Idiopathic pulmonary arterial hypertension

General Information (adopted from Orphanet):

Synonyms, Signs: IPAH
Number of Symptoms 18
OrphanetNr: 275766
OMIM Id:
ICD-10: I27.0
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: > 0.1 of 100 000
Inheritance:
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: Idiopathic and/or familial pulmonary arterial hypertension
 -Rare genetic disease
 -Rare respiratory disease

Comment:

IPAH occurs predominantly in younger women, but is increasingly diagnosed also in elderly patients (PMID:23164592).

Symptom Information: Sort by abundance 

1
 (HPO:0001254) Lethargy 25840091 IBIS 104 / 7739
2
 (HPO:0012378) Fatigue 25840091 IBIS 50 / 7739
3
 (HPO:0001667) Right ventricular hypertrophy 25840091 IBIS 23 / 7739
4
 (HPO:0011025) Abnormality of cardiovascular system physiology 25840091 IBIS 41 / 7739
5
 (HPO:0004930) Abnormality of the pulmonary vasculature 24951767 IBIS 3 / 7739
6
 (HPO:0002092) Pulmonary hypertension 24355641 IBIS 109 / 7739
7
 (HPO:0004890) Elevated pulmonary artery pressure 19307479 IBIS 8 / 7739
8
 (HPO:0005317) Increased pulmonary vascular resistance 25840091 IBIS 7 / 7739
9
 (HPO:0012664) Reduced ejection fraction 25996939 IBIS 32 / 7739
10
 (HPO:0001681) Angina pectoris 25840091 IBIS 22 / 7739
11
 (HPO:0001708) Right ventricular failure 19307479 IBIS 11 / 7739
12
 (HPO:0005308) Pulmonary artery vasoconstriction 19307479 IBIS 4 / 7739
13
 (HPO:0002094) Dyspnea 25840091 IBIS 132 / 7739
14
 (HPO:0012418) Hypoxemia 19307479 IBIS 18 / 7739
15
 (MedDRA:10059165) Oxygen consumption decreased 24905576 IBIS 2 / 7739
16
 (MedDRA:10021143) [DEL] Hypoxia 19307479 IBIS 5 / 7739
17
 (MedDRA:10019311) Heart sounds abnormal 21858979 IBIS 4 / 7739
18
 (OMIM) Plexiform vascular lesions 22449225 IBIS 3 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: