Mario A Cleves1, Sadia Ghaffar, Weizhi Zhao, Bridget S Mosley, Charlotte A Hobbs. 1. Arkansas Center for Birth Defects Research and Prevention, Department of Pediatrics, University of Arkansas for Medical Sciences, College of Medicine, Arkansas Children's Hospital, Little Rock, Arkansas 72211, USA. clevesmarioa@uams.edu
Abstract
BACKGROUND: In the United States and other developed nations, birth defects are the leading cause of infant mortality. Congenital heart defects (CHDs) are among the most prevalent and fatal of all birth defects. Here we report the survival probability of infants born with CHDs in Arkansas and examine the impact of multiple malformations on survival. METHODS: Birth and death certificate records were linked to birth defects registry data for infants born with CHDs from January 1993 through December 1998 in Arkansas. Both neonatal and first-year survival probabilities were estimated. These were computed non-parametrically using Kaplan-Meier's product limit method. A Cox proportional-hazards model was used to evaluate the relative importance of additional malformations on survival. RESULTS: A total of 1,983 infants with CHDs were included in this study. The neonatal survival probability for this cohort was 94.0% (95% CI: 93.0%, 95.1%), and the first-year survival probability was 88.2% (95% CI: 86.8%, 89.6%). The presence of hypoplastic left heart syndrome conferred the greatest reduction in survival, whereas infants with pulmonic valve stenosis and infants with ventricular septal defects had the highest first-year survival. Infants with multiple CHDs had decreased survival compared to those with isolated heart defects. Survival was also adversely affected by the presence of congenital abnormalities in other body systems. CONCLUSIONS: Neonatal and first-year survival of infants with CHDs varies by both the type of cardiac malformation and the presence of additional cardiac and non-cardiac malformations. Further work will focus on the effects of maternal and infant characteristics on survival.
BACKGROUND: In the United States and other developed nations, birth defects are the leading cause of infant mortality. Congenital heart defects (CHDs) are among the most prevalent and fatal of all birth defects. Here we report the survival probability of infants born with CHDs in Arkansas and examine the impact of multiple malformations on survival. METHODS: Birth and death certificate records were linked to birth defects registry data for infants born with CHDs from January 1993 through December 1998 in Arkansas. Both neonatal and first-year survival probabilities were estimated. These were computed non-parametrically using Kaplan-Meier's product limit method. A Cox proportional-hazards model was used to evaluate the relative importance of additional malformations on survival. RESULTS: A total of 1,983 infants with CHDs were included in this study. The neonatal survival probability for this cohort was 94.0% (95% CI: 93.0%, 95.1%), and the first-year survival probability was 88.2% (95% CI: 86.8%, 89.6%). The presence of hypoplastic left heart syndrome conferred the greatest reduction in survival, whereas infants with pulmonic valve stenosis and infants with ventricular septal defects had the highest first-year survival. Infants with multiple CHDs had decreased survival compared to those with isolated heart defects. Survival was also adversely affected by the presence of congenital abnormalities in other body systems. CONCLUSIONS: Neonatal and first-year survival of infants with CHDs varies by both the type of cardiac malformation and the presence of additional cardiac and non-cardiac malformations. Further work will focus on the effects of maternal and infant characteristics on survival.
Authors: Ying Wang; Gang Liu; Mark A Canfield; Cara T Mai; Suzanne M Gilboa; Robert E Meyer; Marlene Anderka; Glenn E Copeland; James E Kucik; Wendy N Nembhard; Russell S Kirby Journal: J Pediatr Date: 2015-01-29 Impact factor: 4.406
Authors: Dongngan T Truong; Shaji C Menon; Linda M Lambert; Phillip T Burch; Xiaoming Sheng; L LuAnn Minich; Richard V Williams Journal: Pediatr Cardiol Date: 2018-05-24 Impact factor: 1.655
Authors: Christopher Wren; Claire A Irving; Josephine Amanda Griffiths; John J O'Sullivan; Milind P Chaudhari; Simon R Haynes; Jon H Smith; J R Leslie Hamilton; Asif Hasan Journal: Eur J Pediatr Date: 2011-07-12 Impact factor: 3.183
Authors: Ming Li; Jingyun Li; Changshuai Wei; Qing Lu; Xinyu Tang; Stephen W Erickson; Stewart L MacLeod; Charlotte A Hobbs Journal: Ann Hum Genet Date: 2015-11-27 Impact factor: 1.670
Authors: Ming Li; Mario A Cleves; Himel Mallick; Stephen W Erickson; Xinyu Tang; Todd G Nick; Stewart L Macleod; Charlotte A Hobbs Journal: Hum Genet Date: 2014-06-04 Impact factor: 4.132