Systemic Semilunar Valve Dysfunction May Be an Underappreciated Component of the Late Morbidity Occurring After Single Ventricle Palliation

2021 ◽  
Vol 12 (6) ◽  
pp. 745-746
Author(s):  
John Lamberti
2021 ◽  
pp. 1-4
Author(s):  
Lilian M. Lopes ◽  
Rodrigo F. Bezerra ◽  
Jose Pedro da Silva ◽  
Luciana da Fonseca da Silva

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.


2019 ◽  
Vol 10 (2) ◽  
pp. 164-170 ◽  
Author(s):  
Valdano Manuel ◽  
Humberto Morais ◽  
Aida L. R. Turquetto ◽  
Gade Miguel ◽  
Leonardo A. Miana ◽  
...  

Introduction: Single ventricle physiology management is challenging, especially in low-income countries. Objective: To report the palliation outcomes of single ventricle patients in a developing African country. Methods: We retrospectively studied 83 consecutive patients subjected to single ventricle palliation in a single center between March 2011 and December 2017. Preoperative data, surgical factors, postoperative results, and survival outcomes were analyzed. The patients were divided by palliation stage: I (pulmonary artery banding [PAB] or Blalock–Taussig shunt [BTS]), II (Glenn procedure), or III (Fontan procedure). Results: Of the 83 patients who underwent palliation (stages I-III), 38 deaths were observed (31 after stage I, six after stage II, and one after stage III) for an overall mortality of 45.7%. The main causes of operative mortality were multiple organ dysfunction due to sepsis, shunt occlusion, and cardiogenic shock. Twenty-eight survivors were lost to follow-up (22 after stage I, six after stage II). Thirteen stage II survivors are still waiting for stage III. The mean follow-up was 366 ± 369 days. Five-year survival was 28.4 % for PAB and 30.1% for BTS, while that for stage II and III was 49.8% and 57.1%, respectively. Age (hazard ratio, 0.61; 95% confidence interval: 0.47-0.7; P = .000) and weight at surgery (hazard ratio, 0.45; 95% confidence interval: 0.31-0.64; P = .002) impacted survival. Conclusion: A high-mortality rate was observed in this initial experience, mainly in stage I patients. A large number of patients were lost to follow-up. A task force to improve outcomes is urgently required.


2011 ◽  
Vol 57 (14) ◽  
pp. E409
Author(s):  
Gabriele Egidy Assenza ◽  
Kimberlee Gauvreau ◽  
Dionne Graham ◽  
Michael Job Landzberg ◽  
Koenraad J. Mortele ◽  
...  

2014 ◽  
Vol 25 (6) ◽  
pp. 1119-1123 ◽  
Author(s):  
Christopher J. Knott-Craig ◽  
Thittamaranahalli Kariyappa S. Kumar ◽  
Alejandro R. Arevalo ◽  
Vijaya M. Joshi

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.


2020 ◽  
Vol 26 (2) ◽  
pp. 46-58
Author(s):  
Zornitsa Vassileva ◽  
Anna Kaneva

Unbalanced atrioventricular septal defect is seen by 10-15% of the patients with this congenital heart disease and poses serious challenges for the treating physicians regarding the choice of the most suitable operative intervention. The forms with moderate degree of unbalance between the two ventricles, especially when the size of the left ventricle is considered borderline, make the choice between two-ventricle correction and single-ventricle palliation quite difficult. The most important factor when judging the degree of unbalance is not the size of the ventricles but rather the redistribution of blood flow. The use of different echocardiographic measurements could contribute to the more precise determination of the indications for one of the two surgical strategies with resulting improvement of patient outcome.


Author(s):  
Sheba John ◽  
Laura Schoeneberg ◽  
Christopher E. Greenleaf ◽  
Jorge D. Salazar ◽  
Dilachew A. Adebo

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