Hitendu Dave1, Barbara Rosser2, Walter Knirsch3, Michael Hübler2, René Prêtre4, Oliver Kretschmar3. 1. Division of Congenital Cardiovascular Surgery, University Children's Hospital Zurich, Switzerland Children's Research Centre, University of Zurich, Switzerland hitendu.dave@kispi.uzh.ch hitendu@hotmail.com. 2. Division of Congenital Cardiovascular Surgery, University Children's Hospital Zurich, Switzerland Children's Research Centre, University of Zurich, Switzerland. 3. Children's Research Centre, University of Zurich, Switzerland Department of Pediatric Cardiology, University Children's Hospital Zurich, Switzerland. 4. Department of Cardiovascular Surgery, University Hospital Lausanne (CHUV), Lausanne, Switzerland.
Abstract
OBJECTIVES: To analyse the results of hybrid palliation of hypoplastic left heart syndrome (HLHS) patients and its variants with an emphasis on the long-term fate of the pulmonary arteries. METHODS: We analysed 28 neonates (2006-11) with HLHS and its variants, who underwent bilateral pulmonary artery banding, patent ductus arteriosus (PDA) stenting and balloon atrial septostomy using a true hybrid approach. Median age and weight were 4 (0-36) days and 3 (1.9-3.7) kg respectively. Diagnoses included 23 HLHS and 5 variants. The fate of all surviving branch pulmonary arteries (PA) after a hybrid approach were compared with their counterparts in 29 Norwood I survivors (2002-11). RESULTS: Four of 28 hybrid procedures needed to be converted to a Norwood procedure. Mortality after stage I hybrid palliation was 3/24 (12.5%). All 21 acute survivors underwent a comprehensive stage II at a median age of 4 (2.3-5.7) months, without any mortality (0%). Eleven of 21 comprehensive stage II survivors have undergone extracardiac Fontan; including 1 who underwent a rescue Fontan at 7 months of age and died (1/11: 9%). While 18/21 (86%) needed branch PA intervention in the hybrid group during the median follow-up duration of 39 (10-81) months, 9/29 (31%) needed the same in the Norwood group during a median follow-up duration of 58 (16-128) months (P < 0.001). Eight of 21 (38%) needed stenting [all on the left pulmonary artery (LPA)] in the hybrid group vs 5/29 (17%) in the Norwood group (P = 0.097). Ten of 21 (48%) patients had surgical/catheter intervention on both branch PA in the hybrid group vs 2/29 (7%) in the Norwood group (P = 0.001). Pre-Fontan Nakata index was significantly better in the Norwood group 206 (118-406) compared with the hybrid group 153 (56-230) mm(2)/m(2) (P = 0.01). The comparable lower lobe indices were 149 (103-333) and 137 (45-178) mm(2)/m(2) (P = 0.04), respectively. CONCLUSIONS: Hybrid approach can be pursued with a low mortality. However, the high frequency of catheter and/or surgical interventions, and the sluggish growth of the branch PA pre-Fontan need innovative solutions. A comparison of the neurodevelopmental outcome for the hybrid vs the Norwood cohort would define the role of the hybrid strategy in the treatment of HLHS and its variants.
OBJECTIVES: To analyse the results of hybrid palliation of hypoplastic left heart syndrome (HLHS) patients and its variants with an emphasis on the long-term fate of the pulmonary arteries. METHODS: We analysed 28 neonates (2006-11) with HLHS and its variants, who underwent bilateral pulmonary artery banding, patent ductus arteriosus (PDA) stenting and balloon atrial septostomy using a true hybrid approach. Median age and weight were 4 (0-36) days and 3 (1.9-3.7) kg respectively. Diagnoses included 23 HLHS and 5 variants. The fate of all surviving branch pulmonary arteries (PA) after a hybrid approach were compared with their counterparts in 29 Norwood I survivors (2002-11). RESULTS: Four of 28 hybrid procedures needed to be converted to a Norwood procedure. Mortality after stage I hybrid palliation was 3/24 (12.5%). All 21 acute survivors underwent a comprehensive stage II at a median age of 4 (2.3-5.7) months, without any mortality (0%). Eleven of 21 comprehensive stage II survivors have undergone extracardiac Fontan; including 1 who underwent a rescue Fontan at 7 months of age and died (1/11: 9%). While 18/21 (86%) needed branch PA intervention in the hybrid group during the median follow-up duration of 39 (10-81) months, 9/29 (31%) needed the same in the Norwood group during a median follow-up duration of 58 (16-128) months (P < 0.001). Eight of 21 (38%) needed stenting [all on the left pulmonary artery (LPA)] in the hybrid group vs 5/29 (17%) in the Norwood group (P = 0.097). Ten of 21 (48%) patients had surgical/catheter intervention on both branch PA in the hybrid group vs 2/29 (7%) in the Norwood group (P = 0.001). Pre-Fontan Nakata index was significantly better in the Norwood group 206 (118-406) compared with the hybrid group 153 (56-230) mm(2)/m(2) (P = 0.01). The comparable lower lobe indices were 149 (103-333) and 137 (45-178) mm(2)/m(2) (P = 0.04), respectively. CONCLUSIONS: Hybrid approach can be pursued with a low mortality. However, the high frequency of catheter and/or surgical interventions, and the sluggish growth of the branch PA pre-Fontan need innovative solutions. A comparison of the neurodevelopmental outcome for the hybrid vs the Norwood cohort would define the role of the hybrid strategy in the treatment of HLHS and its variants.
Authors: William N Evans; Alvaro Galindo; Abraham Rothman; Michael L Ciccolo; Sergio A Carrillo; Ruben J Acherman; Gary A Mayman; Kathleen A Cass; Katrinka T Kip; Carlos F Luna; Joseph M Ludwick; Robert C Rollins; William J Castillo; John A Alexander; Humberto Restrepo Journal: Pediatr Cardiol Date: 2016-03-01 Impact factor: 1.655