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. Author manuscript; available in PMC: 2019 Aug 22.
Published in final edited form as: Birth Defects Res. 2018 Nov 16;110(19):1478–1486. doi: 10.1002/bdr2.1413

TABLE 1.

Birth defects for the National Birth Defects Prevention Network (NBDPN) annual report by disease classification codes

Birth defects ICD-9-CM codes ICD-10-CM codes CDC/BPA codes
Central nervous system
 Anencephaly 740.0–740.1 Q00.0-Q00.1 740.00–740.10
 Spina bifida without anencephaly 741.0, 741.9 w/o 740.0–740.1 Q05.0-Q05.9, Q07.01, Q07.03 w/o Q00.0-Q00.1 741.00–741.99 w/o 740.00–740.10
 Encephalocele 742.0 Q01.0-Q01.9 742.00–742.09
 Holoprosencephaly 742.2 Q04.2 742.26
Eyea
 Anophthalmia/microphthalmia 743.0, 743.1 Q11.0-Q11.2 743.00–743.10
 Congenital cataract 743.30–743.34 Q12.0 743.32
Eara
 Anotia/microtia 744.01, 744.23 Q16.0, Q17.2 744.01, 744.21
Cardiovascular
 Common truncus (TA) 745.0 Q20.0 745.00 (excluding 745.01)
 Transposition of the great arteries (TGA) 745.10, 0.12, 0.19 Q20.3, Q20.5 745.10–745.12, 745.18–745.19
bDextro-Transposition of the great arteries (d-TGA) 745.10 Q20.3 745.10, 745.11, 745.18, 745.19
 Tetralogy of Fallot (TOF) 745.2 Q21.3 745.20–745.21, 747.31
 Ventricular septal defect 745.4 Q21.0 745.40–745.49 (excluding 745.487, 745.498)
 Atrial septal defect 745.5 Q21.1 745.51–745.59
 Atrioventricular septal defect (endocardial cushion defect) 745.60, 0.61, 0.69 Q21.2 745.60–745.69, 745.487
 Pulmonary valve atresia and stenosis 746.01, 746.02 Q22.0, Q22.1 746.00, 746.01
bPulmonary valve atresia 746.01 Q22.0 746.00
 Tricuspid valve atresia and stenosis 746.1 Q22.4 746.100, 746.106 (excluding 746.105)
bTricuspid valve atresia 746.1 Q22.4 746.100
 Ebstein anomaly 746.2 Q22.5 746.20
 Aortic valve stenosis 746.3 Q23.0 746.30
 Hypoplastic left heart syndrome 746.7 Q23.4 746.70
 Coarctation of aorta 747.10 Q25.1 747.10–747.19
 Total anomalous pulmonary venous connection (TAPVC) 747.41 Q26.2 747.42
 Single ventricle 745.3 Q20.4 745.3
 Interrupted aortic arch (IAA) 747.11 Q25.2, Q25.4 747.215–747.217, 747.285
 Double outlet right ventricle (DORV) 745.11 Q20.1 745.13–745.15
Orofacial
 Cleft palate alone (without cleft lip) 749.0 Q35.1-Q35.9 749.00–749.09
 Cleft lip alone (without cleft palate) 749.1 Q36.0-Q36.9 749.10–749.19
 Cleft lip with cleft palate 749.20–749.25 Q37.0-Q37.9 749.20–749.29
 Choanal atresia 748.0 Q30.0 748.00
Gastrointestinal
 Esophageal atresia/tracheoesophageal fistula 750.3 Q39.0-Q39.4 750.30–750.35
 Rectal and large intestinal atresia/stenosis 751.2 Q42.0-Q42.9 751.20–751.24
 Biliary atresia 751.61 Q44.2-Q44.3 751.65
 Small intestinal atresia/stenosis 751.1 Q41.0-Q41.9 751.10–751.19
Genitourinary
 Renal agenesis/hypoplasia 753.0 Q60.0-Q60.6 753.00–753.01
 Bladder exstrophy 753.5 Q64.10, Q64.19 753.50
 Hypospadias 752.61 Q54.0-Q54.9 (excluding Q54.4) 752.60–752.62 (excluding 752.61 and 752.621)
 Congenital posterior urethral valves 753.6 Q64.2 753.60
 Cloacal exstrophy 751.5 Q64.12 751.555
Musculoskeletal
 Gastroschisis 756.73 Q79.3 756.71
 Omphalocele 756.72 Q79.2 756.70
 Diaphragmatic hernia 756.6 Q79.0, Q79.1 756.610–756.617
 Limb deficiencies (reduction defects) 755.2–755.4 Q71.0-Q71.9, Q72.0-Q72.9, Q73.0-Q73.8 755.20–755.49
 Craniosynostosis No specific code Q75.0 756.00–756.03
 Clubfoot 754.51, 754.70 Q66.0, Q66.89 754.50, 754.73 (excluding 754.735)
Chromosomal
 Trisomy 13 758.1 Q91.4-Q91.7 758.10–758.19
 Trisomy 21 (down syndrome) 758.0 Q90.0-Q90.9 758.00–758.09
 Trisomy 18 758.2 Q91.0– 91.3 758.20–758.29
 Turner syndrome 758.6 Q96.0-Q96.9 758.60–758.69
 Deletion 22 q11.2 758.32 Q93.81 758.37

ICD-9-CM: International Classification of Diseases, 9th Revision, Clinical Modification; ICD-10-CM: International Classification of Diseases, 10th Revision, Clinical Modification; CDC/BPA: Centers for Disease Control and Prevention/British Pediatric Association Classification of Diseases; TA: truncus arteriosus; TGA: transposition of the great arteries; d-TGA: d-TGA dextro-transposition of great arteries; TOF: tetralogy of Fallot; TAPVC: total anomalous pulmonary venous connection; IAA: interrupted aortic arch; DORV: double outlet right ventricle.

a

Eye and ear anomalies are the focus of this year’s data brief.

b

These sub-categories of the broader reported conditions (transposition of the great arteries, pulmonary valve atresia and stenosis, and tricuspid valve atresia and stenosis) are presented given the interest in monitoring critical congenital heart defects targeted for pulse oximetry screening (Mai et al., 2012).