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.

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.

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.

Cardiopulmonary exercise test and bioimpedance as prediction tools to predict the outcomes of obesity treatment.

INTRODUCTION Obesity impacts the global population. Bioelectrical impedance analysis (BIA) and cardiopulmonary exercise test [CPET]) may help modify the treatment. OBJECTIVES We aimed to compare BIA and CPET results in obese and lean indivisuals, assess changes in BIA and CPET during obesity treatment, and indentify parameters predicting treatment outcome. PATIENTS AND METHODS We enrolled 200 obese patients, of whom 45 underwent a lifestyle modification treatment, and 32 lean individuals (controls). Lifestyle modifications included diet, rehabilitation, education, and behavioral therapy. The diet was based on body composition assessed by BIA and fat metabolism assessed by CPET. The intensity of exercise in the rehabilitation program was based on CPET, mainly peak oxygen uptake (VO2peak), fat metabolism (FAT), and fat heart rate (FAT HR). The protocol duration was 12 weeks. RESULTS Obese patients differed from lean controls with regard to VO2peak (P <0.0001), oxygen uptake at anaerobic threshold (P <0.0002), respiratory exchange ratio (P <0.014), oxygen uptake to work rate slope (P <0.0004), FAT (P <0.001), FAT HR (P <0.0008), anaerobic threshold heart rate (P <0.0003), as well as fat mass (P = 0.01), fat­free mass (P = 0.007), resting metabolic rate (RMR) (P = 0.007), total body water (P = 0.01), and extracellular water (P = 0.004). The treatment resulted in increased RMR (P <0.02) and VO2peak (P <0.002), as well as reduced fat tissue (P <0.006) and resting heart rate (P <0.017). The prediction model based on FAT HR, resting heart rate, and FAT enabled the prediction of treatment outcomes in 92% of patients. CONCLUSIONS Obese patients had pathological FAT and impaired exercise tolerance. Changes in BIA and CPET prove the metabolic impact of lifestyle modification treatment.

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).

Differences in gene expression related to the outcomes of obesity treatment, peak oxygen uptake, and fatty acid metabolism measured in a cardiopulmonary exercise test.

INTRODUCTION The obesity pandemic requires development of methods that could be used on a large scale, such as the cardiopulmonary exercise test (CPET). Gene expression may explain CPET results on the molecular level. OBJECTIVES The aim of this study was to compare gene expression in obesity, depending on CPET results. PATIENTS AND METHODS The study group consisted of 9 obese patients and 7 controls. The treatment encompassed diet, rehabilitation, and behavioral therapy. Diet was based on the body composition analyzed by bioelectrical impedance, resting metabolic rate, and subjective patient preferences. The rehabilitation depended on the CPET results: maximal oxygen uptake and fatty acid metabolism. Behavioral intervention focused on the diagnosis of health problems leading to obesity, lifestyle modification, training in self­assessment, and development of healthy habits. The intensive treatment lasted for 12 weeks and consisted of consultations with a physician, dietitian, and medical rehabilitation specialist. RNA was isolated from the whole blood. A total of 47 323 transcripts were analyzed, of which 32 379 entities were confirmed to have high quality of RNA. RESULTS We observed differences in gene expression related to the CPET results indicating abnormalities in fat oxidation and maximal oxygen uptake. The genes with major differences in expression were: CLEC12A, HLA­DRB1, HLA­DRB4, HLA­A29.1, IFIT1, and LOC100133662. CONCLUSIONS The differences in gene expression may account for the outcomes of treatment related to inflammation caused by obesity, which affects the muscles, fat tissue, and fatty acid metabolism.

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.

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.

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.

Mountain climbing of the grown-up patient with non-corrected congenital heart defect.

Congenital heart defects (CHD) are the cause of reduced physical performance. The presence of congenital abnormalities in the heart of grown-up patients contributes to excessive hypo-kinesia. We present endurance parameters and a personalized comprehensive cardiac rehabilitation program before an extreme mountain climbing of a 27-year-old patient with an uncorrected ventricular septal defect (VSD). A 26-year-old female patient with an uncorrected congenital VSD was admitted to the department of cardiac rehabilitation before the planned high-mountain expedition. Professional preparation and assessment of actual exercise capacity was performed before scheduled extreme climbing. We conclude that physical activity associated with a heavy load in people with uncorrected CHD who have not developed pulmonary hypertension and reverse right-to-left flow seems to be safe, while participation of grown-up patients with congenital heart disease (GUCH) in extreme mountain climbing requires special preparation, individually designed endurance training and education program, conducted by the team of professionals in specialist centers.

The impact of professional status on the effects of and adherence to the outpatient followed by home-based telemonitored cardiac rehabilitation in patients referred by a social insurance institution.

OBJECTIVES: Legislators and policymakers have expressed strong interest in intervention programs to reduce dependence on social disability benefits. Hybrid: ambulatory followed by home-based cardiac telerehabilitation--hybrid cardiac rehabilitation (HCR) seems to be a novel alternative for standard cardiac rehabilitation for patients with cardiovascular diseases (CVD) as a form of pension prevention paid by the Social Insurance Institution (SII). The kind of professional status may bias the motivation to return to work after HCR. The aim of our study was to evaluate whether the professional status can affect the effects of HCR. MATERIAL AND METHODS: One hundred fifty-two patients with CVD referred by the SII for a 5-week HCR were qualified for the study. Patients (87.7% males), aged 57.31 ± 5.61 years, were divided into 2 subgroups: W) white-collar employees (N = 22) and B) blue-collar employees (N = 130). To evaluate functional capacity, an exercise test on a treadmill was used. RESULTS: The number of days of absence in the cardiac rehabilitation program did not differ between the groups (mean ± standard deviation--B: 1.09 ± 3.10 days, W: 1.95 ± 3.64 days). There were significant improvements (p < 0.05) in measured variables after HCR in both (W and B) groups (max workload: 8.21 ± 2.88 METs (measured in metabolic equivalents) vs. 9.6 ± 2.49 METs, 7.76 ± 2.51 METs vs. 8.73 ± 2.7 METs, resting heart rate (RHR): 77 ± 16.22 bpm vs. 69.94 ± 12.93 bpm, 79.59 ± 14 bpm vs. 75.24 ± 11.87 bpm; double product, i.e., product of heart rate and systolic BP (DP rest) 10 815.22 ± 2968.24 vs. 9242.94 ± 1923.08, 10 927.62 ± 2508.47 vs. 9929.7 ± 2304.94). In group B, a decrease in systolic blood pressure (BP syst. - 137.03 ± 17.14 mm Hg vs. 131.82 ± 21.13 mm Hg), heart rate recovery in the 1st minute after the end of peak exercise (HRR1) (99.38 ± 19.25 vs. 93.9 ± 19.48) and New York Heart Association (NYHA) class (1.22 ± 0.53 vs. 1.11 ± 0.36) was observed. In group W, a decrease in diastolic blood pressure (BP diast.) at rest was observed (88.28 ± 9.79 mm Hg vs. 83.39 ± 8.95 mm Hg). The decrease in resting HR was significantly greater in group W (69.94 ± 12.93 vs. 75.24 ± 11.87, p = 0.034). CONCLUSIONS: Hybrid cardiac rehabilitation is feasible and safe with high adherence to the program regardless of the patient's professional status. Professional status did not influence the beneficial effect of HCR on exercise tolerance.

Influence of a hybrid form of cardiac rehabilitation on exercise tolerance in coronary artery disease patients with and without diabetes.

BACKGROUND: The effectiveness of stationary and ambulatory cardiac rehabilitation of patients with coronary artery disease (CAD) and diabetes has been proven by some authors, but data concerning the effects of hybrid forms of cardiac rehabilitation (HCR) in this population are lacking. A home-based telerehabilitation is a promising form of secondary prevention of cardiovascular diseases in this group of patients. AIM: The objective of the study was to compare the effects of HCR in CAD patients with and without diabetes mellitus (DM). The secondary endpoint was the assessment of CAD risk factors like low exercise capacity and obesity, in both groups of patients. METHODS: This was a retrospective study, which comprised 125 patients with CAD aged 57.31 ± 5.61 years referred for HCR. They were assigned to Group D (with diabetes; n = 37) or Group C (without diabetes; n = 88). HCR was carried out as a comprehensive procedure that included all core components of cardiac rehabilitation according to guidelines. Before and after HCR all patients underwent a symptom-limited exercise test performed according to the Bruce protocol on a treadmill. RESULTS: Before HCR the maximal workload was higher in Group C than in Group D (8.13 ± 2.82 METs vs. 6.77 ± 1.88 METs; p = 0.023), but after HCR the difference was not significant. In both groups an increase in the maximal workload after HCR was observed (Group D: before HCR 6.81 ± 1.91 METs, after HCR 8.30 ± 2.04 METs; p < 0.001; Group C: before HCR 8.31 ± 2.71 METs, after HCR 9.13 ± 2.87 METs; p = 0.001). Resting heart rate, double product, and heart rate recovery 1 (HRR1) declined in both groups. No significant differences in changes in exercise test parameters between both groups' parameters were found. CONCLUSIONS: HCR was effective in patients with DM. The adherence was high. Patients with DM had higher rates of obesity and significantly lower exercise tolerance than patients without DM. Patients from both groups gained similar benefit from HCR in terms of physical capacity, resting heart rate, and heart rate recovery.

Effects of outpatient followed by home-based telemonitored cardiac rehabilitation in patients with coronary artery disease.

BACKGROUND: Cardiac rehabilitation (CR) has been shown to reduce the cardiovascular mortality of patients with coronary artery disease (CAD) and help people to return to professional work. Unfortunately, limited accessibility and low participation levels present persistent challenges in almost all countries where CR is available. Applying telerehabilitation provides an opportunity to improve the implementation of and adherence to CR, and it seems that the hybrid form of training may be the optimal approach due to its cost-effectiveness and feasibility for patients referred by a social insurance institution. AIM: To present the clinical characteristics and evaluate the effects of hybrid: outpatient followed by home-based cardiac telerehabilitation in patients with CAD in terms of exercise tolerance, safety, and adherence to the programme. METHODS: A total of 125 patients (112 men, 13 women) with CAD, aged 58.3 ± 4.5 years, underwent a five-week training programme (TP) consisting of 19-22 exercise training sessions. The first stage of TP was performed in the ambulatory form of CR in hospital; then, patients continued to be telemonitored TP at home (hybrid model of cardiac rehabilitation - HCR). Before and after completing CR, all patients underwent a symptom-limited treadmill exercise stress test. Adherence was reported by the number of dropouts from the TP. RESULTS: The number of days of absence in the HCR programme was 1.50 ± 4.07 days. There were significant improvements (p < 0.05) in some measured variables after HCR in the exercise test: max. workload: 7.86 ± 2.59 METs vs. 8.88 ± 2.67 METs; heart rate (HR) at rest: 77.59 ± 12.53 bpm vs. 73.01 ± 11.57 bpm; systolic blood pressure at rest: 136.69 ± 17.19 mm Hg vs. 130.92 ± 18.95 mm Hg; double product at rest: 10623.33 ± 2262.97 vs. 9567.50 ± 2116.81; HRR1: 97.46 ± 18.27 bpm vs. 91.07 ± 19.19 bpm; and, NYHA class: 1.18 ± 0.48 vs. 1.12 ± 0.35. CONCLUSIONS: In patients with documented CAD, HCR is feasible and safe, and adherence is good. Most patients were on social rehabilitation benefit, had a smoking history, and suffered from hypertension, obesity, or were overweight. A hybrid model of CR improved exercise tolerance.

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.