Long-term Outcomes of Staged Complete Repair of Pulmonary Atresia With Ventricular Septal Defect

Macalister, Samuel J.; Buratto, Edward; Naimo, Phillip S.; Ye, Xin Tao; Fulkoski, Nicholas; Weintraub, Robert G.; Konstantinov, Igor E.

doi:10.1016/j.athoracsur.2022.09.022

Cited by 4 publications

(3 citation statements)

References 16 publications

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“…However, proportional patients did not strictly follow the schedule because of misconceptions about PA/VSD disease, the long distance from home to the hospital, and the family’s poor financial situation, which may have compromised the likelihood of complete repair. Thirdly, the mean period of follow-up was 70.3 months in the RV-PA connection group and 61.1 months in the systemic-to-pulmonary shunt group, which is relatively short compared to some previous studies [ 15 , 16 ]. Of note, the complete repair rate in the PA/VSD/MAPCAs patients was particularly lower than that in the previous studies, compared with PA/VSD patients without MAPCAs.…”

Section: Discussionmentioning

confidence: 74%

“…The previous major studies on PA/VSD patients who initially underwent rehabilitative surgery were summarized in Table 5 (Ref. [ 3 , 6 , 7 , 8 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 ]). The complete repair rate in either the overall PA/VSD cohort or PA/VSD sub-cohort was slightly lower than the previous investigation [ 6 , 14 , 22 ], which is somewhat attributed to the following reasons.…”

Section: Discussionmentioning

confidence: 99%

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Outcomes of Rehabilitation Strategies for Pulmonary Atresia with Ventricular Septal Defect: A Single Center’s Experience

Zhang,

Ma,

Liu

et al. 2024

Rev. Cardiovasc. Med.

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Background: Both systemic-to-pulmonary shunt and right ventricle-pulmonary artery (RV-PA) connection are extensively applied to initially rehabilitate the pulmonary artery in pulmonary atresia with the ventricle septal defect (PA/VSD). However, which of these options is the most ideal for promoting pulmonary artery development and improving outcomes remains controversial. Methods: A total of 109 PA/VSD patients undergoing initial rehabilitative surgery at Guangdong Provincial People’s Hospital from 2010 to 2020 were enrolled in this study. A series of clinical data were collected to compare the perioperative and postoperative outcomes between systemic-to-pulmonary and RV-PA connection. Results: The mean duration of follow-up was 61.1 months in the systemic-to-pulmonary shunt group and 70.3 months in the RV-PA connection group ( p 0.05). The RV-PA connection technique resulted in a significantly higher , lower red blood cells (RBC), lower hemoglobin, and lower hematocrit (Hct) ( p 0.05). The cumulative incidence curve estimated a cumulative complete repair rate of 56 7% after 5 years in the RV-PA connection group, significantly higher than 36 7% after 5 years in the systemic-to-pulmonary shunt group ( p 0.05). The Kaplan-Meier curve revealed a similar estimated survival rate between the two groups ( p = 0.73). The RV-PA connection was identified as an independent predictor for complete repair in the multivariable analysis (HR = 2.348, 95% CI = 1.131–4.873). Conclusions: The RV-PA connection is a more ideal initial rehabilitative technique than systemic-to-pulmonary shunt in treating PA/VSD as a consequence of comparable probability of survival but improved definitive complete repair rate.

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Section: Discussionmentioning

confidence: 74%

Section: Discussionmentioning

confidence: 99%

Outcomes of Rehabilitation Strategies for Pulmonary Atresia with Ventricular Septal Defect: A Single Center’s Experience

Zhang,

Ma,

Liu

et al. 2024

Rev. Cardiovasc. Med.

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“…The follow-up mortality was also comparable between the younger group (10%) and the elder group (9.8%), which was consistent with the previous investigation ( 17 – 20 ). Macalister et al reported a mortality rate ranging from approximately 6%–20% during a median follow-up period of 10.5 years ( 21 ). In the younger group, a higher incidence of delayed sternal closure and longer lengths of hospital stay were observed.…”

Section: Discussionmentioning

confidence: 99%

Long-term outcomes of pulmonary atresia with ventricular septal defect by different initial rehabilitative surgical age

Ma,

Tan,

Zhang

et al. 2023

Front. Cardiovasc. Med.

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BackgroundThere is a lack of evidence guiding the surgical timing selection in pulmonary atresia with ventricular septal defect. This study aims to compare the long-term outcomes of different initial rehabilitative surgical ages in patients with pulmonary atresia with ventricular septal defect (PAVSD).MethodsFrom January 2011 to December 2020, a total of 101 PAVSD patients undergoing the initial rehabilitative surgery at our center were retrospectively reviewed. Receiver-operator characteristics curve analysis was used to identify the cutoff age of 6.4 months and therefore to classify the patients into two groups. Competing risk models were used to identify risk factors associated with complete repair. The probability of survival and complete repair were compared between the two groups using the Kaplan-Meier curve and cumulative incidence curve, respectively.ResultsThe median duration of follow-up was 72.76 months. There were similar ΔMcGoon ratio and ΔNakata index between the two groups. Multivariate analysis showed that age ≤6.4 months (hazard ratio (HR) = 2.728; 95% confidence interval (CI):1.122–6.637; p = 0.027) and right ventricle-to-pulmonary artery connection (HR = 4.196; 95% CI = 1.782–9.883; p = 0.001) were associated with increased probability of complete repair. The cumulative incidence curve showed that the estimated complete repair rates were 64% ± 8% after 3 years and 69% ± 8%% after 5 years in the younger group, significantly higher than 28% ± 6% after 3 years and 33% ± 6% after 5 years in the elder group (p < 0.001). There was no significant difference regarding the estimated survival rate between the two groups.ConclusionCompared with those undergoing the initial rehabilitative surgery at the age >6.4 months, PAVSD patients at the age ≤6.4 months had an equal pulmonary vasculature development, a similar probability of survival but an improved probability of complete repair.

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The Debate Goes On…

Bateson¹,

Louis²

2023

The Annals of Thoracic Surgery

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Long-term Outcomes of Staged Complete Repair of Pulmonary Atresia With Ventricular Septal Defect

Cited by 4 publications

References 16 publications

Outcomes of Rehabilitation Strategies for Pulmonary Atresia with Ventricular Septal Defect: A Single Center’s Experience

Outcomes of Rehabilitation Strategies for Pulmonary Atresia with Ventricular Septal Defect: A Single Center’s Experience

Long-term outcomes of pulmonary atresia with ventricular septal defect by different initial rehabilitative surgical age

The Debate Goes On…

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