Division of Cardiovascular-Thoracic Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Children's Memorial Hospital, Chicago, IL 60614, USA.
J Thorac Cardiovasc Surg. 2010 Jun;139(6):1387-1394.e3. doi: 10.1016/j.jtcvs.2010.02.024. Epub 2010 Apr 14.
Surgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome.
Our cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990-2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0-4 years), and the median weight was 3.5 kg (range, 1.3-15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy.
There were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 +/- 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days).
Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.
全肺静脉异常连接的手术修复与显著的死亡率和发病率相关，尤其是在单心室生理患者中。本研究分析了一家机构全肺静脉异常连接手术修复的结果，以确定阳性结果的趋势和指标。
我们的心脏手术数据库确定了 100 例接受全肺静脉异常连接手术修复的患者（1990-2008 年）：心上型（52 例）、心内型（15 例）、心下型（23 例）和混合型（10 例）。修复时的中位年龄为 14.6 天（范围 0-4 岁），中位体重为 3.5 公斤（范围 1.3-15 公斤）。患者分为两组：双心室（n=83）或单心室（n=17）生理。除 1 例单心室生理患者外，其余均为异构综合征（94%），17 例患者中有 13 例为心上型解剖结构。
共有 12 例手术死亡（双心室组 4 例[5%]，单心室组 8 例[47%]，P<.01）和 9 例晚期死亡（双心室组 6 例[7%]，单心室组 3 例[18%]，P<.05）。全肺静脉连接类型死亡的是心上型（12/52；23.1%）、心内型（1/15；6.7%）、心下型（3/23；13.0%）和混合型（5/10；50%）。双心室组 22 例（27%）和单心室组 7 例（41%）存在肺静脉梗阻（P=0.25）。肺静脉梗阻患者死亡率为 9/29（31%），非肺静脉梗阻患者死亡率为 12/71（17%）（P=0.23）。38 例患者采用深低温停循环（双心室组 27 例，27.5%；单心室组 11 例，64.7%）。深低温停循环平均时间为 31.4+/-10.7 分钟（组间无显著差异，P=不显著）。术后中位住院时间为 11 天（范围 0-281 天）。19 例患者因肺静脉狭窄需要再次手术（双心室组 14 例，单心室组 5 例，P=0.045）；再次手术的中位时间为 104 天（范围 4-753 天）。
具有双心室解剖结构的全肺静脉异常连接患者有良好的结果。具有单心室解剖结构的患者死亡率更高，并且存在需要再次手术的肺静脉狭窄风险增加。混合型全肺静脉异常连接患者的死亡率最高。
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