Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
复制标题
室间隔缺损和主要主肺侧支动脉肺闭锁中肺循环的生长和发育。
DOI：
10.1136/hrt.44.1.14
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发表时间：
1980
影响因子：
--
通讯作者：
F. Macartney
中科院分区：
文献类型：
--
作者：
S. G. Haworth;F. Macartney
总结在11例肺动脉闭锁伴室间隔缺损和主要肺动脉侧支动脉的尸检标本中，所有病例均存在中央肺动脉，由侧支动脉供血，侧支动脉与肺叶肺动脉或较少见的中央肺动脉闭合。这些正常连接的肺动脉经常只灌注少数支气管肺段。其余部分与侧支动脉相连，侧支动脉在肺内与具有正常连接血管结构的血管相连，并以正常方式与腺泡前和腺泡内气道分支，以灌注肺泡毛细血管床。这些侧支动脉和与之相连的肺内血管在肉眼或显微镜下与肺内的中央肺动脉或其分支均无连接。无论近端连接的类型如何，肺段动脉通常都异常小。狭窄发生在58- 8%的侧支动脉中，通常是主动脉和肺之间的厚壁狭窄肌肉部分。显微镜下，定量形态学分析显示，在呼吸单元内，肺循环未能正常生长，除了最年轻的患者在12天龄时死亡。结构变化通常与肺血流量减少一致;只有3例病例包含显示肺动脉高压结构证据的任何肺段。这些解剖学和组织学的结果进行了讨论，在胚胎学，命名，肺动脉闭锁与室间隔缺损和主要的先天性肺动脉侧支动脉的自然史。结构的结果提供了一个合理的基础，这种情况下的临床管理。
SUMMARY In 11 necropsy specimens of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, central pulmonary arteries were present in all cases, supplied by collateral arteries which anastomosed with a lobar pulmonary artery or, less commonly, with a central pulmonary artery. These normally connected pulmonary arteries frequently perfused only a minority of bronchopulmonary segments. The remaining segments were connected to collateral arteries which, within the lung, were continuous with vessels which had the structure of normally connected vessels and branched with the pre-and intra-acinar airways in a normal manner to perfuse the alveolar capillary bed. These collateral arteries and the intrapulmonary vessels with which they anastomosed had no connection, either macroscopically or microscopically, with either the central pulmonary arteries or their branches within the lung. Irrespective ofthe type ofproximal connection, the segmental pulmonary arteries were generally abnormally small. Stenoses occurred in 58-8 per cent of collateral arteries, usually as a thick-walled narrowed muscular section between the aorta and lung. Microscopically, quantitative morphometric analysis showed that within the respiratory unit the pulmonary circulation had failed to grow normally in all save the youngest patient dying at 12 days of age. The structural changes were usually consistent with a reduction in pulmonary blood flow; only three cases contained any segments of lung showing structural evidence of pulmonary hypertension. These anatomical and histological findings are discussed in relation to the embryology, nomenclature, and natural history of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The structural findings provide a rational basis for the clinical management of this condition.