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Ventricular septal defects closure (VSD) depending on the anatomy and clinical setting can be performed surgically or by a hybrid and transcatheter approach. Two cases of children with VSD will be presented. Patients' defects were closed with various types of occluders made of nitinol wire mesh occluder, patent ductus arteriosus (PDA) type. The first case was a 2.5-year-old boy after cardiosurgical correction of tetralogy of Fallot (TOF). After the procedure, a significant haemodynamic residual VSD was observed, which was not successfully closed during the subsequent reoperation. Despite pharmacological treatment, symptoms of heart failure were observed in this patient. In echocardiographic images the residual VSD was presented as a tunnel-like dissection of the ventricular septum (length 6 mm and diameter 3.4 mm). The defect was closed via arterial access with an Amplatzer Duct Occluder II (ADO II). The procedure was successfully performed without any medical complications. In this child, a significant shunt reduction and a noticeable improvement in the patient's clinical status and diminished symptoms of heart failure were noticed. The second patient was a 4-year-old girl suffering from a multi-perforated perimembranous VSD accompanied by a ventricular septal defect with aneurysm. The defect was closed by a venous approach with a PDA Cardio-O-Fix occluder (very similar to ADO I). No short-term or long-term complications were visible during or after the procedure. Only a mild residual shunt through the VSD was observed 6 months afterwards. Transcatheter VSD closure with a proper morphology, with occluders of type Amplatzer Duct Occluder ADO I or ADO II, constitutes a safe and effective therapeutic alternative. [ABSTRACT FROM AUTHOR]

Marfan syndrome (MFS) is an autosomal dominantly inherited disorder with defect FBN1 gene. The major symptoms of MFS include skeletal, cardiovascular, ocular malformations. The diagnosis of MFS is based on clinical findings and GHENT criteria. We present a case of a girl aged 16-years with delayed diagnosis of the Marfan syndrome despite typical clinical pictures since early childhood. [ABSTRACT FROM AUTHOR]

In the pediatric population there is a shortage in the number of hearts available for transplantation. However, sometimes a heart transplant is urgently required to save lives. The problem arises when the patient is hyperimmunized and there is a positive crossmatch with the only available donor. We present such a case of a 9-year-old patient with dilated cardiomyopathy on ventricular assist device, in whom it was possible to perform a successful HT and also apply a successive prevention against graft rejection. [ABSTRACT FROM AUTHOR]

The aim of this study is to analyze the feasibility of performing an isolated heart transplant in patients with severe pulmonary hypertension as a result of restrictive cardiomyopathy. The results present the clinical course from the diagnosis of restrictive cardiomyopathy at the age of 2 until the heart transplant at 8 years old. Initially, the patient was considered for multiorgan transplantation, heart and lungs, due to extremely high pulmonary resistance. However, due to the prolonged waiting period for a donor and the worsening condition of the child, a decision was made to perforate the atrial septum with the implantation of an atrial flow regulator system. After conducting control hemodynamic measurements, the qualification was changed to an isolated heart transplant, accepting the high operative risk associated with the still elevated pulmonary resistance index of 4.9 Wood units. This study describes the medical problems that occurred during postoperative treatment. The patient underwent an orthotopic heart transplant in her eighth year of life. Postsurgery, complications were observed, including generalized seizures and heart transplant rejection reaction. Immunosuppressive therapies were applied, and efforts were made to combat anemia and electrolyte disorders. While the cardiovascular system and heart parameters improved, there were some difficulties in controlling heart rhythm and stabilizing electrolyte levels. • Restrictive cardiomyopathy is the least common type of cardiomyopathy in children and presents significant management challenges, particularly when associated with severe pulmonary hypertension. • 2.Usage of Atrial Flow Regulator (AFR) as a bridge to heart transplantation in patients with high pulmonary resistance. • 3.Rreinforcement of the importance of personalized and innovative approaches in pediatric heart transplantation. [ABSTRACT FROM AUTHOR]

Several reviews have shown that COVID-19 in children is a relatively mild disease. However, a rare complication affecting children and adolescents after COVID-19 has been identified. Pediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS), which in some cases manifests itself as a hyperinflammatory syndrome with a multiorgan failure, may lead to death. We report a case of a 17-year-old patient who was admitted to the hospital with cardiogenic shock of unknown etiology. The disease was life-threatening, thus necessitating mechanical ventilation, circulatory support, and extracorporeal therapy due to renal and liver dysfunction. The patient tested negative for SARS-CoV-2 Reverse Transcription Polymerase Chain Reaction. Other infectious causes of illness were excluded. However, the patient had a positive IgG antibody test result and high levels of interleukin-6, which helped to diagnose PIMS-TS. Intravenous immunoglobulin and steroid therapies were initiated, unfortunately, with poor outcome. The patient's critical condition, particularly end-stage heart failure, led to mechanical circulatory support implantation and finally orthotopic heart transplantation. After the surgery, the patient's condition improved gradually. PIMS-TS manifests itself with different clinical images and as a state of varying severity, ultimately causing multiorgan dysfunction with shock resembling toxic shock syndrome. Ultimately, myocardial complications of PIMS-TS necessitated heart transplantation in the described patient. [ABSTRACT FROM AUTHOR]

Anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome - BWG) is a serious congenital cardiac anomaly leading to myocardial ischemia with severe heart failure. Immediate surgical correction is the treatment of choice, and the risk of postoperative complications depends on the degree of myocardial injury. The authors present two cases of infants with BWG, in whom longterm (175 and 26 days) left ventricular assistance with a Berlin Heart device was used, resulting in successful weaning from the support and subsequent hospital discharge. Because of serious hemorrhagic complications and their neurological consequences observed in the first patient, the anticoagulation protocol was modified in the second patient, providing more stable support and allowing the device to be removed after a shorter period of time. The Berlin Heart left ventricular assist device may be treated not only as a bridge for transplantation but also, considering the shortage of donors in this age group, as a bridge to recovery. [ABSTRACT FROM AUTHOR]