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.

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.

The pulmonary vasculature in a neonatal porcine model with increased pulmonary blood flow and pressure

2001 ◽  
Vol 11 (4) ◽  
pp. 420-430 ◽  
Author(s):  
Elisabeth V. Stenbøg ◽  
Daniel A. Steinbrüchel ◽  
Anne Bloch Thomsen ◽  
Ulrik Baandrup ◽  
Lene Heickendorff ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Vascular Disease ◽  
Pulmonary Blood Flow ◽  
Left Pulmonary Artery ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Systolic Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Circulating Levels

Introduction: Hypertension and hyperperfusion of the pulmonary vascular bed in the setting of congenital cardiac malformations may lead to progressive pulmonary vascular disease. To improve the understanding of the basic mechanisms of this disease, there is a need for clinically relevant animal models which reflect the disease process. Material and Results: We randomly allocated 45 newborn pigs, at the age of 48 hrs, to groups in which there was either construction of a 3 mm central aorto-pulmonary shunt, undertaken in 9, or ligation of the left pulmonary artery, achieved in 13. Controls included sham operations in 13, or no operations in 10 pigs. Follow-up was continued for three months. The interventions were compatible with survival in most pigs. The shunts resulted in an acute 85% increase in systolic pulmonary arterial pressure, and a more than twofold increase in pulmonary blood flow. By three months of age, nearly all shunts had closed spontaneously, and haemodynamics were normal. Ligation of the left pulmonary artery resulted in a normal total pulmonary blood flow, despite only the right lung being perfused, and a 33% increase in systolic pulmonary arterial pressure. These haemodynamic changes were maintained throughout the period of study. In both groups, histomorphometry revealed markedly increased muscularity of the intra-acinar pulmonary arteries. Circulating levels of endothelin were normal in the shunted animals, and elevated in those with ligation of the left pulmonary artery. Conclusion: In neonatal porcine models of pulmonary vascular disease, created by construction of 3 mm central aorto-pulmonary shunts and ligation of one pulmonary artery, we observed histopathological changes of the pulmonary vasculature similar to early hypertensive pulmonary vascular disease in humans. Elevated circulating levels of endothelin were associated with abnormal haemodynamics rather than abnormal pathology. These findings could be valuable for future studies on the pathogenesis of hypertensive pulmonary vascular disease associated with congenital cardiac malformations.

Abstract 14860: Echocardiographic Assessment of Shunt Fraction in Complex Cyanotic Heart Diseases Palliated With Aortopulmonary Shunts

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Joan K Lee ◽  
briana olson ◽  
Neal Jorgensen ◽  
Matthew D Files
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Heart Diseases ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Ventricular Volume ◽  
Systemic Circulation ◽  
Repeated Measurements ◽  
Observer Agreement ◽  
Shunt Fraction

Introduction: Neonates with complex heart defects with inadequate pulmonary blood flow require aortopulmonary shunts (APS) either as surgical-placed modified Blalock-Taussig shunt or a catheter-based stent implantation into a patent ductus arteriosus. An ideal APS provides equal pulmonary blood flow (Qp) as the systemic blood flow (Qs), which is considered balanced circulation. Pulmonary over-circulation leads to complications from inadequate systemic circulation, such as organ dysfunction, shock, and death. Accurately determining Qp:Qs ratio in these patients relies on catheterization, which is invasive and may falsely lower the Qp due to anesthetic effects. To our knowledge, there has not been any studies on echocardiographic comparisons of balanced versus overcirculated APS. We aim to evaluate echocardiographic Doppler assessment of velocity-time integral (VTI) at the aortic arch isthmus distal to APS origin as a measure of shunt fraction in APS patients. We defined shunt fraction ratio (SFR) as retrograde to prograde flow by VTI. Methods: This is a retrospective pilot study of neonates with APS. We selected two cohorts of patients with appropriately balanced circulation (n=12) and those with excessive Qp (n=5) resulting in end-organ damage. We analyzed serial echocardiograms, with repeated measurements for inter-observer agreement. Results: Mean SFR is lower in balanced cohort compared to the overcirculated cohort (0.45 ± 0.07 vs. 0.55 ± 0.09, p=0.02). For both groups, SFR was positively associated with oxygen saturation (r= 0.57, p=0.01), as well as markers of ventricular volume load, such as the highest brain naturetic peptide during hospitalization (Pearson’s correlation r=0.83, p= 0.04) and as outpatient (r= 0.72, p =0.02). Inter-observer agreement was 0.74, suggesting that this is a reproducible technique. Conclusions: SFR by VTI is a simple echocardiographic technique to estimate the volume of APS flow and appears to have important implications for clinical outcomes. Prompt characterization of pulmonary overcirculation without invasive assessment could improve clinical management. Further prospective studies are needed to validate these findings.

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.

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.

scholarly journals Simulations Reveal Adverse Hemodynamics in Patients With Multiple Systemic to Pulmonary Shunts

2015 ◽  
Vol 137 (3) ◽  
Author(s):  
Mahdi Esmaily-Moghadam ◽  
Bari Murtuza ◽  
Tain-Yen Hsia ◽  
Alison Marsden
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Thrombus Formation ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Worst Case ◽  
Thrombotic Risk ◽  
Clinical Scenarios ◽  
Shunt Occlusion ◽  
Flow Competition

For newborns diagnosed with pulmonary atresia or severe pulmonary stenosis leading to insufficient pulmonary blood flow, cyanosis can be mitigated with placement of a modified Blalock–Taussig shunt (MBTS) between the innominate and pulmonary arteries. In some clinical scenarios, patients receive two systemic-to-pulmonary connections, either by leaving the patent ductus arteriosus (PDA) open or by adding an additional central shunt (CS) in conjunction with the MBTS. This practice has been motivated by the thinking that an additional source of pulmonary blood flow could beneficially increase pulmonary flow and provide the security of an alternate pathway in case of thrombosis. However, there have been clinical reports of premature shunt occlusion when more than one shunt is employed, leading to speculation that multiple shunts may in fact lead to unfavorable hemodynamics and increased mortality. In this study, we hypothesize that multiple shunts may lead to undesirable flow competition, resulting in increased residence time (RT) and elevated risk of thrombosis, as well as pulmonary overcirculation. Computational fluid dynamics-based multiscale simulations were performed to compare a range of shunt configurations and systematically quantify flow competition, pulmonary circulation, and other clinically relevant parameters. In total, 23 cases were evaluated by systematically changing the PDA/CS diameter, pulmonary vascular resistance (PVR), and MBTS position and compared by quantifying oxygen delivery (OD) to the systemic and coronary beds, wall shear stress (WSS), oscillatory shear index (OSI), WSS gradient (WSSG), and RT in the pulmonary artery (PA), and MBTS. Results showed that smaller PDA/CS diameters can lead to flow conditions consistent with increased thrombus formation due to flow competition in the PA, and larger PDA/CS diameters can lead to insufficient OD due to pulmonary hyperfusion. In the worst case scenario, it was found that multiple shunts can lead to a 160% increase in RT and a 10% decrease in OD. Based on the simulation results presented in this study, clinical outcomes for patients receiving multiple shunts should be critically investigated, as this practice appears to provide no benefit in terms of OD and may actually increase thrombotic risk.

scholarly journals CLOSING OF INTERVENTRICULAR SEPTUM DEFECT IN ONE-STEP AND STEPWISE SURGICAL TREATMENT IN PATIENTS WITH PULMONARY ATRESIA AND AORTO-PULMONARY COLLATERALS

2015 ◽  
Vol 174 (4) ◽  
pp. 9-12
Author(s):  
A. A. Morozov ◽  
R. R. Movsesyan ◽  
V. G. Lyubomudrov
Keyword(s):  
Blood Flow ◽  
Surgical Treatment ◽  
Pulmonary Blood Flow ◽  
Interventricular Septum ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Definitive Repair ◽  
One Step ◽  
Pulmonary Arterial ◽  
The Right

Pulmonary atresia with defect of interventricular septum and collateral pulmonary blood flow refers to complicated congenital malformation of the heart. Surgical treatment represents itself as very difficult task because of anatomical variability of this abnormality. The main problem of surgery is a definitive repair of the defect including correction of maldistributions of pulmonary arterial bed (unifocalization of pulmonary blood flow), reconstruction of outflow tract of the right ventricle and closing of interventricular septum defect. The performance of closing interventricular septum defect could be successful in patients with pulmonary atresia and collateral pulmonary blood flow in case of stepwise and one-step surgical treatment. The combination of maximal number of pulmonary segments and sufficient development of central pulmonary arteries facilitates to progress of assigned task. Patients with the level of pulmonary-arterial index more than 170 mm/m² and integrity of pulmonary arterial bed, centralization of pulmonary segments (minimum 15) could be considered as a candidate for definite repair of the defect.

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