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[胎儿循环持续存在。两例临床及血管造影评估。文献综述(作者译)]

[Persistence of fetal circulation. Clinical and angiographic evaluation of two cases. Review of the literature (author's transl)].

作者信息

Pieri D, Calcaterra G, Grillo R, Donzelli M, Muscarella S, Sperandeo V

出版信息

G Ital Cardiol. 1981;11(12):2211-26.

PMID:7346316
Abstract

Persistent fetal circulation ("PFC") is a condition characterized by perduring after birth of fetal hemodynamic pattern: persistence of high pulmonary vascular resistance then occurs. "PFC" is a high mortality syndrome recently recognized as a cause of severe cyanosis and tachypnea in full-term newborns. Perinatal hypoxia, hyperviscosity, acidosis etc, may be also responsible of a persistence of high pulmonary vascular resistance. The increased right heart pressures cause a right to left shunt across the ductus arteriosus and foramen ovale not yet completely closed at birth. In this paper the clinical and angiographic evaluation of two full-term newborns, males, 5 days and 5 months old, are described, who presented with tachypnea, cyanosis and heart failure. Moreover we want to make a critical review of medical literature about this subject. The electrocardiogram of both patients showed right ventricular hypertrophy and myocardial ischemia. The chest roentgenograms showed cardiac enlargement and increased pulmonary vascular markings. Lung and central nervous system disorders were excluded by clinical and instrumental exams. Both patients underwent cardiac catheterization and angiocardiography in order to exclude a congenital heart disease. There was just evidence of pulmonary vascular resistance and right to left shunt at ductal and atrial level. O2 100% administration in one patient determined a decrease in pulmonary resistance and the shunting abolition. Clinical and hemodynamic patterns of this syndrome are very different. There are "simple forms" with normal myocardial function and "complex" higher mortality forms with evidence of cardiac involvement. It is often difficult to distinguish such forms from the other causes of central cyanosis and/or cardiac failure in the newborn as transposition of the great arteries, total anomalous pulmonary venous connection: the individualization of the syndrome then may be impossible without cardiac catheterization. A correct diagnosis for a precocious and reasonable treatment are extremely important for the survival of such critical newborns.

摘要

持续性胎儿循环(“PFC”)是一种以胎儿出生后血流动力学模式持续存在为特征的病症:随后会出现高肺血管阻力持续存在的情况。“PFC”是一种高死亡率综合征,最近被确认为足月新生儿严重青紫和呼吸急促的一个病因。围产期缺氧、高粘滞血症、酸中毒等也可能是高肺血管阻力持续存在的原因。右心压力升高导致血液经动脉导管和出生时尚未完全闭合的卵圆孔发生右向左分流。本文描述了两名足月新生儿(男性,分别为5天和5个月大)的临床和血管造影评估情况,这两名新生儿均表现出呼吸急促、青紫和心力衰竭。此外,我们还想对关于该主题的医学文献进行批判性综述。两名患者的心电图均显示右心室肥厚和心肌缺血。胸部X线片显示心脏扩大和肺血管纹理增多。通过临床和器械检查排除了肺部和中枢神经系统疾病。两名患者均接受了心导管检查和心血管造影,以排除先天性心脏病。仅发现有肺血管阻力以及导管和心房水平的右向左分流。对其中一名患者给予100%氧气吸入后,肺阻力降低,分流消失。该综合征的临床和血流动力学模式差异很大。存在心肌功能正常的“简单形式”以及有心脏受累证据、死亡率更高的“复杂形式”。通常很难将这些形式与新生儿中心性青紫和/或心力衰竭的其他病因(如大动脉转位、完全性肺静脉异位连接)区分开来:因此,如果不进行心导管检查,可能无法鉴别该综合征。对于这些病情危急的新生儿的存活而言,正确诊断以便进行早熟且合理的治疗极为重要。

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