Incidence and outcomes of patients with functionally univentricular heart born in Latvia, 2007 to 2015

Funding Information: This study was approved by the Ethics Committee of the Children’s Clinical University Hospital (Decision No. 5-2016; 12 September 2016); given the retrospective nature and absence of any patient identification, the requirement for individual patient or parent consent was waived. Publisher Copyright: © 2018 by the authors. Licensee MDPI, Basel, Switzerland.Background and Objectives: A functionally univentricular heart is the term used to describe congenital heart defects where it is impossible to restore two pumping chambers. These lesions are associated with high mortality, morbidity, and medical resource utilization. The aim of this study was to review incidence and outcomes of patients with a functionally univentricular heart at the only pediatric cardiac surgery center in Latvia. Methods: We performed a retrospective review of medical records of (i) all children with a functionally univentricular heart treated at the Clinic of Pediatric Cardiology and Cardiac Surgery, and (ii) all prenatally diagnosed cases of univentricular heart at Children’s Clinical University Hospital in Latvia. We reviewed data regarding children born from January 1, 2007, to December 31, 2015. The children’s cardiac anatomy and interventions were categorized in accordance with the International Pediatric and Congenital Cardiac Code (v3.3). Results: During the study period, 49 patients with a functionally univentricular heart were admitted to Children’s Clinical University Hospital with a corrected incidence of 0.69 per 1000 live births per year. There were 26 patients that had a hypoplastic left ventricle, and 22 patients that had a hypoplastic right ventricle, while one patient had an indeterminate ventricle. Thirty (61.2%) patients had died by the end of data collection. Twenty-one of the 30 deaths occurred before or immediately after stage I surgical palliation. Cumulative neonatal and 5-year survival of patients with a hypoplastic right ventricle was 81.8% and 63.6%, respectively; for patients with hypoplastic left ventricle—46.2% and 17.3%, respectively. Discussion: This is the first mid-term outcome study of patients with a univentricular heart in Latvia. The high mortality reflects the challenges of a small-volume, developing congenital cardiac surgery center. Data from this study will be used as a baseline for quality improvement.publishersversionPeer reviewe

Echocardiographic follow up after surgical correction of aortic coarctation during the first year of life

Publisher Copyright: Copyright © 2011-2014 by Walter de Gruyter GmbH.Coarctation of the aorta (AoCo) accounts for 6-10% of congenital heart diseases in infants. We analysed echocardiographic findings of patients operated on for AoCo in the University Hospital for Children in Riga during the first year of life to evaluate the long-term findings. Fifty-nine children underwent surgical correction of AoCo at the age of 55 ± 61 days. The methods of surgical correction were anastomosis end-to-end (ETE) in 29% (n = 17), subclavian flap aortoplasty (SFA) in 64% (n = 38) and extended anastomosis end-to-end (EETE) in 7% (n = 4). Recoarctation developed in 15 patients (25%) with no difference between surgical techniques (P > 0.05). The recoarcation patients had left ventricle hypertrophy (left ventricle mass index (LVMi) 76 ± 19 g/m2.7) normalising after angioplasty (LVMi 42 ± 7 g/m2.7). Patients with recoarctation had a decreased pulsed wave (PW) Doppler systolic/diastolic ratio in abdominal aorta 2.3 ± 0.4 versus patients without recoarctation 5.3 ± 1.2 and the control group 6.3 ± 1.4 (P < 0.05). A high incidence of recoarctation exists in patients operated on for AoCo as small infants. Life-long surveillance is required to monitor and to intervene in a timely way. Supplementation of the echocardiographic protocol with an evaluation of PW Doppler flow pattern in abdominal aorta can provide additional information about the presence of obstruction.publishersversionPeer reviewe

Initial experience with Edwards SAPIEN valve transcatheter implantation in native RVOT in Latvia

© Lietuvos mokslų akademija, 2020.Transcatheter pulmonary valve implantation has been a well-known method for more than a decade, but there are still many challenging cases when a personalized solution is needed. We report a case of a 15-year-old female patient with tetralogy of Fallot, who underwent a surgical correction during infancy. Because of progressive pulmonary regurgitation, stenosis, and right ventricle dilatation, transcatheter pulmonary valve implantation in the native right ventricle outflow tract (RVOT) using Edwards SAPIEN valve was performed. A "landing zone" was created prior to the intervention of stenting the RVOT and the right pulmonary artery. The transcatheter approach for pulmonary valve replacement in a native RVOT is a reasonable alternative to the surgical approach.publishersversionPeer reviewe

Comparison of Characteristics and Outcomes of Multisystem Inflammatory Syndrome, Kawasaki Disease and Toxic Shock Syndrome in Children

Publisher Copyright: © 2023 by the authors.Background and Objectives: Since the first cases of multisystem inflammatory syndrome in children (MIS-C) in April 2020, the diagnostic challenge has been to recognize this syndrome and to differentiate it from other clinically similar pathologies such as Kawasaki disease (KD) and toxic shock syndrome (TSS). Our objective is to compare clinical signs, laboratory data and instrumental investigations between patients with MIS-C, KD and TSS. Materials and Methods: This retrospective observational study was conducted at the Children’s Clinical University Hospital, Latvia (CCUH). We collected data from all pediatric patients <18 years of age, who met the Centers for Disease Control and Prevention case definition for MIS-C, and who presented to CCUH between December 2020 and December 2021. We also retrospectively reviewed data from inpatient medical records of patients <18 years of age diagnosed as having KD and TSS at CCUH between December 2015 and December 2021. Results: In total, 81 patients were included in this study: 39 (48.1%) with KD, 29 (35.8%) with MIS-C and 13 (16.1%) with TSS. In comparison with TSS and KD, patients with MIS-C more often presented with gastrointestinal symptoms (abdominal pain (p < 0.001), diarrhea (p = 0.003)), shortness of breath (p < 0.02) and headache (p < 0.003). All MIS-C patients had cardiovascular involvement and 93.1% of MIS-C patients fulfilled KD criteria, showing higher prevalence than in other research. Patients with KD had higher prevalence of cervical lymphadenopathy (p < 0.006) and arthralgias (p < 0.001). In comparison with KD and TSS, MIS-C patients had higher levels of ferritin (p < 0.001), fibrinogen (p = 0.04) and cardiac biomarkers, but lower levels of platelets and lymphocytes (p < 0.001). KD patients tended to have lower peak C-reactive protein (CRP) (p < 0.001), but higher levels of platelets. Acute kidney injury was more often observed in TSS patients (p = 0.01). Pathological changes in electrocardiography (ECG) and echocardiography were significantly more often observed in MIS-C patients (p < 0.001). Conclusions: This research shows that MIS-C, KD and TSS have several clinical similarities and additional investigations are required for reaching final diagnosis. All the patients with suspected MIS-C diagnosis should be examined for possible cardiovascular involvement including cardiac biomarkers, ECG and echocardiography.publishersversionPeer reviewe

Stem cell therapy as one of temporary measures for management of heart failure and pulmonary hypertension in children

Favourable results of experimental studies on animals and several conditions in adult population indicate that bone marrow derived progenitor stem cell (BMPSC) transplantation may play a crucial role. Nevertheless, little is known about possible implementation of the BMPSC transplantation in children, dilated cardiomyopathy and pulmonary hypertension in particular. An increasing understanding of the nature and processes of idiopathic dilated cardiomyopathy and pulmonary arterial hypertension in children, as well as the limited treatment options have led our research towards the use of stem cell transplantation in the management of these patients. We performed intramyocardial BMPSC transplantation in 6 patients (4 months to 17 years) with dilated cardiomyopathy. All patients underwent complete detailed examination before and after the procedure. All patients demonstrated an increase in LVEF and degree of shortening of the left ventricular diameter between end-diastole and end-systole after the procedure. A decreased concentration of natriuretic peptide or LVDd on 2D and 3D echocardiography was observed in 5 and 3 of the 6 patients respectively. Intrapulmonary BMPSC transplantation was performed in two patients (9 and 15 years old) with severe pulmonary hypertension due to uncorrected large ventricular septal defects. Both patients showed improvement in lungs’ vascularization. No serious periprocedural side effects were observed. If applied wisely, the stem cell therapy appears to be a safe and effective way for stabilization of critically ill patients with both severe pulmonary hypertension and idiopathic cardiomyopathy.</p

Paediatric and adult congenital cardiology education and training in Europe

Publisher Copyright: © The Author(s), 2022. Published by Cambridge University Press.Background: Limited data exist on training of European paediatric and adult congenital cardiologists. Methods: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. Results: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41). Conclusion: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.Peer reviewe