raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:275828

Pulmonary arterial hypertension associated with chronic hemolytic anemia

Synonyms: PAH associated with chronic hemolytic anemia

Type
Clinical group
Group
Group of disorders
Inheritance
Multigenic/multifactorial, Not applicable
Age of onset
All ages
Status
active

Pulmonary arterial hypertension associated with chronic hemolytic anemia (PAH-CHA) is a form of PAH characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of chronic hemolytic anemia.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPulmonary arterial hypertension associated with chronic hemolytic anemiaPAH associated with chronic hemolytic anemia
frHypertension artérielle pulmonaire associée à une anémie hémolytique chroniqueHTAP associée à une anémie hémolytique chronique
dePulmonale arterielle Hypertonie mit assoziierter chronisch-hämolytischer AnämiePAH mit assoziierter chronischer hämolytischer Anämie
esHipertensión arterial pulmonar asociada a anemia hemolítica crónicaHAP asociada a anemia hemolítica crónica
itIpertensione arteriosa polmonare associata ad anemia emolitica cronicaPAH associata ad anemia emolitica cronica
nlPulmonale arteriële hypertensie geassocieerd met chronische hemolytische anemiePAH geassocieerd met chronische hemolytische anemie
ptHipertensão arterial pulmonar associada a anemia hemolítica crónica
plPłucne nadciśnienie tętnicze związane z przewlekłą niedokrwistością hemolitycznąPAH związane z przewlekłą niedokrwistością hemolityczną
csPlicní arteriální hypertenze asociovaná s chronickou hemolytickou anémiíPAH asociovaná s chronickou hemolytickou anémií
ukЛегенева артеріальна гіпертензія, асоційована з хронічною гемолітичною анемієюЛАГ, повязана з хронічною гемолітичною анемією
zh慢性溶血性贫血相关肺动脉高压慢性溶血性贫血相关PAH
trKronik hemolitik anemi ilişkili pulmoner arteriyel hipertansiyonKronik hemolitik anemi ile ilişkili PAH

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO17156Evalidated
UMLSC3698315Evalidated

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

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET

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

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card