raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:1461

Criss-cross heart

Synonyms: Criss-cross atrioventricular relationships · Superoinferior ventricles · Twisted atrioventricular connections

Type
Morphological anomaly
Group
Disorder
Inheritance
Unknown
Age of onset
Infancy, Neonatal
Status
active

Criss cross heart (CCH) is a cardiac malformation where the inflow streams of the two ventricles cross due to twisting of the heart about its major axis. The clinical features depend on the particular cardiac defects associated, like simple or corrected transposition of the great arteries and ventricular septal defects.

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Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCriss-cross heartCriss-cross atrioventricular relationships · Superoinferior ventricles · Twisted atrioventricular connections
frCoeur croiséCoeur à ventricules entrecroisés · Ventricules croisés · Ventricules supéro-inférieurs
deCriss-Cross-HerzAtrio-ventrikuläre Verbindungen, verdrehte · Superoinferiore Ventrikellage · Überkreuzte atrioventrikuläre Verhältnisse
esCorazón en criss-crossConexiones auriculoventriculares cruzadas · Conexiones auriculoventriculares en criss-cross · Conexiones auriculoventriculares trenzadas · Ventrículos superoinferiores
itCuore criss-crossConnessioni atrioventricoalri criss-cross · Connessioni atrioventricolari alterate · Ventricoli supero-inferiori
nlCriss-cross hartCriss-cross atrioventriculaire connecties · Gedraaide atrioventriculaire verbindingen · Supero-inferieure ventrikels
ptCoração cruzadoComunicações auriculo-ventriculares cruzadas
plWada serca typu criss-cross heart= en
csCrisscross srdceCriss-cross vztah síní a komor · Superoinferiorní poloha komor · Zkřížené spojení síní a komor
ukCriss-cross heartПерехресні атріовентрикулярні звязки · Наднижній шлуночок · Перекручені атріовентрикулярні зєднання
zh交叉心脏房室传导交叉
trÇapraz çizgili kalpSüper-inferior ventriküller · Bükülmüş atriyoventriküler bağlantılar · Çaprazlama-ters atriyoventriküler ilişkiler

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q24.8NTBTattributedvalidated
ICD-11LA81NTBTindexvalidated
MONDO0015449Evalidated
MeSHD003420Evalidated
UMLSC0010334Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

Codes that Orphanet moved or referred to this one:

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Genes

0 direct

Orphanet records no gene directly on this code.

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Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Prevalence at birth1-9 / 1 000 0000.8 / 100 000EuropeValue and classValidatedISBN:9781416023906[OTHER]_22555779[PMID]
Point prevalenceUnknown—EuropeClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

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HPO phenotypes

13 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0030680Abnormal cardiovascular system morphology
HP:0000961Cyanosis
Frequent (79-30%)
HP:0001633Abnormal mitral valve morphology
HP:0011968Feeding difficulties
HP:0001718Mitral stenosis
HP:0001642Pulmonic stenosis
HP:0002093Respiratory insufficiency
HP:0004381Supravalvular aortic stenosis
HP:0001669Transposition of the great arteries
HP:0010446Tricuspid stenosis
HP:0001629Ventricular septal defect
Occasional (29-5%)
HP:0001999Abnormal facial shape
HP:0000765Abnormal thorax morphology

Run the differential on the 2 obligate / very frequent terms

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Sources for this card