Congenital heart anomaly in newborns with congenital diaphragmatic hernia: A single-center experience

Rodrigo Ruano, P. Javadian, J. A. Kailin, S. A. Maskatia, A. A. Shamshirsaz, D. L. Cass, I. J. Zamora, H. Sangi-Haghpeykar, T. C. Lee, N. A. Ayres, A. Mehollin-Ray, C. I. Cassady, C. Fernandes, S. Welty, M. A. Belfort, O. O. Olutoye

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Objective To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Methods In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively. Results Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤2 for both RACHS-1 (area under the curve (AUC), 0.74 (P=0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P=0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P=0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO. Conclusions In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.

Original languageEnglish (US)
Pages (from-to)683-688
Number of pages6
JournalUltrasound in Obstetrics and Gynecology
Volume45
Issue number6
DOIs
StatePublished - Jan 1 2015
Externally publishedYes

Fingerprint

Newborn Infant
Risk Adjustment
Extracorporeal Membrane Oxygenation
Thoracic Surgery
Area Under Curve
Survival
Congenital Diaphragmatic Hernias
Infant Mortality
ROC Curve
Cohort Studies
Thorax
Survival Rate
Retrospective Studies
Morbidity
Mortality
Population

Keywords

  • cardiac anomaly
  • chromosomal anomaly
  • congenital diaphragmatic hernia
  • ECMO
  • lung-to-head ratio
  • prenatal diagnosis
  • pulmonary hypoplasia
  • survival

ASJC Scopus subject areas

  • Radiological and Ultrasound Technology
  • Reproductive Medicine
  • Radiology Nuclear Medicine and imaging
  • Obstetrics and Gynecology

Cite this

Ruano, R., Javadian, P., Kailin, J. A., Maskatia, S. A., Shamshirsaz, A. A., Cass, D. L., ... Olutoye, O. O. (2015). Congenital heart anomaly in newborns with congenital diaphragmatic hernia: A single-center experience. Ultrasound in Obstetrics and Gynecology, 45(6), 683-688. https://doi.org/10.1002/uog.14648

Congenital heart anomaly in newborns with congenital diaphragmatic hernia : A single-center experience. / Ruano, Rodrigo; Javadian, P.; Kailin, J. A.; Maskatia, S. A.; Shamshirsaz, A. A.; Cass, D. L.; Zamora, I. J.; Sangi-Haghpeykar, H.; Lee, T. C.; Ayres, N. A.; Mehollin-Ray, A.; Cassady, C. I.; Fernandes, C.; Welty, S.; Belfort, M. A.; Olutoye, O. O.

In: Ultrasound in Obstetrics and Gynecology, Vol. 45, No. 6, 01.01.2015, p. 683-688.

Research output: Contribution to journalArticle

Ruano, R, Javadian, P, Kailin, JA, Maskatia, SA, Shamshirsaz, AA, Cass, DL, Zamora, IJ, Sangi-Haghpeykar, H, Lee, TC, Ayres, NA, Mehollin-Ray, A, Cassady, CI, Fernandes, C, Welty, S, Belfort, MA & Olutoye, OO 2015, 'Congenital heart anomaly in newborns with congenital diaphragmatic hernia: A single-center experience', Ultrasound in Obstetrics and Gynecology, vol. 45, no. 6, pp. 683-688. https://doi.org/10.1002/uog.14648
Ruano, Rodrigo ; Javadian, P. ; Kailin, J. A. ; Maskatia, S. A. ; Shamshirsaz, A. A. ; Cass, D. L. ; Zamora, I. J. ; Sangi-Haghpeykar, H. ; Lee, T. C. ; Ayres, N. A. ; Mehollin-Ray, A. ; Cassady, C. I. ; Fernandes, C. ; Welty, S. ; Belfort, M. A. ; Olutoye, O. O. / Congenital heart anomaly in newborns with congenital diaphragmatic hernia : A single-center experience. In: Ultrasound in Obstetrics and Gynecology. 2015 ; Vol. 45, No. 6. pp. 683-688.
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title = "Congenital heart anomaly in newborns with congenital diaphragmatic hernia: A single-center experience",
abstract = "Objective To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Methods In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively. Results Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤2 for both RACHS-1 (area under the curve (AUC), 0.74 (P=0.04); sensitivity, 80.0{\%}; specificity, 87.5{\%}) and STS-EACTS (AUC, 0.83 (P=0.03); sensitivity, 100{\%}; specificity, 87.5{\%}). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0{\%}; P=0.04) than in those with isolated CDH (77.1{\%}) and those with CDH and a minor CHA (81.3{\%}). We found no significant differences among the groups with regard to the need for ECMO. Conclusions In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.",
keywords = "cardiac anomaly, chromosomal anomaly, congenital diaphragmatic hernia, ECMO, lung-to-head ratio, prenatal diagnosis, pulmonary hypoplasia, survival",
author = "Rodrigo Ruano and P. Javadian and Kailin, {J. A.} and Maskatia, {S. A.} and Shamshirsaz, {A. A.} and Cass, {D. L.} and Zamora, {I. J.} and H. Sangi-Haghpeykar and Lee, {T. C.} and Ayres, {N. A.} and A. Mehollin-Ray and Cassady, {C. I.} and C. Fernandes and S. Welty and Belfort, {M. A.} and Olutoye, {O. O.}",
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T1 - Congenital heart anomaly in newborns with congenital diaphragmatic hernia

T2 - A single-center experience

AU - Ruano, Rodrigo

AU - Javadian, P.

AU - Kailin, J. A.

AU - Maskatia, S. A.

AU - Shamshirsaz, A. A.

AU - Cass, D. L.

AU - Zamora, I. J.

AU - Sangi-Haghpeykar, H.

AU - Lee, T. C.

AU - Ayres, N. A.

AU - Mehollin-Ray, A.

AU - Cassady, C. I.

AU - Fernandes, C.

AU - Welty, S.

AU - Belfort, M. A.

AU - Olutoye, O. O.

PY - 2015/1/1

Y1 - 2015/1/1

N2 - Objective To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Methods In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively. Results Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤2 for both RACHS-1 (area under the curve (AUC), 0.74 (P=0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P=0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P=0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO. Conclusions In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.

AB - Objective To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Methods In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively. Results Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤2 for both RACHS-1 (area under the curve (AUC), 0.74 (P=0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P=0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P=0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO. Conclusions In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.

KW - cardiac anomaly

KW - chromosomal anomaly

KW - congenital diaphragmatic hernia

KW - ECMO

KW - lung-to-head ratio

KW - prenatal diagnosis

KW - pulmonary hypoplasia

KW - survival

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