Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery

Bhaskar, Jayapadman; Galati, John C.; Brooks, Paul; Oppido, Guido; Konstantinov, Igor E.; Brizard, Christian P.; d’Udekem, Yves

doi:10.1016/j.jtcvs.2015.01.038

Cited by 35 publications

(34 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…10 Many patients with isomerism die before reaching adulthood. 11 In 2011, Jacobs and associates 12 reported an analysis of The Society of Thoracic Surgeons congenital heart surgery database of patients with heterotaxy. They found that heterotaxy is a challenging problem with increased discharge mortality in most single ventricle palliation subgroups.…”

Section: Commentmentioning

confidence: 99%

Outcomes of the Fontan Operation for Patients With Heterotaxy: A Meta-Analysis of 848 Patients

Marathe

Cao

Celermajer

et al. 2020

The Annals of Thoracic Surgery

View full text Add to dashboard Cite

Background. Heterotaxy is considered a risk factor for adverse events at all stages in the pre-Fontan pathway, and Fontan outcomes are expected to be worse in patients with heterotaxy. The aim of this study was to review existing literature reporting outcomes of the Fontan operation systematically in patients with heterotaxy.Methods. A systematic review and meta-analysis was performed to identify and synthesize early mortality and medium-and long-term survival in heterotaxy patients after the Fontan procedure. Subsequent outcome analyses were stratified by study period era, cohort size, and proportion of right versus left atrial isomerism to explore predictors of outcome.Results. A total of 21 studies were included for analysis, which were composed of 848 post-Fontan heterotaxy patients. Early mortality varied between 1% and 30% with a weighted event rate of 14% (95% confidence interval [CI], 10%-19%). Survival at 1, 5, and 10 years was 86% (95% CI, 79%-91%), 80% (95% CI, 71%-87%), and 74% (95% CI, 59%-85%), respectively. Stratification by study period highlighted that studies with a median study period year of 1995 or later had similar early mortality and 1-and 5-year survival, but superior 10-year survival (P [ .02) compared with earlier studies. Stratification by cohort size and right versus left atrial isomerism did not reveal subgroup differences.Conclusions. Compared with existing literature, in patients with heterotaxy, early mortality after Fontan is higher than for the overall Fontan population. Long-term survival is comparable to the overall Fontan cohort. When heterotaxy patients are successfully transitioned to Fontan, subsequent survival is acceptable and predictable. Long-term follow-up is lacking.

show abstract

Section: Commentmentioning

confidence: 99%

Outcomes of the Fontan Operation for Patients With Heterotaxy: A Meta-Analysis of 848 Patients

Marathe

Cao

Celermajer

et al. 2020

The Annals of Thoracic Surgery

View full text Add to dashboard Cite

show abstract

“…Future studies are required to better understand the pathophysiological mechanisms in this rare subgroup of patients. Though patients with obstructed TAPVD and atrial isomerism are at increased risk of early mortality, 16 it appears that survival after the Fontan completion is associated with a good long-term prognosis.…”

Section: Commentmentioning

confidence: 99%

Long-term Outcomes of the Fontan Operation in Patients With Total Anomalous Pulmonary Venous Drainage

Yong

Zhu

Plessis

et al. 2019

The Annals of Thoracic Surgery

Self Cite

View full text Add to dashboard Cite

“…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

View full text Add to dashboard Cite

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.

show abstract

Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery

Abstract: A significant proportion of patients with isomerism still die before reaching adulthood. Only a fraction of those with obstructed pulmonary veins survive. Having biventricular repair does not confer a survival advantage to those born with right atrial isomerism.

Cited by 35 publications

References 14 publications

Outcomes of the Fontan Operation for Patients With Heterotaxy: A Meta-Analysis of 848 Patients

Outcomes of the Fontan Operation for Patients With Heterotaxy: A Meta-Analysis of 848 Patients

Long-term Outcomes of the Fontan Operation in Patients With Total Anomalous Pulmonary Venous Drainage

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

Contact Info

Product

Resources

About