Haemodynamic and anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect - including a case of persistent fifth aortic arch.
Haemodynamic and anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect - including a case of persistent fifth aortic arch.
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肺动脉闭锁伴室间隔缺损肺血供的血流动力学和解剖学特征——包括一例持续性第五主动脉弓病例。
DOI：
10.1136/hrt.36.11.1049
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发表时间：
1974
影响因子：
--
通讯作者：
P. Deverall
中科院分区：
文献类型：
--
作者：
F. Macartney;O. Scott;P. Deverall
报告8例先天性肺动脉闭锁合并室间隔缺损患儿。通过主动脉造影和多次选择性注射造影剂到主肺吻合口或肺动脉中来建立肺血供的解剖结构。所有患者均显示有中央肺动脉（源自胚胎学第六主动脉弓）。在4例（I组）中，中央肺动脉仅通过永存动脉导管供血（3例随后进行了外科肺动脉分流术）。在3例（第2组）中，中央肺动脉仅通过主要的肺动脉-肺动脉侧支动脉供应，在1例（第3组）中，肺血供来自侧支动脉和持续的左侧第五主动脉弓。在肺动脉-肺动脉吻合口的肺动脉端，无论其性质如何，每例患者的Sybrine压差均为28 ~ 8I mmHg。组I所有患者和组2中的I例患者均为单灶性肺血供，即第六主动脉弓与所有系统性肺血供来源一致，并为所有系统性肺血供来源提供单一病灶。肺阻力相对于第六主动脉弓压力的范围为1.4 ~ 6.4单位M2。其余患者为多灶性肺动脉供血，即一条或多条肺动脉侧支动脉独立于第六主动脉弓提供肺动脉血供，该组患者相对于第六主动脉弓的最小肺动脉阻力范围为3-9至I2-I5单位M2。后一个数字表明存在严重的肺血管疾病。因此
Eight children with congenital pulmonary atresia with ventricular septal defect were investigated. The anatomy of pulmonary blood supply was established by aortography and multiple selective injections of contrast medium into aortopulmonary anastomoses or pulmonary arteries. All patients were shown to have central pulmonary arteries (derived from the embryological sixth aortic arches). In 4 (group I) the central pulmonary arteries had been supplied via a persistent ductus arteriosus alone (3 subsequently had surgical aortopulmonary shunts). In 3 (group 2), the central pulmonary arteries were supplied via major aortopulmonary collateral arteries alone, and in I patient (group 3) pulmonary blood supply was derived both from collateral arteries and a persistent leftfifth aortic arch. Systolic pressure gradients of 28 to 8I mmHg were demonstrated in each patient at the pulmonary artery end of the aortopulmonary anastomosis, whatever its nature. All patients in group I, and I patient in group 2 had unifocalpulmonary blood supply, i.e. the sixth aortic arch asasto,nosed with, and provided a single focusfor, all systemic sources of pulmonary blood supply. Pulmonary resistance relative to sixth aortic arch pressure rangedfrom 1`4 to 6.4 units M2. The remaining patients had multifocal pulmonary blood supply, i.e. one or more aortopulmonary collateral arteries provided pulmonary blood supply independent of the sixth aortic arch. In this group the minimum pulmonary resistance relative to the sixth aortic arch rangedfrom 3-9 to I2-i5 units M2. The latterfigure suggested the presence of severe pulmonary vascular disease. Thus