Left Ventricular Outflow Tract Obstruction Due to an Aneurysm of the Membranous Septum in a Patient with Pulmonary Atresia and Ventricular Septum Defect - Gase Report

Abstract

Although the occurrence of subpulmonary stenosis resulting from displacement of an aneurysm of the membranous septum is well known, resultant subaortic stenosis has not been well documented. We present an unusual case of acquired pulmonary atresia with restrictive ventricular septal defect and associated severe subaortic resulting from an aneurysm of the membranous septum.

Rare cause of dynamic aortic obstruction in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries: a case report

“…At the age of 6 years old, she presented a severe sub-aortic stenosis that was found to be due to a dome-like elongation of the membranous septum and accessory tricuspid valve tissue protruding through the ventricular septal defect into the left ventricle outflow tract. 2 This case highlights a unique finding in the setting of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Left ventricle outflow tract obstruction caused by tricuspid valve aneurysmatic tissue is a complication that has never been reported in the literature, although it can further interfere with the clinical management of this already troublesome and complex CHD.…”

Prenatal diagnosis of membranous ventricular septal aneurysms and their association with absence of atrioventricular valve ‘offsetting’

“…Indeed, the most common presentation of ventricular septal aneurysm is in association with VSDs6, 19. Aneurysms of the membranous portion of the ventricular septum and its related heart defects have been associated with serious complications such as right outflow tract obstruction25–27, aortic valve prolapse8, spontaneous rupture3, 10, tricuspid insufficiency, bacterial endocarditis8 and cerebral infarction secondary to embolism28. In some of these cases the therapeutic approach is resection of the sacular dilatation of the septum and closure of the defect8.…”

“…This observation is consistent with previous reports which demonstrated that in infants and adults, aneurysms of the membranous septum bulge into the right ventricle due to higher pressure in the left ventricle17, 32, 42. Indeed, functional obstruction of the outflow tracts is more frequently seen in the right side, postnatally25–27.…”

Two Cases of Prenatally Diagnosed Membranous and Muscular Ventricular Septal Aneurysms

“…Isolated muscular ventricular septal aneurysms are preferentially asymptomatic. Membranous ventricular septal aneurysms more often become complicated (usually by right outflow tract obstruction but also by arrhythmia, thromboembolism, and even subaortic stenosis and may require surgical correction. There are only few publications about prenatally revealed ventricular septal aneurysms.…”