Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome

Alsoufi, Bahaaldin; McCracken, Courtney; Schlosser, Brian; Sachdeva, Ritu; Well, Andrew; Kogon, Brian; Border, William L.; Kanter, Kirk R.

doi:10.1016/j.jtcvs.2016.01.054

Cited by 63 publications

(68 citation statements)

References 37 publications

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“…Those outcomes are related to the presence of associated cardiac lesions (TAPVC, common atrioventricular valve regurgitation, pulmonary atresia, and heart block) in addition to the extracardiac lesions that all contribute to increased mortality after single ventricle palliation [5, 6, 11, 14-17, 19, 21, 22]. Interestingly, a recent report from our institution showed that patients with heterotaxy syndrome and right dominant ventricle had a trend to better survival than patients with left dominant ventricle, suggesting again that associated lesions rather than dominant ventricle morphology played a larger role in early outcomes in those patients [22].…”

Section: Commentmentioning

confidence: 99%

The Impact of Dominant Ventricle Morphology on Palliation Outcomes of Single Ventricle Anomalies

Alsoufi

Gillespie

Kim

et al. 2016

The Annals of Thoracic Surgery

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Section: Commentmentioning

confidence: 99%

The Impact of Dominant Ventricle Morphology on Palliation Outcomes of Single Ventricle Anomalies

Alsoufi

Gillespie

Kim

et al. 2016

The Annals of Thoracic Surgery

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“…Many previous reports have shown that heterotaxy was associated with increased mortality risk following various palliative procedures including BTS, Norwood, or PAB. [6][7][8][9][10]14,15,[24][25][26] A recent Society of Thoracic Surgeons study examining hospital survival of 1,505 patients with heterotaxy who underwent surgery demonstrated that discharge mortality was higher in patients with heterotaxy compared to patients without heterotaxy for every procedure mortality risk category and for different subgroups of patients such as those who underwent BTS or Fontan. 14 Several recent single institution reports of palliation outcomes in patients with heterotaxy demonstrated increased mortality, respiratory complications, and more complicated postoperative course.…”

Section: Discussionmentioning

confidence: 99%

Outcomes of Multistage Palliation of Infants With Single Ventricle and Atrioventricular Septal Defect

Alsoufi

Kanter

Shashidharan

et al. 2019

World J Pediatr Congenit Heart Surg

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Background: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results. Methods: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention. Results: Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients. Conclusions: Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

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“…These results are in agreement with the literature data, with a rather favourable prognosis in case of doubleinlet left ventricle, 10 as opposed to the right-type single ventricle, 11,12 or heterotaxis with complete atrio-ventricular canal defect. 13…”

Section: Discussionmentioning

confidence: 99%

Surgery of single ventricles in humanitarian practice: surgery for which patients?

Gouton¹,

Bical²,

Zoghbi

et al. 2019

Cardiol Young

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Objectives:To analyse the feasibility and effectiveness in humanitarian practice of surgical management of children with single-ventricle heart condition.Methods:Retrospective study of children with a single ventricle, managed by the association Mécénat-Chirurgie Cardiaque since 1996, with long-term follow-up after their return home.Results:Of the 138 children in our cohort, 119 had one or more surgeries (180 procedures): palliative surgery alone (systemic-pulmonary anastomosis or banding), 41; partial cavo-pulmonary connection, 47; total cavo-pulmonary connection (mean age 8.5 years), 31. Operative mortality is 5.5%. After a mean follow-up of 5.6 years, 18 children (13%) were lost to follow-up. Survival at 10 years is 79% in children receiving surgery (palliative only, 72%; partial cavo-pulmonary connection, 77%; total cavo-pulmonary connection, 97%) versus 29% in children with no surgical intervention. The prognosis is better for tricuspid atresia and double-inlet left ventricle (86 and 83% survival at 10 years) than for double-outlet right ventricle or complete atrio-ventricular canal defect (64 and 68% at 5 years).Conclusion:The surgery of the single ventricle in humanitarian medicine allows a very satisfactory survival after one or more surgeries tending towards a total cavo-pulmonary connection as soon as possible.

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Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome

Cited by 63 publications

References 37 publications

The Impact of Dominant Ventricle Morphology on Palliation Outcomes of Single Ventricle Anomalies

The Impact of Dominant Ventricle Morphology on Palliation Outcomes of Single Ventricle Anomalies

Outcomes of Multistage Palliation of Infants With Single Ventricle and Atrioventricular Septal Defect

Surgery of single ventricles in humanitarian practice: surgery for which patients?

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