Conference: 2014 International PHA Conference and Scientific Sessions
Release Date: 06.22.2014
Presentation Type: Abstracts
File Download: 2014 Conference Abstract - Bhombal S
Download Adobe Acrobat
Background: Small cardiac shunts have not typically been considered significant contributors to pulmonary disease. However, premature neonates, already at risk for development of chronic lung disease, with moderate increases in pulmonary blood flow due to cardiac shunts may be at increased risk for respiratory compromise. This study aims to examine the effects of left to right shunting on preterm infants <1 year of age with pulmonary hypertension.
Methods: We conducted a retrospective review of hospitalized infants in the intensive care units of Children's Hospital Los Angeles who received sildenafil for pulmonary hypertension between 2008-2012. Patients with atrial septal defects, patent foramen ovales, patent ductus arteriosus and ventricular septal defects were included. Patients with complex congenital heart disease were excluded.
Results: In our population of 147 patients started on sildenafil for pulmonary hypertension, 66 (45%) were premature infants, of which 60 (90%) had evidence of intracardiac shunting (atrial septal defects or ventricular septal defects) or patent ductus arteriosus at the time of diagnosis with pulmonary hypertension. Out of these 60 patients, 22 (37%) patients were repaired, of which 1 patient died (5%). Thirty-eight patients (63%) remained unrepaired and 14 died (37%), of which 7 had comorbidities that led to their death (alveolar capillary dysplasia, unrepaired congenital diaphragmatic hernia, and cystic dysplastic kidneys with pulmonary hypoplasia). Thus, there was an 18% mortality in patients with prematurity, pulmonary hypertension, and unrepaired congenital heart defect without other significant comorbidities. In the preterm population with pulmonary hypertension, those with repaired congenital heart disease were more likely to survive than those who had unrepaired congenital heart disease (p = 0.016).
Conclusions: Small cardiac shunts have not typically been considered significant contributors to pulmonary disease. It is not unusual to monitor for spontaneous closure with intervention performed at preschool age if necessary. It is interesting that in our population of premature patients placed on sildenafil for pulmonary hypertension, a majority had cardiac shunting. Our data suggest that premature infants with simple cardiac lesions leading to increased pulmonary blood flow may have increased morbidity and mortality.