Haworth S G, Reid L
Br Heart J. 1977 Jan;39(1):80-92. doi: 10.1136/hrt.39.1.80.
Quantitative morphometric techniques have been applied to the injected and inflated lung and to the heart in 9 infants with total anomalous pulmonary venous return dying with obstruction to pulmonary venous return. In 5 (mean age at death 55 days) pge 20 days) to an infradiaphragmatic site. Structural changes were present in the pulmonary circulation in all patients, even in the youngest, an 8-day-old child. In both types of total anomalous pulmonary venous return increased arterial muscularity was severe, as shown by increase in wall thickness and by extension of muscle into smaller and more peripheral arteries than normal; these changes tended to increase with age. Where the pulmonary venous blood drained to a supradiaphragmatic site, the severity of arterial medial hypertrophy correlated inversely with the magnitude of the pulmonary: systemic flow ratio, increasing as the pulmonary blood flow fell. Vein wall thickness was increased and in all but the youngest child the veins were 'arterialised'. At the lung periphery the arteries and alveoli appeared to have multiplied normally. Arterial size varied according to whether pulmonary venous blood drained above or below the diaphragm; the diameter of pre- and intra-acinar arteries was increased only in cases where the pulmonary venous return drained to a supradiaphragmatic site, being normal when it drained to an infradiaphragmatic site. In the heart the left ventricle was of normal size in all but one case. Dilation and severe hypertrophy of the right ventricle and septum were present only in cases of drainage to a supradiaphragmatic site. In the older patients with the latter anomaly dilation of the pulmonary arteries and right ventricle suggested that a large left-to-right shunt had preceded the onset of obstruction to pulmonary venous return and that the more severe right ventricular and septal hypertrophy in these cases might be the result of a longer duration of pulmonary hypertension. In contrast, in total anomalous pulmonary venous return to an infradiaphragmatic site it appears that obstruction to pulmonary venous return develops soon after birth and prevents a large increase in pulmonary blood flow, and thus neither the pulmonary arteries nor the right ventricle become dilated. In infants with total anomalous pulmonary venous return and obstruction to pulmonary venous return, it is striking how rapidly the pulmonary circulation develops new muscle.
对9例因肺静脉回流梗阻死亡的完全性肺静脉异位引流婴儿的注入和充气肺以及心脏应用了定量形态测量技术。在5例(平均死亡年龄55天)中,前列腺素E2在20天内注入到膈下部位。所有患者的肺循环均存在结构改变,即使是最年幼的8天大婴儿也不例外。在两种类型的完全性肺静脉异位引流中,动脉肌层增厚均很严重,表现为管壁厚度增加以及肌层延伸至比正常更小且更外周的动脉;这些改变往往随年龄增长而加重。当肺静脉血引流至膈上部位时,动脉中层肥厚的严重程度与肺循环:体循环血流比值呈负相关,随着肺血流量下降而增加。静脉壁厚度增加,除最年幼的婴儿外,所有婴儿的静脉均已“动脉化”。在肺周边,动脉和肺泡似乎正常增殖。动脉大小根据肺静脉血是引流至膈上还是膈下而有所不同;仅在肺静脉回流引流至膈上部位的病例中,腺泡前和腺泡内动脉直径增加,而引流至膈下部位时则正常。在心脏方面,除1例病例外,所有病例的左心室大小均正常。仅在引流至膈上部位的病例中出现右心室和室间隔扩张及严重肥厚。在患有后一种异常的年长患者中,肺动脉和右心室扩张表明在肺静脉回流梗阻发生之前存在大量左向右分流,并且这些病例中更严重的右心室和室间隔肥厚可能是肺动脉高压持续时间更长的结果。相比之下,在肺静脉完全性异位引流至膈下部位的病例中,似乎肺静脉回流梗阻在出生后不久就会发生,并阻止肺血流量大幅增加,因此肺动脉和右心室均不会扩张。在患有完全性肺静脉异位引流且肺静脉回流梗阻的婴儿中,肺循环迅速形成新肌肉的情况令人惊讶。