Prenatal identification of the pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia

2009 ◽  
Vol 19 (2) ◽  
pp. 185-191 ◽  
Author(s):  
Anna N. Seale ◽  
Siew Y. Ho ◽  
Elliot A. Shinebourne ◽  
Julene S. Carvalho
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Postmortem Examination ◽  
Aortopulmonary Window ◽  
Cardiac Defect ◽  
Pulmonary Flow ◽  
Collateral Arteries ◽  
Arterial Duct ◽  
Pulmonary Arterial

AbstractObjectiveTo define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia.BackgroundIn this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent. These variables influence the prognosis and management.MethodsWe carried out a retrospective review of cases from a tertiary service for fetal cardiology, identifying all cases of tetralogy of Fallot with pulmonary atresia diagnosed antenatally between January, 1997, and April, 2006. We established pre- and postnatal outcomes, and compared the prenatal diagnosis with postnatal or autopsy findings.ResultsOf 6587 fetuses scanned during this period, 11 were diagnosed as having tetralogy of Fallot with pulmonary atresia and no other cardiac defect. In 5, arterial flow to the lungs was via an arterial duct, and in the other 6, the main identified source of flow was systemic-to-pulmonary collateral arteries. Of the latter 6 pregnancies, 4 were terminated, along with 3 of the 5 with ductal supply. The presence of systemic-to-pulmonary collateral arteries was confirmed at postmortem examination in 3 instances, and in the two delivered neonates, in neither of whom was an infusion of prostaglandin commenced.ConclusionThe patterns of pulmonary flow can be identified prenatally in the setting of tetralogy with pulmonary atresia. Supply through systemic-to-pulmonary collateral arteries impacts on counselling, introducing uncertainty regarding postnatal surgical management.

Tetralogy of Fallot with pulmonary atresia and aortopulmonary window may mimic common arterial trunk

2021 ◽  
pp. 1-5
Author(s):  
Sudesh Prabhu ◽  
Manaswini Keshav ◽  
Prakash Ramachandra ◽  
Vimal Raj ◽  
Colin John ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Pulmonary Atresia ◽  
Aortopulmonary Window ◽  
Collateral Arteries ◽  
Common Arterial Trunk ◽  
Arterial Trunk ◽  
Pulmonary Arterial ◽  
Morphological Differences

Abstract Tetralogy of Fallot with pulmonary atresia is a group of congenital cardiac malformations, which is defined by the absence of luminal continuity between both ventricles and the pulmonary artery, and an interventricular communication. Pulmonary arterial supply in patients with tetralogy of Fallot with pulmonary atresia can be via the arterial duct or from collateral arteries arising directly or indirectly from the aorta (systemic-to-pulmonary artery collaterals), or rarely both. The rarest sources of pulmonary blood flow are aortopulmonary window and fistulous communication with the coronary artery. Herein, we describe an outflow tract malformation, tetralogy of Fallot with pulmonary atresia and aortopulmonary window, which was misdiagnosed as common arterial trunk. We emphasise the morphological differences.

Arterial supply to the lungs in tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis

1992 ◽  
Vol 2 (1) ◽  
pp. 65-72 ◽  
Author(s):  
Siew Yen Ho ◽  
Gualtiero Catani ◽  
Jeong-Wook Seo
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Stenosis ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Arterial Supply ◽  
Collateral Arteries ◽  
Pulmonary Parenchyma ◽  
Different Origins ◽  
Different Sources ◽  
Critical Pulmonary Stenosis

SummaryThe arterial supply to the lungs in cases with tetralogy of Fallot with pulmonary atresia or critical pulmonary stenosis is mostly via the arterial duct or else by collateral arteries arising directly or indirectly from the aorta. Nine anatomical specimens with collateral arteries were studied by blunt dissection to determine the arterial supply to each pulmonary segment. The precise supply varied from case to case but, overall, nearly two-thirds of the pulmonary segments were connected to the central pulmonary arteries. A quarter were supplied exclusively by systemic-to-pulmonary collateral arteries, with the remainder having a dual supply. Coexistence of ductal connections and systemic-to-pulmonary collateral arteries was seen in one case. Anastomoses between vessels of different origins were observed up to the segmental level in eight of the nine cases. In four cases, the anastomoses formed arterial rings. The extent of pulmonary parenchyma supplied by arteries from different sources and the presence of arterial anastomoses in these malformations have implications on the planning of surgical repair.

Systemic-to-pulmonary blood supply in tetralogy of Fallot with pulmonary atresia

2002 ◽  
Vol 12 (4) ◽  
pp. 373-388 ◽  
Author(s):  
Renata N. Rossi ◽  
Alison Hislop ◽  
Robert H. Anderson ◽  
F. Maymone Martins ◽  
Andrew C. Cook
Keyword(s):  
Tetralogy Of Fallot ◽  
Blood Supply ◽  
Pulmonary Atresia ◽  
Arterial Supply ◽  
Pulmonary Vasculature ◽  
Cardiac Malformations ◽  
Collateral Arteries ◽  
Pulmonary Arterial ◽  
Clinical Correlations ◽  
Arterial Anatomy

AbstractTetralogy of Fallot with pulmonary atresia is one of the most challenging congenital cardiac malformations, for the morphologist, cardiologist and surgeon alike. Much of the difficulty in this lesion concerns the nature and development of pulmonary arterial supply, and the manner in which complete segmental supply to the lungs can be successfully restored or maintained. In this review, we discuss the anatomy and nomenclature of the lesion, emphasising the variability that can occur in pulmonary arterial anatomy, particularly in the presence of systemic-to-pulmonary collateral arteries. We speculate on the likely embryologic origins of these connections. Then by means of anatomic-clinical correlations, we emphasise the diagnostic approach to delineating the origin and extent of the pulmonary vasculature.

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.

Tetralogy of Fallot with pulmonary atresia associated with aortopulmonary window and major aortopulmonary collaterals

1995 ◽  
Vol 5 (3) ◽  
pp. 289-290 ◽  
Author(s):  
A. R. Bhagwat ◽  
R. J. Pinto ◽  
Satyavan Sharma
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Atresia ◽  
Postoperative Recovery ◽  
Aortopulmonary Window ◽  
Descending Thoracic Aorta ◽  
Additional Source ◽  
Collateral Arteries ◽  
Arterial Blood ◽  
Aortopulmonary Collaterals ◽  
Major Aortopulmonary Collaterals

SummaryWe report a patient with tetralogy of Fallot and pulmonary atresia in whom the predominant extracardiac supply of arterial blood was via an aortopulmonary window. Major aortopulmonary collateral arteries originating from the descending thoracic aorta provided an additional source of pulmonary blood flow. The patient underwent successful correction and the postoperative recovery was uneventful.

Solitary arterial trunk

1994 ◽  
Vol 4 (1) ◽  
pp. 71-74
Author(s):  
Daniel J. Penny ◽  
Rakesh Dua ◽  
James L. Wilkinson
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Pulmonary Trunk ◽  
Descending Aorta ◽  
Perimembranous Ventricular Septal Defect ◽  
Arterial Trunk ◽  
Pulmonary Arterial

AbstractA heart is described in which there was a solitary arterial trunk that exited from the heart astride a large perimembranous ventricular septal defect, with the pulmonary arterial supply originating entirely from collaterals from the descending aorta. There was no evidence of either hilar pulmonary arteries or an atretic pulmonary trunk. The morphology of the ventricular outlet component, with absence of the outlet septum supports the description of this heart as solitary arterial trunk, rather than tetralogy of Fallot with pulmonary atresia.

Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management

2020 ◽  
pp. 108925322092048
Author(s):  
Madhusudan Ganigara ◽  
Eyal Sagiv ◽  
Sujatha Buddhe ◽  
Aarti Bhat ◽  
Sathish M. Chikkabyrappa
Keyword(s):  
Blood Flow ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Sole Source ◽  
Aortic Insufficiency ◽  
Collateral Arteries ◽  
Antegrade Flow

Tetralogy of Fallot (ToF) with pulmonary atresia (ToF-PA) is a complex congenital heart defect at the extreme end of the spectrum of ToF, with no antegrade flow into the pulmonary arteries. Patients differ with regard to the sources of pulmonary blood flow. In the milder spectrum of disease, there are confluent branch pulmonary arteries fed by ductus arteriosus. In more severe cases, however, the ductus arteriosus is absent, and the sole source of pulmonary blood flow is via major aortopulmonary collateral arteries (MAPCAs). The variability in the origin, size, number, and clinical course of these MAPCAs adds to the complexity of these patients. Currently, the goal of management is to establish pulmonary blood flow from the right ventricle (RV) with RV pressures that are ideally less than half of the systemic pressure to allow for closure of the ventricular septal defect. In the long term, patients with ToF-PA are at higher risk for reinterventions to address pulmonary arterial or RV-pulmonary artery conduit stenosis, progressive aortic root dilation and aortic insufficiency, and late mortality than those with less severe forms of ToF.

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.

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

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.

Sign in / Sign up

Export Citation Format

Share Document