Changed outcomes of fetuses with congenital heart disease

Fesslová, Vlasta; Branković, Jelena; Boschetto, Chiara; Masini, A. C.; Prandstraller, Daniela; Perolo, A.; Ventriglia, Flavia; Macerola, Silvia; Crepaz, R; Romeo, Cristina; Luca, Francesco De; Previtera, Agata; Errico, Gabriella

doi:10.2459/jcm.0b013e328365c325

Cited by 13 publications

(8 citation statements)

References 26 publications

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“…In this contemporary, multicenter cohort of fetuses with EA/TVD, the perinatal mortality was 45%, substantially higher than other forms of congenital heart disease in the current era. 14,15 However, in comparison with previous single-center series of EA/TVD over the past several decades, a greater proportion of fetuses survived to birth. This may be attributable to a combination of factors, including a lower rate of pregnancy termination and progress in prenatal diagnosis resulting in greater likelihood of identifying less severely affected fetuses.…”

Section: Discussionmentioning

confidence: 91%

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

et al. 2015

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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 wereincluded 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;© 2015 American Heart Association, Inc.Circulation characterized by apical displacement of the valve or leaflet deformation, respectively. 3 In severe cases of dysplasia, the tricuspid valve orifice may become unguarded. 4,5 Although there is a broad morphologic spectrum, these malformations lead to the same hemodynamic burden, namely, tricuspid regurgitation (TR) and its pathophysiological sequelae. Although older children and adults with EA/TVD may be asymptomatic for years, the diagnosis of EA/TVD in the perinatal period carries a poor prognosis. In the fetus, severe TR may lead to cardiomegaly, hydrops, and arrhythmia, with demise rates as high as 48%.6 Among prenatally diagnosed patients who survive to live birth, hemodynamic instability, cyanosis, and respiratory compromise are common. Although neonatal mortality approached 80 to 85% in early series, 7,8 various single-center series have reported reduced mortality in the past 2 decades, ranging from 17% to 56%. 9-12 Clinical Perspective on p 489Fetal risk factors for perinatal mortality have been identified, including lack of antegrade flow across the pulmonary valve and retrograde duct flow 9-12 and fetal distress. 12However, studies were limited by small sample sizes, with Yu et al 12 reporting the largest series to date with 46 prenatally diagnosed patients. The prognostic value of indices of cardiomegaly, such as the cardiothoracic area (CTA) ratio and the right atrial area index, has been mixed. [9][10][11][12][13] Importantly, hemodynamic factors with potentially important influences on perinatal mortality, such as right ventricular pressure and the presence of pulmonary regurgitation (PR), have not been investigated.Since our understanding of fetuses diagnose...

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Section: Discussionmentioning

confidence: 91%

“…The overall perinatal outcome for the 243 fetuses is depicted in Figure 1. There were 15 known pregnancy terminations, all but 1 of which occurred at less than 24 weeks GA (median, 21 weeks GA [14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31]). There were 41 fetal demises, and the median GA at demise was 30 weeks (22-37).…”

Section: Resultsmentioning

confidence: 99%

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

et al. 2015

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“…This is well correlated with a previous Italian multicentric study that reported an overall TOP rate of 20%. 23…”

Section: Principal Findingmentioning

confidence: 99%

Prenatal diagnosis of congenital heart defects and voluntary termination of pregnancy

Montaguti

Balducci

Perolo

et al. 2020

American Journal of Obstetrics & Gynecology MFM

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“…Perinatal mortality (defined as fetal demise or death before neonatal discharge) remains high among fetuses diagnosed with Ebstein's anomaly; there are reports of up to 45% in recent large multicentre cohort series of fetuses with Ebstein's anomaly or tricuspid valve dysplasia, substantially higher than other forms of congenital heart disease in the current era [8,9]. In the fetus, severe tricuspid regurgitation may lead to cardiomegaly, hydrops, and arrythmia, with demise rates as high as 48% [10].…”

Section: Introductionmentioning

confidence: 99%

The attempt of pregnancy modification in a case of fetal Ebstein’s anomaly

Rydzewska¹,

Murlewska²,

Krekora³

et al. 2019

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Introduction: Ebstein's anomaly is a rare congenital heart defect (< 1% of all cases of congenital heart disease). Its distinctive feature is the displacement of the septal and posterior leaflets of the tricuspid valve towards the apex. The right atrium is enlarged, and the right ventricle is divided into two parts. The leaflets are often dysplastic. This leads to tricuspid regurgitation. Pulmonary stenosis or atresia is the most common associated defect. All these changes can lead, during the fetal life, to cardiomegaly, lung hypoplasia, heart failure of the fetus, or even intrauterine demise. Case presentation: A 39-year-old G3P3 at 23 weeks of gestation had a detection of heart disease. She was admitted to our Institute for further management. The prenatal echocardiography exam showed the fetus with Ebstein's anomaly, pulmonary valve atresia, and cardiomegaly. Intrauterine digoxin and steroid treatment was started. Subsequent fetal echocardiographic monitoring was performed late in the third trimester. At 39 weeks of gestation the oxygen test was positive and oxygen therapy was ordered. Elective caesarean section was performed at 40 weeks of gestation, and a male newborn weighting 2860 g was delivered. On the 11th day of life, he underwent cardiac surgery in the same hospital and was discharged four weeks later in a good general condition. Conclusion: Despite the fact that there are no recommendations for intrauterine therapy for fetal Ebstein's disease, this case shows the possibility of finding an alternative method of fetal treatment, which can be a combination of digoxin, steroids, and maternal oxygen therapy and early neonatal, handmade, tricuspid valve replacement.

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Changed outcomes of fetuses with congenital heart disease

Abstract: Our data show a reduced overall and surgical mortality, with respect to our previous study, resulting from resulting from an improved perinatal management and treatment of affected fetuses in the more recent era.

Cited by 13 publications

References 26 publications

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

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

Prenatal diagnosis of congenital heart defects and voluntary termination of pregnancy

The attempt of pregnancy modification in a case of fetal Ebstein’s anomaly

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