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Pediatr Cardiol. 2013 Apr;34(4):924-30. doi: 10.1007/s00246-012-0576-1. Epub 2012 Nov 15.

Intact right ventricle-pulmonary artery shunt after stage 2 palliation in hypoplastic left heart syndrome improves pulmonary artery growth.

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  • 1Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, Morgan Stanley Children's Hospital of New York-Presbyterian, 3959 Broadway, 2 North, New York, NY 10032, USA.


For patients with hypoplastic left heart syndrome who have undergone the Norwood procedure with a right ventricle-pulmonary artery (RV-PA) shunt, the shunt can either be removed or left intact at the time of the stage 2 procedure. This study aimed to determine the effects of an intact shunt on pulmonary artery growth and clinical outcomes after the stage 2 procedure. A retrospective review of patients who underwent Norwood with an RV-PA shunt from 2005 to 2010 was performed. Catheterization data, echocardiographic data, postoperative outcome variables, and mortality data were collected. Pulmonary artery size was measured at pre-stage 2 and pre-Fontan catheterizations using the Nakata Index and the McGoon Ratio. Of the 68 patients included in the study, 48 had the shunt removed at the time of stage 2 (group 1), and 20 had the shunt left intact (group 2). The two groups did not differ in terms of pre-stage 2 hemodynamics or pulmonary artery size. After stage 2, group 2 had higher oxygen saturations. The two groups did not differ regarding duration of chest tube drainage, length of hospital stay, need for unplanned interventions, or mortality. Before Fontan, the group 2 patients had higher superior vena cava (SVC) pressures and more venovenous collaterals closed. There was increased pulmonary artery growth between the pre-stage 2 and pre-Fontan catheterizations in group 2 using both the Nakata Index (+148.5 vs -52.4 mm(2)/m(2); p = 0.01) and the McGoon Ratio (+0.36 vs +0.01; p = 0.01). These findings indicate that patients with an intact RV-PA shunt after stage 2 have greater pulmonary artery growth than patients with the shunt removed, with no increased risk of complications.

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