Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis
Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 ce...
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| Format: | Article |
| Language: | English |
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Wiley
2021-06-01
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| Series: | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| Subjects: | |
| Online Access: | https://www.ahajournals.org/doi/10.1161/JAHA.120.019713 |
| _version_ | 1818774904400510976 |
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| author | Anjali Chelliah Anita J. Moon‐Grady Shabnam Peyvandi Joanne S. Chiu James E. Bost David Schidlow Sheila J. Carroll Brooke Davey Allison Divanovic Lisa Hornberger Lisa W. Howley Ann Kavanaugh‐McHugh John P. Kovalchin Stephanie M. Levasseur Christopher L. Lindblade Shaine A. Morris Deliwe Ngwezi Jay D. Pruetz Michael D. Puchalski Jack Rychik Cyrus Samai Theresa A. Tacy Wayne Tworetzky Margaret M. Vernon Jay Yeh Mary T. Donofrio |
| author_facet | Anjali Chelliah Anita J. Moon‐Grady Shabnam Peyvandi Joanne S. Chiu James E. Bost David Schidlow Sheila J. Carroll Brooke Davey Allison Divanovic Lisa Hornberger Lisa W. Howley Ann Kavanaugh‐McHugh John P. Kovalchin Stephanie M. Levasseur Christopher L. Lindblade Shaine A. Morris Deliwe Ngwezi Jay D. Pruetz Michael D. Puchalski Jack Rychik Cyrus Samai Theresa A. Tacy Wayne Tworetzky Margaret M. Vernon Jay Yeh Mary T. Donofrio |
| author_sort | Anjali Chelliah |
| collection | DOAJ |
| description | Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality. |
| first_indexed | 2024-12-18T10:48:33Z |
| format | Article |
| id | doaj.art-633fba80d89446efa6062c3e84fe07b1 |
| institution | Directory Open Access Journal |
| issn | 2047-9980 |
| language | English |
| last_indexed | 2024-12-18T10:48:33Z |
| publishDate | 2021-06-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| spelling | doaj.art-633fba80d89446efa6062c3e84fe07b12022-12-21T21:10:30ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802021-06-01101210.1161/JAHA.120.019713Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal DiagnosisAnjali Chelliah0Anita J. Moon‐Grady1Shabnam Peyvandi2Joanne S. Chiu3James E. Bost4David Schidlow5Sheila J. Carroll6Brooke Davey7Allison Divanovic8Lisa Hornberger9Lisa W. Howley10Ann Kavanaugh‐McHugh11John P. Kovalchin12Stephanie M. Levasseur13Christopher L. Lindblade14Shaine A. Morris15Deliwe Ngwezi16Jay D. Pruetz17Michael D. Puchalski18Jack Rychik19Cyrus Samai20Theresa A. Tacy21Wayne Tworetzky22Margaret M. Vernon23Jay Yeh24Mary T. Donofrio25Division of Cardiology Department of Pediatrics Morgan Stanley Children’s Hospital of New York‐PresbyterianColumbia University Irving Medical Center New York NYDivision of Cardiology Department of Pediatrics UCSF Benioff Children's HospitalUniversity of California‐San Francisco School of Medicine San Francisco CADivision of Cardiology Department of Pediatrics UCSF Benioff Children's HospitalUniversity of California‐San Francisco School of Medicine San Francisco CADivision of Cardiology Department of Pediatrics Massachusetts General HospitalHarvard Medical School Boston MACenter for Translational Research Children’s Research InstituteChildren’s National HospitalGeorge Washington University School of Medicine and Health Sciences Washington DCDepartment of Cardiology Department of Pediatrics Boston Children's HospitalHarvard Medical School Boston MADivision of Cardiology Department of Pediatrics Komansky Children’s Hospital of New York‐PresbyterianWeill Cornell Medicine New York NYDivision of Cardiology Department of Pediatrics Connecticut Children’s Medical Center University of Connecticut Health Center Hartford CTDepartment of Pediatrics The Heart InstituteCincinnati Children's Hospital Medical CenterUniversity of Cincinnati College of Medicine Cincinnati OHDivision of Cardiology Department of Pediatrics Stollery Children’s HospitalUniversity of Alberta Edmonton AB CanadaDivision of Cardiology Department of Pediatrics Heart InstituteChildren's Hospital ColoradoUniversity of Colorado School of Medicine Denver CODivision of Cardiology Department of Pediatrics Monroe Carell Jr. Children's HospitalVanderbilt University School of Medicine Nashville TNDivision of Cardiology Department of Pediatrics Nationwide Children’s HospitalThe Ohio State University College of Medicine Columbus OHDivision of Cardiology Department of Pediatrics Morgan Stanley Children’s Hospital of New York‐PresbyterianColumbia University Irving Medical Center New York NYDivision of Cardiology Department of Pediatrics Phoenix Children’s HospitalUniversity of Arizona College of Medicine Phoenix AZDivision of Cardiology Department of Pediatrics Texas Children’s HospitalBaylor School of Medicine Houston TXDivision of Cardiology Department of Pediatrics Stollery Children’s HospitalUniversity of Alberta Edmonton AB CanadaDivision of Cardiology Department of Pediatrics Children’s Hospital of Los AngelesKeck School of Medicine of USC Los Angeles CADivision of Cardiology Department of Pediatrics Johns Hopkins All Children's Hospital St. Petersburg FLDivision of Cardiology Department of Pediatrics Children's Hospital of PhiladelphiaPerelman School of Medicine at the University of Pennsylvania Philadelphia PADivision of Cardiology Department of Pediatrics Children’s Healthcare of AtlantaEmory University School of Medicine Atlanta GADivision of Cardiology Department of Pediatrics Lucile Packard Children's HospitalStanford School of Medicine Palo Alto CADepartment of Cardiology Department of Pediatrics Boston Children's HospitalHarvard Medical School Boston MADivision of Cardiology Department of Pediatrics Seattle Children’s HospitalUniversity of Washington Seattle WADivision of Cardiology Department of Pediatrics University of California Davis Medical Center Sacramento CADivision of Cardiology Children's National HospitalDepartment of PediatricsGeorge Washington University School of Medicine and Health Sciences Washington DCBackground Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.https://www.ahajournals.org/doi/10.1161/JAHA.120.019713congenital heart diseasefetal cardiologyfetal echocardiographyprenatal diagnosistetralogy of Fallot with absent pulmonary valve |
| spellingShingle | Anjali Chelliah Anita J. Moon‐Grady Shabnam Peyvandi Joanne S. Chiu James E. Bost David Schidlow Sheila J. Carroll Brooke Davey Allison Divanovic Lisa Hornberger Lisa W. Howley Ann Kavanaugh‐McHugh John P. Kovalchin Stephanie M. Levasseur Christopher L. Lindblade Shaine A. Morris Deliwe Ngwezi Jay D. Pruetz Michael D. Puchalski Jack Rychik Cyrus Samai Theresa A. Tacy Wayne Tworetzky Margaret M. Vernon Jay Yeh Mary T. Donofrio Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease congenital heart disease fetal cardiology fetal echocardiography prenatal diagnosis tetralogy of Fallot with absent pulmonary valve |
| title | Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis |
| title_full | Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis |
| title_fullStr | Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis |
| title_full_unstemmed | Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis |
| title_short | Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis |
| title_sort | contemporary outcomes in tetralogy of fallot with absent pulmonary valve after fetal diagnosis |
| topic | congenital heart disease fetal cardiology fetal echocardiography prenatal diagnosis tetralogy of Fallot with absent pulmonary valve |
| url | https://www.ahajournals.org/doi/10.1161/JAHA.120.019713 |
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