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1. Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study

Author

Freud, Lindsay R, McElhinney, Doff B, Kalish, Brian T, Escobar‐Diaz, Maria C, 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, and Tworetzky, Wayne

Subjects
Pediatric, Perinatal Period - Conditions Originating in Perinatal Period, Lung, Cardiovascular, Heart Disease, Infant Mortality, Clinical Research, Good Health and Well Being, Blood Flow Velocity, Ebstein Anomaly, Echocardiography, Female, Heart Valve Diseases, Hospital Mortality, Humans, Infant, Newborn, Logistic Models, Male, Perinatal Mortality, Prenatal Diagnosis, Retrospective Studies, Risk Factors, Tricuspid Valve, congenital heart disease, Ebstein anomaly, mortality, neonate, outcome, palliation, right ventricle, tricuspid regurgitation, Cardiorespiratory Medicine and Haematology
Abstract

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died

Published
2020

2. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era

Author

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, and Tworetzky, Wayne

Subjects
Paediatrics, Reproductive Medicine, Biomedical and Clinical Sciences, Cardiovascular Medicine and Haematology, Perinatal Period - Conditions Originating in Perinatal Period, Pediatric, Infant Mortality, Lung, Clinical Research, Preterm, Low Birth Weight and Health of the Newborn, Congenital Structural Anomalies, Reproductive health and childbirth, Good Health and Well Being, Abortion, Eugenic, Adult, Birth Weight, Cardiac Catheterization, Cardiac Surgical Procedures, Down Syndrome, Ebstein Anomaly, Female, Gestational Age, Heart Defects, Congenital, Hospital Mortality, Humans, Infant, Newborn, Infant, Premature, Infant, Premature, Diseases, Male, Palliative Care, Pericardial Effusion, Pregnancy, Pregnancy Outcome, Retrospective Studies, Risk Factors, Treatment Outcome, Tricuspid Valve, Tricuspid Valve Insufficiency, Ultrasonography, Prenatal, Young Adult, Ebstein anomaly, echocardiography, heart defects, congenital, mortality, tricuspid valve insufficiency, heart defects, congenital, Cardiorespiratory Medicine and Haematology, Clinical Sciences, Public Health and Health Services, Cardiovascular System & Hematology, Cardiovascular medicine and haematology, Clinical sciences, Sports science and exercise
Abstract

BackgroundEbstein 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 resultsFetuses 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

Published
2015

6. Foetal echocardiographic assessment of borderline small left ventricles can predict the need for postnatal intervention

Author

Weber, Roland W., Ayala-Arnez, Ricardo, Atiyah, Merna, Etoom, Yousef, Manlhiot, Cedric, McCrindle, Brian W., Hickey, Edward J., Jaeggi, Edgar T., Nield, Lynne E., Weber, Roland W., Ayala-Arnez, Ricardo, Atiyah, Merna, Etoom, Yousef, Manlhiot, Cedric, McCrindle, Brian W., Hickey, Edward J., Jaeggi, Edgar T., and Nield, Lynne E.

Abstract

Background We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles. Methods Foetuses were included who had a left ventricle that was 2-4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models. Results From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean −3.8 versus −4.2 intervention group, p = 0.04, odds ratio 2.8). Discussion The need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography

Published
2017

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

Author

Freud, Lindsay R, 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, Freud, Lindsay R, 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, and Tworetzky, Wayne

Abstract

BackgroundEbstein 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 resultsFetuses 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).ConclusionIn 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.

Published
2015

9. Abstract 17203: Postnatal Management of Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multi-center Study

Author

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

Published
2015
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10. Foetal echocardiographic assessment of borderline small left ventricles can predict the need for postnatal intervention

Author

Weber, Roland W, Ayala-Arnez, Ricardo, Atiyah, Merna, Etoom, Yousef, Manlhiot, Cedric, McCrindle, Brian W, Hickey, Edward J, Jaeggi, Edgar T, Nield, Lynne E, Weber, Roland W, Ayala-Arnez, Ricardo, Atiyah, Merna, Etoom, Yousef, Manlhiot, Cedric, McCrindle, Brian W, Hickey, Edward J, Jaeggi, Edgar T, and Nield, Lynne E

Abstract

Background We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles. Methods Foetuses were included who had a left ventricle that was 2-4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models. Results From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean −3.8 versus −4.2 intervention group, p = 0.04, odds ratio 2.8). Discussion The need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography

Published
2013

11. Congenital Heart Block Maternal Sera Autoantibodies Target an Extracellular Epitope on the α1G T-Type Calcium Channel in Human Fetal Hearts

Author

Strandberg, Linn S., primary, Cui, Xuezhi, additional, Rath, Arianna, additional, Liu, Jie, additional, Silverman, Earl D., additional, Liu, Xiaoru, additional, Siragam, Vinayakumar, additional, Ackerley, Cameron, additional, Su, Brenda Bin, additional, Yan, Jane Yuqing, additional, Capecchi, Marco, additional, Biavati, Luca, additional, Accorroni, Alice, additional, Yuen, William, additional, Quattrone, Filippo, additional, Lung, Kalvin, additional, Jaeggi, Edgar T., additional, Backx, Peter H., additional, Deber, Charles M., additional, and Hamilton, Robert M., additional

Published
2013
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13. Response to Letter Regarding Article, “Comparison of Transplacental Treatment of Fetal Supraventricular Tachyarrhythmias With Digoxin, Flecainide, and Sotalol: Results of a Nonrandomized Multicenter Study”

Author

Blom, Nico A., primary, De Groot, Ernestine, additional, Jaeggi, Edgar T., additional, McCrindle, Brian W., additional, Manlhiot, Cedric, additional, Carvalho, Julene S., additional, Api, Olus, additional, Clur, Sally-Ann B., additional, Rammeloo, Lukas, additional, and Ryan, Greg, additional

Published
2012
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21. Congenital Heart Block Maternal Sera Autoantibodies Target an Extracellular Epitope on the α1G T-Type Calcium Channel in Human Fetal Hearts.

Author

Strandberg, Linn S., Cui, Xuezhi, Rath, Arianna, Liu, Jie, Silverman, Earl D., Liu, Xiaoru, Siragam, Vinayakumar, Ackerley, Cameron, Su, Brenda Bin, Yan, Jane Yuqing, Capecchi, Marco, Biavati, Luca, Accorroni, Alice, Yuen, William, Quattrone, Filippo, Lung, Kalvin, Jaeggi, Edgar T., Backx, Peter H., Deber, Charles M., and Hamilton, Robert M.

Subjects
CONGENITAL heart disease, AUTOANTIBODIES, TARGETED drug delivery, EPITOPES, CALCIUM channels, FETAL development, MESSENGER RNA, SINOATRIAL node, LABORATORY mice
Abstract

Background: Congenital heart block (CHB) is a transplacentally acquired autoimmune disease associated with anti-Ro/SSA and anti-La/SSB maternal autoantibodies and is characterized primarily by atrioventricular (AV) block of the fetal heart. This study aims to investigate whether the T-type calcium channel subunit α1G may be a fetal target of maternal sera autoantibodies in CHB. Methodology/Principal Findings: We demonstrate differential mRNA expression of the T-type calcium channel CACNA1G (α1G gene) in the AV junction of human fetal hearts compared to the apex (18–22.6 weeks gestation). Using human fetal hearts (20–22 wks gestation), our immunoprecipitation (IP), Western blot analysis and immunofluorescence (IF) staining results, taken together, demonstrate accessibility of the α1G epitope on the surfaces of cardiomyocytes as well as reactivity of maternal serum from CHB affected pregnancies to the α1G protein. By ELISA we demonstrated maternal sera reactivity to α1G was significantly higher in CHB maternal sera compared to controls, and reactivity was epitope mapped to a peptide designated as p305 (corresponding to aa305–319 of the extracellular loop linking transmembrane segments S5–S6 in α1G repeat I). Maternal sera from CHB affected pregnancies also reacted more weakly to the homologous region (7/15 amino acids conserved) of the α1H channel. Electrophysiology experiments with single-cell patch-clamp also demonstrated effects of CHB maternal sera on T-type current in mouse sinoatrial node (SAN) cells. Conclusions/Significance: Taken together, these results indicate that CHB maternal sera antibodies readily target an extracellular epitope of α1G T-type calcium channels in human fetal cardiomyocytes. CHB maternal sera also show reactivity for α1H suggesting that autoantibodies can target multiple fetal targets. [ABSTRACT FROM AUTHOR]

Published
2013
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23. Foetal echocardiographic assessment of borderline small left ventricles can predict the need for postnatal intervention

Author

Weber, Roland W., Ayala-Arnez, Ricardo, Atiyah, Merna, Etoom, Yousef, Manlhiot, Cedric, McCrindle, Brian W., Hickey, Edward J., Jaeggi, Edgar T., Nield, Lynne E., Weber, Roland W., Ayala-Arnez, Ricardo, Atiyah, Merna, Etoom, Yousef, Manlhiot, Cedric, McCrindle, Brian W., Hickey, Edward J., Jaeggi, Edgar T., and Nield, Lynne E.

Abstract

Background We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles. Methods Foetuses were included who had a left ventricle that was 2-4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models. Results From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean −3.8 versus −4.2 intervention group, p = 0.04, odds ratio 2.8). Discussion The need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography

25. Abstract 16215: Success Rate of Fetal Aortic Valvuloplasty in Relation to Center Volumes: A Report From the International Fetal Cardiac Intervention Registry

Author

Gijtenbeek, Manon, Morris, Shaine A, Moon-Grady, Anita, Armstrong, Aimee K, Cruz-Lemini, Monica, Dangel, Joanna, Emery, Stephen P, Galindo, Alberto, Gelehrter, Sarah, Grinenco, Sofia, Herberg, Ulrike, Jaeggi, Edgar T, Malekzadeh-Milani, Sophie, Pedra, Simone R, Pruetz, Jay D, Simpson, John, and Monique, Haak C

Abstract

Introduction:The impact of center volume on the procedural outcomes of fetal aortic valvuloplasty (FAV) is unknown. The relationship between volume and outcome is crucial to understand if regionalization should be implemented worldwide.Hypothesis:We hypothesized that higher center volume is associated with greater technical success in FAV.Methods:Data were included from institutions that performed ?3 procedures (2001-2018) and reported technical success. Technical success was defined as ?1 balloon inflation across the aortic valve, with clear evidence of increased flow across the valve and/or new aortic regurgitation by color Doppler. Secondary endpoint measures were fetal periprocedural complications (pericardial/pleural effusion or bradycardia requiring treatment, balloon rupture, or death within 48 hours of the procedure) and fetal periprocedural death.Results:A total of 162 FAVs from 11 centers were included, with FAV center volumes ranging from 6-31. Overall technical success rate was 80%. Higher center volume was not associated with technical success or fetal complications (p=0.948 and p=0.953 respectively), but was associated with fewer periprocedural fetal deaths (p=0.02, Figure). Periprocedural fetal death rate appeared to plateau after 15 FAVs. Intervention in larger fetuses was associated with greater technical success (p<0.001), fewer complications (p=0.01), and fewer periprocedural deaths (p<0.01). Intervention in older fetuses trended towards greater technical success (p=0.08), with significantly fewer complications (p<0.01) and periprocedural deaths (p<0.01). There were 3 significant maternal complications (1.9%) with no association with center volume.Conclusions:In this registry cohort, higher volume centers did not have more FAV technical success or fewer complications, but they did have fewer fetal deaths. Improved FAV outcomes are associated with larger and older fetuses.

Published
2019
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26. Abstract 15729: A Severe Sustained Decrease in Left Heart Flow Results in Left Ventricular Hypoplasia in Mid-Gestation Fetal Lambs

Author

Chaturvedi, Rajiv R, Keunen, Johannes, de Vrijer, Barbra, Ryan, Greg, and Jaeggi, Edgar T

Abstract

Introduction:The etiology of hypoplastic left heart syndrome (HLHS) is unclear. Supravalvar aortic banding in fetal lambs produced conflicting results as to whether LV hypoplasia or hypertrophy occurred. We hypothesized low flow in the fetal left heart results in hypoplasiaMethods:LV inflow obstruction created in 75day fetal lambs by percutaneous implantation of LA coils and harvest at 125 days.Results:19 coiled fetuses and 1 control died prior to harvest. 15 coiled fetuses reached 125 days, 1 had anasarca (10.6kg) due to severe mitral regurgitation. Effective coiling resulted in tissue growth into the coil scaffold leading to severe acquired mitral stenosis and a slit-like LV. LV inflow and antegrade aortic valve (AoV) flow was so low in 9 that there was retrograde flow in the ascending aorta from the duct. 4 of 9 had no antegrade AoV flow, with the brain and coronaries wholly perfused from the duct. These low-flow LV had the most severe left heart hypoplasia and the LV was no longer apex forming. Due to the variation in fetal size (non-hydropic weight 1.74-4.45kg), each fetus?s left heart measurements were normalized to the corresponding right-heart structure and these were all smaller in the low-flow LV hearts as compared to controls (Table 1).Conclusion:Long after cardiogenesis, a severe, prolonged (1/3 gestation) decrease in left heart flow is sufficient to produce LV and ascending aortic hypoplasia and retrograde perfusion of the brain and coronaries, similar to human HLHS. This model will allow investigation of HLHS development and therapies.graphic_{3CF29FE0-3EAF-40EA-AAA1-AE98366853C0}

Published
2019
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27. Abstract 13349: Prediction of Perinatal Outcomes in Fetuses With Ebstein?s Anomaly or Tricuspid Valve Dysplasia

Author

Torigoe, Tsukasa, Freud, Lindsay R, Puchalski, Michael, Szwast, Anita L, Freire, Grace A, Kavanaugh-Mchugh, Ann K, Levasseur, Stephanie, Fan, Chun-Po, Tworetzky, Wayne, and Jaeggi, Edgar T

Abstract

Background:Ebstein?s anomaly and tricuspid valve dysplasia (EA/TVD) carry high risks of fetal (F) and neonatal (N) death. Factors previously shown to be associated with mortality include increased cardio-thoracic ratio (CTR), right atrial area index (RAAI), absent pulmonary flow, pulmonary regurgitation (PR), and prognostic scores (SAS score; SickKids (SK) score). Scores combine the results of 5 echocardiographic findings according to severity (value 0-2 per variable; score range 0-10) with higher scores connoting higher odds of mortality. For SAS, the variables include CTR, RAAI, right/left ventricular ratio, pulmonary and ductal flows. The SK score includes CTR, RAAI, pulmonary flow, tricuspid regurgitation (TR) severity ? TR peak gradient (TRPG), and PR. We sought to validate factors associated with F/N outcomes in this ancillary multicenter study.Methods:Echocardiograms of fetuses with EA/TVD from 18 institutions were reviewed without prior knowledge of patient outcomes. Excluded were cases with incomplete exams and without intended active care. Area under receiver operating curves were used to compare the SAS and SK scores.Results:Of 129 subjects, 53 (41%) died in-utero or as neonates, while 76 survived to neonatal hospital discharge. Factors at the initial exam (27.6?5.5 weeks gestation) associated with mortality (p<0.001) included hydrops (OR 28.58; [95%CI: 1.61-507.3]), moderate/severe TR with lower TRPG (per mmHg decrease: OR 1.22 [1.14-1.30]), PR (OR 11.81 [4.9-28.2]), CTR (OR per % increase: 1.20 [1.13-1.28], RAAI (OR 97.1 [16.0-589]), and absent pulmonary flow (OR 31.5 [7.12-138]). Baseline SK and SAS scores were 5.0?3.0 and 4.8?2.7, respectively. Areas under the curve were 0.93 (SK) vs 0.83 (SAS) for F/N death (p<0.001). Survival to hospital discharge with a score <6 was 93.5% (SK: 58/62) vs 87.3% (SAS: 55/63) and for scores >7 2.9% (SK: 1/34) vs 15.8% (SAS: 3/19). A prenatal TRPG ?30 mmHg was significantly associated with biventricular survival to hospital discharge (OR 110.5 [20.90-584.35]; p<0.001).Conclusion:The SK score system is superior to SAS in predicting perinatal outcomes. F/N death was associated with significant hemodynamic alterations already present on the baseline exam, including significant TR with a low TRPG.

Published
2019
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