Congenital Aneurysm of the Muscular Interventricular Septum

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Congenital aneurysms of the muscular interventricular septum are rare. We report the diagnosis of such an aneurysm by fetal echocardiography at 19 weeks' gestation and the subsequent follow-up through early neonatal life, with the ultimate progression to a dilated cardiomyopathy requiring cardiac transplantation. This case highlights the early diagnosis of this rare finding and the unusual clinical course to include the confounding features of endocardial fibroelastosis, left ventricular noncompaction, and a lymphocytic infiltrate demonstrated on pathologic examination. Most infants with congenital septal aneurysms are asymptomatic, but this case demonstrates the need for careful serial assessment and clinical follow-up because of the unclear origin and natural history of this entity.

Section snippets

Case Report

A 30-year-old woman (gravida 2, para 1) presented to the fetal echocardiography laboratory at our institution at 19 weeks' gestation for further evaluation of an abnormal 4-chamber cardiac view, concerning for right ventricular hypoplasia, discovered on an obstetric ultrasound 2 weeks earlier. The obstetric ultrasound revealed otherwise normal findings. Her pregnancy had been uncomplicated. There was no family history of congenital heart disease, congenital malformations, sudden cardiac death,

Discussion

Congenital aneurysms of the muscular ventricular septum are rare. To date, a total of 19 patients have been reported in the medical literature.1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14 In children, septal aneurysms may be acquired or congenital, in contrast to the adult population where they are most often found postmyocardial infarction. Acquired causes include anomalous origin of the left coronary artery from the pulmonary artery, blunt chest trauma, infection, cardiac surgery, and

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The article was written by LCDR Michael Carr, MC, USN, while a fellow at Texas Children's Hospital training in pediatric cardiology. The views expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, or the US Government.

I am military service member. This work was prepared as part of my official duties. Title 17 USC 105 provides that “Copyright protection under this title is not available for any work of the United States Government.” Title 17 USC 101 defines a US Government work as a work prepared by a military service member or employee of the US Government as part of that person's official duties.

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