先天性心脏病肺动脉高压X线和血液动力学、病理组织学的对照研究

A CORRELATIVE STUDY ON RADIOGRAPHIC FINDINGS WITH HEMODYNAMIC AND HISTOPATHOLOGIC CHANGES OF PULMONARY ARTERIAL HYPERTENSION IN CONGENITAL HEART DISEASE

本文分析１００例左向右分流先天性心脏病肺动脉高压的Ｘ线平片所见，并和血液动力学、病理组织学检查结果进行了对照研究，首次明确提出了Ｘ线分级标准，并着重阐述了Ⅲｂ的诊断标准：二尖瓣型心脏，轻度增大或不大，仅有右心室增大或以右心室增大为主，中外带肺血管纹理明显变细、扭曲，肺血无明显增多甚至减少，肺野清亮。Ⅲｂ反映肺动脉压力和全肺阻力重度升高，肺血管病变为Ⅲ级或Ⅳ级，属不可复阶段，不宜手术，预后较差。

Radiographic findings of 100 cases of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) with left to right shunt were analysed, and those findings were correlated with the hemodynamic and histopathologic changes. Based on the present study a radiographic classification system of PAH in CHD was firstly described with emphasis on the diagnostic standard of Grade Ⅲb PHA and its clinical significance.The heart with mitral configuration was not or mildly enlarged,only or mainly with right ventricular involvement. Marked and diffused narrowing and twisting of vascular markings on the middle to peripheral lung fields with increased transparency showing pulmonary oligemia, and even no signs of increased pulmonary arterial blood flow were found. Grade Ⅲb reflects markedly elevated pulmonary arterial pressure and vascular resistance as well as Grade Ⅲ or Ⅳ histopathologic changes of the pulmonary vasculature.The radiographic findings of Grade Ⅲb indicate irreversible morphologic lesions of the pulmonary vasculature.Patients with Ⅲb PAH is not suitable for surgery and is usually with poor prognosis.