Infants with pulmonary atresia intact ventricular septum who require balloon atrial septostomy have significantly higher 18-month mortality.
Cardiol Young
; 31(10): 1613-1618, 2021 Oct.
Article
in En
| MEDLINE
| ID: mdl-33641693
ABSTRACT
INTRODUCTION:
Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences.METHODS:
This is a 30-year (1988-2018) retrospective study of patients with pulmonary atresia intact ventricular septum.RESULTS:
Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock-Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001).DISCUSSION:
Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pulmonary Atresia
/
Ventricular Septum
/
Heart Defects, Congenital
Type of study:
Observational_studies
/
Qualitative_research
Limits:
Humans
/
Infant
/
Newborn
Language:
En
Journal:
Cardiol Young
Journal subject:
ANGIOLOGIA
/
CARDIOLOGIA
/
PEDIATRIA
Year:
2021
Document type:
Article
Affiliation country:
Estados Unidos