Cardiovascular magnetic resonance of pulmonary artery growth and ventricular function after Norwood procedure with Sano

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Cardiovascular magnetic resonance of pulmonary artery growth and ventricular function after Norwood procedure with Sano modification D Scott Lim*1, Benjamin B Peeler2, G Paul Matherne1 and Christopher M Kramer3 Address: 1Department of Pediatrics, University of Virginia, Charlottesville, USA, 2Department of Surgery, University of Virginia, Charlottesville, USA and 3Departments of Medicine & Radiology, University of Virginia, Charlottesville, USA Email: D Scott Lim* - [email protected]; Benjamin B Peeler - [email protected]; G Paul Matherne - [email protected]; Christopher M Kramer - [email protected] * Corresponding author

Published: 6 July 2008 Journal of Cardiovascular Magnetic Resonance 2008, 10:34 34

doi:10.1186/1532-429X-10-

Received: 25 March 2008 Accepted: 6 July 2008

This article is available from: http://www.jcmr-online.com/content/10/1/34 © 2008 Lim et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract For hypoplastic left heart syndrome (HLHS), there have been concerns regarding pulmonary artery growth and ventricular dysfunction after first stage surgery consisting of the Norwood procedure modified with a right ventricle-to-pulmonary artery conduit. We report our experience using cardiovascular magnetic resonance (CMR) to determine and follow pulmonary arterial growth and ventricular function in this cohort. Following first stage palliation, serial CMR was performed at 1 and 10 weeks post-operatively, followed by cardiac catheterization at 4 – 6 months. Thirty-four of 47 consecutive patients with HLHS (or its variations) underwent first stage palliation. Serial CMR was performed in 20 patients. Between studies, ejection fraction decreased (58 ± 9% vs. 50 ± 5%, p < 0.05). Pulmonary artery growth occurred on the left (6 ± 1 mm vs. 4 ± 1 mm at baseline, p < 0.05) but not significantly in the right. This trend continued to cardiac catheterization 4–6 months post surgery, with the left pulmonary artery of greater size than the right (8.8 ± 2.2 mm vs. 6.7 +/- 1.9 mm, p < 0.05). By CMR, 5 had pulmonary artery stenoses initially, and at 2 months, 9 had stenoses. Three of the 9 underwent percutaneous intervention prior to the second stage procedure. In this cohort, reasonable growth of pulmonary arteries occurred following first stage palliation with this modification, although that growth was preferential to the left. Serial studies demonstrate worsening of ventricular function for the cohort. CMR was instrumental for detecting pulmonary artery stenosis and right ventricular dysfunction.

Background Hypoplastic left heart syndrome is a congenital cardiac malformation involving hypoplasia of the ascending aorta, aortic valve atresia or stenosis, a hypoplastic left

ventricle, and mitral atresia or hypoplasi