Heterotaxy Is Not a Risk Factor for Adverse Long-Term Outcomes After Fontan Completion

Zannino, Diana; Cao, Jacob; Plessis, Karin du; Marathe, Supreet P; Ayer, Julian; Celermajer, David S.; Gentles, Thomas L.; Sholler, Gary F.; Justo, Robert; Alphonso, Nelson; d’Udekem, Yves; Winlaw, David S.

doi:10.1016/j.athoracsur.2019.11.015

Cited by 22 publications

(31 citation statements)

References 20 publications

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“…This study reviewed our experience in the management of 86 patients with atrial isomerism in our center over the past 13-years. Our data showed that despite even greater clinical diversity, the intermediate outcomes were improving, and 10year survival rate reached 79.3%, which is comparable to many of the previous reports (5,6). Consistent with previous studies, we found that extracardiac TAPVC, more than moderate AVV regurgitation, and requirement for first-stage palliative surgery were independent risk factors for mortality.…”

Section: Discussionsupporting

confidence: 91%

“…There was no significant difference compared to patients without isomerism (10). This result indicated that once Fontan circulation is successfully completed, it does not appear to be an important risk factor for its failure, which was similar to Marathe's latest report (5). We also found that the difference of 5-year survival between RAI and LAI was also not statistically significant (p = 0.506).…”

Section: Discussionsupporting

confidence: 88%

“…Cardiovascular mortality in early childhood is estimated at about 10% or more, even in the current era (2,3). Fortunately, recent reports from developed countries showed that the survival of atrial isomerism with cardiac malformation is continuing to improve, and most patients can survive into adulthood (4)(5)(6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

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Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China

Zou

Cao

et al. 2021

Front. Cardiovasc. Med.

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Objectives: The management of atrial isomerism with complex congenital heart disease remains challenging. Experience has been largely obtained in advanced countries. The clinical diversity is greater in China. We evaluated the early- and medium-term outcomes of surgical treatment of these patients.Methods: We reviewed 86 patients of atrial isomerism with complex congenital heart disease undergoing varied surgeries in our center in 2008–2020. Cox regression models were used to analyze the risk factors for mortality.Results: There were 75 cases of right and 11 of left atrial isomerism. Eighty-three (96.5%) patients underwent single-ventricle staged palliation approach, with 10 early and 7 late deaths. The overall 1-, 5-, and 10-year survival rates were 84.7, 79.3, and 79.3%, respectively. Thirty-six (43.4%) patients completed the Fontan procedure with median age of 48 months and freedom from death or Fontan failure at 1-, 5-, and 8-years were 94.4, 87.4, and 80.7%, respectively. Concomitant total anomalous pulmonary venous connection [hazard ratio (HR): 5.15 (1.95–12.94), p = 0.008], more than moderate atrioventricular valve regurgitation [HR: 4.82 (2.42–6.79), p = 0.003], and the need for first-stage palliative surgery [HR: 4.58 (1.64–10.76), p = 0.015] were independent risk factors for mortality.Conclusions: Despite even greater clinical diversity, the surgical outcomes of atrial isomerism with complex congenital heart disease are improving in China. The early and intermediate outcomes are comparable to many previous reports. Concomitant total anomalous pulmonary venous connection, moderate or severe atrioventricular valve regurgitation, and the need for a first-stage palliative surgery are still independent risk factors for mortality.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 88%

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Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China

Zou

Cao

et al. 2021

Front. Cardiovasc. Med.

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“…Patients undergoing FP with the diagnosis of heterotaxy syndromes is always a challenge and results of FP in these patients are variable in literature (31,32). The IECF modification is easy and reproducible in these patients no matter the anatomy is complex or not, since the cardiopulmonary bypass and aortic cross clamp times are not prolonged with IECF in these patients (27).…”

Section: Discussionmentioning

confidence: 99%

Mid Term Results of Fenestrated Intra-Extracardiac Fontan Procedure: Insights, Experiences and Expectations

Özyüksel¹,

Şimşek²,

Demiroluk³

et al. 2021

Preprint

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Background: Intraextracardiac Fontan procedure aimed to combine the advantages of lateral tunnel and extracardiac conduit modifications of the original technique. Herein, we present our experience in our patients with intraextracardiac fenestrated Fontan Procedure. Methods: A retrospective analysis was performed in order to evaluate intraextracardiac fenestrated Fontan patients between 2014 and 2021. Seventeen patients were operated on with a mean age and body weight of 9.1 ± 5.5 years and 28.6 ± 14.6 kg. Results: Sixteen patients (94%) were palliated as univentricular physiology with hypoplasia of one of the ventricles. One patient (6%) with well-developed two ventricles with double outlet right ventricle and complete atrioventricular septal defect had straddling of the chordae prohibiting a biventricular repair. All of the patients had cavopulmonary anastomosis prior to Fontan completion, except one case. Fenestration was performed in all cases. Postoperative mean pulmonary artery pressures and arterial oxygen saturation levels at follow up were 10 ± 2.4 mmHg and 91.3 ± 2.7 %, respectively. Mean duration of pleural drainage was 5.4 ± 2.3 days. All of the fenestrations are patent at a mean follow up period of 4.8 ± 7.7 years, except one case. Any morbidity and mortality were not encountered. Conclusions: The mid-term results of intraextracardiac fenestrated Fontan procedure are encouraging. This procedure may improve the results in a patient population who should be palliated as univentricular physiology, especially in cases with complex cardiac anatomy.

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“…Marathe and colleagues 1 have recently reported their experience of 109 patients with heterotaxy who underwent univentricular repair and completed Fontan operation. Interestingly, they found that although heterotaxy has always been considered a risk factor for poor outcomes, once Fontan circulation is successfully achieved it does not appear to be an important risk factor for its failure.…”

mentioning

confidence: 99%

Heterotaxy: Elder Age, Improving Outcomes

Chen

Guariento

Chen

2020

The Annals of Thoracic Surgery

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Heterotaxy Is Not a Risk Factor for Adverse Long-Term Outcomes After Fontan Completion

Cited by 22 publications

References 20 publications

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China

Mid Term Results of Fenestrated Intra-Extracardiac Fontan Procedure: Insights, Experiences and Expectations

Heterotaxy: Elder Age, Improving Outcomes

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