| Abstract|| |
In the normal lung, the only communications between the systemic and pulmonary arterial systems are the connections between the bronchial and pulmonary arteries that occur at the respiratory bronchioles, where pulmonary and bronchial capillaries freely anastomose. Rarely, anomalous connections can occur between normal or aberrant systemic arteries and pulmonary vessels. We performed a comprehensive literature review of all available manuscripts on PubMed and Google Scholar that included a case report or case series with diagnosis of systemic artery to pulmonary venous fistulas who underwent percutaneous treatment. Furthermore, we report three cases of children diagnosed and treated in our Pediatric Cardiology Center.
Keywords: Cardiac catheterization, children, percutaneous treatment, pulmonary fistulas
|How to cite this article:|
Mazza GA, Garofalo M, Agnoletti G. Percutaneous treatment of anomalous systemic artery to pulmonary venous fistulas in children: Description of three cases and review of the literature. Ann Pediatr Card 2021;14:536-40
|How to cite this URL:|
Mazza GA, Garofalo M, Agnoletti G. Percutaneous treatment of anomalous systemic artery to pulmonary venous fistulas in children: Description of three cases and review of the literature. Ann Pediatr Card [serial online] 2021 [cited 2022 May 22];14:536-40. Available from: https://www.annalspc.com/text.asp?2021/14/4/536/340948
| Introduction and Background|| |
In the normal lung, the only communications between the systemic and pulmonary arterial systems are the connections between the bronchial and pulmonary arteries that occur at the respiratory bronchioles, where pulmonary and bronchial capillaries freely anastomose. Rarely, anomalous connections can occur between normal or aberrant systemic arteries and pulmonary vessels. These lesions are acyanotic, and the lack of parenchymal abnormalities together with normal pulmonary arterial and bronchial supply distinguishes systemic arterial supply without sequestration from pseudo and true sequestration [Table 1] and [Table 2].
|Table 1: Definition of types of sequestration complex from Jariwala et al.|
Click here to view
|Table 2: Classification of arterio-venous fistula/malformation of lungs from Jariwala et al.|
Click here to view
The characteristic features of systemic arterial supply without sequestration (i.e., systemic artery to pulmonary venous fistulas) are resumed in [Table 3].
|Table 3: Characteristic features of anomalous systemic artery to pulmonary venous fistulas from Jariwala et al.|
Click here to view
Although etiology remains unknown, the most likely theory is a persistent embryonic connection between the aorta and the pulmonary parenchyma. Furthermore, acquired causes exist and include pulmonary suppuration, pleurotomy, and iatrogenic anastomosis., Disease is usually asymptomatic in children, and it may be discovered following the incidental finding of a continuous chest murmur or left ventricular dilation due to the left-to-left shunt, but sometimes, heart failure is the first symptom at the onset., In older children and young adults, cases of massive life-threatening hemoptysis, and pulmonary hypertension have been reported. However, this latter occurs more frequently in case of systemic artery to pulmonary artery (left-to-right) shunts than systemic artery to pulmonary vein (left-to-left) shunts., In addition, endocarditis is considered another possible complication.,
To date, definite indications for treatment still have not been established. However, several authors recommend intervention both in all symptomatic patients and in asymptomatic ones with incidental diagnosis due to the risk of congestive heart failure, endocarditis, and hemorrhagic complications.,
In the past, surgical ligation of collaterals with or without lobectomy was the therapy of choice. Recently, transcatheter embolization has proved to be a feasible therapeutic alternative.
We performed a comprehensive literature review of all available manuscripts on PubMed and Google Scholar that included a case report or case series with diagnosis of systemic artery to pulmonary venous fistulas who underwent percutaneous treatment. Furthermore, we report three cases of children diagnosed and treated in our Pediatric Cardiology Center of Regina Margherita Hospital in Turin.
| Case Series from the Literature|| |
Considering the rarity of this condition, we found only 23 papers of which 5 reporting percutaneous closures, performed in a total of 6 patients [Table 4].
|Table 4: Review of the literature of anomalous systemic artery to pulmonary venous fistulas percutaneously treated|
Click here to view
Campbell et al., Scott and Perry (1969), and Ernst and Bruschke reported systemic artery to pulmonary vein fistulas with left-to-left shunt first. Later on, other isolated case reports, mostly congenital, were published.
In the past, surgery has been the treatment of choice, but in 1998, Brühlmann et al. described the first therapeutic transarterial embolization with coils of a systemic artery to pulmonary vein fistula in a 51-year-old man presenting with massive hemoptysis. In their case report, a large anomalous artery arising from the lower thoracic aorta and supplying the basal lung segments of the left lower lobe was diagnosed with a computed tomography (CT) scan.
Since then, other authors reported successful percutaneous embolization of this kind of lesions.
Jariwala et al. reported the case of a 7-month-old girl who was referred to the hospital by a general practitioner with failure to thrive and suspected congenital heart disease.
CT angiography showed anomalous systemic feeders of the left lung from the branches of the left mammary and left axillary arteries and from an aberrant vessel originating from the upper abdominal aorta. Collaterals were embolized using vascular plugs and coils.
Chabbert et al. described the case of a 17-year-old boy who was admitted to the hospital for chest pain. CT scan confirmed the presence of an aberrant aneurysmal artery arising from the aorta above the celiac trunk and supplying the basal segments of the right lower lobe without any parenchymal abnormality. Aberrant artery was successfully embolized using several coils.
Kosutic et al. reported the case of a 3-month-old infant with signs and symptoms of vascular tracheal compression and congestive heart failure. Aortography showed two major aberrant arteries arising from the descending thoracic aorta and entering the right upper pulmonary lobe. One of them was successfully embolized with two coils and the other one spontaneously closed after cardiac catheterization.
Singhi et al. reported the case of a 74-day-old term baby who had tachypnea, feeding difficulty, and failure to thrive. An anomalous artery from the descending aorta to the lower segment of the left lung was diagnosed and then embolized with three coils.
They also reported the case of a 90-day-old term baby presenting with tachypnea and feeding difficulty. Two collaterals arising from the abdominal aorta and supplying the lower lobe of the left lung were identified and then embolized using a vascular plug and five coils.
| Case Series from our Center|| |
Case #1 is a 5-year-old healthy boy, who was hospitalized at the age of 3 years for an episode of right basilar bronchopneumonia, treated with antibiotic therapy. During hospitalization, a continuous murmur was detected at the right hemithorax. Echocardiography was normal; therefore, he underwent CT scan that showed an aberrant artery connecting the mean thoracic aorta to the laterobasal segment of the right lung, without any parenchymal abnormality. Because of the potential hemorrhagic risk and development of pulmonary hypertension, we decided to embolize the vessel. Hence, a conventional arterial catheterization (femoral, 5F) was performed. Aortography showed a large collateral arising from the thoracic aorta, nourishing the inferior lobe of the right lung, and an additional minor collateral arising from the right mammary artery [Video 1 [Additional file 1]].
Selective angiography of the aberrant artery showed a large left-to-left shunt that was successfully embolized with a vascular plug (Amplatzer vascular Plug1 8 mm).
Selective angiography of the right mammary artery confirmed perfusion of the right inferior pulmonary lobe by an aberrant vessel that was occluded with a second vascular plug (Amplatzer vascular Plug1 6 mm).
Postembolization aortography showed complete occlusion of the aberrant artery from the aorta and scarce residual flow through the mammary artery beyond the plug [Video 2 [Additional file 2]].
Control cardiac catheterization performed 7 months later showed the development of other additional collateral vessels arising from the thoracic aorta and the right mammary artery nourishing the inferior lobe of the right lung [Video 3 [Additional file 3]].
All anomalous vessels were embolized with vascular plugs of various sizes (Amplatzer vascular Plug1 8 and 10 mm large and Plug4 5 and 6 mm large). Postembolization arteriographies showed scarce residual flow in the feeding arteries beyond the plugs [Video 4 [Additional file 4]].
At the last clinical follow-up, 8 months later, the child was in good health and he did not assume any drug.
Patient #2 is an ex very low birth weight preterm male (birth weight 550 g) with a history of a severe bronchopulmonary dysplasia and respiratory failure.
After hospital discharge, he was readmitted at the age of 6 months for respiratory failure. Cardiac examination showed pulmonary hypertension, mitral and tricuspid valve insufficiency, and left ventricular enlargement. He underwent thoracic CT scan that showed partial lung consolidation with widespread images of ground glass.
At the age of 3 years, clinicians noticed a murmur at the right hemithorax and therefore he underwent a diagnostic 6F femoral artery approach catheterization. Aortography showed several collateral vessels from the thoracic aorta and the right mammary artery to the entire right lung [Video 5 [Additional file 5]].
Occlusion of three collaterals arising from the aorta was performed using vascular plugs (Amplatzer vascular Plug4 7 mm and Amplatzer vascular Plug1 10 mm) and microparticles, while the hypertrophic mammary artery was embolized with eight coils and a vascular plug (Amplatzer vascular Plug1 10 mm).
Final aortography showed poor residual flow to the right lung [Video 6 [Additional file 6]].
Control cardiac catheterization performed 10 months later still showed large anomalous vascularization to the right lung arising from the thoracic aorta and right subclavian artery [Video 7 [Additional file 7]].
We embolized two collaterals from the subclavian artery: one with a plug (Amplatzer vascular Plug4 7 mm) and one with a coil and two collaterals from the thoracic aorta: one with a plug (Amplatzer vascular Plug4 6 mm) and one with a coil.
Although final aortography showed moderate residual flow to the right lung, we decided not to prolong the length of the examination, considering that the child was very fragile.
At the last clinical follow-up, 8 months later, parents reported overall good health with improved respiratory condition. At physical examination, murmur was still hearable and left ventricle appeared still dilated at echocardiography. He will undergo a new planned cardiac catheterization in the next months.
Patient #3 is a 3-year-old boy who entered into the emergency room with hemoptysis. Thoracic CT scan showed lung parenchymal consolidation of the anterolateral segment of the left lower lobe with images of vascular malformations in its structure.
Therefore, he underwent a 5F femoral arterial catheterization which showed two large collateral vessels from the thoracic aorta to both lungs. The left one gave rise to a complex arteriovenous malformation with a large connection with the inferior left pulmonary vein [Video 8 [Additional file 8]].
We performed embolization of both collaterals with two plugs (Amplatzer vascular Plugs4 5 and 6 mm).
Final aortography showed no residual vascularization through the collaterals to the lungs [Video 9 [Additional file 9]].
The patient was discharged in good clinical conditions.
All procedures we described were uneventful.
| Discussion and Conclusions|| |
Anomalous systemic arterial supply to normal lungs represents a subtype of a rare group of bronchovascular anomalies which can have varied presentation. Although predominately reported in the adult population, pediatric case reports are also available in the literature.
In the past, surgical ligation of collaterals with or without lobectomy was the therapy of choice. Recently, transcatheter embolization has proved to be a feasible therapeutic alternative in these cases without sequestration.
In our opinion, the best approach in case of suspicion is to perform contrast CT first in order to confirm the diagnosis and plan the best therapeutic strategy, also considering the low X-rays and contrast doses needed with the newest CT scanners.
Then, the patient can undergo cardiac catheterization aiming at the closure of as many collaterals as possible.
As reported, the Amplatzer vascular plugs are largely used for transcatheter occlusion of abnormal vessel communications with a high success rate. The only disadvantage may be the need for relatively large catheters (5F at least) in small children. Controlled-release coils are other common devices consisting of a metallic coil with attached fibers that increase their occlusive effect. Their advantage is that they can be implanted through 4F catheters.,
In our cases, we successfully used plugs for larger fistulas and coils for smaller ones.
In the literature, immediate and short-term follow-up results proved good, but long-term postembolization data are scarce. Based on our experience, some embolized anomalous connections can show persistent residual flow, as well as new collaterals can develop over time, suggesting that these lesions likely represent a heart–lung disease more than an isolated anomaly of one or more vessels.
For these reasons, a careful follow-up remains mandatory, and in case of disease relapse, a new interventional cardiac catheterization is needed, in order to prevent the severe complications reported in untreated patients.
We recommend a control cardiac catheterization after 6–12 months, preceded by a new contrast CT examination in case of doubts about the anatomy or extensive disease.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's guardian has given consent for patient's images and other clinical information to be reported in the journal. The patient's guardian understands that patient's name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Geyik S, Yavuz K, Keller FS. Unusual systemic artery to pulmonary artery malformation without evidence of systemic disease, trauma or surgery. Cardiovasc Intervent Radiol 2006;29:897-901.
Jariwala P, Ramesh G, Sarat Chandra K. Congenital anomalous/aberrant systemic artery to pulmonary venous fistula: Closure with vascular plugs and coil embolization. Indian Heart J 2014;66:95-103.
Kirks DR, Kane PE, Free EA, Taybi H. Systemic arterial supply to normal basilar segments of the left lower lobe. AJR Am J Roentgenol 1976;126:817-21.
Viamonte M. Intrathoracic extracardiac shunts. Semin Roentgenol 1967;2:342-67.
Scott LP 3rd
, Perry LW. Systemic artery – Pulmonary vein fistulas, congenital and acquired left to left shunt. Am J Cardiol 1969;23:872-6.
Wong MN, Joshi P, Sim KH. Large anomalous systemic arterial supply to the left lung without pulmonary sequestration: A rare cause of heart failure in a child. Pediatr Cardiol 2009;30:85-6.
Pernot C, Simon P, Hoeffel JC, Worms AM, Marcon F, Prevot J. Systemic artery-pulmonary vein fistula without sequestration. Pediatr Radiol 1991;21:158-9.
Singhi AK, Nicholson I, Francis E, Kumar RK, Hawker R. Anomalous systemic arterial supply to normal basal segment of the left lung. Heart Lung Circ 2011;20:357-61.
Pouwels HM, Janevski BK, Penn OC, Sie HT, ten Velde GP. Systemic to pulmonary vascular malformation. Eur Respir J 1992;5:1288-91.
Shi YP, Li YD, Lv XZ, Yang YH. Systemic-pulmonary arteriovenous fistulae with pulmonary hypertension: A case report. Medicine (Baltimore) 2018;97:e9959.
Li JF, Zhai ZG, Kuang TG, Liu M, Ma ZH, Li YD, et al
. A case of pulmonary hypertension due to fistulas between multiple systemic arteries and the right pulmonary artery in an adult discovered for occulted dyspnoea. Heart Lung Circ 2017;26:e54-8.
Jacheć W, Tomasik A, Kurzyna M, Pietura R, Torbicki A, Głowacki J, et al.
The multiple systemic artery to pulmonary artery fistulas resulting in severe irreversible pulmonary arterial hypertension in patient with previous history of pneumothorax. BMC Pulm Med 2019;19:80.
Lee JK, Park JH, Kim J, Kim SJ, Lee AR, Lee CH, et al.
Embolization of multiple systemic artery to pulmonary artery fistula with recurrent hemoptysis. Tuberc Respir Dis (Seoul) 2013;75:120-4.
Saito T, Matsuda M, Yamaguchi T, Matsuzaki T, Nakagawa S. A case of a traumatic systemic-pulmonary arteriovenous fistula. Jpn Heart J 1975;16:196-203.
Chabbert V, Doussau-Thuron S, Otal P, Bouchard L, Didier A, Joffre F, et al.
Endovascular treatment of aberrant systemic arterial supply to normal basilar segments of the right lower lobe: Case report and review of the literature. Cardiovasc Intervent Radiol 2002;25:212-5.
Brühlmann W, Weishaupt D, Goebel N, Imhof E. Therapeutic embolization of a systemic arterialization of lung without sequestration. Eur Radiol 1998;8:355-8.
Kosutic J, Minic P, Sovtic A, Prijic S. Upper lung lobe systemic artery-pulmonary vein fistula with signs and symptoms of congestive heart failure: Successful treatment with coil embolization. J Vasc Interv Radiol 2007;18:299-302.
Campbell DC Jr., Murney JA, Dominy DE. Systemic arterial blood supply to a normal lung. JAMA 1962;182:497-9.
Ernst SM, Bruschke AV. An aberrant systemic artery to the right lung with normal pulmonary tissue. Chest 1971;60:606-8.
Currarino G, Willis K, Miller W. Congenital fistula between an aberrant systemic artery and a pulmonary vein without sequestration. A report of three cases. J Pediatr 1975;87:554-7.
Girona J, Martı G, Betrian P, Gran F, Casaldaliga J. Percutaneous embolization of vascular fistulas using coils or Amplatzer vascular plugs. Revista Espanola de Cardiologıa (English Edition) 2009;62:765-73.
Perry SB, Radtke W, Fellows KE, Keane JF, Lock JE. Coil embolization to occlude aortopulmonary collateral vessels and shunts in patients with congenital heart disease. J Am Coll Cardiol 1989;13:100-8.
Dr. Giuseppe Antonio Mazza
Division of Pediatric Cardiology, Poland Square, 10124 Turin
Source of Support: None, Conflict of Interest: None
[Table 1], [Table 2], [Table 3], [Table 4]