Abstract 12635: Nationwide Long-term Follow-up of Pulmonary Atresia With Ventricular Septal Defect

Introduction: The naturally poor survival of pulmonary atresia with ventricular septal defect (PA+VSD) has improved due to evolved perioperative and surgical treatment. Studies including PA+VSD patients, both with and without major aortopulmonary collateral arteries (MAPCAs), with extensive follow-up are scarce. This nationwide study aimed to investigate survival and surgical treatment in PA+VSD patients with and without MAPCAs. Methods: Study comprised 109 PA+VSD patients born in Finland between 1970 and 2007. We reviewed retrospectively medical records and operative reports through December 2011, as well as first available angiograms and preoperative angiograms prior to repair attempt. Results: The median follow-up time for the total study population, including also patients who died during the follow-up, was 11.4 years (IQR 0.8 - 21.1). The incidence of PA+VSD, which could be determined reliably from 1995 to 2007, was 6.1 per 100 000 live births. Although the patients with (n = 43) or without MAPCAs (n = 66) showed no difference in survival (p = 0.74), the patients without MAPCAs had better probability to achieve repair (64% vs. 28%, p < 0.0001). The bigger size of true central pulmonary arteries assessed by McGoon index at first angiogram [HR 0.66 (CI95% 0.49 - 0.88) per 0.5 McGoon index units, p = 0.006] and achievement of repair [HR 0.07 (CI95% 0.03 - 0.17), p < 0.0001] improved the overall survival. After successful repair survival was 93% at 1 year and 91% from 2 years on. Palliated patients, instead, had survival at 1, 5, 10, and 20 years of age of 55%, 42%, 34%, and 20% respectively. However, patients with right ventricle - pulmonary artery connection and septal fenestration had better survival than rest of the palliated patients (p = 0.001). Palliation with a systemic-pulmonary artery shunt increased McGoon index by 41% (p < 0.0001). Conclusions: The patients with MAPCAs had higher risk to remain palliated than patients without, although their survival was similar. Survival of PA+VSD was influenced by the initial size of true central pulmonary arteries and whether repair was achieved. Although palliative procedures may not improve the final outcome of PA+VSD, palliative surgery may have a role in its treatment.

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

10.22541/au.163275129.98299783/v1 ◽

2021 ◽

Author(s):

Pieter van de Woestijne ◽

M. Mokhles ◽

Ingrid van Beynum ◽

Peter de Jong ◽

Jeroen Wilschut ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Septal Defect ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Pulmonary Vasculature ◽

Collateral Arteries ◽

Staged Approach ◽

The Right ◽

Rv Function

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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Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135117717266 ◽

2017 ◽

Vol 8 (5) ◽

pp. 564-569 ◽

Cited By ~ 4

Author(s):

Edon J. Rabinowitz ◽

Shilpi Epstein ◽

Nina Kohn ◽

David B. Meyer

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Right Ventricle ◽

Septal Defect ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Arterial Tree ◽

Collateral Arteries ◽

Complete Repair ◽

Pulmonary Arterial

Background: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. Methods: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. Results: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. Conclusion: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

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Morphologic and hemodynamic results of staged repair of pulmonary atresia with ventricular septal defect in the presence of hypoplastic pulmonary arteries and systemic-to-pulmonary collateral arteries

Cardiology in the Young ◽

10.1017/s1047951100001347 ◽

1993 ◽

Vol 3 (2) ◽

pp. 98-103

Author(s):

James L. Wilkinson ◽

Yin Ming Ng ◽

Krishnan S. Iyer ◽

Roger B. B. Mee

Keyword(s):

Ventricular Septal Defect ◽

Septal Defect ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Ventricular Pressure ◽

Pulmonary Vascular Disease ◽

Time Interval ◽

Collateral Arteries ◽

Pulmonary Arterial

AbstractFollow-up cardiac catheterization and angiography was performed in 23 patients after complete, staged repair of pulmonary atresia with ventricular septal defect and multiple systemic-to-pulmonary collateral arteries. Time interval from repair varied from 0.2 to 89 months (mean 26 months). Major residual anomalies of arborization of the pulmonary arteries, with or without pulmonary vascular disease, were present in six patients, two of whom had die patch on the ventricular septal defect perforated because of systemic or suprasystemic right ventricular pressure in the early postoperative period after repair. Both of these children later died, while the other four patients were found to have moderately to severely elevated ventricular pressure ratios and pulmonary arterial pressure measurements, though they are all alive at follow-up intervals from 20 months to 6.8 years. The remaining 17 patients all had hemodynamically satisfactory or good results, with pulmonary arterial mean pressures ranging from 8 to 30 mm Hg (mean 22 mm Hg) and pressure ratios between the ventricles from 0.24 to 0.75 (mean 0.43)—the higher ratios generally reflecting gradients across the conduit valve. Angiography demonstrated that the hypoplastic pulmonary arteries increased in size to normal dimensions, during staging, in most patients, though abnormalities of arborization persisted in several. Analysis of the data from the entire cohort of 58 patients, who were accepted for staging towards repair, showed a significant association between early age at entry to the program and likelihood of complete repair being achievable.

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Prenatal ultrasound diagnosis of pulmonary atresia with a ventricular septal defect and major aortopulmonary collateral arteries (MARCA)

10.21516/2413-1458-2020-19-4-370-375 ◽

2020 ◽

Author(s):

V.V. Ezhova

Keyword(s):

Prenatal Diagnosis ◽

Surgical Treatment ◽

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Septal Defect ◽

Pulmonary Atresia ◽

Type B ◽

Major Aortopulmonary Collateral Arteries ◽

Collateral Arteries ◽

Aortopulmonary Collateral

The case of prenatal diagnosis of pulmonary artery atresia with a ventricular septal defect and major aortopulmonary collateral arteries at 34 weeks is presented. Atresia of the pulmonary artery with a ventricular septal defect and major aortopulmonary collateral arteries (type B) was diagnosed. At 39 weeks birth occurred. After the examination, the prenatal diagnosis was confirmed. The condition of the newborn was rated as satisfactory. On the 7th day the child was discharged under the supervision of a cardiologist. Surgical treatment is planned. The article discusses the issues of differential prenatal diagnosis, modern possibilities of operative treatment and prognosis.

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Outcomes of management of major aortopulmonary collaterals for pulmonary atresia and ventricular septal defect

Cardiology in the Young ◽

10.1017/s1047951120004047 ◽

2020 ◽

pp. 1-9

Author(s):

Dong Zhao ◽

Keming Yang ◽

Wei Feng ◽

Shoujun Li ◽

Jun Yan ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Septal Defect ◽

Pulmonary Atresia ◽

Survival Curves ◽

Major Aortopulmonary Collateral Arteries ◽

Palliative Procedure ◽

Collateral Arteries ◽

Kaplan Meier ◽

Aortopulmonary Collateral

Abstract Objective: This study aimed to investigate the association between long-term survival and different management of major aortopulmonary collateral arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Methods: From November, 2009 to October, 2018, a total of 98 consecutive patients with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries, and hypoplastic pulmonary arteries treated with modified Blalock–Taussig shunt or right ventricle–pulmonary artery connection were included. Fifty-five patients who received occlusion or ligation of major aortopulmonary collateral arteries during or after palliative procedure were occlusion group, and the other 43 patients were no occlusion group. The early and late outcomes were compared. Results: The mean duration of follow-up was 30.9 months in no occlusion group and 49.8 months in the occlusion group (p < 0.001). Multivariate analysis showed that only no occlusion of major aortopulmonary collateral arteries was predictive of total mortality (Hazard Ratio: 4.42, 95% CI: 1.27 to 15.42, p = 0.02). The Kaplan–Meier survival curves confirmed that patients without occlusion of major aortopulmonary collateral arteries demonstrated worse survival as compared with the occlusion group (p = 0.013). The Kaplan–Meier survival curves of patients who underwent different palliative procedures showed no differences. Conclusions: For patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries when a primary repair is not feasible, those without occlusion of major aortopulmonary collateral arteries have a higher risk of death following an initial palliative procedure compared with patients who underwent occlusion of major aortopulmonary collateral arteries. The occlusion of major aortopulmonary collateral arteries is not associated with a higher rate of complete repair or better improvement of pulmonary artery growth.

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Double aortic arch: an elusive diagnosis in a child with CHD

Cardiology in the Young ◽

10.1017/s1047951121004856 ◽

2021 ◽

pp. 1-3

Author(s):

Mariana Lemos ◽

Miguel Fogaça da Mata ◽

Ana Coutinho Santos

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Aortic Arch ◽

Ct Angiography ◽

Septal Defect ◽

Vascular Ring ◽

Pulmonary Atresia ◽

Double Aortic Arch ◽

Shunt Surgery

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

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Stenting of modified Blalock–Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytz201 ◽

2019 ◽

Vol 3 (4) ◽

pp. 1-4

Author(s):

Julia Illner ◽

Holger Reinecke ◽

Helmut Baumgartner ◽

Gerrit Kaleschke

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Congenital Heart ◽

Septal Defect ◽

Pulmonary Atresia ◽

Complex Congenital Heart Disease ◽

Bare Metal

Abstract Background Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. Case summary We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock–Taussig (BT) shunt and a severely stenosed central shunt (Waterston–Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. Discussion This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.

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Prenatal Diagnosis and Postnatal Outcome of Fetuses with Pulmonary Atresia and Ventricular Septal Defect

Ultraschall in der Medizin - European Journal of Ultrasound ◽

10.1055/a-0770-2832 ◽

2019 ◽

Vol 41 (05) ◽

pp. 514-525

Author(s):

Ingo Gottschalk ◽

Brigitte Strizek ◽

Christel Jehle ◽

Rüdiger Stressig ◽

Ulrike Herberg ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Septal Defect ◽

Pulmonary Atresia ◽

Intrauterine Fetal Death ◽

Chromosomal Anomalies ◽

Cardiac Anomalies ◽

One Stage ◽

Collateral Arteries ◽

Postnatal Outcome

AbstractPurpose To assess the intrauterine course, associated conditions and postnatal outcome of fetuses with pulmonary atresia with ventricular septal defect (PAVSD).Methods All cases of PAVSD diagnosed prenatally over a period of 10 years with a minimum follow-up of 6.5 years were retrospectively collected in 3 tertiary referral centers.Results 50 cases of PAVSD were diagnosed prenatally. 44.0 % of fetuses had isolated PAVSD, 4.0 % had associated cardiac anomalies, 10.0 % had extra-cardiac anomalies, 38.0 % had chromosomal anomalies, 4.0 % had non-chromosomal syndromes. Among the 32 liveborn children, 56.3 % had reverse flow in the patent arterial duct, 25.0 % had major aortopulmonary collateral arteries (MAPCAs) with ductal agenesis and 18.7 % had a double supply. 17 pregnancies were terminated (34.0 %), there was 1 intrauterine fetal death (2.0 %), 1 neonatal death (2.0 %), and 6 deaths (12.0 %) in infancy. 25 of 30 (83.3 %) liveborn children with an intention to treat were alive at the latest follow-up. The mean follow-up among survivors was 10.0 years (range 6.5–15.1). 56.0 % of infants underwent staged repair, 44.0 % had one-stage complete repair. After exclusion of infants with additional chromosomal or syndromal anomalies, 88.9 % were healthy, and 11.1 % had mild limitations. The presence of MAPCAs did not differ significantly between survivors and non-survivors (p = 0.360), between one-stage or staged repair (p = 0.656) and healthy and impaired infants (p = 0.319).Conclusion The prognosis in cases without chromosomal or syndromal anomalies is good. MAPCAs did not influence prognosis or postoperative health. The incidence of repeat interventions due to recurrent stenoses is significantly higher after staged compared with single-stage repair.

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