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HYPOPLASTIC LEFT HEART SYNDROME WITH INTACT OR RESTRICTIVE ATRIAL SEPTUM:WHAT HAPPENS AFTER SURVIVAL?
619 JACC March 21, 2017 Volume 69, Issue 11
Congenital Heart Disease HYPOPLASTIC LEFT HEART SYNDROME WITH INTACT OR RESTRICTIVE ATRIAL SEPTUM:WHAT HAPPENS AFTER SURVIVAL? Poster Contributions Poster Hall, Hall C Saturday, March 18, 2017, 9:45 a.m.-10:30 a.m. Session Title: Congenital Heart Disease: Innovations in Single Ventricle Heart Disease Abstract Category: 10. Congenital Heart Disease: Pediatric Presentation Number: 1182-009 Authors: Brian Snarr, Michael Liu, Benjamin Zielonka, Jack Rychik, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Background: Hypoplastic left heart syndrome (HLHS) with intact or restrictive atrial septum (IRAS) represents a subset of patients with single ventricle cardiac disease (SVCD) associated with higher mortality. In recent years, management strategies have been developed for catheter-based creation of an inter-atrial communication in the prenatal or immediate postnatal period. Despite these advances, mortality still remains high. For those who survive, little is known about the long term morbidity and overall outcome. We sought to characterize the long-term outcome for those who underwent fetal or postnatal catheter-based intervention for IRAS. Methods: We performed a retrospective study reviewing all patients presenting to our center with a prenatal diagnosis of HLHS or other SVCD between 2004 and 2012. Those with HLHS or other right-dominant SVCD with IRAS who received either prenatal or immediate postnatal catheter-based intervention upon their atrial septum were identified. This cohort was followed longitudinally to death or 6 months post-Fontan and outcomes were characterized. Results: Nearly 500 patients with HLHS or SVCD were identified. Among those, 19 patients underwent fetal (2) or immediate postnatal (17) catheter-based intervention. There were 10 patients (53%) that survived to 6 months post-Fontan. Gestational age, birth weight, fetal AV valve regurgitation and fetal ventricular function were not found to be significantly different from non-survivors. Compared to survivors without IRAS, there was no significant difference in length of stay (LOS) from birth to discharge, or combined total LOS through 6 months post-Fontan.
Conclusions: The mortality for patients with IRAS undergoing catheter-based intervention still remains high with only 50% survival. Prenatal variables such as ventricular function and AV valve regurgitation were not found to be significant risk factors associated with survival. Once patients survive, however, their overall hospital course through Fontan palliation does not appear to be significantly different from their non-IRAS counterparts.
Congenital Heart Disease HYPOPLASTIC LEFT HEART SYNDROME WITH INTACT OR RESTRICTIVE ATRIAL SEPTUM:WHAT HAPPENS AFTER SURVIVAL? Poster Contributions Poster Hall, Hall C Saturday, March 18, 2017, 9:45 a.m.-10:30 a.m. Session Title: Congenital Heart Disease: Innovations in Single Ventricle Heart Disease Abstract Category: 10. Congenital Heart Disease: Pediatric Presentation Number: 1182-009 Authors: Brian Snarr, Michael Liu, Benjamin Zielonka, Jack Rychik, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Background: Hypoplastic left heart syndrome (HLHS) with intact or restrictive atrial septum (IRAS) represents a subset of patients with single ventricle cardiac disease (SVCD) associated with higher mortality. In recent years, management strategies have been developed for catheter-based creation of an inter-atrial communication in the prenatal or immediate postnatal period. Despite these advances, mortality still remains high. For those who survive, little is known about the long term morbidity and overall outcome. We sought to characterize the long-term outcome for those who underwent fetal or postnatal catheter-based intervention for IRAS. Methods: We performed a retrospective study reviewing all patients presenting to our center with a prenatal diagnosis of HLHS or other SVCD between 2004 and 2012. Those with HLHS or other right-dominant SVCD with IRAS who received either prenatal or immediate postnatal catheter-based intervention upon their atrial septum were identified. This cohort was followed longitudinally to death or 6 months post-Fontan and outcomes were characterized. Results: Nearly 500 patients with HLHS or SVCD were identified. Among those, 19 patients underwent fetal (2) or immediate postnatal (17) catheter-based intervention. There were 10 patients (53%) that survived to 6 months post-Fontan. Gestational age, birth weight, fetal AV valve regurgitation and fetal ventricular function were not found to be significantly different from non-survivors. Compared to survivors without IRAS, there was no significant difference in length of stay (LOS) from birth to discharge, or combined total LOS through 6 months post-Fontan.
Conclusions: The mortality for patients with IRAS undergoing catheter-based intervention still remains high with only 50% survival. Prenatal variables such as ventricular function and AV valve regurgitation were not found to be significant risk factors associated with survival. Once patients survive, however, their overall hospital course through Fontan palliation does not appear to be significantly different from their non-IRAS counterparts.