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.

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.

scholarly journals Patent Ductus Arteriosus Stenting for All Ductal-Dependent Cyanotic Infants

2021 ◽  
Vol 14 (3) ◽  
Author(s):  
Kanishka Ratnayaka ◽  
Stephen J. Nageotte ◽  
John W. Moore ◽  
Peter W. Guyon ◽  
Krishna Bhandari ◽  
...  
Keyword(s):  
Blood Flow ◽  
Length Of Stay ◽  
Patent Ductus Arteriosus ◽  
Pulmonary Blood Flow ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Practice Change ◽  
Almost All ◽  
Blalock Taussig Shunt ◽  
Patent Ductus

Background: Ductal-dependent cyanotic newborns require a secure source of pulmonary blood flow. There has been a recent migration to selective ductal (patent ductus arteriosus [PDA]) stenting for some of these children. Universal (nonselective) ductal stenting for all infants with ductal-dependent pulmonary blood flow is controversial. We examine outcomes from a single center with this practice change. Methods: We compare outcomes of all ductal-dependent pulmonary blood flow infants (2013–2020 [January–June]) in the following treatment eras: Era 1 (selective PDA stenting; 2013–2017) or Era 2 (universal PDA stenting; 2018–2020 [January–June]). Results: Eighty-eight patients (Blalock-Taussig shunt, n=41; PDA stent, n=47) met inclusion criteria. In Era 1, most received Blalock-Taussig shunt (62% [41/66]). In Era 2, all received PDA stents (100% [22/22]). There were more females in Era 2, but otherwise no demographic differences between eras. There were no differences in mortality, treatment failures, complications, or reinterventions between eras. Postprocedure length of stay was shorter in Era 2 (8 versus 22 days, P =0.02). There were less surgical revisions for PDA stent patients (2% versus 20%, P =0.02). Postprocedure recovery surrogate end points favored Era 2 and PDA stenting. Additional analysis revealed PDA stent (compared with Blalock-Taussig shunt) patients had shorter post-procedure (10 versus 29 days, P ≤0.001) length of stay and more symmetrical branch pulmonary arteries (0.9 versus 0.7, P =0.001) at subsequent surgery. Conclusions: PDA stenting for almost all ductal dependent cyanotic newborns can be safe and effective and may have lower morbidity than selective PDA stenting.

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.

THE ROLE OF THE PULMONARY VASCULAR BED IN CONGENITAL HEART DISEASE

PEDIATRICS ◽  
1953 ◽  
Vol 12 (3) ◽  
pp. 307-325
Author(s):  
J. FRANCIS DAMMANN ◽  
WILLIAM H. MULLER
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Single Ventricle ◽  
Ductus Arteriosus ◽  
Pulmonary Stenosis ◽  
Pulmonary Arteries ◽  
Signs And Symptoms ◽  
Pulmonary Resistance ◽  
Patient Will ◽  
Systemic Arteries

In the normal human being systemic blood pressure is maintained at a high level by means of the high resistance offered to the flow of blood by the smaller systemic arteries and arterioles. In contrast, pressure in the pulmonary circulation is maintained at a low level because of the relatively large size of the pulmonary vessels. In the fetal and newborn period pulmonary arteries resemble systemic arteries in that they have a small lumen and thick media. In this period pulmonary pressure and resistance are high. As these vessels gradually thin out and enlarge, resistance to pulmonary blood flow falls and consequently pulmonary blood pressure falls. There is evidence to suggest that the fetal state of thick-walled, thin-lumened pulmonary arteries is retained in instances where the two circulations are joined, such as in patients with a single ventricle, large ventricular defect, aortic septal defect or large patent ductus arteriosus. As the patient grows, resistance to pulmonary blood flow may: (1) decrease. If the small pulmonary arteries develop normally and become thin-walled and large-lumened, pulmonary resistance will fall. More and more blood will be shunted into the lungs and the patient will develop the signs and symptoms of cardiac decompensation. (2) remain the same. If the small pulmonary arteries retain their fetal characteristics, pulmonary resistance will remain elevated. A balance between systemic and pulmonary blood flow will be maintained compatible with a relatively normal life. (3) increase. If, because of secondary intimal changes, the pulmonary arteries become thicker-walled and smaller-lumened, pulmonary resistance will increase. Progressively less blood will be shunted to the lungs and finally the shunt will reverse and become predominantly venous arterial. The patient will develop the signs and symptoms of pulmonary stenosis with increasing dyspnea and cyanosis. Data from two groups of patients are presented to illustrate the importance of the pulmonary vascular bed. In the first group, early and severe symptoms of cardiac failure necessitated closure of a large patent ductus arteriosus early in life. The ductus was large, comparable to the size of the aorta, pressures from the aorta and pulmonary artery were similar and a study of the lung biopsies demonstrated that the lumens of the small pulmonary arteries were decreased in size and the media were abnormally thick. In each instance closure of the ductus resulted in a cure. In the second group the two circulations were joined by either a large ventricular defect or a single ventricle. Three patients were in cardiac failure due to excessive pulmonary blood flow. Pulmonary blood flow was decreased by the creation of pulmonary stenosis, the main pulmonary artery being narrowed 60 to 80%. The postoperative course in these three patients illustrates the value of the creation of pulmonary stenosis in instances where the two circulations are joined by a large communication between the ventricles or great vessels.

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.

scholarly journals Echocardiographic findings in children with Tetralogy of Fallot

2015 ◽  
Vol 12 (2) ◽  
pp. 65-68
Author(s):  
Urmila Shakya ◽  
Poonam Sharma ◽  
Manish Shrestha
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Anatomical Variations ◽  
Left Pulmonary Artery ◽  
Double Outlet Right Ventricle ◽  
Echocardiographic Findings

Background and Aims: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in children. The aim of this study was to identify various cardiac defects associated with TOF by transthoracic echocardiography.Methods: All children presenting to Shahid Gangalal National Heart Centre, with the diagnosis of TOF from 2014 to 2015 were included in the study. Echocardiograpic reports of all eighty eight children were reviewed.Results: The mean age of presentation was 3.5 } 3.9 years. Fifty six (64%) children were male and thirty two (36%) were female. Pulmonary artery anomaly was found in nine (10.2%) children which included hypoplastic pulmonary arteries, absent left pulmonary artery, post stenotic and aneurysmal dilatation of pulmonary artery. Pulmonary valve anomalies were seen in 13(14.8%) children which include pulmonary atresia, pin-hole opening of valve and bicuspid and absent pulmonary valve. Double outlet right ventricle, patent ductus arteriosus/major aortico-pulmonary collaterals, atrial septal defect and persistent left superior venacava were present in 12 (13.6%), 21 (23.9%), 22 (22.7%) and nine (10.2%) respectively. Right sided aortic arch was seen in 20 (22.7%).Conclusion: TOF is associated with various anatomical variations which need to be identified to avoid surgical complications. These can be diagnosed by echocardiography which has greatly reduced the need for invasive cardiac catheterization for these patients.Nepalese Heart Journal 2015;12(2):65-68

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