Yamaki S, Abe A, Tabayashi K, Endo M, Mohri H, Takahashi T
Department of Cardiology, Katta General Hospital, Shiroishi, Japan.
Ann Thorac Surg. 1998 Nov;66(5):1565-70. doi: 10.1016/s0003-4975(98)00779-6.
Among 120 infants less than 12 months of age who had lung biopsy and autopsy, 20 were inoperable because of severe irreversible pulmonary vascular disease.
The infants were classified into three groups. Group 1 comprised 6 patients who showed complete obstruction of the small pulmonary arterial lumen and atrophy of the peripheral arterial media and who were considered to have absolute operative contraindications. Group 2 comprised 6 patients who had no pathologic findings of absolute operative contraindication and had an index of pulmonary vascular disease of more than 2.2. They were isolated as having advanced plexogenic pulmonary arteriopathy. Group 3 comprised 8 patients who had extremely thickened media of small pulmonary arteries, with abnormally thickened media extending into the small peripheral arteries characterized by extremely narrow lumina and medial thickness exceeding luminal diameter.
Six of the 9 patients in whom operative repair was abandoned on the basis of preoperative or intraoperative lung biopsy are still alive. Of the 11 patients who underwent operation without biopsy, none survived.
Preoperative or intraoperative lung biopsy and assessment of arteriopathy based on the above criteria are recommended in all patients in whom fatal pulmonary vascular disease is suspected.
在120例年龄小于12个月且接受了肺活检和尸检的婴儿中，20例因严重不可逆的肺血管疾病而无法进行手术。
将这些婴儿分为三组。第1组包括6例患者，其小肺动脉管腔完全阻塞，外周动脉中层萎缩，被认为有绝对手术禁忌证。第2组包括6例无绝对手术禁忌证的病理表现且肺血管疾病指数大于2.2的患者。他们被单独列为患有晚期丛状肺血管病。第3组包括8例小肺动脉中层极度增厚的患者，异常增厚的中层延伸至外周小动脉，其特点是管腔极窄，中层厚度超过管腔直径。
9例根据术前或术中肺活检放弃手术修复的患者中有6例仍存活。11例未进行活检即接受手术的患者均未存活。
对于所有怀疑患有致命性肺血管疾病的患者，建议进行术前或术中肺活检，并根据上述标准评估血管病。
