CORONARY TO PULMONARY ARTERY FISTULA AS AN IMPORTANT SOURCE OF PULMONARY BLOOD FLOW IN TETRALOGY OF FALLOT WITH PULMONARY ATRESIA

2020 ◽  
Vol 75 (11) ◽  
pp. 3070
Author(s):  
Sruti Rao ◽  
Christian Pizarro ◽  
Mary Harty ◽  
Mark Cartoski
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Pulmonary Atresia ◽  
Pulmonary Artery Fistula

Coronary-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia

2013 ◽  
Vol 22 (8) ◽  
pp. 1003-1009 ◽  
Author(s):  
Sachin Talwar ◽  
Robert H Anderson ◽  
Vikas Kumar Keshri ◽  
Shiv Kumar Choudhary ◽  
Gurpreet Singh Gulati ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Atresia ◽  
Pulmonary Artery Fistula

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.

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.

scholarly journals P1727 Large coronary artery to pulmonary artery fistula as primary source of pulmonary blood supply in tetralogy of fallot with pulmonary atresia

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
N Aslam ◽  
Z Rashid ◽  
M Mohsin ◽  
D Chowdhury ◽  
B Sultan Hasan
Keyword(s):  
Coronary Artery ◽  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Blood Supply ◽  
Left Main Coronary Artery ◽  
Pulmonary Atresia ◽  
Primary Source ◽  
Left Main ◽  
Grade 3 ◽  
Pulmonary Artery Fistula

Abstract Funding Acknowledgements Not Applicable OnBehalf Not Applicable Introduction Pulmonary blood supply in patients of Tetralogy of Fallot with pulmonary atresia is usually from patent arterial duct or major aortopulmonary collaterals (MAPCAs) arising from descending thoracic aorta. We describe a case in which large coronary to pulmonary artery fistula was the primary source of pulmonary blood supply. Case Report A 17 years old female was referred to our hospital for diagnostic workup of suspected congenital heart disease. She was previously undiagnosed and now complains of progressive shortness of breath for last few months. On physical examination she was non-dysmorphic with oxygen saturation of ∼ 77 % in room air, blood pressure of ∼ 117/72 mmHg, pulse rate of ∼ 89 beats per minute and respiratory rate of ∼ 24 breaths per minute. She was clinically cyanosed with grade 3 clubbing and polycythemic. Cardiovascular examination revealed quiet precordium with normally placed apex beat, grade 2 parasternal heave with single second heart sound and grade 3/6 continuous murmur along left mid sternal border. Twelve lead electrocardiogram (ECG) showed normal sinus rhythm, right axis deviation and right ventricular hypertrophy. There was no evidence of ischemia. Chest X-ray revealed "boat shaped heart" with oligaemic lung fields. Transthoracic echocardiography showed large conoventricular ventricular septal defect with bidirectional flow. There was aortic over-ride with dilated left main coronary artery. No forward flow was seen across right ventricular outflow tract. Considering hugely dilated left main coronary artery, suspicion of coronary to pulmonary artery fistula was made and cardiac computed tomography followed by conventional angiography was done, both confirmed the diagnosis of Tetralogy of Fallot with pulmonary atresia and large coronary artery to main pulmonary artery fistula as a primary pulmonary blood supply. Two small collaterals (MAPCAs) were also identified supplying small part of right and left lungs. Conclusion This case highlights unusual source of pulmonary blood supply in Tetralogy of Fallot with pulmonary atresia. Correct pre-operative diagnosis is essential for appropriate surgical planning and better outcome. Abstract P1727 Figure. TOF-PA with CA to PA Fistula

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