scholarly journals 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

2021 ◽  
pp. 1-4
Author(s):  
Lilian M. Lopes ◽  
Rodrigo F. Bezerra ◽  
Jose Pedro da Silva ◽  
Luciana da Fonseca da Silva
Keyword(s):  
Single Ventricle ◽  
Ductus Arteriosus ◽  
Ebstein’S Anomaly ◽  
Biventricular Repair ◽  
Fetal Demise ◽  
Ebstein's Anomaly ◽  
Gestational Week ◽  
Inflammatory Drugs ◽  
Anti Inflammatory ◽  
Single Ventricle Palliation

Abstract 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.

Surgical management of symptomatic neonates with Ebstein’s anomaly: choice of operation

2014 ◽  
Vol 25 (6) ◽  
pp. 1119-1123 ◽  
Author(s):  
Christopher J. Knott-Craig ◽  
Thittamaranahalli Kariyappa S. Kumar ◽  
Alejandro R. Arevalo ◽  
Vijaya M. Joshi
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Surgical Management ◽  
Single Ventricle ◽  
Pulmonary Atresia ◽  
Early Mortality ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Single Ventricle Palliation ◽  
Artery Conduit

AbstractObjective:Symptomatic neonates with Ebstein’s anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset.Methods:A total of 32 consecutive symptomatic neonates with Ebstein’s anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients’ weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein’s anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes’ palliation and 2 Blalock–Taussig shunts. Six recent patients with Ebstein’s anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair.Results:Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein’s anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4).Conclusions:Surgical management of neonates with Ebstein’s anomaly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.

Modified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein’s anomaly: A paradigm shift

2021 ◽  
Keyword(s):  
Tricuspid Valve ◽  
Single Ventricle ◽  
Hemodynamic Instability ◽  
Ebstein’S Anomaly ◽  
Tricuspid Valve Repair ◽  
Valve Regurgitation ◽  
Valve Repair ◽  
Tricuspid Valve Regurgitation ◽  
Ebstein's Anomaly ◽  
Single Ventricle Palliation

Severe tricuspid valve regurgitation secondary to Ebstein’s anomaly represents several challenges in neonates. It can result in significant respiratory and/or hemodynamic compromise that mandates urgent interventions. When conservative management fails, 2 surgical options are available: tricuspid valve repair or single ventricle palliation. The overall results of neonatal tricuspid valve repair are unsatisfactory especially in sick neonates and those with preoperative hemodynamic instability. Single ventricle palliation utilizing the Starnes procedure with right ventricular exclusion provides a quicker way to improve hemodynamics and allows rapid decompression of the right ventricle but carries the long-term disadvantages of the single ventricle pathway. We were recently faced with a challenging case of neonatal Ebstein’s anomaly resulting in severe tricuspid valve regurgitation (TR) and significant hemodynamic and respiratory instability. We performed an initial stage I palliation with a modified Starnes’ procedure, which allowed stabilization and rapid recovery of the patient to be followed 5 months later with conversion to 2-ventricle repair using the cone technique. We believe combining these 2 strategies for suitable neonatal candidates may be a useful technique that should be considered in the algorithm for neonatal Ebstein’s anomaly.

scholarly journals Half-turned truncal switch operation after single ventricle palliation in a patient with borderline left heart hypoplasia

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Tak-Hyuk Oh ◽  
Hanna Jung ◽  
Joon Yong Cho ◽  
Youngok Lee
Keyword(s):  
Single Ventricle ◽  
Ductus Arteriosus ◽  
Pulmonary Stenosis ◽  
Fontan Operation ◽  
Main Pulmonary Artery ◽  
Good Alternative ◽  
Systemic Circulation ◽  
Biventricular Repair ◽  
Switch Operation ◽  
Single Ventricle Palliation

Abstract Background The optimal surgical strategy for the correction of double outlet right ventricle (DORV, transposition of the great arteries [TGA] type) or TGA with ventricular septal defect (VSD), pulmonary stenosis (PS), and borderline small left ventricle (LV) is still controversial. The half-turned truncal switch operation (HTTSO) introduced by Yamagishi and colleagues is a good option, but it is still challenging in a patient with borderline small LV. We aimed to describe our experience of a case of HTTSO conversion from single ventricle palliation. Case presentation A 5-year-old girl with single ventricle physiology was referred to our hospital from Kazakhstan for a Fontan operation. At the time of birth, she was diagnosed with DORV (TGA type), PS, and situs inversus totalis, with moderate valvar and subvalvar stenosis and a relatively small LV cavity. Her LV volume was not adequate to support the systemic circulation; therefore, doctors in Kazakhstan selected the single ventricle palliation course of treatment for the infant. At 4 months of age, she underwent left-sided modified Blalock-Taussig shunt, patent ductus arteriosus ligation, and atrial septectomy. At 2 years of age, shunt takedown, left bidirectional cavopulmonary shunt, and main pulmonary artery division were performed. Annual echocardiography of the patient showed that the LV size was growing too adequately to persist with the single ventricle palliation course of treatment. Via a multidisciplinary approach, we considered her LV to be suitable for biventricular repair and HTTSO was planned. The operation and postoperative course were uneventful. The patient was discharged on postoperative day 6 and went back to Kazakhstan. Conclusions Based on our successful surgical outcome, in patients diagnosed with DORV (TGA type) or TGA with VSD, PS, and borderline LV, HTTSO after achieving adequate LV growth by single ventricle palliation may be considered a good alternative to conventional operations in patients at a high risk for initial biventricular repair.

scholarly journals Mid-term Outcome Of Surgical Treatment In Pediatric Patients With Ebstein's Anomaly: A Single-center Cohort Study.

2020 ◽  
Author(s):  
Jia-chen Li ◽  
Xian-chao Jiang ◽  
Si-meng Zhang ◽  
Jin-yang Liu ◽  
Ya-juan Zhang ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Reoperation Rate ◽  
Term Effect ◽  
Ebstein’S Anomaly ◽  
Biventricular Repair ◽  
Ebstein's Anomaly ◽  
Long Term Follow Up ◽  
Surgical Treatments

Abstract Background: Ebstein’s anomaly is a malformation of the tricuspid valve and myopathy of the right ventricle. Surgery is now the main treatment for the defect. To summarize our surgical results and experience based on patients with Ebstein’s anomaly who were under 7 years of age and treated with different surgical treatments.Materials and Methods: From January 2010 to December 2019, 80 patients under 7 years old who were diagnosed of Ebstein’s anomaly and underwent different surgical treatments were consecutively enrolled and followed up in detail. Results: The median age of the 80 patients at the time of surgery was 3.63 years. Sixty-four (80.00%) patients underwent biventricular repair while 13 (16.25%) underwent 1.5-ventricle repair. With the median follow-up 27.50 months, the long-term survival of the total cohort, 1.5-ventricular repair and biventricular repair was 82.35%, 91.67% and 100%, respectively. The long-term freedom from reoperation rate was 97.50%, 92.31% and 98.44%, respectively. Mild, moderate and severe TR before surgery occurred in 6 (7.50%), 18 (22.50%) and 56 (70.00%), respectively. The early outcomes of 78 patients were 65 (83.33%), 11 (14.11%) and 2 (2.56%); the mid-term outcomes of 72 patients were 49 (68.06%), 19 (26.38%) and 4 (5.56%). Both early and long-term valve regurgitation were significantly decreased (p< 0.001) compared with preoperative condition. No more severe regurgitation occurred (p=0.404), though some early mild regurgitation became acceptable moderate regurgitation during long-term follow-up (p=0.036). Compared with Carpentier procedure, cone procedure had better long-term effect, while the effect of whole-valve technique needed more operation and long-term follow-up.Conclusion: The reoperation rate and mid-term mortality of surgical treatment for Ebstein’s anomaly were both low, tricuspid regurgitation was significantly improved during mid-term follow up. Cone procedure had the best mid-term effect among anatomic repair.

Abstract 20041: Single Ventricle Outcome Following Neonatal Palliation of Severe Ebstein’s Anomaly With Modified Starnes Procedure

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
S. Ram Kumar ◽  
Nathan Noh ◽  
Novell Castillo ◽  
Brian Fagan ◽  
Grace Kung ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Nyha Class ◽  
Pacemaker Implantation ◽  
Outcome Data ◽  
Left Ventricular ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly

Background: We have previously shown that neonates in profound cardiogenic shock due to severe Ebstein’s anomaly can be successfully salvaged with fenestrated right ventricular (RV) exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of 26 patients who underwent neonatal Starnes procedure between 1989 and 2011. Patient demographics, clinical variables and outcome data were collected. Data is presented as mean ± standard errors or median (interquartile ranges). Results: 26 patients (12, 46% boys) underwent Starnes procedure at 7 (5-9) days of life. All were intubated and on prostacyclin infusion, 24 (92%) were inotrope-dependent and 23 (88%) had no antegrade flow from the RV. Two patients had had prior intervention (one tricuspid annuloplasty and one shunt alone). Three patients underwent non-fenestrated RV exclusion, two (67%) of whom died. Of the remaining 23, 3 (13%) died during the same hospitalization. The 21 neonatal survivors have been followed for 7 (6-8) years. One patient died after Glenn. The remaining 20 have successfully undergone Fontan completion with an indexed pulmonary resistance of 1.8 (1.2-2.3) W/m2 and mean pulmonary pressure of 12 (9-18) mm Hg. At last follow-up, all patients have normal left ventricular function, and all but one patient are in NYHA Class I symptoms. Two patients have required pacemaker implantation, while the rest are in sinus rhythm. Survival at 1, 5 and 10 years are 81±4%, 77±3% and 77±3%, respectively. Conclusion: Long-term single ventricle outcomes amongst neonatal survivors of modified Starnes procedure are excellent. There is reliable remodeling of the excluded RV and excellent function of the left ventricle.

scholarly journals Ebstein’s anomaly with ‘reversible’ functional pulmonary atresia

2019 ◽  
Vol 12 (12) ◽  
pp. e229809
Author(s):  
João Rato ◽  
Ana R Sousa ◽  
Ana Teixeira ◽  
Rui Anjos
Keyword(s):  
Pulmonary Valve ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Ebstein’S Anomaly ◽  
Peripheral Oxygen Saturation ◽  
Pulmonic Valve ◽  
Ebstein's Anomaly ◽  
Chest X Ray ◽  
Team Decision

We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein’s anomaly without anterograde flow from right ventricular to pulmonary atresia (PA)—functional PA with flow reversal in the ductus arteriosus. Prostaglandin E1 was started after birth. Chest X-ray showed severe cardiomegaly and echocardiogram confirmed Ebstein’s anomaly with a thickened non-opening pulmonary valve without anterograde flow but with mild regurgitation. Multidisciplinary team decision was to progressively reduce prostaglandins and have an expectant attitude. Peripheral oxygen saturation above 85% was maintained and serial echocardiograms documented progressive reduction of the ductus arteriosus and the opening of the pulmonic valve cusps, with the development of anterograde flow. The newborn was discharged at day 19 of life without the need for any intervention, and at last follow-up remains asymptomatic, with anterograde normal flow in the pulmonary valve.

Non-steroid anti-inflammatory drugs in the treatment of patent ductus arteriosus in European newborns

2009 ◽  
Vol 22 (sup3) ◽  
pp. 77-80 ◽  
Author(s):  
Hercília Guimarães ◽  
Gustavo Rocha ◽  
Teresa Tomé ◽  
Fani Anatolitou ◽  
Kosmas Sarafidis ◽  
...  
Keyword(s):  
Patent Ductus Arteriosus ◽  
Ductus Arteriosus ◽  
Inflammatory Drugs ◽  
Anti Inflammatory ◽  
Patent Ductus

Echocardiographic factors discriminating biventricular versus univentricular approach in the foetus with borderline left ventricle

2014 ◽  
Vol 25 (5) ◽  
pp. 941-950 ◽  
Author(s):  
David W. Jantzen ◽  
Sarah K. Gelehrter ◽  
Sunkyung Yu ◽  
Janet E. Donohue ◽  
Carlen G. Fifer
Keyword(s):  
Mitral Valve ◽  
Left Ventricle ◽  
Single Ventricle ◽  
Left Ventricular ◽  
Z Score ◽  
Biventricular Repair ◽  
Z Scores ◽  
Valve Annulus ◽  
Single Ventricle Palliation ◽  
Dimension Ratio

AbstractBackground: The term “borderline left ventricle” describes a small left heart that may be inadequate to provide systemic cardiac output and implies the potential need for a single-ventricle palliation. The aim of this study was to identify foetal echocardiographic features that help discriminate which infants will undergo single-ventricle palliation versus biventricular repair to aid in prenatal counselling. Methods: The foetal database at our institution was searched to identify all foetuses with borderline left ventricle, as determined subjectively by a foetal cardiologist, from 2000 to 2011. The foetal images were retrospectively analysed for morphologic and physiologic features to determine which best predicted the postnatal surgical choice. Results: Of 39 foetuses identified with borderline left ventricle, 15 were planned for a univentricular approach, and 24 were planned for a biventricular approach. There were significant differences between the two outcome groups in the Z-scores of the mitral valve annulus, left ventricular end-diastolic dimension, aortic valve annulus, and ascending aorta diameter (p<0.05). With respect to discriminating univentricular outcomes, cut-offs of mitral valve Z-score ⩽−1.9 and tricuspid:mitral valve ratio ⩾1.5 were extremely sensitive (100%), whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 provided the highest specificity (95.8%). Conclusion: In foetuses with borderline left ventricle, a mitral valve Z-score ⩾−1.9 or a tricuspid:mitral valve ratio ⩽1.5 suggests a high probability of biventricular repair, whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 confers a likelihood of single-ventricle palliation.

scholarly journals Organ and prenatal toxicity of nonsteroidal anti-inflammatory drugs

2015 ◽  
Vol 28 (3) ◽  
pp. 200-203
Author(s):  
Katarzyna Dyndor ◽  
Wojciech Dworzanski ◽  
Małgorzata Pliszczynska-Steuden ◽  
Monika Cendrowska-Pinkosz ◽  
Tomasz Chroscicki ◽  
...  
Keyword(s):  
Ductus Arteriosus ◽  
Late Pregnancy ◽  
Congenital Defects ◽  
Abdominal Wall Defects ◽  
Cox Inhibitors ◽  
Prenatal Toxicity ◽  
Developing Heart ◽  
Increased Risk ◽  
Inflammatory Drugs ◽  
Anti Inflammatory

Abstract Non-selective cyclooxygenase (COX) inhibitors, commonly referred to as nonsteroidal anti-inflammatory drugs (NSAIDs), are among the most taken pharmaceuticals. In adults, they can have a series of side effects, including especially gastroenterotoxicity, hepatotoxicity, nephrotoxicity, chondrotoxicity, and neurotoxicity, and they can induce allergic reactions. Any exacerbation of symptoms depends on the chemical structure of the drug, its dosage and duration of exposure, individual sensitivity, comorbidities and the degree of inhibition of basic COX isoenzymes - the constitutive (COX-2) and induced (COX-1) expressions. However, data on prenatal toxicity are inconsistent. Classic nonselective COX inhibitors do not result in an increase in the risk of developing significant congenital defects; however, if used in the late-pregnancy period, they can have an adverse effect on the foetus, by inducing the premature closure of the ductus arteriosus and by producing a tocolytic effect. Individual reports also indicate the increased risk of developing heart and anterior abdominal wall defects, as well as hypospadias.

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