Development of Pulmonary Arteries after a Central End-to-Side Shunt in Patients with Pulmonary Atresia, Ventricular Septal Defect, and Diminutive Pulmonary Arteries

Fang, Minhua; Wang, Huishan; Jin, Yan; Wang, Zengwei; Wang, Zhenlong; Zhang, Chunzhen

doi:10.1055/s-0033-1354247

Cited by 7 publications

(4 citation statements)

References 16 publications

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“…After our initial experience with central shunts with disappointing results with regard to growth and increase of the pulmonary artery size [23] we use these procedure for diminutive pulmonary arteries to promote outgrowth as mentioned by other authors [24,25]. In our opinion rehabilitation of the pulmonary artery as promoted by several authors [15,26,27] may be useful, but still does not preclude further unifocalization in most of our cases.…”

Section: Discussionmentioning

confidence: 85%

Staged Correction of Pulmonary Atresia, Ventricular Septal Defect and Collateral Arteries

Woestijne

Mokhles

Beynum

et al. 2021

Preprint

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Objectives Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCA’s) has a variable anatomy with regard to the pulmonary vasculature, asking for an individualized surgical treatment. A protocol was applied consisting of staged unifocalization and correction. Methods Since 1989 39 consecutive patients were included (median age at first operation 13 months). In selected cases a central aorto-pulmonary shunt was performed as first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Postoperatively and at follow up echocardiographic data were obtained. Results In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, four of them died. One patient is awaiting further correction. Correction was done successfully in 28 patients. Operative mortality was 3% and late mortality 11%. Median follow-up after correction was 19 years. Eleven patients needed homograft replacement. Freedom from conduit replacement was 88%, 73% and 60% at 5, 10 and 15 years respectively. Right ventricular function was reasonable or good in 75 % of the patients. Conclusions After complete unifocalization 30/37 patients (81%) were considered correctable. The main reasons for palliative treatment without correction were pulmonary hypertension and/or inadequate outgrowth of pulmonary arteries. Staged approach of PA, VSD and SPCA’s results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.

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Section: Discussionmentioning

confidence: 85%

Staged Correction of Pulmonary Atresia, Ventricular Septal Defect and Collateral Arteries

Woestijne

Mokhles

Beynum

et al. 2021

Preprint

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“…Based on this concept, an opposite approach aiming at promoting native PAs development has been devised. According to this approach, MAPCAs are not utilized unless they represent the unique pulmonary supply of a lung segment, relying on the genetic commitment of PAs to grow under the proper stimulus of flow [8][9][10][11][12][13][14][15][16][17][18][19][20].…”

Section: Discussionmentioning

confidence: 99%

“…Based on this background alternative approaches aiming at promoting native pulmonary arteries growth have been explored [8][9][10][11][12][13][14][15][16][17][18][19][20]. Published results of different strategies are contrasting in terms of mortality and reintervention rate, depending on the particular institution [7,[21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36].…”

Section: Introductionmentioning

confidence: 99%

Long Term Outcome of Pulmonary Atresia with Major Aorto-Pulmonary Collaterals: Data from a Pooled Analysis

Piazza¹,

Ciuffreda²,

Ferrero³

et al. 2020

JICOA

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Prognosis of Tetralogy of Fallot and Pulmonary atresia with MAPCAs is variable. We aimed to assess the outcome of patients who had undergone repair of TOF and PA with MAPCAs by different institutions and approaches, pooling together data on mortality and reinterventions. Abstracts were screened and full text papers were examined retrieving data on mortality, reinterventions and percentage of complete repair. Variables abstracted from the studies were pooled together and compared among different strategies. 28 papers out of 124 were selected, accounting for 1983 patients. 704 patients had undergone single stage unifocalization, 544 recruitment of PAs, 645 patients multistage and combined approaches. Median follow up was 49 months (36-70). 93 deaths occurred in the first month (5.4 %): 38 in group one (5.8%), 10 in group 2 (2.3%) and 45 in group 3 (8.6%), p<0.0001. At last follow up a total of 305 deaths occurred (16%) and did not differ among the three groups: 0.21 (0.15-0.25), 0.18 (0.07-0.26), 0.32 (0,13-0.38) deaths/100patients year, respectively p=0.43. 465 out of 969 patients, underwent at least one re-intervention (47.9%). Reintervention rate was similar among the three subsets and inversely correlated with the volume of institution (r= -0.45, p= 0.04). Although strategies of pulmonary atresia with MAPCAs repair are based on contrasting philosophies and extreme anatomic varibility, pooling together data from different studies, outcomes do not differ significantly and are characterized by a high rate of reinterventions. The latter appears to be significantly influenced by the volume of patients.

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“…While some reported remarkable outcomes using the early complete unifocalization strategy, others recommended the rehabilitation strategy to promote development of native pulmonary arteries (1,2). Currently both are used in many centers with favorable results (3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

Outcomes After Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: A Tailored Approach in a Developing Setting

Zou

Cui

et al. 2021

Front. Cardiovasc. Med.

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Objectives: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is complex and diverse that has led to a variety of treatment strategies. Experience has been largely obtained in the advanced countries. The clinical diversity is greater in China. We evaluated our surgical approaches and outcomes of these patients.Methods: We reviewed 127 patients undergoing varied surgeries in our center in 2010–2019.Results: Thirty patients underwent single-stage complete repair by unifocalizing MAPCAs and VSD closure (aged 3.9–131.4 months, median 22) with 3 (10%) early deaths. Ninety-seven underwent the first-stage rehabilitation strategy including systemic-to-pulmonary shunt in 29 (aged 0.5–144 month, median 8), and palliative RV-PA conduit in 68 (aged 2.2–209.6 months, median 14) with 5 (5.2%) early deaths. Eight-one patients (63.8%) eventually achieved complete repair with a median right/left ventricular (RV/LV) pressure ratio of 0.7 (ranged 0.4–1.0). Fourteen patients (11.0%) accepted palliation as final destination. Survival for the entire cohort was 89.5, 85.2, and 76.1% at 1, 5, and 10 years, respectively. Survival for those undergoing complete repair was 88.2 and 76.6% at 1 and 5 year, respectively. RV/LV pressure ratio ≥0.8 was risk factor for mortality (HR10.3, p = 0.003).Conclusions: Our cohort, the largest from China, had distinctive clinical features with substantially wider age range and higher RV/LV pressure ratio. Using the combined approaches tailored to individual patients, complete repair was achieved in 64% of patients. The early and intermediate outcomes are acceptable compared to many of the previous reports.

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Development of Pulmonary Arteries after a Central End-to-Side Shunt in Patients with Pulmonary Atresia, Ventricular Septal Defect, and Diminutive Pulmonary Arteries

Cited by 7 publications

References 16 publications

Staged Correction of Pulmonary Atresia, Ventricular Septal Defect and Collateral Arteries

Staged Correction of Pulmonary Atresia, Ventricular Septal Defect and Collateral Arteries

Long Term Outcome of Pulmonary Atresia with Major Aorto-Pulmonary Collaterals: Data from a Pooled Analysis

Outcomes After Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: A Tailored Approach in a Developing Setting

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