Fixed Subaortic Stenosis: A Clinical Dilemma for Clinicians and Patients

Ezon, David

doi:10.1111/chd.12127

Cited by 20 publications

(22 citation statements)

References 55 publications

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“…Recommendations range from early operation to longer periods of observation, depending on patient characteristics. Ezon et al reported that ≥2 studies recommended surgery at diagnosis, regardless of the severity of the obstruction . Brauner et al suggested that early surgery prevents AR .…”

Section: Treatmentmentioning

confidence: 99%

Subvalvular aortic stenosis: a review of current literature

Devabhaktuni

Chakfeh

Malik

et al. 2018

Clinical Cardiology

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Subvalvular aortic stenosis (SAS) is one of the common adult congenital heart diseases, with a prevalence of 6.5%. It is usually diagnosed in the first decade of life. Echocardiography is the test of choice to diagnose SAS. Surgical correction is the best treatment modality, and the prognosis is usually excellent. In this review, we describe the pathophysiology, diagnosis, prognosis, and management of SAS with a focus on different pathophysiologic mechanisms, diagnostic approach, and prognosis of the disease by reviewing the current literature.

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Section: Treatmentmentioning

confidence: 99%

Subvalvular aortic stenosis: a review of current literature

Devabhaktuni

Chakfeh

Malik

et al. 2018

Clinical Cardiology

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“…The majority of interatrial communications can be divided anatomically into two groups: those located within the rims of the oval fossa, and those located outside the oval fossa [ 5 ]. The latter lesions include the sinus venosus, coronary sinus, and “ostium primum” defects.…”

Section: Discussionmentioning

confidence: 99%

Identification and Morphogenesis of Vestibular Atrial Septal Defects

Loomba

Tretter

Mohun

et al. 2020

JCDD

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Background: The vestibular atrial septal defect is an interatrial communication located in the antero-inferior portion of the atrial septum. Reflecting either inadequate muscularization of the vestibular spine and mesenchymal cap during development, or excessive apoptosis within the developing antero-inferior septal component, the vestibular defect represents an infrequently recognized true deficiency of the atrial septum. We reviewed necropsy specimens from three separate archives to establish the frequency of such vestibular defects and their associated cardiac findings, providing additional analysis from developing mouse hearts to illustrate their potential morphogenesis. Materials and methods: We analyzed the hearts in the Farouk S. Idriss Cardiac Registry at Ann and Robert H. Lurie Children’s Hospital in Chicago, IL, the Van Mierop Archive at the University of Florida in Gainesville, Florida, and the archive at Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida, identifying all those exhibiting a vestibular atrial septal defect, along with the associated intracardiac malformations. We then assessed potential mechanisms for the existence of such defects, based on the assessment of 450 datasets of developing mouse hearts prepared using the technique of episcopic microscopy. Results: We analyzed a total of 2100 specimens. Of these, 68 (3%) were found to have a vestibular atrial septal defect. Comparable defects were identified in 10 developing mouse embryos sacrificed at embryonic data 15.5, by which stage the antero-inferior component of the atrial septum is usually normally formed. Conclusion: The vestibular defect is a true septal defect located in the muscular antero-inferior rim of the oval fossa. Our retrospective review of autopsied hearts suggests that the defect may be more common than previously thought. Increased awareness of the location of the defect should optimize its future clinical identification. We suggest that the defect exists because of failure, during embryonic development, of union of the components that bind the leading edge of the primary atrial septum to the atrioventricular junctions, either because of inadequate muscularisation or excessive apoptosis.

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“…The fibromuscular fixed subaortic stenosis is the most frequent, and in many cases the tunnel-type lesions are associated with a greater degree of stenosis. They are isolated or acquired and their prevalence in adults with congenital heart disease is 6.5%, with a male to female ratio of 2:1 [4][5][6][7]. We reported an interesting and reliable case of double orifice fixed subaortic membrane in adults.…”

Section: Introductionmentioning

confidence: 99%

Double Orifice Fissured Subaortic Membrane in the Adult

Zampi

Cottini

Pergolini

et al. 2017

ijcp

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Left ventricle outflow tract obstruction is a relatively common form of congenital heart disease, occurring in 2.8 out of 10,000 live births and accounts for 3-6% of congenital heart diseases. Subvalvular aortic stenosis can be either a fixed stenosis resulting from subaortic membrane or a dynamic stenosis because of hypertrophic cardiomyopathy. We described an original and rare image of double orifice fissured subaortic membrane in the adult.

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Fixed Subaortic Stenosis: A Clinical Dilemma for Clinicians and Patients

Cited by 20 publications

References 55 publications

Subvalvular aortic stenosis: a review of current literature

Subvalvular aortic stenosis: a review of current literature

Identification and Morphogenesis of Vestibular Atrial Septal Defects

Double Orifice Fissured Subaortic Membrane in the Adult

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