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: 4.17). 12/2010; 140(6):1245-50. DOI: 10.1016/j.jtcvs.2010.05.022
Source: PubMed


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.

Download full-text


Available from: Thomas G Travison
  • [Show abstract] [Hide abstract]
    ABSTRACT: A boundary element method featuring the Green's function for the circular cylinder has been used to compute particle hydrodynamic mobilities in a channel. The Green's function has very slowly converging Fourier-Bessel sums; consequently, construction of the integral equation kernel, i.e. the matrix assembly, accounts for essentially all of the CPU time in the sequential algorithm. A Single Program Multiple Data (SPMD) model was used to assign the matrix assembly to N processors. The validity of the SPMD model was verified using Condor, a batch job scheduler designed to pool idle workstation cycles in the network. Our work is typical of many CPU intensive computations in engineering and suggests that a SPMD approach that uses idle resources over a network is an attractive option. Our results are in excellent agreement with established mobilities for simple test cases, and can he readily extended to more interesting geometries.
    No preview · Article · Jun 1995 · Computers & Chemical Engineering
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Up to 20% of fetuses with critical left heart obstructive lesions have highly restrictive or intact atrial septae. Although this condition is generally tolerated in utero, severe hypoxemia requiring emergency atrial septostomy often develops in newborns with restrictive atrial septum. We have reported that a pulmonary venous Doppler forward/reverse time-velocity integral ratio less than 5 is highly predictive of the need for emergency atrial septostomy. We reviewed our subsequent experience using fetal pulmonary venous Doppler patterns to identify and manage fetuses with critical left heart obstruction and suspected restrictive atrial septum. A retrospective review of neonates with a prenatal diagnosis of critical left heart obstruction was performed. Fetal restrictive atrial septum was defined as a small/absent interatrial shunt on 2-dimensional imaging and a mean forward/reverse time-velocity integral ratio of 5 or less. Available serial pulmonary venous Doppler data were reviewed. The primary outcome was postnatal confirmation of restrictive atrial septum or severe left atrial hypertension. Eight of 39 infants had a forward/reverse time-velocity integral ratio of 5 or less. A restrictive atrial septum was confirmed postnatally in 6 of 8 infants. Overall, a forward/reverse time-velocity integral ratio of 5 or less had a sensitivity of 100% and specificity of 94% for emergency atrial septostomy. Lowering the cutoff value to 3 or less would have eliminated false-positive diagnoses in the current series. Serial data demonstrated that late second trimester values did not change in later gestation with respect to either threshold in 30 of 32 fetuses. In the fetus with critical left heart obstruction, a threshold forward/reverse time-velocity integral ratio of 3 or less optimizes specificity for predicting emergency atrial septostomy. Most late second trimester values will not change over time with regard to threshold levels.
    Full-text · Article · Dec 2010 · The Journal of thoracic and cardiovascular surgery
  • [Show abstract] [Hide abstract]
    ABSTRACT: As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry. From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9-5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia. In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.
    No preview · Article · Mar 2011 · Congenital Heart Disease
Show more