Infantile Isolated Absence of Right Pulmonary Artery with an Absent Stump and No Collaterals

IF 0.3 Q4 PEDIATRICS Journal of Child Science Pub Date : 2021-01-01 DOI:10.1055/s-0040-1722567
S. Patil, Sayantan Ghoshbag, U. Pillay
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Abstract

Abstract Unilateral absence of pulmonary artery (UAPA) is a rare entity. Diagnosis is often incidental based on echocardiography (ECHO)/computed tomography (CT) findings done for a cardiac evaluation or recurrent pulmonary pathology not responding to conventional treatment. Childhood-onset symptoms are often rare, and most of these cases are reported in adulthood or postpuberty. Here, we present a 5-month-old child with an isolated absence of right pulmonary artery without even an arterial stump or collaterals. Treatment options depend on the severity of symptoms and anatomy of the abnormal vessels, and in some cases, patients get better, if not completely well with the available medical and surgical treatments.
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婴儿孤立性右肺动脉缺失,残端缺失,无络腮
摘要单侧肺动脉缺失(UAPA)是一种罕见的疾病。诊断通常是偶然的,基于超声心动图(ECHO)/计算机断层扫描(CT)所做的心脏评估或复发性肺部病理对常规治疗无效。儿童期发病的症状通常很少见,这些病例大多发生在成年期或青春期后。在这里,我们提出了一个5个月大的孩子孤立的没有右肺动脉甚至没有动脉残端或侧支。治疗方案取决于症状的严重程度和异常血管的解剖结构,在某些情况下,患者即使不能完全康复,也会在现有的药物和手术治疗下好转。
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0.50
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19
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