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Perspectives in Disease Prevention and Health Promotion Premature Mortality Due to Congenital Anomalies -- United States

In 1986, as in previous years (1,2), congenital anomalies (CAs) were the fifth leading cause of years of potential life lost before age 65 (YPLL). They accounted for 651,523 or approximately 5.4% of all YPLL (3).

An examination of detailed mortality data for 1985 from the National Center for Health Statistics indicated that agenesis, hypoplasia, and dysplasia of the lung (ICD-9 code 748.5) were the leading causes of YPLL, accounting for 9.1% of CA-attributable YPLL (Table 1). Six types of CAs of the cardiovascular system were among the 15 leading causes of premature mortality attributed to CAs; hypoplastic left heart syndrome was the third leading cause (Table 1). Three chromosomal defects-- trisomies of chromosomes 13 and 18 and Down syndrome--were also among the leading 15 causes of CA-attributable YPLL. The two major neural tube defects, anencephalus and spina bifida, were the second and 14th leading causes, respectively, together accounting for 8.1% of YPLL attributed to CAs.

The proportional distribution of CA-attributable YPLL varied by race (Table 1). For example, anencephalus and spina bifida accounted for a higher percentage of YPLL for whites, while congenital hydrocephalus accounted for a larger proportion of YPLL for other races. Reported by: Birth Defects and Genetic Diseases Br, Div of Birth Defects and Developmental Disabilities, Center for Environmental Health and Injury Control, CDC.

Editorial Note

Editorial Note: As infant mortality due to other causes has been reduced, CAs have become the leading cause of infant mortality (4) and are the fifth leading cause of YPLL.

Variation in the proportional distribution of CA-attributable YPLL by race is due to several factors, including variations in the incidence of birth defects. For example, neural tube defects occur more frequently among whites than among other races. In addition, some of the variation for other anomalies may result from differences in access to medical care and in the likelihood of medical intervention to correct malformations, which in turn affects survival rates.

YPLL estimates may understate the public health impact of CAs for at least two reasons. First, because anomalies in infants who die shortly after birth may not be diagnosed, these infant deaths may not be attributed to CAs. Second, because YPLL statistics are based only on live births, the impact of CAs may be underestimated since a substantial number of fetuses with anomalies are stillborn or spontaneously aborted. In addition, because prenatal diagnosis of neural tube and chromosomal defects is possible in some instances, pregnancies may be terminated and are not represented in the YPLL statistics.

Improvements in the care of persons with some types of CAs may reduce YPLL in the future. However, because many infants survive with irreparable CAs and live for decades with disabilities, primary prevention of CAs is the ultimate goal. Primary prevention will require further understanding of the causes of CAs.


  1. CDC. Premature mortality due to congenital anomalies. MMWR 1986;35:97-9.

  2. CDC. Premature mortality due to congenital anomalies--United States, 1984. MMWR 1987;36:370.

  3. CDC. Table V. Estimated years of potential life lost (YPLL) before age 65 and cause-specific mortality, by cause of death--United States, 1986. MMWR 1988;37:45.

  4. Wegman ME. Annual summary of vital statistics--1986. Pediatrics 1987;80:817-27.

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