VARIABILITY OF PRACTICE AND RISK FOR ADVANCED DIAGNOSTIC MODALITIES PRIOR TO SUPERIOR CAVOPULMONARY CONNECTION AS PART OF STAGED SINGLE VENTRICLE PALLIATION: A MULTI-INSTITUTION STUDY

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.

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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

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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

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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.

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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.

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