Situs inversus with levocardia

MedGen UID:: 7331
•Concept ID:: C0023569
•: Congenital Abnormality

Synonyms:	Isolated Levocardia; Levocardia; Levocardia, Isolated; Situs Inversus with Levocardia
SNOMED CT:	Levocardia (205769006); Isolated levocardia (205769006); Situs inversus with levocardia (205769006)

HPO:	HP:0031592
Monarch Initiative:	MONDO:0019862
Orphanet:	ORPHA95854

Definition

Situs inversus of thoracic and abdominal viscera with the heart remaining normally situated on the left; usually associated with congenital cardiac abnormalities such as transposition of the great vessels and/or spleen defects including asplenia or polysplenia. [from HPO]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSitus inversus with levocardia

Non-Neoplastic Disorder
- Non-Neoplastic Disorder by Special Category
  - Deformity
    - Heterotaxy
      - Abnormality of abdominal situs
        Abdominal situs inversus
        Situs inversus with levocardia

Follow this link to review classifications for Situs inversus with levocardia in Orphanet.

Professional guidelines

PubMed

Prenatal diagnosis of isolated perimembranous ventricular septal defects undergoing primary surgical repair in infancy.

Evans WN, Acherman RJ, Ciccolo ML, Lehoux J, Restrepo H
J Matern Fetal Neonatal Med 2022 Dec;35(25):8001-8005. Epub 2021 Jun 17 doi: 10.1080/14767058.2021.1940933. PMID: 34139939

Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects.

Khayata M, Alkharabsheh S, Shah NP, Verma BR, Gentry JL, Summers M, Xu B, Asher C, Klein AL
Open Heart 2020;7(1):e001103. Epub 2020 Jan 8 doi: 10.1136/openhrt-2019-001103. PMID: 32076559 Free PMC Article

Prenatal echocardiographic diagnosis of cardiac right/left axis and malpositions according to standardized Cordes technique.

Ozkutlu S, Bostan OM, Deren O, Onderoğlu L, Kale G, Güçer S, Orhan D
Anadolu Kardiyol Derg 2011 Mar;11(2):131-6. Epub 2011 Feb 8 doi: 10.5152/akd.2011.033. PMID: 21303758

See all (7)

Recent clinical studies

Etiology

The extracardiac total cavopulmonary connection for definitive conversion to the Fontan circulation: summary of early experience and results.

Laschinger JC, Ringel RE, Brenner JI, McLaughlin JS
J Card Surg 1993 Sep;8(5):524-33. doi: 10.1111/j.1540-8191.1993.tb00407.x. PMID: 8219531

See all (1)

Diagnosis

Situs inversus with levocardia in a 15-year-old male adolescent: a case report.

Mesfin T, Kedir I, Tilahun T, Seyoum K, Dadi S, Ejigu N, Desta F, Geta G, Tsegaye M, Salih H
J Med Case Rep 2023 Dec 3;17(1):499. doi: 10.1186/s13256-023-04254-9. PMID: 38042875 Free PMC Article

Situs anomalies on prenatal MRI.

Nemec SF, Brugger PC, Nemec U, Bettelheim D, Kasprian G, Amann G, Rimoin DL, Graham JM Jr, Prayer D
Eur J Radiol 2012 Apr;81(4):e495-501. Epub 2011 Jun 28 doi: 10.1016/j.ejrad.2011.06.016. PMID: 21715116

See all (2)

Prognosis

Situs inversus with levocardia in a 15-year-old male adolescent: a case report.

Mesfin T, Kedir I, Tilahun T, Seyoum K, Dadi S, Ejigu N, Desta F, Geta G, Tsegaye M, Salih H
J Med Case Rep 2023 Dec 3;17(1):499. doi: 10.1186/s13256-023-04254-9. PMID: 38042875 Free PMC Article

Dynamic left ventricular outflow obstruction in situs inversus with levocardia.

Hirata K, Tengan T, Mototake H, Wake M
BMJ Case Rep 2015 Dec 1;2015 doi: 10.1136/bcr-2015-213030. PMID: 26628311 Free PMC Article

The extracardiac total cavopulmonary connection for definitive conversion to the Fontan circulation: summary of early experience and results.

Laschinger JC, Ringel RE, Brenner JI, McLaughlin JS
J Card Surg 1993 Sep;8(5):524-33. doi: 10.1111/j.1540-8191.1993.tb00407.x. PMID: 8219531

See all (3)