Pediatric Heart Transplantation in the Context of Severe Pulmonary Hypertension Secondary to Restrictive Cardiomyopathy-Case Report.

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.

Long-term results of pediatric heart transplantations: Single-center experiences.

BACKGROUND: Heart failure (HF) is characterized by significant mortality in both adults and children. Characteristics of pediatric HF are feeding problems, poor weight gain, exercise intolerance, or dyspnea. These changes are often accompanied by endocrine disorders. The main causes of HF are congenital heart defects (CHD), cardiomyopathies, arrhythmias, myocarditis, or heart failure secondary to oncological treatment. Heart transplantation (HTx) is the method of choice for treatment of end-stage HF in pediatric patients. AIMS: This article aimed to summarize the single-center experience in heart transplantation in children. METHODS: Between 1988 and 2021 in the Silesian Center for Heart Diseases in Zabrze, 122 pediatric cardiac transplantations were performed. In the group of recipients with failing Fontan circulation, HTx was performed in 5 children. The study group was evaluated for the postoperative course: rejection episodes depending on the medical treatment scheme, coinfections, and mortality. RESULTS: One-, 5-, and 10-year survival rates between 1988 and 2001 were 53%, 53%, and 50%, respectively. One-, 5-, and 10-year survival rates between 2002 and 2011 were 97%, 90%, and 87%, respectively; between 2012 and 2021 (1-year of follow-up), the survival rate was 92%. The main cause of mortality both in early and late periods after transplantation was graft failure. CONCLUSIONS: Cardiac transplantation in children remains the main method of treatment for endstage heart failure. Our results at both early and long-term posttransplant periods are comparable to those obtained in the most experienced foreign centers.

First Polish analysis of the treatment of advanced heart failure in children with the use of BerlinHeart EXCOR mechanical circulatory support.

BACKGROUND: The treatment of advanced heart failure (HF) in children and infants poses a serious management problem. Heart failure in that patient group is usually of congenital aetiology. The treatment schedules for paediatric patients are in most cases adapted from the guidelines for treatment of adults. Up to 2009, the treatment of that extremely difficult group of patients was limited to pharmacological therapy and occasional heart transplantations. Constantly increasing problems with recruiting donors, especially for the paediatric group, contribute to the fact that mechanical support with the use of ventricular assist devices is for many children the only chance of surviving the period of waiting for a heart donor. AIM: The aim of the study was to analyse the outcomes of circulatory support in Poland and to assess the advisability of this method for treatment of children with severe HF. METHODS: This treatment of paediatric patients is currently used in three Polish centres. From December 28, 2009 to August 1, 2015, 27 implantations of BerlinHeart EXCOR® mechanical circulatory support system were performed in children aged from one month to 16 years (10 patients below one year of age; 37%). Left ventricular assist devices were implanted to 21 patients, whereas the remaining children received biventricular support. The most common reason for using this method was HF developed in the course of cardiomyopathy. In one case, HF after Fontan operation was the indication. RESULTS: The duration of the circulatory support period ranged from six to 1215 days. It was followed by successful heart transplantations in 10 (37%) patients, in five (18.1%) it resulted in regeneration of the heart, enabling explantation of the device, whereas three children are still waiting for transplantations. Nine (33%) children died during the therapy because of thromboembolic complications. CONCLUSIONS: As follows from our data, circulatory support utilising the BerlinHeart EXCOR® system is an effective and promising method used as a bridge to cardiac transplantation, or for regeneration of the myocardium in paediatric patients. In the group of the youngest and the most difficult patients, the method requires close cooperation of the medical and nursing personnel.

Temporary left ventricular assistance for extreme postoperative heart failure in two infants with Bland-White-Garland syndrome.

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 long-term (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.

Transcatheter closure of ventricular septal defects with nitinol wire occluders of type patent ductus arteriosus.

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.