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Abnormal ventricular septum morphology

MedGen UID:
866908
•Concept ID:
C4021264
•
Anatomical Abnormality
Synonyms: Abnormal interventricular septum morphology; Abnormality of the ventricular septum; Ventricular septum abnormality
 
HPO: HP:0010438

Definition

A structural abnormality of the interventricular septum. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Abnormal ventricular septum morphology

Conditions with this feature

Interventricular septum aneurysm
MedGen UID:
234648
•Concept ID:
C1387721
•
Disease or Syndrome
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.
See: Condition Record
Interventricular septum aneurysm

Professional guidelines

PubMed

Advances in the treatment of pulmonary atresia with intact ventricular septum: palliative and definitive repair.
Laks H, Billingsley AM
Cardiol Clin 1989 May;7(2):387-98. PMID: 2471593
Effect of beta-blocker administration in the post-valvotomy management of infants with pulmonary atresia and intact ventricular septum.
Yamaguchi M, Ohashi H, Tachibana H, Hosokawa Y, Tei T, Mito H
Kobe J Med Sci 1987 Dec;33(6):235-9. PMID: 2896267
Diagnosis and surgical treatment of infants with critical pulmonary outflow obstruction. Study of thirty-four infants with pulmonary stenosis or atresia, and intact ventricular septum.
Gersony WM, Bernhard WF, Nadas AS, Gross RE
Circulation 1967 Apr;35(4):765-76. doi: 10.1161/01.cir.35.4.765. PMID: 4225808

Recent clinical studies

Etiology

Rare variants in genes encoding structural myocyte contribute to a thickened ventricular septum in sudden death population without ventricular alterations.
Alcalde M, Nogué-Navarro L, Tiron C, Fernandez-Falgueras A, Iglesias A, Simon A, Buxó M, Pérez-Serra A, Puigmulé M, López L, Picó F, Del Olmo B, Corona M, Campuzano O, Moral S, Castella J, Coll M, Brugada R
Forensic Sci Int Genet 2022 May;58:102688. Epub 2022 Mar 16 doi: 10.1016/j.fsigen.2022.102688. PMID: 35316720
Fetal cardiac interventions: Where do we stand?
Friedman KG, Tworetzky W
Arch Cardiovasc Dis 2020 Feb;113(2):121-128. Epub 2020 Feb 26 doi: 10.1016/j.acvd.2019.06.007. PMID: 32113817
Discontinuous Pulmonary Artery.
Eltayeb O, Mongé MC, Popescu AR, Sarwark AE, Harris T, Backer CL
World J Pediatr Congenit Heart Surg 2017 Jan;8(1):106-110. Epub 2016 Jun 22 doi: 10.1177/2150135115618871. PMID: 26993757
Left ventricular cleft.
Cappai A, Malvindi PG, Raffa GM, Basciu A, Monti L
Eur Heart J Cardiovasc Imaging 2013 Jan;14(1):14. Epub 2012 Jul 29 doi: 10.1093/ehjci/jes157. PMID: 22848022
The role of intraoperative device closure in the management of muscular ventricular septal defects.
Konstantinov IE, Coles JG
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003;6:84-9. doi: 10.1053/pcsu.2003.50001. PMID: 12740774

Diagnosis

A rare ventriculoarterial connection: double outlet of both ventricles.
Genc HZ, Ozyilmaz I, Baş S, Kose B, Ozturk E, Hatemi AC, Tanidir IC
Cardiol Young 2023 Nov;33(11):2390-2392. Epub 2023 May 19 doi: 10.1017/S1047951123001130. PMID: 37203793
Double Posterior Descending Artery Arising from a Right Coronary Artery.
Wróbel G, Spałek M, Kuder T
Int Heart J 2019 Sep 27;60(5):1226-1229. Epub 2019 Sep 4 doi: 10.1536/ihj.19-018. PMID: 31484871
Left ventricular cleft.
Cappai A, Malvindi PG, Raffa GM, Basciu A, Monti L
Eur Heart J Cardiovasc Imaging 2013 Jan;14(1):14. Epub 2012 Jul 29 doi: 10.1093/ehjci/jes157. PMID: 22848022
Complete atrioventricular canal.
Calabrò R, Limongelli G
Orphanet J Rare Dis 2006 Apr 5;1:8. doi: 10.1186/1750-1172-1-8. PMID: 16722604Free PMC Article
Ventricular septal defect.
Soto B, Bargeron LM Jr, Diethelm E
Semin Roentgenol 1985 Jul;20(3):200-13. doi: 10.1016/0037-198x(85)90004-5. PMID: 3898389

Therapy

The Secrets of the Frogs Heart.
Corno AF, Zhou Z, Uppu SC, Huang S, Marino B, Milewicz DM, Salazar JD
Pediatr Cardiol 2022 Oct;43(7):1471-1480. Epub 2022 Mar 15 doi: 10.1007/s00246-022-02870-8. PMID: 35290490
Fetal cardiac interventions: Where do we stand?
Friedman KG, Tworetzky W
Arch Cardiovasc Dis 2020 Feb;113(2):121-128. Epub 2020 Feb 26 doi: 10.1016/j.acvd.2019.06.007. PMID: 32113817
Hamartomas and other tumor-like malformations of the lungs and heart.
Wick MR
Semin Diagn Pathol 2019 Jan;36(1):2-10. Epub 2018 Nov 16 doi: 10.1053/j.semdp.2018.11.002. PMID: 30473462
Hypertrophic cardiomyopathy.
Santos Mateo JJ, Sabater Molina M, Gimeno Blanes JR
Med Clin (Barc) 2018 Jun 8;150(11):434-442. Epub 2017 Nov 14 doi: 10.1016/j.medcli.2017.09.013. PMID: 29150126
Hypertrophic cardiomyopathy in childhood.
Colan SD
Heart Fail Clin 2010 Oct;6(4):433-44, vii-iii. doi: 10.1016/j.hfc.2010.05.004. PMID: 20869644Free PMC Article

Prognosis

Rare variants in genes encoding structural myocyte contribute to a thickened ventricular septum in sudden death population without ventricular alterations.
Alcalde M, Nogué-Navarro L, Tiron C, Fernandez-Falgueras A, Iglesias A, Simon A, Buxó M, Pérez-Serra A, Puigmulé M, López L, Picó F, Del Olmo B, Corona M, Campuzano O, Moral S, Castella J, Coll M, Brugada R
Forensic Sci Int Genet 2022 May;58:102688. Epub 2022 Mar 16 doi: 10.1016/j.fsigen.2022.102688. PMID: 35316720
Double Posterior Descending Artery Arising from a Right Coronary Artery.
Wróbel G, Spałek M, Kuder T
Int Heart J 2019 Sep 27;60(5):1226-1229. Epub 2019 Sep 4 doi: 10.1536/ihj.19-018. PMID: 31484871
Left ventricular cleft.
Cappai A, Malvindi PG, Raffa GM, Basciu A, Monti L
Eur Heart J Cardiovasc Imaging 2013 Jan;14(1):14. Epub 2012 Jul 29 doi: 10.1093/ehjci/jes157. PMID: 22848022
Intracardiac bronchogenic cyst.
Vaideeswar P, Agnihotri MA, Patwardhan AM
J Card Surg 2011 May;26(3):266-8. Epub 2011 Mar 28 doi: 10.1111/j.1540-8191.2011.01206.x. PMID: 21443547
Complete atrioventricular canal.
Calabrò R, Limongelli G
Orphanet J Rare Dis 2006 Apr 5;1:8. doi: 10.1186/1750-1172-1-8. PMID: 16722604Free PMC Article

Clinical prediction guides

Prevalence of Left Ventricular Myocardial Crypts in Japanese Patients.
Watanabe J, Kawano H, Sueyoshi E, Koike H, Yoshimuta T, Ikeda S, Maemura K
Intern Med 2024 Jun 15;63(12):1675-1681. Epub 2023 Nov 13 doi: 10.2169/internalmedicine.2771-23. PMID: 37952949Free PMC Article
Rare variants in genes encoding structural myocyte contribute to a thickened ventricular septum in sudden death population without ventricular alterations.
Alcalde M, Nogué-Navarro L, Tiron C, Fernandez-Falgueras A, Iglesias A, Simon A, Buxó M, Pérez-Serra A, Puigmulé M, López L, Picó F, Del Olmo B, Corona M, Campuzano O, Moral S, Castella J, Coll M, Brugada R
Forensic Sci Int Genet 2022 May;58:102688. Epub 2022 Mar 16 doi: 10.1016/j.fsigen.2022.102688. PMID: 35316720
Rare association of absent pulmonary valve with intact ventricular septum and patent ductus arteriosus: Assessment in a newborn with CT Angiography.
Porayette P, Reinking B, Ashwath R
J Cardiovasc Comput Tomogr 2021 Jan-Feb;15(1):e1-e2. Epub 2020 Aug 1 doi: 10.1016/j.jcct.2020.07.001. PMID: 32807704
A Tubular Aortopulmonary Window: An Embryological Curiosity.
Chidambarathanu S, Agarwal R, Hussain ZM, Brown NA, Anderson RH
World J Pediatr Congenit Heart Surg 2016 May;7(3):411-3. Epub 2016 Jan 21 doi: 10.1177/2150135115596586. PMID: 26795903
Doppler echocardiographic evaluation of ventricular septal defects in adults.
Rahko PS
Echocardiography 1993 Sep;10(5):517-31. doi: 10.1111/j.1540-8175.1993.tb00067.x. PMID: 10146328

Recent systematic reviews

Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review.
Li FF, Du XL, Chen S
J Huazhong Univ Sci Technolog Med Sci 2015 Oct;35(5):656-661. Epub 2015 Oct 22 doi: 10.1007/s11596-015-1485-3. PMID: 26489617

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