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.

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.

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.

Management of Pulmonary Arterial Supply Dependent on a Coronary Arterial Fistula in a Patient With Tetralogy of Fallot With Pulmonary Atresia

2012 ◽  
Vol 3 (4) ◽  
pp. 499-503 ◽  
Author(s):  
Pankaj Garg ◽  
Sachin Talwar ◽  
Shyam Sundar Kothari ◽  
Palleti Rajashekar ◽  
Gurpreet Singh Gulati ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Atresia ◽  
Arterial Supply ◽  
Pulmonary Arterial

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.

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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