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.

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.

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.

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.

Dimensions of the pulmonary arteries in rat fetuses with congenital heart disease

1994 ◽  
Vol 4 (3) ◽  
pp. 291-297
Author(s):  
Kazuo Momma ◽  
Masahiko Ando ◽  
Masaaki Yoshigi
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Arteries ◽  
Common Arterial Trunk ◽  
Arterial Trunk ◽  
Right Pulmonary Artery ◽  
The Right

AbstractSo as to understand better the pathogenesis of enlargement or hypoplasia of pulmonary arteries in congenital heart disease, we studied the dimensions of the pulmonary arteries in 74 fetuses with congenital heart disease induced by administration of bis-diamine to pregnant rats. The congenital malformations induced included 12 with large ventricular septal defect, 17 with tetralogy of Fallot, 15 with tetralogy together with severe valvar pulmonary stenosis and absence of the arterial duct, 17 with tetralogy with absent pulmonary valve syndrome and absence of the arterial duct, and 13 with common arterial trunk with a confluent segment supplying the pulmonary arteries. For comparison, 16 fetuses of the same gestational age with normal hearts were studied. After rapid whole-body freezing on the 21st day of gestation, the fetuses were studied by means of serial cross-sectional photographs of the thorax. The diameter of the right pulmonary artery of the fetus was of comparable dimensions in the normal hearts (480±10 µm) (mean±SEM), those with ventricular septal defects (470±10 µm), common arterial trunk (520±20 µm), and tetralogy of Fallot (500±10 µm). These findings suggest that the commonly observed enlargement of the right pulmonary artery in patients with ventricular septal defect and common arterial trunk, and hypoplasia of the right pulmonary artery in tetralogy of Fallot, occur postnatally in response to abnormal postnatal pulmonary blood flow.

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.

Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia

2021 ◽  
Vol 25 (3) ◽  
pp. 218-228 ◽  
Author(s):  
Casey A. Quinlan ◽  
Gregory J. Latham ◽  
Denise Joffe ◽  
Faith J. Ross
Keyword(s):  
Blood Flow ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Anesthetic Management ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Systemic Circulation ◽  
Arterial System ◽  
Pulmonary Vasculature ◽  
Pulmonary Arterial

Tetralogy of Fallot with pulmonary atresia (ToF-PA) is a rare diagnosis that includes an extraordinarily heterogeneous group of complex anatomical findings with significant implications for physiology and prognosis. In addition to the classic findings of ToF, this particular diagnosis is characterized by complete failure of forward flow from the right ventricle to the pulmonary arterial system. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, most frequently via a patent ductus arteriosus, major aortopulmonary collaterals, or a combination of the two. The pathophysiology of ToF-PA is largely attributable to the abnormalities of the pulmonary vasculature. Ultimately, these patients require operative intervention to create a reliable, controlled source of pulmonary blood flow and ideally complete intracardiac repair. Even after operative correction, these patients remain at risk for pulmonary arterial stenoses and pulmonary hypertension. Although there have been significant advances in surgical and interventional management of ToF-PA leading to dramatic improvements in survival and long-term functional status, there is ongoing debate about the optimal management strategy given the risk of development of irreversible abnormalities of the pulmonary vasculature and the morbidity and mortality associated with sometimes multiple, complex operative interventions often occurring early in infancy. This review will discuss the findings in patients with ToF-PA with a focus on the perioperative and anesthetic management and will highlight challenges faced by the anesthesiologist in caring for these patients.

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