Circular Shunt Research Articles

The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly

ObjectivesSymptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. MethodsThe EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. ResultsWhen evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. ConclusionsRisk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.

Fetal circular shunt in Ebstein's anomaly and non-steroidal anti-inflammatory treatment.

A circular shunt is a poor prognostic factor associated with Ebstein's anomaly. Targeting the constriction of the ductus arteriosus (DA) in order to limit or resolve the circular shunt, has been shown to improve fetal outcomes. Prenatal non-steroidal anti-inflammatory drugs (NSAIDs) have been known to constrict the DA. Recently, prenatal NSAIDs have been used for that purpose in the treatment of circular shunt. Limited research shows that it may be an effective treatment leading to improved fetal outcomes. In this article, we did an extensive review of literature to describe this therapy's effectiveness and outcomes. 82% of fetuses were able to achieve ductal constriction with prenatal NSAID therapy. For fetuses who achieved ductal constriction, fetal demise was less likely (6%) when compared to those who were unable to achieve the same (50%). Of all the fetuses with hydrops, 50% had resoluation of hydrops with prenatal NSAID treatment.

Modified Starnes Procedure With Patch Occlusion of the Main Pulmonary Artery and Other Technical Modifications to Facilitate Subsequent Biventricular Repair of Ebstein Anomaly

The Starnes procedure, introduced in 1991, has been a valuable advancement in the surgical treatment of critically ill neonates with Ebstein's anomaly (EA). However, it used to be followed by the single ventricle pathway, which presents long-term outcome limitations. In 2019, this management concept was modified by taking down the Starnes procedure and performing the Cone repair of the tricuspid valve, which resulted in a biventricular reconstruction. This approach rehabilitates the right ventricle, likely improving the patients' long-term outcomes. However, it requires modifications in the classical Starnes operation and observing certain surgical details. In cases of circular shunting, we use a patch to occlude the pulmonary artery instead of dividing and oversewing it, simplifying the succeeding pulmonary artery reconstruction. We use a PTFE fenestrated patch placed in a supra-valvar position, preserving the underneath tricuspid valve integrity, facilitating the tricuspid valve repair, and decreasing the possibility of heart block.Central message: In the current era of biventricular repair after the Starnes procedure, using a non-stick supravalvar patch to exclude the tricuspid valve and preserving the pulmonary valve structure by patch occlusion of the main pulmonary artery instead of division and oversewing it are valuable modifications of the classic Starnes operation to optimize a future biventricular repair.

Transplacental non-steroidal anti-inflammatory drugs versus expectant management in fetal Ebstein anomaly with circular shunt: Systematic review and meta-analysis.

Ebstein anomaly (EA) is a rare congenital cardiac malformation associated with high perinatal mortality. In this systematic review and meta-analysis, we aimed to investigate the outcomes of pregnancies affected by EA or tricuspid valve dysplasia (TVD) with circular shunt, focusing on two prenatal management approaches: (1) expectant management (EM) and (2) transplacental non-steroidal anti-inflammatory drugs (NSAID) therapy. We searched PubMed, Scopus, and Web of Science systematically from its inception until June 2023. The random-effect model was used to pool the data. Heterogeneity was assessed using the I2 value. Twenty-one studies with a total of 610 fetuses with EA/TVD with circular shunt were included in the synthesis, of which 17 studies (583 fetuses) were on EM and 4 studies (27 fetuses) used transplacental NSAID therapy. The NSAID group had higher rates of moderate to severe tricuspid regurgitation, hydrops, and pericardial effusion on prenatal ultrasound compared with the EM group. However, ductal constriction was achieved in 81% of NSAID cases, mitigating the disease pathophysiology, although 65% of them experienced oligohydramnios. Notably, the NSAID group showed significantly higher rates of live birth (86%) and survival to hospital discharge (89%) compared with the EM group (67% and 43%, respectively). Despite these promising results, it's important to acknowledge that the number of cases treated with NSAIDs was small, with limited safety data. Therefore, caution is advised in interpreting these findings, and patients considering NSAID therapy should be informed about these limitations. Future multicenter studies are necessary to further explore the safety and effectiveness of NSAID therapy in this particular population.

Circular shunt in fetal absent pulmonary valve with tricuspid stenosis.

Pediatrics InternationalAccepted Articles e15480 CLINICAL NOTE Circular shunt in fetal absent pulmonary valve with tricuspid stenosis Tomiko Toyokawa, Tomiko Toyokawa Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Yao Municipal Hospital, Yao, Osaka, JapanSearch for more papers by this authorNoboru Inamura, Corresponding Author Noboru Inamura ki88mi@gmail.com orcid.org/0000-0002-6270-1158 Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan Correspondence Noboru Inamura, Department of Pediatrics, Kindai University, Faculty of Medicine; 377-2 Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan, Phone: +81-72-366-0221; Fax: +81-72-368-1566. Email: ki88mi@gmail.comSearch for more papers by this authorYukiko Kawazu, Yukiko Kawazu Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Fukuyama City Hospital, Fukuyama, Hiroshima, JapanSearch for more papers by this authorFutoshi Kayatani, Futoshi Kayatani Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, JapanSearch for more papers by this author Tomiko Toyokawa, Tomiko Toyokawa Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Yao Municipal Hospital, Yao, Osaka, JapanSearch for more papers by this authorNoboru Inamura, Corresponding Author Noboru Inamura ki88mi@gmail.com orcid.org/0000-0002-6270-1158 Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan Correspondence Noboru Inamura, Department of Pediatrics, Kindai University, Faculty of Medicine; 377-2 Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan, Phone: +81-72-366-0221; Fax: +81-72-368-1566. Email: ki88mi@gmail.comSearch for more papers by this authorYukiko Kawazu, Yukiko Kawazu Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Fukuyama City Hospital, Fukuyama, Hiroshima, JapanSearch for more papers by this authorFutoshi Kayatani, Futoshi Kayatani Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, JapanSearch for more papers by this author First published: 19 January 2023 https://doi.org/10.1111/ped.15480 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi:10.1111/ped.15480. AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future.e15480 RelatedInformation

An unexpected circular shunt: Novel method to treat semilunar valve insufficiency in a single ventricle patient on mechanical circulatory support

Severe semilunar valve insufficiency in single ventricle patients supported with mechanical circulatory support while awaiting transplant remains a complex clinical scenario with few favorable options for management. We present the first case, to our knowledge, of transcatheter closure of the pulmonic valve in a patient palliated with a hybrid stage 1 procedure for hypoplastic left heart syndrome.

Impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in patients with Ebstein anomaly and tricuspid valve dysplasia.

To evaluate the impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in severe Ebstein anomaly and tricuspid valve dysplasia. Thirty-four fetuses with Ebstein anomaly/tricuspid valve dysplasia were referred from 2013 to 2019 for fetal echocardiography and clinical management. Nineteen fetuses with Ebstein anomaly/tricuspid valve dysplasia and 30 controls underwent cardiovascular magnetic resonance to quantify the fetal blood flow and to calculate cerebral oxygen delivery (cDO2) and consumption (cVO2). The 3D steady-state free precession acquisition was used to measure fetal brain volume. Surgical outcome, brain MRI, and neurodevelopmental follow-up were reviewed. Twenty-six fetuses were live born (76%) and survival (65%) at a mean follow-up of 4 years. Nine fetuses had a brain MRI before discharge, and all had clinically silent injuries and volume loss. At 18 months, five single-ventricle patients had a neurodevelopmental delay in cognition and language (mean percentile: 11th), with gross-motor skills more affected than fine-motor skills (mean percentiles: 4th and 34th). Fetuses with Ebstein anomaly/tricuspid valve dysplasia had smaller brains, lower combined ventricular output, ascending aorta, superior caval vien and umbilical vein flows, lower oxygen saturation in ascending aorta and superior caval vien, lower cDO2 and cVO2 (p < 0.05). Superior caval vien/combined ventricular output and descending aorta/combined ventricular output ratios were lower in fetuses with circular shunt (p < 0.05). Fetuses requiring the Starnes procedure tended to have smaller brains, lower combined ventricular output, superior caval vien, descending aorta, and umbilical vein flows. All patients with Ebstein anomaly/tricuspid valve dysplasia are at high risk of neurodevelopmental delay and warrant follow-up. Fetal cardiovascular magnetic resonance revealed impaired brain growth with diminished cerebral blood flow and cDO2, the extenting dependent on the severity of the haemodynamic compromise.

VP39.03: Spontaneous resolution of transient right heart circular shunt in Twin–twin transfusion syndrome: a case of recipient before and another case of donor after laser surgery

VP39.03: Spontaneous resolution of transient right heart circular shunt in Twin–twin transfusion syndrome: a case of recipient before and another case of donor after laser surgery

Effects and side effects of maternal administration of indomethacin for fetal tricuspid valve dysplasia

We report on the course of two fetuses with tricuspid valve dysplasia (TVD) who were treated with indomethacin. Case 1 presented at 24 weeks' gestation with severe tricuspid regurgitation (TR), retrograde flow in the arterial duct, no antegrade flow across the pulmonary valve and free pulmonary regurgitation (PR). There was cardiomegaly and fetal hydrops with ascites, skin edema and pleural effusion. The Simpson–Andrews–Sharland (SAS) prenatal severity score for TVD was 7, which predicted fetal demise. The SAS score ranges from 0 to 10, with no predicted survivors scoring ≥ 51. Case 2 presented at 16 weeks' gestation with moderate TR, antegrade flow across the pulmonary valve, progressing to functional pulmonary atresia by 21 weeks' gestation, and a SAS score of 4. At 33 + 5 weeks, there was evidence of further progression with increasing cardiomegaly (cardiothoracic ratio (CTR): 0.80), severe TR and PR and a SAS score of 8. Estimated fetal weight (EFW) dropped from 17th to 3rd centile, umbilical artery (UA) pulsatility index was > 95th centile and amniotic fluid index (AFI) was normal (8.9 cm). Maternal indomethacin (100 mg three times a day (TDS)) was commenced in both cases (Figure 1), leading to constriction/closure of the arterial duct, reduction in PR and antegrade flow across the pulmonary valve. In Case 1, by 26 weeks' gestation, fetal hydrops had completely resolved, indomethacin was replaced by ibuprofen (200 mg TDS) due to a more favorable side-effect profile. However, the arterial duct reopened and indomethacin was reintroduced. Following this, the arterial duct constricted, but oligohydramnios developed (AFI: 4.4 cm). At 30 weeks' gestation, there was faltering growth, EFW of 1.2 kg and anhydramnios. Indomethacin was stopped and the AFI normalized (17.1 cm), but cardiomegaly persisted with severe TR. Intrauterine death occurred at 31 + 6 weeks' gestation. In Case 2, following commencement of indomethacin, CTR reduced to 0.65 but oligohydramnios worsened (AFI: 4.2 cm). Indomethacin was stopped and AFI normalized. Indomethacin was restarted at a lower dose (50 mg TDS), but despite failure to constrict the arterial duct, oligohydramnios recurred (AFI: 1.5 cm) and therapy was ceased. The baby was delivered at 36 weeks' gestation due to absent end-diastolic UA flow, weighing 1.4 kg, and was stable without intervention at 1 year of age. Prenatally detected cases of TVD are often those at the most severe end of the spectrum, presenting with cardiomegaly secondary to severe TR1. Cardiomegaly leads to compression of the developing lung tissue, pulmonary hypoplasia and increased risk of postnatal demise. The fetal environment is disadvantageous for the fetus with TVD as the right ventricle (RV) faces systemic pressures and high pulmonary vascular resistance, worsening TR and subsequent right atrial dilatation, which in itself will exacerbate TR. Severe TR causes reduced antegrade flow across the pulmonary valve and reversal of flow in the arterial duct. This ‘steal’ from the systemic arterial circulation inhibits antegrade pulmonary blood flow and causes PR, which results in further volume loading of the RV. Furthermore, RV dilatation has an adverse effect on left ventricular geometry and function, causing additional fetal hemodynamic compromise2, explaining the high perinatal mortality of 45–71%3. Whilst there have been apparently favorable reports of indomethacin for a circular shunt in TVD2, 4, this treatment led to anhydramnios/oligohydramnios in both our cases. This may be due to pre-existing renal hypoperfusion secondary to poor ventricular-ventricular interaction and ductal steal. Furthermore, through its action on prostaglandins, indomethacin can lead to renal vasoconstriction and may cause oligohydramnios5. The potential benefits of ductal constriction for circular shunts need to be balanced against the risk of renal perfusion, necessitating close cardiac and fetal medicine supervision for affected fetuses. A prospective trial is needed to evaluate the risk-benefit ratio of this new treatment for fetal TVD. We acknowledge the contribution of Dr Wayne Tworetzky from the Children's Hospital in Boston, USA, whose group published the rationale for indomethacin therapy in this group of fetuses. Dr Tworetzky advised on the indomethacin and ibuprofen treatment used in the first case. Data available on request due to privacy/ethical restrictions

Critical Ebstein’s anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique

We report an innovative treatment strategy for fetal Ebstein's anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.

A Rare Pulmonary Arterio-venous Malformation with Circular Shunting Due to Inferior Phrenic Artery Supply with Pulmonary Venous Drainage: a Case Report

A rare case of a pulmonary arterio-venous malformation not requiring treatment is reported. An incidental peripheral right lower lobe intrapulmonary pulmonary arterio-venous malformation was identified on computed tomography. On catheter angiography, no pulmonary artery supply was identified. Systemic artery catheter arteriography identified systemic arterial supply from a conjoined inferior phrenic artery with pulmonary venous drainage to the left atrium. Due to this lung abnormality being a systemic arterial circulation to pulmonary venous circulation (left to left or circular) shunt, no treatment was indicated.

Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly)

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21 = 71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p = 0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

EP09.14: Surgical treatment for neonatal Ebstein's anomaly with circular shunting

EP09.14: Surgical treatment for neonatal Ebstein's anomaly with circular shunting

Prenatal Diagnosis of Ebstein's Anomaly: An Autopsy Correlation Predicting Poor Prognosis

Ebstein's anomaly (EA) is rare but not an uncommon congenital heart disease in the fetus. Fetal echocardiogram has progressed to detect many forms of congenital heart diseases, especially to assess the prognosis of EA based on their anatomy and presentation in utero causing nonimmune hydrops. Fetal echocardiographic findings of cardiomegaly, apical displacement of tricuspid valve with severe regurgitation, can confirm the diagnosis of EA in utero. Severe cardiomegaly and nonimmune hydrops further contribute poor prognosis leading to fetal demise. Identifying fetal risk factors for predicting perinatal mortality is important, including the lack of antegrade flow across the pulmonary valve and retrograde duct flow. Here, we describe fetal echocardiographic findings of a case of EA causing severe cardiomegaly, restricted growth of the lungs, and hydrops in a 26-year-old primigravida at 20 weeks of gestation. The diagnosis was further correlated with the autopsy, characterized by severe cardiomegaly, huge right atrium, apical displacement, and tethering of septal and posterior leaflet, with markedly reduced lung volumes. Autopsy findings of dilated ductus further suggestive of possible reversed ductal flow causing circular shunt in EA raising the possibility of the poor postnatal outcome, even intrauterine death, and warranted for safe termination. Poor prognostic factors in EA have also been discussed with autopsy findings.

Recipient Twin Circular Shunt Physiology Before Fetal Laser Surgery: Survival and Risks for Postnatal Right Ventricular Outflow Tract Obstruction.

To evaluate risk for congenital heart disease (CHD) in recipient twins with circular shunt physiology (CSP). This prospective study enrolled twin-twin transfusion syndrome (TTTS) cases from 2006 to 2015. Fetal echocardiography (FE) was performed before laser surgery when cardiac involvement was suspected. Diagnosis of recipient twin CSP required tricuspid and pulmonary regurgitation, right ventricular dysfunction, and flow reversal in the ductus arteriosus. Outcomes were assessed at 30 days after birth. Of the 496 TTTS pregnancies, 20 (4%) met the criteria for CSP. Among those born alive, who had documented cardiac outcomes (n = 457), patients with CSP were more likely to have CHD, specifically right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 22 of 439 [5.0%], odd ratio [OR] 7.29, 95% confidence interval [CI] 2.05-24.72, P = .0025). Of the recipient twins with preoperative FE (n = 259, 52%) who were born alive and had documented cardiac outcomes (n = 242), those with CSP were still more likely to have right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 14 of 224 [6.3%], OR 5.77, CI 1.54-20.92, P = .0077). With both analyses, twins with CSP had higher Quintero stage, but similar patient characteristics and 30-day mortality compared with those without CSP. Subgroup analyses of the CSP cohort identified no differences in preoperative characteristics or FE findings predictive of CHD. Recipient twins with preoperative CSP were at increased risk for postnatal right ventricular outflow tract obstruction, but appeared to have comparable survival after fetal laser surgery despite these dramatic pathophysiological prenatal findings. Preoperative FE in TTTS remains important for prediction of postnatal CHD.

Contemporary Outcomes and Factors Associated With Mortality After a Fetal or Neonatal Diagnosis of Ebstein Anomaly and Tricuspid Valve Disease

BackgroundEbstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare anomalies and data on outcomes after a fetal or neonatal EA/TVD diagnosis are conflicting. MethodsTo examine the outcome and identify markers predictive of mortality, we reviewed our single-centre experience from 2000-2014. Variables were analyzed separately for cases diagnosed in utero without pregnancy termination and for all live-born patients. ResultsOf 47 fetal cases, 8 (17%) died in utero and 10 (21%) as neonates. Independent predictors associated with fetal demise included severe tricuspid regurgitation with a Doppler gradient < 40 mm Hg (odds ratio, 1.22 per mm Hg deduction; P = 0.003) and pulmonary regurgitation (odds ratio, 11.4; P = 0.03) at the baseline examination. A novel prognostic score (range, 0-10) combining the severity of 5 echocardiographic findings was independently associated with overall mortality (hazard ratio [HR], 1.39 per point increase; P = 0.01). Survival rates of 66 live births at 1 month, 1 year, and 5 years were 86%, 82%, and 80% respectively, and 75%, 60%, and 55% remained free from surgery at the same points in time. Factors associated with postnatal death in multivariate analysis included a younger gestational age at birth (HR per week, 1.59; P < 0.001), tricuspid annulus diameter (HR per z-score increase, 1.76; P = 0.004), and no pulmonary forward flow (HR, 4.63; P = 0.03). ConclusionsOur experience with fetal and neonatal EA/TVD shows better survival rates than previously reported. Mortality after a fetal diagnosis was significantly associated with hemodynamic changes indicative of a circular shunt, including pulmonary and tricuspid regurgitation severe enough to cause diastolic umbilical arterial flow reversal.

862: Circular shunt physiology in recipient twins is associated with increased risk for postnatal right ventricular outflow tract obstruction

862: Circular shunt physiology in recipient twins is associated with increased risk for postnatal right ventricular outflow tract obstruction

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

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 &lt;32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5–21.0; P &lt;0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1–1.5; P &lt;0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4–6.2; P &lt;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 &lt;0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P &lt;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.

Demonstration of circular shunt in fetal Ebstein anomaly.

Ebstein's anomaly was diagnosed in a fetus at 24 weeks of gestation. There was significant cardiomegaly and severe tricuspid regurgitation (TR). There was functional pulmonary atresia with severe pulmonary regurgitation (PR) and this was causing a circular shunt. There was no fetal hydrops.

Prenatal ultrasonographic diagnosis of Uhl anomaly

Abstract Uhl anomaly is an extremely rare condition, and herein, we present a case of prenatally diagnosed Uhl anomaly to illustrate the associated echocardiographic features. Fetal echocardiography at 26 weeks indicated an enlarged right ventricle, severe tricuspid and pulmonary regurgitation, and ruptured ventricular septal aneurysm, resulting in a circular shunt. At 36 weeks, based on the presence of a thin ventricular wall and absence of apical trabeculation, a diagnosis of Uhl anomaly was made. The neonate, delivered by cesarean section, underwent main pulmonary artery ligation to eliminate the circular shunt immediately after birth. Moreover, he underwent ductus arteriosus ligation and received a pulmonary artery to aorta shunt for progressive lung congestion. He is currently well and awaiting a staged Fontan operation involving a bidirectional Glenn procedure. Understanding and accurate prenatal diagnoses of Uhl anomaly may help in parental counseling, planning appropriate perinatal care, and increasing the chances of survival.