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Ebstein malformation of the tricuspid valve

ORPHA:1880Morphological anomalyAutosomal dominant, Not applicableAll ages

Ассоциированные гены (1)

MYH7
myosin heavy chain 7
Disease-causing germline mutation(s) in
OMIM: 160760

Фенотипы (27)

Очень частый (80–99%)7
HP:0001622Premature birth
HP:0001631Atrial septal defect
HP:0002093Respiratory insufficiency
HP:0010316Ebstein anomaly of the tricuspid valve
HP:0011575Imperforate tricuspid valve
HP:0012378Fatigue
HP:0030680Abnormal cardiovascular system morphology
Частый (30–79%)10
HP:0000961Cyanosis
HP:0001643Patent ductus arteriosus
HP:0001671Abnormal cardiac septum morphology
HP:0001962Palpitations
HP:0005110Atrial fibrillation
HP:0005180Tricuspid regurgitation
HP:0011675Arrhythmia
HP:0011712Right bundle branch block
HP:0031667Holosystolic murmur
HP:0100749Chest pain
Периодический (5–29%)8
HP:0001635Congestive heart failure
HP:0001645Sudden cardiac death
HP:0002094Dyspnea
HP:0002637Cerebral ischemia
HP:0004306Abnormality of the endocardium
HP:0004420Arterial thrombosis
HP:0010741Pedal edema
HP:0012418Hypoxemia
Очень редкий (1–4%)2
HP:0001297Stroke
HP:0001658Myocardial infarction

Эпидемиология (22)

Point prevalence
1-9 / 100 000
Europe
Prevalence at birth
1-9 / 100 000
Europe
Prevalence at birth
1-9 / 100 000
France
Prevalence at birth
1-5 / 10 000
Austria
Prevalence at birth
1-9 / 100 000
Belgium
Prevalence at birth
1-5 / 10 000
Croatia
Prevalence at birth
1-5 / 10 000
Denmark
Prevalence at birth
1-5 / 10 000
Germany
Prevalence at birth
1-9 / 100 000
Hungary
Prevalence at birth
1-9 / 100 000
Ireland
Prevalence at birth
1-9 / 100 000
Italy
Prevalence at birth
1-5 / 10 000
Malta
Prevalence at birth
1-9 / 100 000
Netherlands
Prevalence at birth
1-9 / 100 000
Poland
Prevalence at birth
1-9 / 1 000 000
Spain
Prevalence at birth
1-5 / 10 000
Switzerland
Prevalence at birth
1-9 / 100 000
United Kingdom
Prevalence at birth
1-9 / 100 000
Ukraine
Prevalence at birth
1-9 / 100 000
Taiwan, Province of China
Prevalence at birth
1-9 / 100 000
United States
Prevalence at birth
1-9 / 100 000
Specific population
Prevalence at birth
1-9 / 100 000
Norway

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы