Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
J Thorac Cardiovasc Surg. 2012 Mar;143(3):569-75. doi: 10.1016/j.jtcvs.2011.08.012. Epub 2011 Sep 9.
Management of pulmonary atresia with intact ventricular septum is challenging and depends on the severity of right ventricular hypoplasia. Clinical outcomes of biventricular repair seem favorable to univentricular palliation, but data on superiority of biventricular repair regarding exercise capacity are conflicting. We investigated the response to physical and pharmacologic stress in patients with surgically corrected pulmonary atresia with intact ventricular septum.
Sixteen patients (7 patients after univentricular palliation, age 11.8 ± 2.6 years; 7 patients after biventricular repair, age 12.9 ± 2.7 years; and 2 patients after 1.5 ventricular repair, age 12 and 19 years) underwent cardiopulmonary exercise test, dobutamine stress magnetic resonance imaging, and delayed contrast-enhanced magnetic resonance imaging.
The univentricular group showed impaired exercise capacity in contrast with normal exercise capacity in the biventricular group. Left ventricular ejection fraction increased in both groups. With dobutamine, left ventricular stroke volume increased only in the biventricular group (+11.3 ± 5.0 mL, P < .001) and not in the univentricular group (-0.04 ± 3.6 mL, P = .9). Heart rate increase was inadequate in the univentricular group. Maximum oxygen consumption and oxygen pulse were strongly correlated with left ventricular stroke volume during stress but not at rest. The results of the 2 patients after 1.5 ventricular repair were comparable to those of the univentricular group. No myocardial fibrosis was detected.
Impaired exercise capacity in children and adolescents with pulmonary atresia with intact ventricular septum after univentricular palliation is related to decreased cardiac reserve and inadequate chronotropic response. Young patients with pulmonary atresia with intact ventricular septum after biventricular repair show normal exercise capacity and cardiac reserve.
肺动脉瓣闭锁伴完整室间隔的处理具有挑战性，取决于右心室发育不良的严重程度。双心室修复的临床结果似乎优于单心室姑息治疗，但关于双心室修复在运动能力方面优势的数据存在冲突。我们研究了手术矫正的肺动脉瓣闭锁伴完整室间隔患者对体力和药物应激的反应。
16 名患者（7 名接受单心室姑息治疗，年龄 11.8±2.6 岁；7 名接受双心室修复，年龄 12.9±2.7 岁；2 名接受 1.5 心室修复，年龄 12 和 19 岁）接受了心肺运动试验、多巴酚丁胺应激磁共振成像和延迟对比增强磁共振成像。
与双心室组正常的运动能力相比，单心室组的运动能力受损。左心室射血分数在两组中均增加。多巴酚丁胺作用下，左心室每搏量仅在双心室组中增加（+11.3±5.0 毫升，P<0.001），而在单心室组中未增加（-0.04±3.6 毫升，P=0.9）。单心室组心率增加不足。最大摄氧量和氧脉搏在应激时与左心室每搏量密切相关，但在休息时无关。2 名接受 1.5 心室修复的患者的结果与单心室组相似。未检测到心肌纤维化。
单心室姑息治疗后肺动脉瓣闭锁伴完整室间隔的儿童和青少年运动能力受损与心脏储备降低和心动过速反应不足有关。双心室修复后的年轻肺动脉瓣闭锁伴完整室间隔患者表现出正常的运动能力和心脏储备。
