Hybrid approach for hypoplastic left heart syndrome and its variants: the fate of the pulmonary arteries

• Published in: European journal of cardio-thoracic surgery Vol. 46; no. 1; pp. 14 - 19
• Main Authors: Dave, Hitendu, Rosser, Barbara, Knirsch, Walter, Hübler, Michael, Prêtre, René, Kretschmar, Oliver
• Format: Journal Article
• Language: English
• Published: Germany Oxford University Press 01-07-2014
• Subjects: Atrial Septum - surgery; Blood Pressure; Blood Vessel Prosthesis; Cardiac Catheterization; Ductus Arteriosus, Patent - surgery; Follow-Up Studies; Fontan Procedure; Humans; Hypoplastic Left Heart Syndrome - mortality; Hypoplastic Left Heart Syndrome - surgery; Infant; Infant, Newborn; Norwood Procedures; Oxygen - blood; Pulmonary Artery - surgery; Reoperation; Retrospective Studies; Stents; Hybrid approach; Bilateral pulmonary artery banding; Hypoplastic left heart syndrome; PDA stenting; Pulmonary artery
• ISSN: 1010-7940; 1873-734X
• DOI: 10.1093/ejcts/ezt604

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) mm2/m2 (P = 0.01). The comparable lower lobe indices were 149 (103–333) and 137 (45–178) mm2/m2 (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.