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. Author manuscript; available in PMC: 2013 Jan 22.
Published in final edited form as: Am J Med Genet A. 2012 Sep 10;158A(10):2499–2505. doi: 10.1002/ajmg.a.35582

TABLE IV.

Types of Congenital Heart Defects and CdLS Severity

Defect (# of probands) Total number (279) Mild (130) Moderate (89) Severe (60)
Septal defects
 VSD 20 8 5 5
 ASD 19 6 5 3
 AVSD 6 1 2 3
 Other (unspecified) 5 2 2
Overall prevalence 18% 12% 16% 22%
Outflow obstruction
 Pulmonic valve 23 4 8 8
 Aortic 11 3 6 1
 Mitral 3 1 2 0
Overall prevalence 13% 6% 18% 15%
Vascular anomalies
 Coarctation/hypoplastic aorta 15 5 5 4
 PS/PPS 15 4 5 5
 Enlarged aortic root 1 1
Overall prevalence 11% 8% 11% 15%
Complex defect
 TOF 3 2 1
 TOF/DORV 2 2
 DORV 1 1
 AV canal defect 1 1 1
 TAPVR 1 1
 Truncus art 1
Overall prevalence 5% 1% 3% 10%
Dextrocardia 2 1 1
Fetal persistent
 PFO 28 8 9 8
 PDA 27 6 4 11
Innocent murmur 42 23 10 8

VSD, ventricular septal defect; ASD, atrial septal defect; AVSD, atrioventricular septal defect; PS, pulmonic stenosis; PPS, peripheral pulmonic stenosis; TOF, tetralogy of Fallot; DORV, double-outlet right ventricle; AV, atrioventricular; TAPVR, total anomalous pulmonary venous return; PFO, patent foramen ovale; PDA, patent ductus arteriosus.