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

ISOLATED COR TRIATRIATUM SINISTER : A CASE REPORT

Cor triatriatum sinister is a rare congenital cardiac anomaly that has been identified in 0.1% of children with congenital heart disease. It is defined as a fibromuscular membrane that divides the left atrium into two chambers: a superior (proximal) that in most cases receives drainage from the pulmonary veins and an inferior (distal) chamber that communicates with the mitral valve and the left atrium. Cor triatriatum sinister can be an isolated lesion (approximately 25% of cases), but in many cases it is associated with other congenital cardiovascular anomalies, the most common one being – atrial septal defect(3). Symptoms in patients with cor triatriatum sinister are related to obstruction of pulmonary venous drainage, pressure loading of the right side of the heart and congestive cardiac failure. Depending on the severity of the obstruction and presence of associated cardiac anomalies it can be diagnosed at any age. Diagnosis is usually achieved by echocardiography in early infancy. Elective treatment method is surgical excision of the membrane. Here we present a pediatric patient (4 months old) presenting in cardiogenic shock with a successful correction of isolated cor triatriatum sinister. To confirm diagnosis and success of surgical repair, transthoracic and transesophageal echocardiography were used.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