Outcomes and Predictors of Perinatal Mortality in Fetuses with Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study

Lindsay R. Freud, Maria C. Escobar-Diaz, Brian T. Kalish, Rukmini Komarlu, Michael D. Puchalski, Edgar T. Jaeggi, Anita L. Szwast, Grace Freire, Stéphanie M. Levasseur, Ann Kavanaugh-Mchugh, Erik C. Michelfelder, Anita J. Moon-Grady, Mary T. Donofrio, Lisa W. Howley, Elif Seda Selamet Tierney, Bettina F. Cuneo, Shaine A. Morris, Jay D. Pruetz, Mary E. Van Der Velde, John P. KovalchinCatherine M. Ikemba, Margaret M. Vernon, Cyrus Samai, Gary M. Satou, Nina L. Gotteiner, Colin K. Phoon, Norman H. Silverman, Doff B. McElhinney, Wayne Tworetzky

Research output: Contribution to journalArticle

35 Citations (Scopus)

Abstract

Background-Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results-Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion-In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

Original languageEnglish (US)
Pages (from-to)481-489
Number of pages9
JournalCirculation
Volume132
Issue number6
DOIs
StatePublished - Aug 11 2015

Fingerprint

Ebstein Anomaly
Tricuspid Valve
Perinatal Mortality
Multicenter Studies
Pulmonary Valve Insufficiency
Fetus
Gestational Age
Odds Ratio
Confidence Intervals
Fetal Death
Fetal Mortality
Pericardial Effusion
Lost to Follow-Up
Birth Weight
Survival
Mortality

Keywords

  • Ebstein anomaly
  • echocardiography
  • heart defects, congenital
  • mortality
  • tricuspid valve insufficiency

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Freud, L. R., Escobar-Diaz, M. C., Kalish, B. T., Komarlu, R., Puchalski, M. D., Jaeggi, E. T., ... Tworetzky, W. (2015). Outcomes and Predictors of Perinatal Mortality in Fetuses with Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study. Circulation, 132(6), 481-489. https://doi.org/10.1161/CIRCULATIONAHA.115.015839

Outcomes and Predictors of Perinatal Mortality in Fetuses with Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era : A Multicenter Study. / Freud, Lindsay R.; Escobar-Diaz, Maria C.; Kalish, Brian T.; Komarlu, Rukmini; Puchalski, Michael D.; Jaeggi, Edgar T.; Szwast, Anita L.; Freire, Grace; Levasseur, Stéphanie M.; Kavanaugh-Mchugh, Ann; Michelfelder, Erik C.; Moon-Grady, Anita J.; Donofrio, Mary T.; Howley, Lisa W.; Tierney, Elif Seda Selamet; Cuneo, Bettina F.; Morris, Shaine A.; Pruetz, Jay D.; Van Der Velde, Mary E.; Kovalchin, John P.; Ikemba, Catherine M.; Vernon, Margaret M.; Samai, Cyrus; Satou, Gary M.; Gotteiner, Nina L.; Phoon, Colin K.; Silverman, Norman H.; McElhinney, Doff B.; Tworetzky, Wayne.

In: Circulation, Vol. 132, No. 6, 11.08.2015, p. 481-489.

Research output: Contribution to journalArticle

Freud, LR, Escobar-Diaz, MC, Kalish, BT, Komarlu, R, Puchalski, MD, Jaeggi, ET, Szwast, AL, Freire, G, Levasseur, SM, Kavanaugh-Mchugh, A, Michelfelder, EC, Moon-Grady, AJ, Donofrio, MT, Howley, LW, Tierney, ESS, Cuneo, BF, Morris, SA, Pruetz, JD, Van Der Velde, ME, Kovalchin, JP, Ikemba, CM, Vernon, MM, Samai, C, Satou, GM, Gotteiner, NL, Phoon, CK, Silverman, NH, McElhinney, DB & Tworetzky, W 2015, 'Outcomes and Predictors of Perinatal Mortality in Fetuses with Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study', Circulation, vol. 132, no. 6, pp. 481-489. https://doi.org/10.1161/CIRCULATIONAHA.115.015839
Freud, Lindsay R. ; Escobar-Diaz, Maria C. ; Kalish, Brian T. ; Komarlu, Rukmini ; Puchalski, Michael D. ; Jaeggi, Edgar T. ; Szwast, Anita L. ; Freire, Grace ; Levasseur, Stéphanie M. ; Kavanaugh-Mchugh, Ann ; Michelfelder, Erik C. ; Moon-Grady, Anita J. ; Donofrio, Mary T. ; Howley, Lisa W. ; Tierney, Elif Seda Selamet ; Cuneo, Bettina F. ; Morris, Shaine A. ; Pruetz, Jay D. ; Van Der Velde, Mary E. ; Kovalchin, John P. ; Ikemba, Catherine M. ; Vernon, Margaret M. ; Samai, Cyrus ; Satou, Gary M. ; Gotteiner, Nina L. ; Phoon, Colin K. ; Silverman, Norman H. ; McElhinney, Doff B. ; Tworetzky, Wayne. / Outcomes and Predictors of Perinatal Mortality in Fetuses with Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era : A Multicenter Study. In: Circulation. 2015 ; Vol. 132, No. 6. pp. 481-489.
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abstract = "Background-Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results-Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5{\%}), 15 terminations (6{\%}), and 41 demises (17{\%}). In the live-born cohort of 176 live-born patients, 56 (32{\%}) died before discharge, yielding an overall perinatal mortality of 45{\%}. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95{\%} confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95{\%} confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95{\%} confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95{\%} confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61{\%} versus 34{\%}; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion-In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.",
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T1 - Outcomes and Predictors of Perinatal Mortality in Fetuses with Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era

T2 - A Multicenter Study

AU - Freud, Lindsay R.

AU - Escobar-Diaz, Maria C.

AU - Kalish, Brian T.

AU - Komarlu, Rukmini

AU - Puchalski, Michael D.

AU - Jaeggi, Edgar T.

AU - Szwast, Anita L.

AU - Freire, Grace

AU - Levasseur, Stéphanie M.

AU - Kavanaugh-Mchugh, Ann

AU - Michelfelder, Erik C.

AU - Moon-Grady, Anita J.

AU - Donofrio, Mary T.

AU - Howley, Lisa W.

AU - Tierney, Elif Seda Selamet

AU - Cuneo, Bettina F.

AU - Morris, Shaine A.

AU - Pruetz, Jay D.

AU - Van Der Velde, Mary E.

AU - Kovalchin, John P.

AU - Ikemba, Catherine M.

AU - Vernon, Margaret M.

AU - Samai, Cyrus

AU - Satou, Gary M.

AU - Gotteiner, Nina L.

AU - Phoon, Colin K.

AU - Silverman, Norman H.

AU - McElhinney, Doff B.

AU - Tworetzky, Wayne

PY - 2015/8/11

Y1 - 2015/8/11

N2 - Background-Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results-Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion-In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

AB - Background-Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results-Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion-In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

KW - Ebstein anomaly

KW - echocardiography

KW - heart defects, congenital

KW - mortality

KW - tricuspid valve insufficiency

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