Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it?1

Abstract

LBW newborns with HLHS and physiologic variants have an increased early surgical risk but have acceptable intermediate survival rates for subsequent palliation including Fontan. LBW and prematurity should not be contraindications to early surgical palliation.

Impact of Patient Characteristics and Anatomy on Results of Norwood Operation for Hypoplastic Left Heart Syndrome

“…The association between genetic syndromes and extracardiac malformations and mortality after the Norwood operation is well established in older studies and, similar to our current series, continues to be evident in more recent studies including the SVR trial [1,3,5,9,13]. A recent study showed that genetic and extracardiac anomalies were associated with increased operative death and hospital stay in neonates who underwent the Norwood operation as recorded in the Society of Thoracic Surgeons (STS) database, in addition to increased early hazard of death and decreased 10-year survival in neonates who underwent the Norwood operation as recorded in the Congenital Hearts Surgeons Society database [13]. We recently analyzed the effect of genetic and extracardiac anomalies on the outcomes of cardiac operations on neonates at our institution and identified that those anomalies were associated with increased resource utilization, ventilation duration, intensive care and hospital stays, and hospital mortality.…”

Single Ventricle Palliation in Low Weight Patients Is Associated With Worse Early And Midterm Outcomes

“…Low weight has persistently been demonstrated as a risk factor for mortality after the Norwood operation, including in recent series from experienced institutions [11,12,14]. Similarly, low weight has been associated with increased mortality risk after BTS due to shunt occlusion related to the small sized shunts or systemic steal due to excessive pulmonary blood flow that is likely more common in smaller babies [16,17].…”

“…In addition to early death, low weight at time of firststage surgery was suggested to have an adverse effect on late survival [3,11,12]. Gelehrter and colleagues [11] reviewed outcomes of 47 neonates with HLHS patients who were 2.5 kg or less at time of Norwood palliation.…”

“…In their series, they had low interstage mortality and their operative survival after Glenn and Fontan was 91% and 94%, respectively. Pizarro and colleagues [12] studied outcomes of 20 neonates with HLHS and other SV variants who were 2.5 kg or less at time of Norwood palliation. In their series, 55% survived to initial hospital discharge and on follow-up they had 1 interstage mortality, while the remaining patients progressed to Glenn operation.…”

“…First-stage palliation in low weight neonates is challenging due to numerous technical and physiologic concerns. Despite several recent improvements in the management of neonates with complex congenital heart disease, low weight (2.5 kg or less) at time of surgery continues to be a significant risk factor for operative mortality after different SV palliative surgeries [6,7,[10][11][12]. While few reports have suggested that low weight at time of first-stage surgery might have an additional impact on late outcomes, little information is available about the effect of low weight on later progression toward subsequent palliative surgeries and overall survival [6,11,12].…”