Idiopathic/heritable pulmonary arterial hypertension
ORPHA:422DiseaseAutosomal dominant, Autosomal recessive, Not applicableAll ages
Фенотипы (HPO)13
Очень частый (80–99%)1
HP:0002092Pulmonary arterial hypertension
Частый (30–79%)2
HP:0002094Dyspnea
HP:0011025Abnormality of cardiovascular system physiology
Периодический (5–29%)9
HP:0001279Syncope
HP:0001962Palpitations
HP:0002240Hepatomegaly
HP:0005133Right ventricular dilatation
HP:0005180Tricuspid regurgitation
HP:0012378Fatigue
HP:0030148Heart murmur
HP:0030848Elevated jugular venous pressure
HP:0100749Chest pain
Очень редкий (1–4%)1
HP:0010741Pedal edema
Эпидемиология7
| Тип оценки | Класс распространённости | Среднее (на 100к) | Регион | Квалификация |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.11 | United States | Value and class |
| Point prevalence | 1-9 / 1 000 000 | 0.65 | France | Value and class |
| Annual incidence | 1-9 / 1 000 000 | 0.1 | France | Value and class |
| Point prevalence | 1-9 / 100 000 | 1.4 | Czech Republic | Value and class |
| Annual incidence | 1-9 / 1 000 000 | 0.62 | Czech Republic | Value and class |
| Point prevalence | 1-9 / 100 000 | 1 | Europe | Value and class |
| Point prevalence | 1-9 / 100 000 | 6.25 | China | Value and class |
Лекарства и терапия
Источник: Open Targets Platform (в реальном времени)
Клинические исследования
Источник: ClinicalTrials.gov (в реальном времени)