First‐year mortality rates for selected birth defects, Hawaii, 1986–1999

Abstract

Birth defects have been the leading cause of infant death in the United States for over the last decade. However, there is little population-based data on the first-year mortality rates for many specific birth defects and the factors that may affect these mortality rates. This investigation examined the first-year mortality rates for 54 selected birth defects of various organ systems in Hawaii during 1986-1999 using data from a population-based birth defects registry and evaluated the impact of the presence of chromosomal abnormalities and other structural birth defects and the year of delivery on the mortality rates. Mortality rates varied widely by defect, being highest for anencephaly (100%), trisomy 13 (82%), and trisomy 18 (74%), while no first-year deaths were reported for glaucoma, bladder exstrophy, and persistent cloaca. The majority (36 of 54 or 67%) of the birth defects had a mortality rate of less than 25%. Among the 51 structural birth defects, 38 (75%) had higher first-year mortality rate for cases with chromosomal abnormalities and 42 (82%) had higher first-year mortality rates for cases with other major structural birth defects. The mortality rate among 1986-1992 deliveries was higher than the mortality rate among 1993-1999 deliveries for 37 (69%) of the 54 birth defects. This study indicates that first-year mortality rates vary widely by type of birth defect, although the mortality rate for the majority of birth defects is relatively low. The presence of a chromosomal abnormality or other structural birth defect increases the mortality rate, and mortality rates for the majority of birth defects have declined in Hawaii during the study period.

Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003

“…This effect of multiple congenital anomalies on survival has been described in other population-based studies of birth defects. 2,8,15,16 Racial/ethnic disparities in survival were found in the present study, as reported by others. After multivariable adjustment, NH blacks with functional single ventricle had a 41% greater risk of death compared with NH whites.…”

“…4 -7 Furthermore, prior published population-based studies have the limitation of using broadly defined heart defect groups and reporting survival of cases born Ͼ10 years ago. [1][2][3] The strengths of our study rest on it being population based, having carefully edited specific diagnostic categories, having births from a more recent era (1996 to 2003), and having follow-up to 5 years of age. Survival of these infants with functional single ventricle was significantly less than that of infants with d-transposition requiring neonatal arterial switch surgery.…”

“…An earlier study, covering 1986 to 1999, of first-year mortality rates for selected birth defects using data from the Hawaii Birth Defects Program reported 1-year survival for HLHS of 29% and for SV of 65%. 2 Cleves et al 9 reported 1-year survival for infants born with congenital heart defects in Arkansas in 1993 to 1998 based on a state registry in which cardiac cases were edited by a pediatric cardiologist. They reported 1-year survival for HLHS of 22.6%; for TA, 76.7%; and for PA-IVS, 63.3%.…”

“…[1][2][3] Several tertiary cardiac centers have reported, from their own surgical experience, improvement in survival for specific single ventricle defects. 4 -7 However, such center-specific data may not be applicable to all centers and do not include patients born in regional hospitals who died before transfer to the tertiary center.…”

La mortalidad infantil por malformaciones congénitas en México: un problema de oportunidad y acceso al tratamiento

“…De haber crecido esta a la par de la capacidad diagnós-tica, se habría observado un aumento en la supervivencia de los infantes con MC graves y se habría detenido -o incluso invertido-la tendencia creciente de las TMIe por MC graves. Así ha ocurrido en los países con mayor renta per cápita (18,19), pero no en los de Amé-rica Latina y de Europa Oriental (20), lo que sugiere que este renglón de la atención infantil es sensible a la magnitud de la inversión en salud.…”