Alamoudi Loujen O, Alboloshi Ethar A, Alhnaidi Malek, Waggass Rahaf, Alsharif Salwan, Gazzaz Abrar M
College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia; King Abdullah International Medical Research Center, Jeddah, Saudi Arabia.
College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia; King Abdullah International Medical Research Center, Jeddah, Saudi Arabia.
Int J Surg Case Rep. 2022 Dec;101:107782. doi: 10.1016/j.ijscr.2022.107782. Epub 2022 Nov 21.
While only a few cases have been reported in pediatrics, subaortic stenosis (SAS) is a gradually progressive disorder rarely seen at birth and infancy, however, it is the most common type of aortic stenosis. It obstructs the blood flow across the left ventricular outflow tract (LVOT). Although the cause is still not well known, different etiologies have been suggested by the literature. While surgical resection is the definitive treatment, recurrence is observed in many patients, nonetheless, LVOT gradient usually progresses over years of follow-up.
We report the clinical and diagnostic course of a 41-months-old Saudi boy, asymptomatic child who was found to have progressive recurrent subaortic stenosis within a few months which required two redo sternotomy for sub-aortic membrane resection throughout a period of two years.
SAS is usually detected incidentally in asymptomatic patients requiring an echocardiogram to assess other accompanying congenital heart defects (CHD), or rather potentially arising after repair of CHD. Patient close monitoring is important aspect given the nature of disease progression, re-operation for recurrence demonstrate significant increase over years, re-resection rate was 0 % after one year, 6 % after five years, and 8 % after 10 years.
Recurrence of LVOT obstruction following sub-aortic membrane resection is common. Long-term follow-up care in postoperative patients is crucial. Majority of patients will need re-operation for recurrence at certain point during course of the disease.
虽然儿科中仅报道了少数病例，但主动脉瓣下狭窄（SAS）是一种逐渐进展的疾病，在出生时和婴儿期很少见，然而，它是最常见的主动脉狭窄类型。它阻碍了左心室流出道（LVOT）的血流。虽然病因仍不清楚，但文献提出了不同的病因。虽然手术切除是确定性治疗方法，但许多患者会出现复发，尽管如此，LVOT梯度在多年随访中通常会进展。
我们报告了一名41个月大的沙特男孩的临床和诊断过程，该无症状儿童在几个月内被发现患有进行性复发性主动脉瓣下狭窄，在两年内需要两次再次开胸进行主动脉瓣下膜切除术。
SAS通常在无症状患者中偶然发现，这些患者需要进行超声心动图检查以评估其他伴随的先天性心脏病（CHD），或者可能在CHD修复后出现。鉴于疾病进展的性质，密切监测患者是一个重要方面，复发后的再次手术多年来显著增加，一年后的再次切除率为0%，五年后为6%，十年后为8%。
主动脉瓣下膜切除术后LVOT梗阻复发很常见。术后患者的长期随访护理至关重要。大多数患者在疾病过程中的某个时候需要因复发而再次手术。
