Olivet R T, Rupp W M, Telander R L, Kaye M P
J Pediatr Surg. 1978 Jun;13(3):231-5. doi: 10.1016/s0022-3468(78)80392-3.
Despite surgical treatment, congenital foramen of Bochdalek diaphragmatic hernia continues to carry a high mortality rate due to associated respiratory insufficiency. We studied the pathologic and hemodynamic changes that result from this condition in lambs. Surgical creation of diaphragmatic hernias in fetal lambs was performed in utero with subsequent delivery by cesarean section near term. Cardiac catheterization was performed on these newborn lambs immediately after delivery, before and after surgical repair of the defect. Data from five of these lambs were compared to data obtained from seven normal lambs. Pulmonary hypertension was found only in lambs with hernias. Pulmonary artery pressures and the mean ratio of pulmonary to systemic resistance were higher in experimental lambs. Oxygen saturation and average cardiac index were higher in normals. Lambs in the experimental group, but not in the control group, had large left-to-right shunts at the level of the ductus arteriosus in the early neonatal period. Arterial oxygen saturations were consistently low with no differences in pre- and post-ductal samples, which suggests right-to-left shunting at the atrial level or physiologic shunting across the unexpanded lungs. No further expansion of the hypoplastic lungs occurred following decompression by surgical hernia repair, but transient hemodynamic improvement was noted in some cases. Surgical ligation of the ductus arteriosus did not significantly alter the clinical condition of the lambs. Lungs in lambs with hernias were grossly abnormal, the left appearing more hypoplastic than the right.
尽管进行了手术治疗，但由于伴有呼吸功能不全，先天性Bochdalek孔膈肌疝的死亡率仍然很高。我们研究了这种情况在羔羊中引起的病理和血流动力学变化。在子宫内对胎羊进行膈肌疝手术造模，随后在接近足月时进行剖宫产分娩。在这些新生羔羊分娩后立即、在缺损手术修复前后进行心脏导管检查。将其中五只羔羊的数据与七只正常羔羊的数据进行比较。仅在患有疝的羔羊中发现肺动脉高压。实验组羔羊的肺动脉压和肺与体循环阻力的平均比值更高。正常羔羊的血氧饱和度和平均心脏指数更高。实验组羔羊在新生儿早期动脉导管水平有较大的左向右分流，而对照组没有。动脉血氧饱和度持续较低，导管前和导管后样本无差异，这表明在心房水平存在右向左分流或未扩张肺脏存在生理性分流。手术修复疝减压后，发育不全的肺脏没有进一步扩张，但在某些情况下观察到短暂的血流动力学改善。动脉导管结扎术并未显著改变羔羊的临床状况。患有疝的羔羊的肺脏明显异常，左侧比右侧发育更差。
