title: Hybrid approach for hypoplastic left heart syndrome and its variants: the fate of the pulmonary arteries.
abstract: OBJECTIVE
  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.affiliation: 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.
authors.forename: Hitendu
authors.initials: H
authors.lastname: Dave
authors.affiliation: Division of Congenital Cardiovascular Surgery, University Children's Hospital Zurich, Switzerland Children's Research Centre, University of Zurich, Switzerland.
authors.forename: Barbara
authors.initials: B
authors.lastname: Rosser
authors.affiliation: Children's Research Centre, University of Zurich, Switzerland Department of Pediatric Cardiology, University Children's Hospital Zurich, Switzerland.
authors.forename: Walter
authors.initials: W
authors.lastname: Knirsch
authors.affiliation: Division of Congenital Cardiovascular Surgery, University Children's Hospital Zurich, Switzerland Children's Research Centre, University of Zurich, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Hübler
authors.affiliation: Department of Cardiovascular Surgery, University Hospital Lausanne (CHUV), Lausanne, Switzerland.
authors.forename: René
authors.initials: R
authors.lastname: Prêtre
authors.affiliation: Children's Research Centre, University of Zurich, Switzerland Department of Pediatric Cardiology, University Children's Hospital Zurich, Switzerland.
authors.forename: Oliver
authors.initials: O
authors.lastname: Kretschmar
mesh_terms: D054087:Atrial Septum / Q000601:surgery; D001794:Blood Pressure; D001807:Blood Vessel Prosthesis; D006328:Cardiac Catheterization; D004374:Ductus Arteriosus, Patent / Q000601:surgery; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality* / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D058327:Norwood Procedures; D010100:Oxygen / Q000097:blood; D011651:Pulmonary Artery / Q000601:surgery*; D012086:Reoperation; D012189:Retrospective Studies; D015607:Stents
chemical_list: D010100:Oxygen
keywords: Bilateral pulmonary artery banding; Hybrid approach; Hypoplastic left heart syndrome; PDA stenting; Pulmonary artery