Lim D Scott, Peeler Benjamin B, Matherne G Paul, Kramer Christopher M
Department of Pediatrics, University of Virginia, Charlottesville, USA.
J Cardiovasc Magn Reson. 2008 Jul 6;10(1):34. doi: 10.1186/1532-429X-10-34.
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.
对于左心发育不全综合征（HLHS），在采用右心室至肺动脉导管改良的诺伍德手术进行一期手术后，肺动脉生长和心室功能障碍一直备受关注。我们报告了使用心血管磁共振成像（CMR）来确定并跟踪该队列中肺动脉生长和心室功能的经验。在一期姑息治疗后，术后1周和10周进行了连续的CMR检查，随后在4 - 6个月时进行了心导管检查。47例连续的HLHS（或其变异型）患者中有34例接受了一期姑息治疗。20例患者进行了连续的CMR检查。在各次检查之间，射血分数下降（58±9%对50±5%，p<0.05）。肺动脉在左侧生长（基线时为6±1mm，对4±1mm，p<0.05），但右侧生长不明显。这种趋势在术后4 - 6个月的心导管检查中持续存在，左肺动脉尺寸大于右肺动脉（8.8±2.2mm对6.7±1.9mm，p<0.05）。通过CMR检查，最初有5例存在肺动脉狭窄，在2个月时，有9例存在狭窄。9例中的3例在二期手术前接受了经皮介入治疗。在这个队列中，采用这种改良的一期姑息治疗后，肺动脉出现了合理的生长，尽管这种生长在左侧更为明显。连续研究表明该队列的心室功能恶化。CMR有助于检测肺动脉狭窄和右心室功能障碍。
