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Interventricular septum aneurysm

MedGen UID:
234648
Concept ID:
C1387721
Disease or Syndrome
Synonym: Aneurysm of interventricular septum
 
Monarch Initiative: MONDO:0007112
OMIM®: 105805
Orphanet: ORPHA99092

Definition

Interventricular septum aneurysm is a rare, non-syndromic, congenital heart malformation characterized by the presence of a congenital aneurysm of the membranous portion of the interventricular septum. Patients may be asymptomatic or may present with ventricular or supraventricular tachycardia, fatigue, exertional dyspnea, palpitations, and cardiac murmur. Ventricular septal defects and conduction defects, such as first-degree atrio-ventricular block or incomplete right bundle branch block, may also be also associated. [from ORDO]

Clinical features

From HPO
Vascular dilatation
MedGen UID:
8076
Concept ID:
C0002940
Pathologic Function
Abnormal outpouching or sac-like dilatation in the wall of an atery, vein or the heart.
Abnormal ventricular septum morphology
MedGen UID:
866908
Concept ID:
C4021264
Anatomical Abnormality
A structural abnormality of the interventricular septum.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInterventricular septum aneurysm

Professional guidelines

PubMed

Akbal OY, Kaymaz C, Tanboga IH, Hakgor A, Yilmaz F, Turkday S, Dogan C, Tanyeri S, Demir D, Bayram Z, Cicek MB, Acar RD, Ozdemir N
Eur Heart J Cardiovasc Imaging 2018 Nov 1;19(11):1302-1308. doi: 10.1093/ehjci/jex303. PMID: 29237020
Kothur K, Singh S, Sharma Y, Mittal BR
J Cardiol 2007 Nov;50(5):299-307. PMID: 18044459
Ren JF, Schwartzman D, Callans DJ, Brode SE, Gottlieb CD, Marchlinski FE
Ultrasound Med Biol 1999 Sep;25(7):1077-86. doi: 10.1016/s0301-5629(99)00064-2. PMID: 10574340

Recent clinical studies

Etiology

Szkutnik M, Kusa J, Białkowski J
Kardiol Pol 2008 Sep;66(9):941-7; discussion 948-9. PMID: 18924021

Clinical prediction guides

Szkutnik M, Kusa J, Białkowski J
Kardiol Pol 2008 Sep;66(9):941-7; discussion 948-9. PMID: 18924021

Supplemental Content

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