Autosomal recessive hereditary disorder	Cardiac complication	Cardiovascular system hereditary disorder	Congenital heart disease	Developmental hereditary disorder	Hereditary disorder of endocrine system	Hereditary disorder of nervous system	Hypogonadism with anosmia	Reproductive system hereditary disorder
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Kallman syndrome with heart disease (disorder)
	Kallman syndrome with heart disease
	Kallman syndrome with cardiopathy
	Syndrome with characteristics of hypogonadotropic hypogonadism associated with gonadotropin-releasing hormone (GnRH) deficiency, anosmia or hyposmia (with hypoplasia or aplasia of the olfactory bulbs) and complex congenital cardiac malformations (double-outlet right ventricle, dilated cardiomyopathy, right aortic arch). It represents a distinct clinical entity from Kallmann syndrome. Less than 10 cases have been described so far.
	Kallmann-syndroom met hartziekte
	syndroom van Kallmann met hartziekte

Id	722027009
Status	Primitive

Due to

Isolated gonadotropin deficiency

Associated morphology	Morphologically abnormal structure
Finding site	Heart structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Finding site	Gonadal endocrine structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Has interpretation	Absent
Interprets	Sense of smell, function

Finding site	Structure of distal part of pituitary
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Structure of olfactory system
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	E23.0
Rule	TRUE
Advice	ALWAYS E23.0
Correlation	SNOMED CT source code to target map code correlation not specified

Target	Q30.8
Rule	TRUE
Advice	ALWAYS Q30.8
Correlation	SNOMED CT source code to target map code correlation not specified

Target	Q24.8
Rule	TRUE
Advice	ALWAYS Q24.8
Correlation	SNOMED CT source code to target map code correlation not specified