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罕见关联的主动脉下膜与动脉导管未闭——病例系列

Subaortic Membrane and Patent Ductus Arteriosus in Rare Association-Case Series.

作者信息

Moafa Hussain, Alnasef Mohammed, Diraneyya Obayda M, Alhabshan F

机构信息

Department of Cardiac Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.

King Abdullah International Medical Research Center, Riyadh, Saudi Arabia.

出版信息

J Saudi Heart Assoc. 2020 Sep 11;32(3):410-414. doi: 10.37616/2212-5043.1199. eCollection 2020.

Abstract

BACKGROUND

The combination of subaortic membrane (SAM) and patent ductus arteriosus is very rare. Subaortic stenosis is the second most common form of left ventricular outflow tract (LVOT) obstruction after valvular aortic stenosis. We are reporting the largest case series of SAM and PDA.

METHODS

We included all patients that were diagnosed with the combination of SAM and PDA at our cardiac center. We have reviewed patients echocardiographic studies, cardiac catheterizations, surgical notes and all the outpatients notes.

RESULTS

We have a total of 7 patients. The age at presentation was in the early childhood with 3 patients diagnosed in infancy. Four patients had severe and moderate LVOT obstruction with SAM being very close to the aortic valve and all required surgical intervention. The last three patients had mild LVOT obstruction 2 of them with the SAM being > 4mm away from the aortic valve. Six out of the seven patients had intervention while the last one is under clinical follow up currently. PDA closure did not change the outcome. There were no other postoperative complication like developing new AI or developing complete heart block. There was no relation between gender, height, weight or age at diagnosis to the SAM clinical course.

CONCLUSION

SAM and PDA association is very rare. The underlying pathophysiology is not well understood. When the SAM is closer to aortic valve (≤ 4mm), it carries higher risk of progressive LVOT obstruction. The interventions for SAM and PDA were safe procedures.

摘要

背景

主动脉瓣下隔膜(SAM)与动脉导管未闭(PDA)并存的情况非常罕见。主动脉瓣下狭窄是继主动脉瓣狭窄之后左心室流出道(LVOT)梗阻的第二常见形式。我们报告了最大的一组SAM合并PDA的病例系列。

方法

我们纳入了在我们心脏中心被诊断为SAM合并PDA的所有患者。我们回顾了患者的超声心动图研究、心导管检查、手术记录以及所有门诊记录。

结果

我们共有7例患者。就诊时年龄处于幼儿期,3例在婴儿期被诊断。4例患者有严重和中度LVOT梗阻,SAM非常靠近主动脉瓣,所有患者均需要手术干预。最后3例患者有轻度LVOT梗阻,其中2例的SAM距离主动脉瓣大于4mm。7例患者中有6例接受了干预,最后1例目前正在临床随访中。PDA封堵术并未改变结果。没有出现诸如新发主动脉瓣反流或发生完全性心脏传导阻滞等其他术后并发症。诊断时的性别、身高、体重或年龄与SAM的临床病程之间没有关联。

结论

SAM与PDA并存非常罕见。其潜在的病理生理学机制尚未完全了解。当SAM更靠近主动脉瓣(≤4mm)时,发生进行性LVOT梗阻的风险更高。针对SAM和PDA的干预措施是安全的手术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af83/7721455/8d5a1874ea93/sha-32-03-410f1.jpg

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