Maine
Birth Defects Counts and Prevalence 2018-2022 (Prevalence per 10,000 Live Births)
Defect
Maternal Race/Ethnicity
 Total * Notes
White,
Non-Hispanic		 Black,
Non-Hispanic		 Hispanic,
Non-Hispanic		 Asian or
Pacific Islander,
Non-Hispanic		 American Indian
or Alaskan Native,
Non-Hispanic
Anencephaly 5 (1.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 5 (0.9)
Anophthalmia / microphthalmia <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Anotia / microtia 8 (1.5) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†) 9 (1.5)
Aortic valve stenosis <5 (†) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 5 (0.9)
Atrial septal defect 165 (31.9) 16 (54.3) <5 (†) 6 (56.2) <5 (†) 193 (33.1)
Atrioventricular septal defect (endocardial cushion defect) 8 (1.5) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 10 (1.7)
Biliary atresia <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Bladder exstrophy <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Choanal atresia 8 (1.5) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†) 9 (1.5)
Cleft lip alone (without cleft palate) 15 (2.9) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 15 (2.6)
Cleft lip with cleft palate 32 (6.2) 0 (0.0) <5 (†) 0 (0.0) <5 (†) 34 (5.8)
Cleft palate alone (without cleft lip) 25 (4.8) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†) 28 (4.8)
Cloacal exstrophy 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Clubfoot 81 (15.7) <5 (†) 0 (0.0) <5 (†) <5 (†) 86 (14.8)
Coarctation of aorta 8 (1.5) <5 (†) <5 (†) 0 (0.0) 0 (0.0) 11 (1.9)
Common truncus (truncus arteriosus or TA) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Congenital cataract <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Congenital posterior urethral valves 24 (9.1) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 24 (8.1) 1
Craniosynostosis 13 (2.5) <5 (†) 0 (0.0) 0 (0.0) <5 (†) 15 (2.6)
Deletion 22q11.2 <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Dextro-Transposition of great arteries (d-TGA) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Diaphragmatic hernia 5 (1.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 5 (0.9)
Double outlet right ventricle (DORV) <5 (†) 0 (0.0) 0 (0.0) <5 (†) 0 (0.0) <5 (†)
Ebstein anomaly 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Encephalocele 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Esophageal atresia / tracheoesophageal fistula 8 (1.5) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†) 9 (1.5)
Gastroschisis 10 (1.9) 0 (0.0) <5 (†) <5 (†) 0 (0.0) 13 (2.2)
Holoprosencephaly <5 (†) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Hypoplastic left heart syndrome <5 (†) 0 (0.0) 0 (0.0) <5 (†) 0 (0.0) <5 (†)
Hypospadias 137 (51.9) 7 (47.1) <5 (†) <5 (†) <5 (†) 150 (50.5) 1
Interrupted aortic arch (IAA) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Limb deficiencies (reduction defects) 13 (2.5) <5 (†) 0 (0.0) <5 (†) 0 (0.0) 15 (2.6)
Omphalocele 5 (1.0) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 6 (1.0)
Pulmonary valve atresia 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Pulmonary valve atresia and stenosis 7 (1.4) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 7 (1.2)
Rectal and large intestinal atresia / stenosis 9 (1.7) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†) 10 (1.7)
Renal agenesis / hypoplasia 19 (3.7) 0 (0.0) 0 (0.0) <5 (†) <5 (†) 21 (3.6)
Single ventricle <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Small intestinal atresia / stenosis 7 (1.4) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 7 (1.2)
Spina bifida without anencephaly 10 (1.9) <5 (†) 0 (0.0) <5 (†) 0 (0.0) 12 (2.1)
Tetralogy of Fallot (TOF) 11 (2.1) 0 (0.0) 0 (0.0) <5 (†) <5 (†) 14 (2.4)
Total anomalous pulmonary venous connection (TAPVC) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Transposition of the great arteries (TGA) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Tricuspid valve atresia 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0)
Tricuspid valve atresia and stenosis 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Trisomy 13 <5 (†) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) <5 (†)
Trisomy 18 5 (1.0) <5 (†) 0 (0.0) 0 (0.0) 0 (0.0) 6 (1.0)
Trisomy 21 (Down syndrome) 42 (8.1) 7 (23.8) <5 (†) <5 (†) <5 (†) 55 (9.4)
Turner syndrome 6 (2.4) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 6 (2.1) 2
Ventricular septal defect 129 (24.9) <5 (†) <5 (†) <5 (†) <5 (†) 139 (23.9)
Total live births 51753 2944 1331 1068 522 58226 3
Male Live Births 26394 1486 691 550 283 29717
Female Live Births 25323 1457 640 518 238 28470
Notes
1- Data for this condition include male and unknown infant sex cases only. Prevalence is calculated per 10,000 male live births.
2- Data for this condition include female and unknown infant sex cases only. Prevalence is calculated per 10,000 female live births.
3- Data for total live births include unknown infant sex.

General comments
†- Counts <5 and associated prevalences are suppressed
*- Data for totals include unknown/other race
Maine
Birth Defects Counts and Prevalence 2018-2022 (Prevalence per 10,000 Live Births)
Defect
Maternal Age
 Total *
Less than 35 years 35 of more years
Gastroschisis 12 (2.6) <5 (†) 13 (2.2)
Trisomy 13 <5 (†) <5 (†) <5 (†)
Trisomy 18 <5 (†) <5 (†) 6 (1.0)
Trisomy 21 (Down syndrome) 27 (5.7) 28 (24.9) 55 (9.4)
Total live births 46974 11227 58226
Notes
†- Counts <5 and associated prevalences are suppressed
*- Data for totals include unknown/other maternal age
Maine
Notes on Specific Birth Defects
Defect Site Comment
Ventricular septal defect Data for this condition does not exclude Inlet VSD or common atrioventricular (AV) canal type VSD.
Atrioventricular septal defect (endocardial cushion defect) Data for this condition does not include Inlet VSD or common atrioventricular (AV) canal type VSD.
Tricuspid valve atresia and stenosis Data for this condition include cases with Tricuspid stenosis or hypoplasia.
Tricuspid valve atresia Data for this condition include cases with Tricuspid stenosis or hypoplasia.
Hypospadias Data for this condition include male cases only.
Turner syndrome Data for this condition include female cases only.
Congenital posterior urethral valves Data for this condition include male cases only.
Universal comment Data for total live births include non-binary and unknown gender.
Universal comment Data for conditions include possible/probable cases.
Universal comment Data for conditions include only live births. Data do not include stillbirths, terminations, or other pregnancy outcomes.