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

Yong, Matthew S.; Zhu, Michael Z.L.; Plessis, Karin du; Weintraub, Robert G.; Hornung, Tim; Winlaw, David S.; Alphonso, Nelson; d’Udekem, Yves; Konstantinov, Igor E.

doi:10.1016/j.athoracsur.2019.04.073

Cited by 10 publications

(10 citation statements)

References 17 publications

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“…In the current study, we did not find that to be justifiable. This is consistent with the reporting of outcomes of patients who underwent TAPVD repair with single ventricle anomalies and then went on to receive a Fontan operation, Yong et al [13]. The outcomes of patients who had TAPVD repair then Fontan operation were comparable to Fontan patients without TAPVD repair.…”

Section: Discussionsupporting

confidence: 88%

“…However, in univariable analysis, we did not find that neonatal age, weight, TAPVD anatomical type, or presentation with obstruction were significantly associated with operative or long-term death. This contrasts with the findings of the larger and more long-term studies like Yong et al [13] and Sakamoto et al [4] which showed that neonatal age, weight less than 2 kg, and mixed TAPVD were factors associated with reduced survival. The current study has shown that pulmonary vein stenosis is an important predictor of long-term freedom from death or reoperation.…”

Section: Discussioncontrasting

confidence: 78%

“…Anatomical type [5], stenosis of the drainage pathway [6], surgical technique [6][7][8][9][10][11][12], and time era all are reported to have an important effect of short-and long-term outcomes. Associated single ventricle anomalies carry a particularly poor prognosis [13]. The advent of the so-called sutureless techniques is thought to prevent stenosis and have an impact on long term outcomes [14,15].…”

Section: Introductionmentioning

confidence: 99%

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Total anomalous pulmonary venous drainage repair: the effect of anatomical type and pulmonary vein stenosis on outcomes

et al. 2020

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Background: Surgical repair of total anomalous venous drainage (TAPVD) is lifesaving. The operative mortality is reported between 4 and 35%. Anatomical type, obstructed presentation, associated single ventricle, and heterotaxy syndromes are thought to influence short-and long-term outcomes. The effect of simple versus sutureless repair for primary surgery is unclear. This study reports the outcomes of the surgical repair and the effect of these variables in a contemporary setting. Results: Between 2011 and 2019, all patients undergoing surgical repair for TAPVD were included. Operative mortality, length of hospital stay, and long-term survival were reported. The effect of anatomical type, surgical technique, obstruction, and associated lesions was assessed. Pearson's test, Wilcoxson's test, and generalized linear regression with Poisson distribution were used. Forty-nine patients from two centers underwent TAPVD repair. The operative mortality was 4%. Postoperative pulmonary vein stenosis occurred in three patients, and reoperation was done in one patient. Survival free from reoperation was 98%, 98%, and 90% at 1, 24, and 60 months in the absence of pulmonary vein stenosis. However, all three patients who developed pulmonary vein stenosis died, at 0.5, 2.7, and 6.3 months of follow-up, respectively. We were unable to detect a significant effect of anatomical type, preoperative obstruction, associated single ventricle, or heterodoxy syndrome on operative mortality or long-term freedom from death or reoperation. Patients who presented with obstruction and infracardiac or supracardiac TAPVD had longer hospital stay. Conclusion: TAPVD repair outcomes are excellent except for patients who develop postoperative pulmonary venous stenosis. Anatomical type, obstructed presentation, associated single ventricle, or heterotaxy are not significant predictors of survival.

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Section: Discussionsupporting

confidence: 88%

Section: Discussioncontrasting

confidence: 78%

Section: Introductionmentioning

confidence: 99%

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Total anomalous pulmonary venous drainage repair: the effect of anatomical type and pulmonary vein stenosis on outcomes

et al. 2020

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confidence: 99%

“…Treatment of patients associated with FSV and TAPVC has a high mortality rate and poor prognosis [1][2][3][4] . Patients with FSV and TAPVC often have visceral heterotaxy syndrome 3,5,6 , which may led to a worse clinical outcomes. In addition, Pulmonary atresia 2,7 , pulmonary artery stenosis 3 , preoperative PVO 1,7 , right ventricular dominance 3,8 , infracardiac or mixed TAPVC 7 , low weight and younger age 9 are considered risk factors that increase the mortality of these patients.…”

mentioning

confidence: 99%

Mid-term results and risk factors for functional single ventricles associated with total anomalous pulmonary venous connection

Liu

Yang³

et al. 2020

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Backgroud: Surgical results of functional single ventricle (FSV) patients with totally anomalous pulmonary venous connection (TAPVC), have a poor outcome. We retrospectively analyzed our 10-year surgical clinical experience and risk factors of mortality of these patients. Methods: Between March 2008 and August 2018, 43 consecutive patients with FSV and TAPVC underwent initial surgical palliation and TAPVC repair or not. The median body weight and age were 12 (range 5-44) kg and 32 (range 2-256) months, respectively. Among these cases, there are 19 cases of supracardiac TAPVC, 22 of intracardiac type, and 2 of mixed type. 12 patients need to perform TAPVC repair during initial surgical palliation (supracardiac in 10 and mixed type in 2). Results: Overall survival at 1 and 5 years were 89.5% and 83.3%, respectively. In TAPVC repair group and non-TAPVC repair group, overall survival after the initial surgical palliation were 58.3 and 87.1% at 1 year, 40.0% and 87.1% at 3 years, respectively. Cox univariate analysis detected that preoperative pulmonary vein obstruction (PVO) (p=0.047) and concomitant TAPVC repair (p=0.007) were risk factors for mortality, and multivariable analysis indicated concomitant TAPVC repair as the only factor (p=0.033). Conclusions: The mid-term results of surgical results of FSV associated with TAPVC, especially for patients who need to concomitant TAPVC repair, remain poor. Preoperative PVO is identified as risk factor that increases mortality of these patients.

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Pulmonary Venous Gradients in Healthy Controls and Following the Repair of Total Anomalous Pulmonary Venous Return

et al. 2022

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

Cited by 10 publications

References 17 publications

Total anomalous pulmonary venous drainage repair: the effect of anatomical type and pulmonary vein stenosis on outcomes

Total anomalous pulmonary venous drainage repair: the effect of anatomical type and pulmonary vein stenosis on outcomes

Mid-term results and risk factors for functional single ventricles associated with total anomalous pulmonary venous connection

Pulmonary Venous Gradients in Healthy Controls and Following the Repair of Total Anomalous Pulmonary Venous Return

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