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Atrial septal defect, ostium secundum type

ORPHA:99103Clinical subtypeAutosomal dominant, Not applicableAntenatal, Infancy, Neonatal

Ассоциированные гены (9)

ACTC1
actin alpha cardiac muscle 1
Disease-causing germline mutation(s) in
OMIM: 102540
GATA4
GATA binding protein 4
Disease-causing germline mutation(s) in
OMIM: 600576
MYH6
myosin heavy chain 6
Disease-causing germline mutation(s) in
OMIM: 160710
NKX2-5
NK2 homeobox 5
Disease-causing germline mutation(s) in
OMIM: 600584
TBX20
T-box transcription factor 20
Disease-causing germline mutation(s) (loss of function) in
OMIM: 606061
TBX20
T-box transcription factor 20
Disease-causing germline mutation(s) (gain of function) in
OMIM: 606061
TLL1
tolloid like 1
Disease-causing germline mutation(s) in
OMIM: 606742
CITED2
Cbp/p300 interacting transactivator with Glu/Asp rich carboxy-terminal domain 2
Disease-causing germline mutation(s) in
OMIM: 602937
GATA6
GATA binding protein 6
Disease-causing germline mutation(s) in
OMIM: 601656

Фенотипы (35)

Очень частый (80–99%)1
HP:0012382Left-to-right shunt
Частый (30–79%)6
HP:0001962Palpitations
HP:0002875Exertional dyspnea
HP:0003546Exercise intolerance
HP:0012378Fatigue
HP:0030718Right atrial enlargement
HP:0031664Systolic heart murmur
Периодический (5–29%)19
HP:0001633Abnormal mitral valve morphology
HP:0001635Congestive heart failure
HP:0001653Mitral regurgitation
HP:0002092Pulmonary arterial hypertension
HP:0002094Dyspnea
HP:0004749Atrial flutter
HP:0004755Supraventricular tachycardia
HP:0005110Atrial fibrillation
HP:0005115Supraventricular arrhythmia
HP:0005133Right ventricular dilatation
HP:0005162Abnormal left ventricular function
HP:0005180Tricuspid regurgitation
HP:0005957Breathing dysregulation
HP:0010741Pedal edema
HP:0011675Arrhythmia
HP:0011705First degree atrioventricular block
HP:0011710Bundle branch block
HP:0012250ST segment depression
HP:0012764Orthopnea
Очень редкий (1–4%)9
HP:0000961Cyanosis
HP:0001279Syncope
HP:0001297Stroke
HP:0001708Right ventricular failure
HP:0002090Pneumonia
HP:0002326Transient ischemic attack
HP:0002718Recurrent bacterial infections
HP:0005317Increased pulmonary vascular resistance
HP:0006536Airway obstruction

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы