Article

Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction Trial(∗).

Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, SC 29425, USA.
The Journal of thoracic and cardiovascular surgery (Impact Factor: 3.41). 12/2010; 140(6):1245-50. DOI: 10.1016/j.jtcvs.2010.05.022
Source: PubMed

ABSTRACT The purpose of this analysis was to assess preoperative risk factors before the first-stage Norwood procedure in infants with hypoplastic left heart syndrome and related single-ventricle lesions and to evaluate practice patterns in prenatal diagnosis, as well as the role of prenatal diagnosis in outcome.
Data from all live births with morphologic single right ventricle and systemic outflow obstruction screened for the Pediatric Heart Network's Single Ventricle Reconstruction Trial were used to investigate prenatal diagnosis and preoperative risk factors. Demographics, gestational age, prenatal diagnosis status, presence of major extracardiac congenital abnormalities, and preoperative mortality rates were recorded.
Of 906 infants, 677 (75%) had prenatal diagnosis, 15% were preterm (<37 weeks' gestation), and 16% were low birth weight (<2500 g). Rates of prenatal diagnosis varied by study site (59% to 85%, P < .0001). Major extracardiac congenital abnormalities were less prevalent in those born after prenatal diagnosis (6% vs 10%, P = .03). There were 26 (3%) deaths before Norwood palliation; preoperative mortality did not differ by prenatal diagnosis status (P = .49). In multiple logistic regression models, preterm birth (P = .02), major extracardiac congenital abnormalities (P < .0001), and obstructed pulmonary venous return (P = .02) were independently associated with preoperative mortality.
Prenatal diagnosis occurred in 75%. Preoperative death was independently associated with preterm birth, obstructed pulmonary venous return, and major extracardiac congenital abnormalities. Adjusted for gestational age and the presence of obstructed pulmonary venous return, the estimated odds of preoperative mortality were 10 times greater for subjects with a major extracardiac congenital abnormality.

0 Bookmarks
 · 
81 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Congenital heart defects (CHDs) occur in nearly 1% of live births. We sought to assess factors associated with prenatal CHD diagnosis in the National Birth Defects Prevention Study (NBDPS). We analyzed data from mothers with CHD-affected pregnancies from 1998-2005. Prenatal CHD diagnosis was defined as affirmative responses to questions about abnormal prenatal ultrasounds and/or fetal echocardiography obtained during a structured telephone interview. Fifteen percent (1,097/7,299) of women with CHD-affected pregnancies (excluding recognized syndromes and single-gene disorders) reported receiving a prenatal CHD diagnosis. Prenatal CHD diagnosis was positively associated with advanced maternal age, family history of CHD, type 1 or type 2 diabetes, twin or higher order gestation, CHD complexity and presence of extracardiac defects. Prenatal CHD diagnosis was inversely associated with maternal Hispanic race/ethnicity, prepregnancy overweight or obesity, and pre-existing hypertension. Prenatal CHD diagnosis varied by time to NBDPS interview and NBDPS study site. Further work is warranted to identify reasons for the observed variability in maternal reports of prenatal CHD diagnosis and the extent to which differences in health literacy or health system factors such as access to specialized prenatal care and/or fetal echocardiography may account for such variability. This article is protected by copyright. All rights reserved.
    Prenatal Diagnosis 11/2013; · 2.68 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: An online survey for parents of children with congenital heart disease (CHD) was developed to study the perceptions and experiences of parents when receiving the diagnosis. The survey was distributed to online support groups. A total of 841 responses from parents of children with CHD in the United States were received over a 4-week period in 2010. The authors hypothesized that the counseling and demeanor of the pediatric cardiologist (PC) may be important factors in determining whether parents of children with CHD seek second opinions, and that the terminology used in counseling may be variably interpreted. Of the 841 respondents, 349 (41 %) received the diagnosis prenatally. A minority of the respondents received: support group information (14 %), Internet resources (21 %), success rates at other hospitals (16 %), or maximum ages of survivors (29 %). Among 26 % of the parents who reported seeking a second opinion from another PC, the majority (71 %) chose the second PC for long-term follow-up care. Those receiving a prenatal diagnosis were more likely to seek a second opinion than those receiving the diagnosis postnatally (32 vs 22 %; p < 0.01). Parents' perception of the PC's compassion and empathy was inversely related to the likelihood of seeking a second opinion. Parents were more likely to seek a second opinion when they were not optimistic about their child's life expectancy, felt pressured by the PC to terminate the pregnancy, were told that their child's death was "somewhat" or "very" likely, or were told the child's CHD was "rare" (all p < 0.01). Two thirds (66 %) of the respondents were told that their child's condition was "rare." The majority of these (77 %) reported that the term was used by the PC. "Rare" was interpreted as "occurring in less than a million births" by 25 %, and as "few or no other people alive with this defect" by 27 %. Parental interpretation of "rare" was unrelated to their levels of education. As reported by the respondents, 13 % felt pressured to terminate the pregnancy by the PC. Those with hypoplastic left heart syndrome were more likely to report feeling pressure to terminate the pregnancy by the PC (21 vs 9 %; p < 0.001) or the perinatologist (23 vs 14 %; p = 0.026). The approach to counseling and the demeanor of the PC have important implications for parents' perceptions of their child's chance of survival. The information given at diagnosis, the manner in which it is presented, and the parents' understanding and interpretation of that information are critical factors in shaping parents' perceptions and management decisions.
    Pediatric Cardiology 08/2013; · 1.20 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Among infants with single ventricle congenital heart disease (SVD) requiring Stage I palliation (S1P), the impact of prenatal diagnosis (PD) on outcomes has been variably characterized. We investigated the impact of PD in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) registry. Retrospective analysis of demographic and outcomes data among infants enrolled in the NPCQIC database; eligibility includes SVD requiring S1P and survival to discharge. From 43 contributing surgical centers, 591 infants had data available through time of BDG (519) or interstage death (55). Median gestational age was 39 weeks (31-46), and 66 % had variants of hypoplastic left heart syndrome. PD was made in 445 (75 %), with significant variation by center (p = 0.004). While infants with PD had slightly lower gestational age at birth (p < 0.001), there were no differences in birth weight, the presence of major syndromes or other organ system anomalies. Those without PD were more likely to have atrioventricular valve regurgitation (p = .002), ventricular dysfunction (p = 0.06), and pre-operative risk factors including acidosis (p < 0.001), renal insufficiency (p = 0.007), and shock (p = 0.05). Post-operative ventilation was shorter in the PD group (9 vs. 12 d, p = 0.002). Other early post-operative outcomes, interstage course, and outcomes at BDG were similar between groups. In a large cohort of infants with SVD surviving to hospital discharge after S1P, PD showed significant inter-site variation and was associated with improved pre-operative status and shorter duration of mechanical ventilation. The significance of such associations merits further study.
    Pediatric cardiology. 08/2014;

Full-text (2 Sources)

Download
10 Downloads
Available from
Jun 1, 2014