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.
References
CDC. Premature mortality due to congenital anomalies. MMWR
1986;35:97-9.
CDC. Premature mortality due to congenital anomalies--United
States,
1984. MMWR 1987;36:370.
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.
Wegman ME. Annual summary of vital statistics--1986. Pediatrics
1987;80:817-27.
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