Santoro G, Marino B, Giannico S, Cicini M P, Formigari R, De Zorzi A, Marcelletti C, Ragonese P
Ospedale Bambino Gesù, Roma.
G Ital Cardiol. 1993 May;23(5):459-65.
The bidirectional cavo-pulmonary anastomosis is a useful surgical procedure for patients with complex congenital heart disease characterized by univentricular physiology. This operation significantly increases effective pulmonary blood flow and rarely causes distortion of the main pulmonary arterial branches. The aim of this study was to evaluate the clinical and pathophysiologic consequences of bidirectional cavo-pulmonary anastomosis as well as the effectiveness of this surgical approach as a definitive palliation.
Clinical, hemodynamic and functional data of 12 patients who had undergone a bidirectional cavo-pulmonary anastomosis were reviewed. Each patient underwent clinical examination, chest x-ray, ambulatory EKG, bidimensional color-Doppler echocardiography, spirometric analysis, lung perfusion scintigraphy, cardiac catheterization and angiography, and stress test. Stress test data were compared to those obtained by a normal population of age-matched children.
After a mean follow-up interval of 28 +/- 20 months all patients showed a good clinical condition. Improved oxygenation, exercise tolerance and growth velocity were noted in all patients. Only 34% of patients showed abnormalities of chest x-ray, 29% manifested significant arrhythmias during ambulatory EKG and 20% showed abnormal ventricular function echocardiographically. Spirometry was normal in all patients, while lung perfusion scintigraphy was constantly abnormal with right/left and upper/lower perfusion mismatch. At cardiac catheterization a reduction of end diastolic volume (from 106.9 +/- 50.6 to 67.3 +/- 41.6 ml, p < 0.05) and end systolic volume (from 58.0 +/- 27.9 to 32.5 +/- 33.5 ml, p < 0.05) was noted. The functional evaluation of these patients was highly abnormal due to an impaired response to effort as evidenced by work time (p < 0.0001 vs normal) and stress-induced cardiovascular modifications.
On the basis of these findings, we suggest that: 1) bidirectional cavo-pulmonary anastomosis is useful as an intermediate step towards a Fontan procedure, as it improves the hemodynamic performance of the systemic ventricle; 2) conversely, bidirectional cavo-pulmonary anastomosis should not be considered a form of definitive palliation for complex congenital heart disease because in these patients the response to exercise remains unsatisfactory.
双向腔肺吻合术是一种用于治疗以单心室生理为特征的复杂先天性心脏病患者的有效外科手术。该手术显著增加了有效的肺血流量，且很少导致主肺动脉分支扭曲。本研究的目的是评估双向腔肺吻合术的临床和病理生理后果，以及这种手术方法作为最终姑息治疗的有效性。
回顾了12例接受双向腔肺吻合术患者的临床、血流动力学和功能数据。每位患者均接受了临床检查、胸部X线检查、动态心电图、二维彩色多普勒超声心动图、肺量计分析、肺灌注闪烁扫描、心导管检查和血管造影以及应激试验。将应激试验数据与年龄匹配的正常儿童群体的数据进行比较。
平均随访28±20个月后，所有患者临床状况良好。所有患者均出现氧合改善、运动耐量提高和生长速度加快。仅34%的患者胸部X线检查异常，29%的患者在动态心电图检查时出现明显心律失常，20%的患者超声心动图显示心室功能异常。所有患者肺量计检查正常，而肺灌注闪烁扫描持续异常，存在右/左和上/下灌注不匹配。心导管检查时发现舒张末期容积减少（从106.9±50.6ml降至67.3±41.6ml，p<0.05）和收缩末期容积减少（从58.0±27.9ml降至32.5±33.5ml，p<0.05）。这些患者的功能评估高度异常，因为工作时间（与正常相比p<0.0001）和应激诱导的心血管改变表明其对运动的反应受损。
基于这些发现，我们建议：1）双向腔肺吻合术作为迈向Fontan手术的中间步骤是有用的，因为它改善了体循环心室的血流动力学性能；2）相反，双向腔肺吻合术不应被视为复杂先天性心脏病的一种最终姑息治疗形式，因为在这些患者中，对运动的反应仍然不令人满意。
