Current outcomes of the Glenn bidirectional cavopulmonary connection for single ventricle palliation

The development of the superior cavopulmonary connection is a rich illustration of international influences in congenital cardiac surgery. The bidirectional Glenn and hemi-Fontan procedures have improved survival as both definitive and staged functional single ventricle palliation. The optimal timing of the second-stage superior cavopulmonary procedures varies by center but for low- and intermediate-risk patients, this may be within three to six months after the Norwood procedure. The list of risk factors continues to grow but the most frequently cited factors include atrioventricular valve regurgitation, decreased ventricular function, need for reintervention, and failure to attain nutritional and growth goals. Ongoing prospective, multi-institutional studies, particularly those fostered internationally by the World Society for Pediatric and Congenital Heart Surgery and other associations, will hopefully provide further clarification of the complex management issues related to patients with functional single ventricle physiology.

Download Full-text

The comparative role of echocardiography and MRI for identifying critical lesions in patients with single-ventricle physiology, before bidirectional cavopulmonary connection

Cardiology in the Young ◽

10.1017/s1047951115002693 ◽

2016 ◽

Vol 26 (7) ◽

pp. 1373-1382 ◽

Cited By ~ 2

Author(s):

Sylvia Krupickova ◽

Michael A. Quail ◽

Robert Yates ◽

Roman Gebauer ◽

Marina Hughes ◽

...

Keyword(s):

Pulmonary Artery ◽

Odds Ratio ◽

Single Ventricle ◽

Pulmonary Arteries ◽

Left Pulmonary Artery ◽

Atrioventricular Valve ◽

Valve Regurgitation ◽

Atrioventricular Valve Regurgitation ◽

Cavopulmonary Connection ◽

Bidirectional Cavopulmonary Connection

AbstractBackgroundIn the era of multi-modality imaging, this study compared contemporary, pre-operative echocardiography and cardiac MRI in predicting the need for intervention on additional lesions before surgical bidirectional cavopulmonary connection.MethodsA total of 72 patients undergoing bidirectional cavopulmonary connection for single-ventricle palliation between 2007 and 2012, who underwent pre-operative assessment using both echocardiography and MRI, were included. The pre-determined outcome measure was any additional surgical or catheter-based intervention within 6 months of bidirectional cavopulmonary connection. Indices assessed were as follows: indexed dimensions of right and left pulmonary arteries, coarctation of the aorta, adequacy of interatrial communication, and degree of atrioventricular valve regurgitation.ResultsMedian age at bidirectional cavopulmonary connection was 160 days (interquartile range 121–284). The following MRI parameters predicted intervention: Z score for right pulmonary artery (odds ratio 1.77 (95% confidence interval 1.12–2.79, p=0.014)) and left pulmonary artery dimensions (odds ratio 1.45 (1.04–2.00, p=0.027)) and left pulmonary artery report conclusion (odds ratio 1.57 (1.06–2.33)). The magnetic resonance report predicted aortic arch intervention (odds ratio 11.5 (3.5–37.7, p=0.00006)). The need for atrioventricular valve repair was associated only with magnetic resonance regurgitation fraction score (odds ratio 22.4 (1.7–295.1, p=0.018)). Echocardiography assessment was superior to MRI for predicting intervention on interatrial septum (odds ratio 27.7 (6.3–121.6, p=0.00001)).ConclusionFor branch pulmonary arteries, aortic arch, and atrioventricular valve regurgitation, MRI parameters more reliably predict the need for intervention; however, echocardiography more accurately identified the adequacy of interatrial communication. Approaching bidirectional cavopulmonary connection, the diagnostic strengths of MRI and echocardiography should be acknowledged when considering intervention.

Download Full-text

Faculty Opinions recommendation of Long-term outcomes of single-ventricle palliation for unbalanced atrioventricular septal defects: Fontan survivors do better than previously thought.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.727254837.793545038 ◽

2018 ◽

Author(s):

P Syamasundar Rao

Keyword(s):

Single Ventricle ◽

Long Term Outcomes ◽

Atrioventricular Septal Defects ◽

Septal Defects ◽

Single Ventricle Palliation ◽

Better Than

Download Full-text

Faculty Opinions recommendation of Neurodevelopmental outcome in children with single ventricle after total cavopulmonary connection.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.732588180.793547138 ◽

2018 ◽

Author(s):

Willem Helbing

Keyword(s):

Single Ventricle ◽

Neurodevelopmental Outcome ◽

Total Cavopulmonary Connection ◽

Cavopulmonary Connection

Download Full-text

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

Cardiology in the Young ◽

10.1017/s1047951121000081 ◽

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.

Download Full-text

Single Ventricle Palliation in a Developing Sub-Saharan African Country: What Should be Improved?

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118822671 ◽

2019 ◽

Vol 10 (2) ◽

pp. 164-170 ◽

Cited By ~ 3

Author(s):

Valdano Manuel ◽

Humberto Morais ◽

Aida L. R. Turquetto ◽

Gade Miguel ◽

Leonardo A. Miana ◽

...

Keyword(s):

Confidence Interval ◽

African Country ◽

Single Ventricle ◽

Hazard Ratio ◽

Stage I ◽

Stage Ii ◽

Stage Iii ◽

Lost To Follow Up ◽

Single Ventricle Palliation

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.

Download Full-text