Juaneda E, Haworth S G
Am J Cardiol. 1984 Dec 1;54(10):1314-20. doi: 10.1016/s0002-9149(84)80089-2.
Pulmonary vascular structure was analyzed using quantitative morphometric techniques in lung biopsy (n = 17) and/or postmortem specimens (n = 18) from 23 patients aged 18 days to 13 years, with truncus arteriosus (TA) type I or II. All 14 patients younger than age 1 year, showed abnormal extension of muscle and an increase in pulmonary arterial medial thickness (p less than 0.01 in 18 of 24 biopsy and autopsy specimens), whereas 6 showed intimal proliferation. Structural abnormalities were usually potentially reversible, even in 5 infants with a pulmonary arteriolar resistance of greater than or equal to 8 units X m2. Abnormalities were more severe in 9 patients aged 3.5 to 13 years, 6 with medial atrophy and 4 with pulmonary arteriolar resistance of greater than 8 units X m2 that also showed intimal fibrosis, with or without plexiform lesions or hyalinized arteries. Findings indicate that biopsy specimens are helpful in assessing the severity of pulmonary vascular disease, and are representative of the entire pulmonary vascular bed (avoiding the lingula); however, interpretation can be difficult if a biopsy specimen is taken of only the peripheral intraacinar arteries, distal to the most damaged vessels. An intracardiac repair is recommended by 6 months, at which time severe pulmonary arterial medial hypertrophy and intimal proliferation can be expected to prejudice the repair.
采用定量形态计量学技术，对23例年龄在18天至13岁、患有I型或II型动脉干（TA）的患者的肺活检标本（n = 17）和/或尸检标本（n = 18）进行肺血管结构分析。所有14例1岁以下的患者均表现出肌肉异常延伸和肺动脉中层厚度增加（24例活检和尸检标本中有18例p<0.01），而6例表现为内膜增生。结构异常通常可能是可逆的，即使在5例肺小动脉阻力大于或等于8单位X平方米的婴儿中也是如此。9例年龄在3.5至13岁的患者异常更为严重，6例有中层萎缩，4例肺小动脉阻力大于8单位X平方米，还表现出内膜纤维化，伴有或不伴有丛状病变或玻璃样变动脉。研究结果表明，活检标本有助于评估肺血管疾病的严重程度，并且代表整个肺血管床（避开舌叶）；然而，如果活检标本仅取自最受损血管远端的外周腺泡内动脉，则可能难以解释。建议在6个月时进行心内修复，此时预计严重的肺动脉中层肥厚和内膜增生会影响修复。
