Vascular remodeling process in reversibility of pulmonary arterial hypertension secondary to congenital heart disease
Vascular remodeling process in reversibility of pulmonary arterial hypertension secondary to congenital heart disease
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DOI：
10.1078/0344-0338-00457
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发表时间：
2003-01-01
影响因子：
2.8
通讯作者：
Capelozzi, VL
中科院分区：
文献类型：
--
作者：
Egito, EST;Aiello, VD;Capelozzi, VL
采用形态计量学方法分析了26例6 ~ 160个月先天性心脏病合并肺动脉高压患儿肺活检标本的肺血管重构过程。修复性手术22例，姑息性手术4例。1例患者术后死亡，4例患者出院后死亡。血管重塑检查显示了特征性病理学图像：明显的中膜增厚伴胶原含量增加（纤维化），无显著的动脉内膜增生。在平均44个月的随访中，72%的幸存者没有症状，没有药物治疗。经超声心动图诊断，22%的患者显示有肺动脉高压。上述特征性病理学特征发生在38%的术后死亡或肺动脉高压的患者中。这些发现有助于识别结果不符合经典I级和II级变化预期的高风险组。我们的结论是，孤立的中膜增厚的存在并不能确保生存或正常的术后肺动脉压在后期随访，胶原蛋白含量可以是一个更好的参考良好的结果。建议在发生严重的中膜纤维化之前进行早期心内修复。
Pulmonary vascular remodeling process was analyzed using morphometry in lung biopsy specimens taken from 26 children, aged 6 to 160 months, who had congenital heart disease and significant pulmonary arterial hypertension. Reparative surgery was performed in 22 patients and palliative surgery was performed in four patients. One patient expired postoperatively and four others after hospital discharge. Vascular remodeling examination revealed a characteristic pathological picture: pronounced medial thickening with increased collagen content (fibrosis), without significant arterial intimal proliferation. At a mean follow-up of 44 months, 72% of the survivors were asymptomatic with no medication. Diagnosed by echocardiogram, 22% of these patients were shown to have pulmonary arterial hypertension. The characteristic pathological features described above occurred in 38% of the patients who either expired or had pulmonary hypertension postoperatively. These findings were an aid to identifying a high risk group in which the outcome does not meet expectations for the classical grade I and II changes. We concluded that the presence of isolated medial thickening does not ensure either survival or a normal postoperative pulmonary arterial pressure at late follow-up and that the collagen content can be a better reference for good outcome. Early intracardiac repair is recommended before the development of significant medial fibrosis.