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
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.
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ABSTRACT: Purpose of review Fetal cardiology is a rapidly evolving field. Imaging technology continues to advance as do approaches to in-utero interventions and care of the critically ill neonate, with even greater demand for improvement in prenatal diagnosis of congenital heart disease (CHD) and arrhythmias. Recent findings Reviewing the advances in prenatal diagnosis of CHD in such a rapidly developing field is a broad topic. Therefore, we have chosen to focus this review of recent literature on challenges in prenatal detection of CHD, challenges in prenatal counseling, advances in fetal arrhythmia diagnosis, and potential benefits to patients with CHD who are identified prenatally. Summary As methods and tools to diagnose and manage CHD and arrhythmias in utero continue to improve, future generations will hopefully see a reduction in both prenatal and neonatal morbidity and mortality. Prenatal diagnosis can and should be used to optimize location and timing of delivery and postnatal interventions. Video abstract http://links.lww.com/MOP/A21Current Opinion in Pediatrics 08/2014; 26(5). DOI:10.1097/MOP.0000000000000136 · 2.74 Impact Factor
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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.
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ABSTRACT: Objectives: The technical performance score (TPS) has been reported in a single center study to predict the outcomes after congenital cardiac surgery. We sought to determine the association of the TPS with outcomes in patients undergoing the Norwood procedure in the Single Ventricle Reconstruction trial. Methods: We calculated the TPS (class 1, optimal; class 2, adequate; class 3, inadequate) according to the predischarge echocardiograms analyzed in a core laboratory and unplanned reinterventions that occurred before discharge from the Norwood hospitalization. Multivariable regression examined the association of the TPS with interval to first extubation, Norwood length of stay, death or transplantation, unplanned postdischarge reinterventions, and neurodevelopment at 14 months old. Results: Of 549 patients undergoing a Norwood procedure, 356 (65%) had an echocardiogram adequate to assess atrial septal restriction or arch obstruction or an unplanned reintervention, enabling calculation of the TPS. On multivariable regression, adjusting for preoperative variables, a better TPS was an independent predictor of a shorter interval to first extubation (P = .019), better transplant-free survival before Norwood discharge (P < .001; odds ratio, 9.1 for inadequate vs optimal), shorter hospital length of stay (P < .001), fewer unplanned reinterventions between Norwood discharge and stage II (P = .004), and a higher Bayley II psychomotor development index at 14 months (P = .031). The TPS was not associated with transplant-free survival after Norwood discharge, unplanned reinterventions after stage II, or the Bayley II mental development index at 14 months. Conclusions: TPS is an independent predictor of important outcomes after Norwood and could serve as a tool for quality improvement.Journal of Thoracic and Cardiovascular Surgery 06/2014; 148(5). DOI:10.1016/j.jtcvs.2014.05.076 · 3.99 Impact Factor