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. Author manuscript; available in PMC: 2026 Sep 1.
Published in final edited form as: Clin Perinatol. 2025 Jul 15;52(3):589–608. doi: 10.1016/j.clp.2025.06.010

Table 3.

Genes associated with non-syndromic congenital heart disease (selected)

Gene Cardiovascular malformation OMIM References
Transcription factors
CITED2 ASD, VSD 602937 16287139
GATA4 ASD, VSD, AVSD, PS, TOF 600576 12845333
15235040
15810002
15689439
18055909
18076106
GATA5 ASD, VSD, DORV, TOF, BAV 611496 23031282
24638895
GATA6 PTA, TOF, ASD, BAV 601656 19666519
20581743
20631719
37700164
HAND1 AVSD, DORV, HLHS, ASD, VSD 602406 18276607
19586923
HAND2 TOF, LVNC, VSD 602407 20819618
26865696
MED13L TGA 608771 14638541
NR2F2 AVSD, AS, CoA, VSD, HLHS, TOF 107773 24702954
NKX2–5 ASD, atrioventricular conduction delay, TOF, HLHS, VSD 600584 9651244
10587520
11714651
14607454
NKX2–6 PTA 611770 15649947
24421281
TBX1 DORV, TOF, IAA, PTA, VSD 602054 14585638
TBX5 AVSD, TOF, BAV, CoA, ASD, VSD 601620 22543974
TBX20 ASD, VSD, MS, DCM 606061 17668378
27510170
ZFPM2/FOG2 TOF, DORV 603693 14517948
Cell signaling and adhesion proteins
ACVR1/ALK2 AVSD 102576 19506109
CRELD1 ASD, AVSD 607170 12632326
FOXH1 TOF, TGA, VSD 603621 18538293
GJA1 HLHS, VSD, PA 121014 11470490
19615768
HEY2 AVSD 604674 16329098
JAG1 TOF, PS 601920 9207788
9207787
11152664
NOTCH1 BAV, AS, HLHS, TOF, PS, ASD, VSD, CoA, DORV 190198 16025100
26820064
PDGFRA2 TAPVR 173490 20071345
SMAD6 BAV, CoA, AS 602931 22275001
TAB2 BAV, AS, TOF 605101 20493459
VEGFA TOF, PDA, AS, BAV, CoA, IAA, VSD 192240 30232381
20420808
Structural proteins
ACTC1 ASD, HCM, DCM, LVNC 102540 17947298
DCHS1 MVP 603057 26258302
ELN SVAS 130160 7693128
11175284
MYH6 ASD, HCM, DCM 160710 15735645
20656787
MYH7 Ebstein’s anomaly, LVNC, HCM, DCM 160760 18159245
21127202
MYH11 PDA, TAA 160745 16444274
27418595

AS, aortic stenosis; ASD, atrial septal defect; AVSD, atrioventricular septal defect; BAV, bicuspid aortic valve; CoA, coarctation of the aorta; DCM, dilated cardiomyopathy; DORV, double-outlet right ventricle; HCM, hypertrophic cardiomyopathy; HLHS, hypoplastic left heart syndrome; IAA, interruption of aortic arch; LVNC, left ventricular noncompaction cardiomyopathy; MS, mitral valve stenosis; MVP, mitral valve prolapse; PA, pulmonary atresia; PDA, patent ductus arteriosus; PS, pulmonary stenosis; PTA, persistent truncus arteriosus; SVAS, supravalvular aortic stenosis; TAA thoracic aortic aneurysm; TAPVR total anomalous pulmonary venous return; TGA, transposition of great arteries; TOF, tetralogy of Fallot; and VSD, ventricular septal defect.