Lan Yueh-Tze, Chang Ruey-Kang, Drant Stacey, Odim Jonah, Laks Hillel, Wong Ah Lin, Allada Vivek
Division of Cardiology, Department of Pediatrics, UCLA Medical Center, Los Angeles, California, USA.
Am J Cardiol. 2002 Apr 15;89(8):959-63. doi: 10.1016/s0002-9149(02)02246-4.
Neonates with double-inlet left ventricle or tricuspid atresia with transposed great arteries and a bulboventricular foramen (BVF) area <2 cm(2)/m(2) develop BVF obstruction. This study examined the outcome of neonates with BVF area between 1 and 2 cm(2)/m(2) whose BVF was bypassed after the neonatal period. We reviewed 29 neonates with double-inlet left ventricles (n = 18) or tricuspid atresia (n = 11) and transposed great arteries. The study group consisted of 9 patients with neonatal BVF areas of 1 to 2 cm(2)/m(2) who did not undergo repair of the BVF obstruction as a neonate. The comparison group consisted of 8 "ideal" patients without BVF obstruction. Precavopulmonary shunt data from cardiac catheterization and echocardiogram and outcomes of the cavopulmonary shunt were compared. Study group patients developed a mild BVF gradient (18 +/- 10 mm Hg by cardiac catheterization) by a mean of 7 months. Left ventricular wall thickness, however, remained in the normal range (4.2 +/- 0.3 mm) and was not statistically different from the comparison group (4.1 +/- 0.4 mm). No difference was found in the precavopulmonary mean pulmonary artery pressure (15 +/- 5 vs 15 +/- 6 mm Hg), transpulmonary gradient (8 +/- 4 vs 8 +/- 5 mm Hg), and left ventricular end-diastolic pressure (7 +/- 2 vs 8 +/- 3 mm Hg). One patient in the study group died from respiratory syncytial virus pneumonia while awaiting cavopulmonary shunt. Neither group had mortality from the cavopulmonary shunt. The lengths of hospital stay were comparable (8.3 +/- 3.7 vs 8.9 +/- 6.0 days). Thus, neonates with BVF area between 1 and 2 cm(2)/m(2) develop mild but hemodynamically insignificant BVF gradient by 7 months of age. This group of patients can be managed safely with relief of BVF obstruction later in infancy.
患有双入口左心室或三尖瓣闭锁合并大动脉转位且球室孔（BVF）面积<2 cm²/m²的新生儿会发生BVF梗阻。本研究调查了新生儿期后BVF被绕过的BVF面积在1至2 cm²/m²之间的新生儿的预后情况。我们回顾了29例患有双入口左心室（n = 18）或三尖瓣闭锁（n = 11）合并大动脉转位的新生儿。研究组由9例新生儿期BVF面积为1至2 cm²/m²且未在新生儿期进行BVF梗阻修复的患者组成。对照组由8例无BVF梗阻的“理想”患者组成。比较了心导管检查和超声心动图的腔肺分流数据以及腔肺分流的结果。研究组患者平均在7个月时出现轻度BVF梯度（心导管检查为18±10 mmHg）。然而，左心室壁厚度仍保持在正常范围内（4.2±0.3 mm），与对照组（4.1±0.4 mm）无统计学差异。腔肺分流前平均肺动脉压（15±5 vs 15±6 mmHg）、跨肺梯度（8±4 vs 8±5 mmHg）和左心室舒张末期压力（7±2 vs 8±3 mmHg）均无差异。研究组中有1例患者在等待腔肺分流时死于呼吸道合胞病毒肺炎。两组均无腔肺分流导致的死亡。住院时间相当（8.3±3.7 vs 8.9±6.0天）。因此，BVF面积在1至2 cm²/m²之间的新生儿在7个月大时会出现轻度但血流动力学上无显著意义的BVF梯度。这组患者在婴儿期后期进行BVF梗阻解除后可得到安全管理。
