Abstract
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.
Highlights
Congenital heart disease (CHD) is the most frequent congenital disorder in newborns, occurring with a frequency of approximately 1 in 40 neonates [1]
There is a group of diagnoses called ‘functionally univentricular heart’, which are congenital cardiovascular malformations where it is impossible to restore normal physiology with one ventricular pump committed to the systemic and another to the pulmonary circulation [3]
We searched this registry for all the patients born in Latvia from 1 January 2007, until 31 December 2015, with a functionally univentricular heart according to the definition from the International Society for Nomenclature of Pediatric and Congenital Heart
Summary
Congenital heart disease (CHD) is the most frequent congenital disorder in newborns, occurring with a frequency of approximately 1 in 40 neonates [1]. Univentricular heart has one of the highest mortality rates among congenital birth defects. These children require many resources and present many management challenges to a multidisciplinary clinical team. 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. 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. The children’s cardiac anatomy and interventions were categorized in accordance with the International Pediatric and Congenital Cardiac Code (v3.3)
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