TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE - NATURAL-HISTORY AND HEMODYNAMIC CONSIDERATIONS
TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE - NATURAL-HISTORY AND HEMODYNAMIC CONSIDERATIONS
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DOI：
10.1161/01.cir.50.1.167
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发表时间：
1974-01-01
期刊：
影响因子：
37.8
通讯作者：
RUDOLPH, AM
中科院分区：
文献类型：
--
作者：
LAKIER, JB;STANGER, P;RUDOLPH, AM
肺动脉瓣缺如或发育不良是一种罕见的情况，通常与室间隔缺陷和阻塞性肺瓣环有关。继发性病变累及肺动脉和主支气管是常见的，许多继发性病变会导致严重的肺部并发症。本文报告8例肺动脉瓣缺如、室间隔缺损和肺动脉瓣环闭塞的病例。本文介绍了其临床特点、心导管检查、电影血管造影和尸检结果。讨论了可能的胎儿血流动力学及其影响，以及出生后血流动力学及其临床后果。有这种联合病变的患者被认为预后非常差，最近的出版物都主张早期手术治疗；然而，大多数发表的系列文章都是小的，只有106例被记录在案。我们回顾了这些病例的临床和解剖学特征，以及对预后和手术治疗结果的概述。
Absent or hypoplastic pulmonary valve is an uncommon condition usually associated with a ventricular septal defect and an obstructive pulmonary valve ring. Secondary abnormalities involving the pulmonary arteries and major bronchi are common, and many result in severe pulmonary complications. Eight patients with absent pulmonary valve, ventricular septal defect and obstructed pulmonary valve ring are herein described. The clinical features, cardiac catheterization, cineangiographic and postmortem findings are presented. Possible fetal hemodynamics and their influences are discussed, as are postnatal hemodynamics and their clinical consequences. Patients with this combination of lesions have been regarded as having a very poor prognosis, and recent publications have advocated early surgical intervention; however, most published series are small, and only 106 cases have been documented. These cases have been reviewed for clinical and anatomic features as well as an overview of prognosis and results of surgical intervention.