disease was not proved; nevertheless we would like to emphasize the need for oncologic vigilance in similar cases.

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.

Delayed closure of multiple muscular ventricular septal defects in an infant after coarctation repair and a hybrid procedure--a case report.

There are several strategies of surgical approach for the repair of multiple muscular ventricular septal defects (mVSDs), but none leads to a fully predictable, satisfactory therapeutic outcome in infants. We followed a concept of treating multiple mVSDs consisting of a hybrid approach based on intraoperative perventricular implantation of occluding devices. In this report, we describe a 2-step procedure consisting of a final hybrid approach for multiple mVSDs in the infant following initial coarctation repair with pulmonary artery banding in the newborn. At 7 months, sternotomy and debanding were performed, the right ventricle was punctured under transesophageal echocardiographic guidance, and the 8-mm device was implanted into the septal defect. Color Doppler echocardiography results showed complete closure of all VSDs by 11 months after surgery, probably via a mechanism of a localized inflammatory response reaction, ventricular septum growth, and implant endothelization.

Correction: Muscle strength, muscle power and body composition in college-aged young women and men with Generalized Joint Hypermobility.

[This corrects the article DOI: 10.1371/journal.pone.0236266.].

Muscle strength, muscle power and body composition in college-aged young women and men with Generalized Joint Hypermobility.

The aim of this study was an evaluation of the musculoskeletal system in women and men with Generalized Joint Hypermobility (GJH). The study included 87 participants- 40 with Generalized Joint Hypermobility (aged 21.2 ±1.8 years) and 47 (aged 21.0 ±1.3 years) in the control group (CG). The study included the Beighton score, the measurements of body composition, muscle flexibility (Straight Leg Raise test, Popliteal Angle test, Modified Thomas Test), and the measurements of muscle strength and muscle power. T-test and Mann-Whitney U Test were applied to assess the differences between independent groups. The study showed that there were no significant differences (p>.05) in the assessed body composition and the muscle flexibility between both women and men with GJH and the participants in the CG. Under isokinetic conditions for the non-dominant lower extremity, men from the CG received significantly higher (p = .02) flexion peak torque at 180°/s angular velocity. Women from the CG received a statistically significantly lower (p = .04) F/E ratio at 180°/s velocity. Under isometric conditions for both women and men with GJH, there were no statistically significant differences (p>.05) in the maximum torques in knee extension and flexion compared to the CG. For women and men with GJH, the maximum power in the lower extremities and jumping ability were not significantly different (p>.05) compared to the CG participants. The body composition, muscle flexibility, muscle strength, and muscle power of adults with Generalized Joint Hypermobility did not differ compared to healthy participants. The fact that there are no differences does not exclude the efficacy of strength training in increasing levels of muscle strength and its impact on body posture and proprioception or coordination.

Assessment of cardiac function in donor and recipient fetuses during a 7-day follow-up after selective laser photocoagulation of communicating vessels due to TTTS.

OBJECTIVES: The aim of the study was to analyze the changes in cardiac function and myocardial contractility of donor and recipient fetuses with twin-to-twin transfusion syndrome (TTTS) subjected to selective laser photocoagulation of the communicating vessels (SLPCV), between and after the procedure. Finally, we verified if fetuses with Quintero's stage I TTTS presented with early impairment of myocardial contractility. MATERIAL AND METHODS: We selected 77 consecutive women with twin pregnancies, whose both fetuses survived at least seven days post-SLPCV. Myocardial contractility of both fetuses was evaluated ultrasonographically, and their myocardial performance indices (Tei-Index values) and shortening fractions (SF) were determined. RESULTS: In donor fetuses, the Tei-Index values for both right and left ventricle remained within the respective reference ranges both before the procedure and during a 7-day follow-up. A significant change in shortening fraction values for the left ventricle in recipient fetuses and the right ventricle of in the donors was observed during a 7-day follow-up. CONCLUSIONS: Comparison of the cardiac parameters of donors and recipients revealed significant differences in Tei-indices during the entire follow-up period. The group with Quintero's I stage TTTS included 74% of recipient fetuses with abnormal Tei-Index values for the right ventricle (mean 0.53).

Cost-effectiveness of mini-circuit cardiopulmonary bypass in newborns and infants undergoing open heart surgery.

BACKGROUND AND AIM: Miniaturisation of the extracorporeal circuit is a current trend in modern paediatric cardiac surgery. Many investigators stress that reduction of priming volume and artificial surface area of extracorporeal circulation could lead to clinical and economic benefits. The aim of this paper was to evaluate the costs of mini-circuit use in infants undergoing open heart surgery. METHODS: We assessed post-operative course and cost of treatment in 60 infants undergoing open heart surgery. This group was prospectively randomised and divided into 2 equal subgroups: with miniaturised (group M) and conventional cardio pulmonary bypass circuits (group C). The study groups were clinically comparable. Surgical complications, duration of hospitalisation and cost of postoperative treatment were assessed in both groups. RESULTS: Miniaturisation of the extracorporeal circuit led to a significant reduction of priming volume and artificial surface area (by 46.6% and 68.8% respectively, p=0.0000001). Post-operative cardio-respiratory insufficiency (2 vs. 8, p=0.038), and infection (3 vs. 9, p=0.049) occurred less often in children from group M. Hospital stay was significantly shorter in group M. Total cost of treatment was significantly lower in children from group M (median: 4361.4 vs. 6660.5 euro, p=0.037). CONCLUSIONS: Miniaturisation of the extracorporeal circulation significantly improve post-operative outcome in infants undergoing open heart surgery. The mini-circuit significantly reduces cost of treatment in small children undergoing open heart surgery.

Monitoring both procalcitonin and C-reactive protein in the early period after tetralogy of Fallot correction in children promotes rational antibiotic use.

PURPOSE: This retrospective cohort study aimed to identify the early postoperative kinetics of C-reactive protein (CRP) and procalcitonin (PCT) in children undergoing tetralogy of Fallot (ToF) correction. The ability of these inflammatory markers to guide rational antibiotic usage was also determined. MATERIALS AND METHODS: All consecutive children who underwent ToF correction in 2009-2016 in our referral pediatric cardiac surgery clinic in Gdansk, Poland and did not exhibit infection signs on early postoperative days (POD) were identified. All patients received 48h antibiotic prophylaxis. Antibiotic treatment was extended or empirical antibiotic therapy was introduced if the clinician considered it necessary. CRP and PCT levels were measured on POD1-4 and 1-3, respectively. RESULTS: Of the 60 eligible children, 44 underwent CRP testing only. The remaining 16 patients underwent both CRP and PCT testing. All patients had abnormally high CRP values after surgery. All patients who also underwent PCT testing also displayed elevated PCT levels. The CRP and PCT levels peaked on POD2 (median=99.8mg/L) and POD1 (median=4.08ng/mL), respectively. In the CRP-alone patients, antibiotic prophylaxis was prolonged or empirical antibiotic therapy was started in 59%; in the CRP and PCT group, this was 25% (p<0.05). CONCLUSIONS: The children had elevated CRP and PCT levels after ToF correction, with peaks observed on POD2 and POD1, respectively. Monitoring both CRP and PCT in the early postoperative period may guide antibiotic therapy, thus reducing unnecessary treatment, additional toxicity, and adverse drug interactions without increasing treatment failure. Rational antibiotic treatment may also reduce antibiotic resistance.

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.

Percutaneous closure of recanalised ductus arteriosus--a single-centre experience.

INTRODUCTION: Restoration of blood flow through a previously occluded ductus arteriosus may occur in some patients. Treatment strategy in patients with such residual shunts has not yet been uniformly established. AIM: To present single-centre experience and to attempt to establish a strategy of management of patients with residual ductus arteriosus shunts following percutaneous closure. METHODS: Of 352 patients who underwent percutaneous closure of ductus arteriosus, in 13 subjects complete closure failed (coils and Rashkind occluders were used in 10 and 3 patients, respectively). In these patients other percutaneous interventions aiming at total closure of residual shunt were attempted. RESULTS: In 12 patients coils were inserted (one patient received two coils). Introduction of implant in one patient failed, but total occlusion of the shunt was confirmed one day after the procedure. Trivial residual shunt was observed in one patient after one-year follow-up. CONCLUSIONS: Percutaneous treatment of residual shunts within the ductus arteriosus is an effective and safe procedure. In our opinion identifying and treating such leaks is important, as it prevents complications and long-term need for antibiotic prevention of infective endocarditis. In the case of a small residual shunt, insertion of a coil seems to be the optimal therapy due to the low cost of the device, favourable design and high effectiveness. For patients in whom anatomy of the ductus arteriosus has been significantly changed, particularly in previously treated subjects, techniques using vascular loops or insertion using a catheter wedge may be helpful.