Risk factors for mortality after the Norwood procedure

Gaynor, J. William; Mahle, William T.; Cohen, Mitchell I.; Ittenbach, Richard F.; DeCampli, William M.; Steven, James M.; Nicolson, Susan C.; Spray, Thomas L.

doi:10.1016/s1010-7940(02)00198-7

Cited by 291 publications

(222 citation statements)

References 12 publications

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“…42 Certain factors are associated with worse outcomes from congenital heart defects, including the type and complexity of the malformation, associated extracardiac anomalies and congestive heart failure. [203][204][205][206][207][208][209][210][211] Children born with congenital heart defects have a higher incidence of neurological abnormalities, including microcephaly, hypotonia, hypertonia, seizures, feeding difficulties and lethargy in the newborn period, 173,175 and these do not appear to be predicted by difficulties at birth, such as birth asphyxia. 164,166,212 Preoperative cerebral infarctions have been noted in 4% of children with congenital heart defects and are associated with subsequent abnormal neurological development.…”

Section: Antenatal and Perinatal Factorsmentioning

confidence: 99%

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

Knowles¹,

Griebsch²,

Dezateux

et al. 2005

Health Technol Assess

214

143

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Section: Antenatal and Perinatal Factorsmentioning

confidence: 99%

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

Knowles¹,

Griebsch²,

Dezateux

et al. 2005

Health Technol Assess

214

143

View full text Add to dashboard Cite

show abstract

“…In addition, there is a rapidly growing population of adult survivors with chronic morbidities that are only now beginning to be understood (8). Although some surgical risk factors have been identified, such as the presence of a genetic syndrome, low birth weight (LBW, <2.5 kg), or a prohibitively small ascending aorta, our ability to predict adverse clinical outcomes remains limited (4,9,10). Understanding the noncardiac parameters of fetal HLHS may lead to the identification of new risk factors that may ultimately improve clinical management.…”

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confidence: 99%

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome

et al. 2013

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Background: Hypoplastic left heart syndrome (HLHS) is associated with significant mortality and morbidity. Fetal head growth abnormalities have been identified in a subset of HLHS fetuses, but it is unclear whether specific patterns of maladaptive growth affect clinical outcomes. We hypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes. Methods: We retrospectively examined a cohort of HLHS patients from midgestation to 1 y of age. Fetal and birth anthropometric measurements were analyzed using the Olsen standard, and clinical outcomes were obtained. results: A total of 104 HLHS patients were identified over a 12-y period; fetal data were available in 38 cases. HLHS neonates demonstrated a high incidence of microcephaly (12%), small head size (27%), and poor head growth (32%). Allcause mortality was 31% at 30 d and 43% at 1 y. Neurologic outcomes were observed in 12% of patients and were significantly increased with microcephaly (43 vs. 4%; P = 0.02). The average length of hospital stay following stage I palliation was 33.4 ± 33 d, correcting for early death. conclusion: In term nonsyndromic HLHS, fetal and neonatal microcephaly are associated with early adverse neurologic outcomes but not mortality.

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“…H ighly restrictive or intact atrial septum (RAS) in the setting of hypoplastic left heart syndrome (HLHS) occurs in approximately 6% to 11% of cases [1][2][3] and is associated with poor short-and medium-term outcomes [1][2][3][4][5][6] despite evolving strategies [1][2][3]7,8 to urgently decompress the left atrium and diminish left atrial hypertension in the immediate postnatal period. Persistent mortality despite relief of RAS in these patients may be partly a result of muscularization of the pulmonary arteries and arterialization of the pulmonary veins because of chronic in utero pulmonary venous hypertension.…”

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confidence: 99%

Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle

et al. 2005

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Background-Pulmonary venous Doppler (PVD) flow patterns in the fetus with hypoplastic left heart syndrome (HLHS) have been correlated with restrictive interatrial communication or intact atrial septum (RAS) postnatally; however, the ability of PVD to identify the neonate requiring emergent atrial septoplasty (EAS) for severe left atrial hypertension and hypoxemia has not been critically evaluated. It was the purpose of this study to determine the predictive power of fetal PVD in identifying the need for EAS in newborns with HLHS and RAS. Methods and Results-Forty-one patients with fetal PVD flow analysis and postnatally confirmed HLHS were studied.Pulsed-wave assessment of PVD flow included S-, D-, and A-wave velocity, time-velocity integral (VTI) of forward and reverse flow, and S/D velocity and forward/reverse VTI ratio. Neonatal EAS was used as the primary clinical outcome variable. Receiver operating characteristic curves were used to determine cutpoints at which PVD indices best predicted EAS. Cutpoints were evaluated for clinical accuracy and usefulness by use of Bayesian analysis. Eight of 41 subjects underwent EAS. Need for EAS was most accurately predicted by forward/reverse VTI ratio Ͻ5 (sensitivity, 0.88, 95% CI, 0.49 to 0.99; specificity, 0.97, 95% CI, 0.82 to 0.99), which, when present, increases the posttest likelihood of EAS to 74%, assuming a pretest prevalence of 10%. Accuracy and usefulness of other PVD indices were affected by false-positive results. Conclusions-In the fetus with HLHS, a PVD forward/reverse VTI ratio of Ͻ5 is the strongest predictor of the need for EAS in the newborn period. These observations should improve our ability to identify and expectantly manage the fetus with HLHS and RAS.

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Risk factors for mortality after the Norwood procedure

Abstract: The Norwood procedure is currently being applied to a heterogeneous group of patients. Operative and 1-year survival are equivalent for patients with HLHS and those with other cardiac defects. The presence of additional cardiac or extra-cardiac anomalies are predictors of poor outcome.

Cited by 291 publications

References 12 publications

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome

Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle

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