Pulmonary atresia with ventricular septal defect: multicenter analysis of prenatal diagnosis cases

2017 ◽  
Author(s):  
M.V. Medvedev , M.V. Kubrina , O.L. Galkina et all
Keyword(s):  
Prenatal Diagnosis ◽  
Ventricular Septal Defect ◽  
Aortic Arch ◽  
Gestational Age ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Mean Value ◽  
Collateral Arteries

A retrospective analysis of 12 cases of prenatal diagnosed of pulmonary atresia with ventricular septum defect (PA-VSD) is presented. In the study of the fetal four chambers view cardiomegaly was detected in 2 (16.7 %) cases. The axis of the heart changed in 8 (66.7 %) cases. Ventricular septal defect (VSD) and overriding dilated ascending aorta were identified in all cases. Central pulmonary arteries were identified in 12 (100%) cases. In 7 (58.3 %) cases the dimensions of the pulmonary arteries were at least 2SD below the mean value for gestational age in the remaining 5 (41.7 %) cases were very narrow. Pulmonary blood supply was prenatally identified as ductus arteriosus (DA) in 8 (66.7 %) and major aortopulmonary collateral arteries (MARSAs) in 3 (25 %) and mixed (DA + MARSAs) in 1 (8.3 %). Fetal hydrops is detected in 2 (16.7 %) cases. The left aortic arch was in 9 (75 %) cases and right aortic arch — 3 (25 %). The average gestational age at prenatal diagnosis was 19.6 (13–23) weeks of gestation. Early diagnosis is represented by one case in 13 weeks of gestation. Еxtracardiac defects were registered in 3 (25 %) cases. Outcomes in fetuses with PA-VSD: termination of pregnancy — 10 (83.4 %), fetal death — 1 (8.3 %), only 1 (8.3 %) survivor

Accuracy of Fetal Echocardiography in the Differential Diagnosis between Truncus Arteriosus and Pulmonary Atresia with Ventricular Septal Defect

2015 ◽  
Vol 39 (2) ◽  
pp. 90-99 ◽  
Author(s):  
Olga Gómez ◽  
Iris Soveral ◽  
Mar Bennasar ◽  
Fatima Crispi ◽  
Narcis Masoller ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Fetal Echocardiography ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Image Correlation ◽  
Truncus Arteriosus ◽  
Chromosomal Anomalies ◽  
Collateral Arteries ◽  
Arterial Trunk

Objectives: To report on the accuracy of fetal echocardiography in the distinction between truncus arteriosus communis (CAT) and pulmonary atresia with ventricular septal defect (PA-VSD) and to describe the association with extracardiac and chromosomal anomalies. Methods: This was a retrospective study on 31 fetuses with a single arterial trunk overriding a VSD with a nonidentifiable right ventricle outflow tract with anterograde flow. Data on the type of cardiac defect, gestational age, characteristics of the arterial trunk valve, presence of additional vascular, chromosomal and extracardiac abnormalities and postnatal outcome were obtained. Misdiagnosed cases were reevaluated by four-dimensional spatiotemporal image correlation (4D-STIC) echocardiography. Results: The overall diagnostic accuracy was 81% and increased to 93.5% with 4D-STIC. Chromosomal and extracardiac anomalies were detected in 40 and 27%, respectively. In the PA-VSD group, patent ductus arteriosus and major aortopulmonary collateral arteries (MAPCAs) were present in 70 and 50% of the cases, respectively, coexisting in 1 of 5 cases. MAPCAs were significantly associated with a right aortic arch and with a 22q11 microdeletion in 50% of cases. Conclusions: A prenatal distinction between CAT and PA-VSD can currently be achieved in most cases. MAPCAs should be actively searched for when PA-VSD is suspected, as they are associated with a higher risk of 22q11 microdeletion and potentially complicate postnatal treatment.

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

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.

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

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Anu Kaskinen ◽  
Juha-Matti Happonen ◽  
Ilkka P Mattila ◽  
Olli M Pitkänen
Keyword(s):  
Surgical Treatment ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Palliative Surgery ◽  
Total Study Population ◽  
Collateral Arteries

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.

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

2017 ◽  
Vol 8 (5) ◽  
pp. 564-569 ◽  
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.

Deposition of collagen in the alveolar wall of lungs from patients with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries—an ultrastructural study

1994 ◽  
Vol 4 (3) ◽  
pp. 277-284
Author(s):  
Hikaru Matsuda ◽  
Tohru Kuratani ◽  
Yasuhisa Shimazaki ◽  
Keishi Kadoba ◽  
Jyunjiro Kobayashi ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Ultrastructural Study ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Alveolar Wall ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

AbstractAbstract Lung biopsies were taken at surgery from five patients (age 2–13, average 7.6 years) with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. The biopsies were then processed for ultrastructural study, comparing paired samples taken, on the one hand, from a segment connected to central pulmonary arteries and, on the other hand, from a segment supplied directly by collateral arteries. Specimens from patients with isolated ventricular septal defect, and from those without cardiac disease, were used as controls. In the patients with tetralogy of Fallot and pulmonary atresia, all biopsies taken from segments supplied by the major collateral arteries showed marked deposition of collagen in the alveolar wall, with an increase in the thickness of the basement membrane (3.6±1.2 µm, mean±SD) greater than seen in those taken from segments connected to central pulmonary arteries (0.9±0.6, p<0.05). The proportional fibrosis of the alveolar interstitial space was also significantly greater in the biopsies from the segments supplied by collateral arteries (30±9%) compared to those from segments fed by central pulmonary arteries (15±8%, p<0.05). No significant differences were found in these indices between the biopsies from segments connected to the central pulmonary arteries in patients with tetralogy of Fallot and pulmonary atresia and those from patients with isolated ventricular septal defect or normal controls. There was no apparent relation to the pulmonary arterial pressure in these findings. The results suggest that the pulmonary segments fed directly by major aortopulmonary collateral arteries in patients with tetralogy of Fallot and pulmonary atresia seem likely to be afflicted by alveolar wall fibrosis, although the etiology and clinical implications of this finding remain unclear.

Hybrid procedure of right ventricle outflow tract stenting in small infants with pulmonary atresia and ventricular septal defect: early and mid-term results from a single centre

2019 ◽  
Vol 29 (3) ◽  
pp. 375-379 ◽  
Author(s):  
Sara Bondanza ◽  
Maria Grazia Calevo ◽  
Maria Elena Derchi ◽  
Francesco Santoro ◽  
Maurizio Marasini
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Hybrid Procedure ◽  
Outflow Tract ◽  
Congenital Defects ◽  
Hybrid Technique ◽  
Collateral Arteries

AbstractIntroductionPulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Percutaneous techniques such as radiofrequency perforation can be challenging. The hybrid technique consists of perventricular stenting of the right ventricle outflow tract through medial sternotomy, to restore native pulmonary flow.MethodsWe retrospectively reviewed the cardiovascular database of our centre in order to analyse our experience in hybrid procedure. We detected six patients who underwent hybrid first approach between November 2007 and December 2015. We report our early results and mid-term outcomes.ResultsMedian age at the procedure was 26 days, median weight was 3150 g, and median Nakata index was 52 mm2/m2. All procedures were successful except for one: this patient underwent a surgical shunt. No immediate and early deaths or major complications occurred and oxygen saturation levels increased in all the patients. Patients were followed up for a period of 12–103 months, and four of them underwent a procedure of unifocalisation at the mean age of 12.5 months.ConclusionsWe reported data from the largest series of patients who underwent this hybrid procedure. Our experience demonstrated encouraging results to expand the use of this approach to bridge high-risk patients with diminutive pulmonary arteries to a second step of surgical repair.

Use of pulmonary arterial unifocalization into cryopreserved homograft pulmonary arterial bifurcation grafts to facilitate complete correction of pulmonary atresia with ventricular septal defect, nonconfluent (absent) central pulmonary arteries and multiple major aortopulmonary collateral arteries

1995 ◽  
Vol 5 (3) ◽  
pp. 217-224 ◽  
Author(s):  
Richard A. Hopkins ◽  
Deborah A. Imperato ◽  
John T. Cockerham ◽  
Stephen R. Shapiro
Keyword(s):  
Ventricular Septal Defect ◽  
Vascular Disease ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Pulmonary Vascular Disease ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Pulmonary Arterial ◽  
Aortopulmonary Collateral

AbstractThree children with pulmonary atresia and ventricular septal defect with absent central pulmonary arteries underwent successful complete correction following preliminary staging procedures for unifocalization and deletion of multiple major aortopulmonary collateral arteries. The unifocalization procedures were accomplished using valveless pulmonary arterial cryopreserved allografts. This technique was found to facilitate and simplify the final repair. All patients survived the complete correction. The patients have been followed for 1½-4 years following the final surgery. One child died two years after repair from pneumonia complicated by asthma. One patient appears to have progression of pulmonary vascular disease despite the complete repair, and one patient continues to do well both symptomatically and hemodynamically. The results suggest that this method of unifocalization solves many of the problems inherent to more classical techniques, but the overall prognosis of patients in this diagnostic entity has yet to be completely defined.

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