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Interventional Case Record
Interventional Case Record
Endovascular management of pulmonary arteriovenous malformation with a vascular plug
Endovascular management of pulmonary arteriovenous malformation with a vascular plug
Contributed by : Aishwarya Dahake
Contributed by : Aishwarya Dahake
Introduction:
Introduction:
Pulmonary arteriovenous malformations are abnormal direct connections between the pulmonary artery and pulmonary vein which result in a right‐to‐left shunt. Although these pulmonary AVMs are expected to be inherited and present at birth, they rarely manifest clinically before adult life. Embolization is the mainstream treatment for pulmonary arteriovenous malformations. During the procedure, balloon or coil embolization devices (or both combined) are used to block the feeding artery or arteries to the malformation. Endovascular therapy of this type is an outpatient-based procedure with minimal invasiveness. We discuss a case wherein the patient presented with sudden onset right haemothorax secondary to pulmonary AV malformation managed with plug embolization.
Pulmonary arteriovenous malformations are abnormal direct connections between the pulmonary artery and pulmonary vein which result in a right‐to‐left shunt. Although these pulmonary AVMs are expected to be inherited and present at birth, they rarely manifest clinically before adult life. Embolization is the mainstream treatment for pulmonary arteriovenous malformations. During the procedure, balloon or coil embolization devices (or both combined) are used to block the feeding artery or arteries to the malformation. Endovascular therapy of this type is an outpatient-based procedure with minimal invasiveness. We discuss a case wherein the patient presented with sudden onset right haemothorax secondary to pulmonary AV malformation managed with plug embolization.
Case presentation:
Case presentation:
A 43 year old woman presented with the complaints of sudden onset breathlessness on exertion grade III, associated with right sided chest pain since seven days. The patient had had multiple episodes of epistaxis since the last five years. There was no history of palpitation, syncope or cough, expectoration and fever. No history of any comorbidities was obtained. A chest radiograph done in at a district hospital which was suggestive of right pleural effusion ; an hence ICD was inserted. The drain output was red ; hence patient was referred to to our institution for further management.
A 43 year old woman presented with the complaints of sudden onset breathlessness on exertion grade III, associated with right sided chest pain since seven days. The patient had had multiple episodes of epistaxis since the last five years. There was no history of palpitation, syncope or cough, expectoration and fever. No history of any comorbidities was obtained. A chest radiograph done in at a district hospital which was suggestive of right pleural effusion ; an hence ICD was inserted. The drain output was red ; hence patient was referred to to our institution for further management.
On clinical examination patient gave hitory also of recurrent epistaxis. There were mucocutaneous patches.
On clinical examination patient gave hitory also of recurrent epistaxis. There were mucocutaneous patches.
A chest radiograph was performed (Figure 1 ).
A chest radiograph was performed (Figure 1 ).
Fig. 1 : Chest radiograph : Shows right basal pleural effusion and consolidation. A chest tube on the right side.
CT pulmonary angio (Figure 2) showed the right interlobar artery draining directly into the right inferior pulmonary vein via multiple dilated tortuous channels (nidus) in the right lower lobe. Coarse calcification is seen in the walls of the dilated collaterals. The nidus measured approximately 4.8 x 6 x 5.7cm. There was subsegmental collapse in the basal segments of right lower lobe. There was moderate subpulmonic hemothorax. An ICD tube was seen in an expected position. Reactive pleural thickening and early vascular enhancement was seen . These findings indicated a ruptured pulmonary AVM with right subpulmonic hemothorax.
CT pulmonary angio (Figure 2) showed the right interlobar artery draining directly into the right inferior pulmonary vein via multiple dilated tortuous channels (nidus) in the right lower lobe. Coarse calcification is seen in the walls of the dilated collaterals. The nidus measured approximately 4.8 x 6 x 5.7cm. There was subsegmental collapse in the basal segments of right lower lobe. There was moderate subpulmonic hemothorax. An ICD tube was seen in an expected position. Reactive pleural thickening and early vascular enhancement was seen . These findings indicated a ruptured pulmonary AVM with right subpulmonic hemothorax.
Fig. 2 CT pul angio
An MRI of the brain brain (Figure 3) was done to screen for brain AVM and brain abscess in view of right to left shunting of blood due to pulmonary AVM
An MRI of the brain brain (Figure 3) was done to screen for brain AVM and brain abscess in view of right to left shunting of blood due to pulmonary AVM
Figure 3:MRI
Interventional treatment : The patient was planned for pulmonary AVM plug embolization. Under general anaesthesia, a right femoral vein access was obtained using 4 Fr H1 catheter and Terumo guide wire, the right descending pulmonary artery was catheterised. The Terumo glide wire was exchanged for Cook AES stiff wire and a 80 cm 10 Fr Raabe sheath was placed with its tip in the right descending pulmonary artery. An angiogram was obtained (Video1) and measurements were taken. The feeding artery measured 14 mm in width. So embolization was planned using Abbott Amplatzer vascular plug II of 20 mm size. Post embolization there was isolation of the nidus from the main pulmonary circulation(Video 2), Post extubation, there was complete clinical improvement in form of increase in SPO2 from 80% to above 95 % on room air. The patient was discharged uneventfully on the 5th. post procedure day.
Interventional treatment : The patient was planned for pulmonary AVM plug embolization. Under general anaesthesia, a right femoral vein access was obtained using 4 Fr H1 catheter and Terumo guide wire, the right descending pulmonary artery was catheterised. The Terumo glide wire was exchanged for Cook AES stiff wire and a 80 cm 10 Fr Raabe sheath was placed with its tip in the right descending pulmonary artery. An angiogram was obtained (Video1) and measurements were taken. The feeding artery measured 14 mm in width. So embolization was planned using Abbott Amplatzer vascular plug II of 20 mm size. Post embolization there was isolation of the nidus from the main pulmonary circulation(Video 2), Post extubation, there was complete clinical improvement in form of increase in SPO2 from 80% to above 95 % on room air. The patient was discharged uneventfully on the 5th. post procedure day.
Fig. 4,
Discussion:
Discussion:
Pulmonary arteriovenous malformations (AVMs) are abnormal connections between a pulmonary artery and a pulmonary vein. These usually bypass the capillary bed and lead to right-to-left shunting of blood, which in turn can lead to symptoms depending on the degree of blood shunting (1).
Pulmonary arteriovenous malformations (AVMs) are abnormal connections between a pulmonary artery and a pulmonary vein. These usually bypass the capillary bed and lead to right-to-left shunting of blood, which in turn can lead to symptoms depending on the degree of blood shunting (1).
Most patients with pulmonary AVMs have the autosomal dominant disease - hereditary hemorrhagic telangiectasia (HHT). Pulmonary AVMs may also be acquired and found in patients with liver cirrhosis. Other rare associations include chest trauma, schistosomiasis of the lung, and metastatic lung disease (2) They are also seen with conditions such as mitral stenosis, metastatic thyroid carcinoma, hepato-pulmonary syndrome, bronchiectasis, schistosomiasis, and actinomycosis (3).
Most patients with pulmonary AVMs have the autosomal dominant disease - hereditary hemorrhagic telangiectasia (HHT). Pulmonary AVMs may also be acquired and found in patients with liver cirrhosis. Other rare associations include chest trauma, schistosomiasis of the lung, and metastatic lung disease (2) They are also seen with conditions such as mitral stenosis, metastatic thyroid carcinoma, hepato-pulmonary syndrome, bronchiectasis, schistosomiasis, and actinomycosis (3).
Pulmonary AVMs have been classified into simple and complex, primary and secondary, large and small. They are classified as large if their size is more than 5 cm. and small if they are less than 5 cm. They are found primarily in the subpleural in 81% of cases, lower lobes in almost 70%, unilaterally in around 75%, and multiple in nearly 36% of the cases (4).
Pulmonary AVMs have been classified into simple and complex, primary and secondary, large and small. They are classified as large if their size is more than 5 cm. and small if they are less than 5 cm. They are found primarily in the subpleural in 81% of cases, lower lobes in almost 70%, unilaterally in around 75%, and multiple in nearly 36% of the cases (4).
The most common complaint in patients is epistaxis. The second most common complaint is dyspnoea on exertion, which is more common in patients with large or multiple pulmonary AVMs. Rarely massive haemoptysis can occur. Murmurs or bruits over the location of the pulmonary AVMs are heard in patients with large pulmonary AVMs. These murmurs are also audible during inspiration and are called machinery murmurs. Occasionally, patients complaining of dyspnoea will be noted to have improvement of the peripheral oxygen saturation upon lying in the supine position, a syndrome known as platypnea-orthodeoxia (5).
The most common complaint in patients is epistaxis. The second most common complaint is dyspnoea on exertion, which is more common in patients with large or multiple pulmonary AVMs. Rarely massive haemoptysis can occur. Murmurs or bruits over the location of the pulmonary AVMs are heard in patients with large pulmonary AVMs. These murmurs are also audible during inspiration and are called machinery murmurs. Occasionally, patients complaining of dyspnoea will be noted to have improvement of the peripheral oxygen saturation upon lying in the supine position, a syndrome known as platypnea-orthodeoxia (5).
For diagnosis. CT is superior in identifying pulmonary AVMs than invasive angiography. However, pulmonary angiography is better at assessing individual AVM anatomy and is also used for treatment (6). Other adjunct tests that can be used in the evaluation of pulmonary AVMs include radionuclide perfusion lung scanning as well as magnetic resonance imaging. (7, 8)
For diagnosis. CT is superior in identifying pulmonary AVMs than invasive angiography. However, pulmonary angiography is better at assessing individual AVM anatomy and is also used for treatment (6). Other adjunct tests that can be used in the evaluation of pulmonary AVMs include radionuclide perfusion lung scanning as well as magnetic resonance imaging. (7, 8)
As the most common clinical presentations are recurrent epistaxis and haemoptysis, surgical resection was the best curative treatment to prevent future episodes and recurrence of haemoptysis (4). Procedures performed included ligation, excision, lobectomy, segmentectomy, and pneumonectomy (9). However, it is claimed that percutaneous embolization is an efficient, safe, and sustained therapy for pulmonary AVMs (10). The method involves localization of the pulmonary AVM by angiography after which a catheter is inserted, and either a coil or inflatable balloon is released to impede the communication (11). Periprocedural mortality has not been reported with this technique, and the most common complication is pleuritic chest pain, which resolves on its own. Prognosis after treatment is usually good as the success rate from coil or balloon embolization is 98%.(12, 13). Patients should be followed with repeat CT of the chest between 6 months and one year after embolization to ensure patients remain successfully treated as well as to evaluate for possible new pulmonary arteriovenous malformations.
As the most common clinical presentations are recurrent epistaxis and haemoptysis, surgical resection was the best curative treatment to prevent future episodes and recurrence of haemoptysis (4). Procedures performed included ligation, excision, lobectomy, segmentectomy, and pneumonectomy (9). However, it is claimed that percutaneous embolization is an efficient, safe, and sustained therapy for pulmonary AVMs (10). The method involves localization of the pulmonary AVM by angiography after which a catheter is inserted, and either a coil or inflatable balloon is released to impede the communication (11). Periprocedural mortality has not been reported with this technique, and the most common complication is pleuritic chest pain, which resolves on its own. Prognosis after treatment is usually good as the success rate from coil or balloon embolization is 98%.(12, 13). Patients should be followed with repeat CT of the chest between 6 months and one year after embolization to ensure patients remain successfully treated as well as to evaluate for possible new pulmonary arteriovenous malformations.
After successful embolization, the occlusion of feeding arteries leads to regression of pulmonary AVMs, resulting in the resolution of right-to-left shunt, improvement to oxygenation, and the prevention of embolic complications (4). Inspite of the recent advances in angiographic interventions, there are still technical limitations to embolization in some cases. It has been reported that the increasing feeding artery diameter, the use of small numbers of coils, the use of oversized coils, and proximal coil location are associated with the recanalization of pulmonary AVMs after embolization (9). Large pulmonary AVMs with multiple large feeding arteries are difficult to occlude completely.
After successful embolization, the occlusion of feeding arteries leads to regression of pulmonary AVMs, resulting in the resolution of right-to-left shunt, improvement to oxygenation, and the prevention of embolic complications (4). Inspite of the recent advances in angiographic interventions, there are still technical limitations to embolization in some cases. It has been reported that the increasing feeding artery diameter, the use of small numbers of coils, the use of oversized coils, and proximal coil location are associated with the recanalization of pulmonary AVMs after embolization (9). Large pulmonary AVMs with multiple large feeding arteries are difficult to occlude completely.
Surgical resection is necessary in cases that are not amenable to transcatheter embolization. However, there is no consensus about which pulmonary AVMs should be treated with surgical resection rather than transcatheter embolization, and this choice often depends on the skill, experience, and preference of the interventionist. The primary advantage of coil embolization is ease of deployment and due to their small size can be delivered through a 3-4 F sheath. The disadvantages are inadequate occlusion and distal embolization of coils. Paradoxical embolization of coils/devices, thrombus or air during occlusion of large high flow PAVMs is a dangerous complication that all interventionalists should be aware of. Several techniques have been described to mitigate the risks of paradoxical embolization including bioptome assisted delivery of coils, use of detachable coils/devices, diligent flushing of catheters/long sheaths, and retrograde balloon occlusion of the pulmonary venous channel, accessed after a transseptal puncture of atrial septum.(10). For the past decade, the St Jude Amplatzer™ vascular plugs have routinely been used for successful embolization of PAVM in both adults and children. (11,12) The four types of AVPs (I-IV) were described in detail in a recent publication by Wang et al. (13) Reported advantages of the AVP device over coils are complete occlusion with a single device resulting in shorter procedure time, the ability to occlude shorter length of vessel and potentially faster time. (14,15).
Surgical resection is necessary in cases that are not amenable to transcatheter embolization. However, there is no consensus about which pulmonary AVMs should be treated with surgical resection rather than transcatheter embolization, and this choice often depends on the skill, experience, and preference of the interventionist. The primary advantage of coil embolization is ease of deployment and due to their small size can be delivered through a 3-4 F sheath. The disadvantages are inadequate occlusion and distal embolization of coils. Paradoxical embolization of coils/devices, thrombus or air during occlusion of large high flow PAVMs is a dangerous complication that all interventionalists should be aware of. Several techniques have been described to mitigate the risks of paradoxical embolization including bioptome assisted delivery of coils, use of detachable coils/devices, diligent flushing of catheters/long sheaths, and retrograde balloon occlusion of the pulmonary venous channel, accessed after a transseptal puncture of atrial septum.(10). For the past decade, the St Jude Amplatzer™ vascular plugs have routinely been used for successful embolization of PAVM in both adults and children. (11,12) The four types of AVPs (I-IV) were described in detail in a recent publication by Wang et al. (13) Reported advantages of the AVP device over coils are complete occlusion with a single device resulting in shorter procedure time, the ability to occlude shorter length of vessel and potentially faster time. (14,15).
Thus, the decision of embolization of pulmonary AVM using AMPLATZER II vascular plug was made. The points which favoured this decision were:
Thus, the decision of embolization of pulmonary AVM using AMPLATZER II vascular plug was made. The points which favoured this decision were:
a. Sufficiently large diameter of the feeding artery which is amenable for embolization by vascular plug.(6)
a. Sufficiently large diameter of the feeding artery which is amenable for embolization by vascular plug.(6)
b. Rapid occlusion time of vascular plugs. (6)
b. Rapid occlusion time of vascular plugs. (6)
c. Ability of the vascular plugs to place and retrieve them prior to detachment. (6)
c. Ability of the vascular plugs to place and retrieve them prior to detachment. (6)
d. Fairly straight course of the feeding artery.
d. Fairly straight course of the feeding artery.
e. Lower recanalization rates of the AMPLATZER II vascular plugs.
e. Lower recanalization rates of the AMPLATZER II vascular plugs.
References:
References:
1.Gill SS, Roddie ME, Shovlin CL, Jackson JE. Pulmonary arteriovenous malformations and their mimics. Clin Radiol. 2015 Jan;70(1):96-110. [PubMed]
1.Gill SS, Roddie ME, Shovlin CL, Jackson JE. Pulmonary arteriovenous malformations and their mimics. Clin Radiol. 2015 Jan;70(1):96-110. [PubMed]
2. Ghelani SJ, Rathod RH. Pulmonary arteriovenous malformations: The consequences of bypassing the capillary bed. J Thorac Cardiovasc Surg. 2015 Sep;150(3):717-9. [PubMed]
2. Ghelani SJ, Rathod RH. Pulmonary arteriovenous malformations: The consequences of bypassing the capillary bed. J Thorac Cardiovasc Surg. 2015 Sep;150(3):717-9. [PubMed]
3.Biswas D, Dey A, Chakraborty M, Biswas S. Pulmonary arteriovenous malformation: An uncommon disease with common presentation. Lung India. 2010 Oct;27(4):247-9. [PMC free article] [PubMed]
3.Biswas D, Dey A, Chakraborty M, Biswas S. Pulmonary arteriovenous malformation: An uncommon disease with common presentation. Lung India. 2010 Oct;27(4):247-9. [PMC free article] [PubMed]
4. Kuhajda I, Milosevic M, Ilincic D, Kuhajda D, Pekovic S, Tsirgogianni K, Tsavlis D, Tsakiridis K, Sakkas A, Kantzeli A, Zarogoulidis K, Zarogoulidis P, Zissimopoulos A, Durić D. Pulmonary arteriovenous malformation-etiology, clinical four case presentations and review of the literature. Ann Transl Med. 2015 Jul;3(12):171. [PMC free article] [PubMed]
4. Kuhajda I, Milosevic M, Ilincic D, Kuhajda D, Pekovic S, Tsirgogianni K, Tsavlis D, Tsakiridis K, Sakkas A, Kantzeli A, Zarogoulidis K, Zarogoulidis P, Zissimopoulos A, Durić D. Pulmonary arteriovenous malformation-etiology, clinical four case presentations and review of the literature. Ann Transl Med. 2015 Jul;3(12):171. [PMC free article] [PubMed]
5. Agrawal A, Palkar A, Talwar A. The multiple dimensions of Platypnea-Orthodeoxia syndrome: A review. Respir Med. 2017 Aug;129:31-38. [PubMed]
5. Agrawal A, Palkar A, Talwar A. The multiple dimensions of Platypnea-Orthodeoxia syndrome: A review. Respir Med. 2017 Aug;129:31-38. [PubMed]
6. Remy J, Remy-Jardin M, Wattinne L, Deffontaines C. Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology. 1992 Mar;182(3):809-16. [PubMed]
6. Remy J, Remy-Jardin M, Wattinne L, Deffontaines C. Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology. 1992 Mar;182(3):809-16. [PubMed]
7.Whyte MK, Peters AM, Hughes JM, Henderson BL, Bellingan GJ, Jackson JE, Chilvers ER. Quantification of right to left shunt at rest and during exercise in patients with pulmonary arteriovenous malformations. Thorax. 1992 Oct;47(10):790-6. [PMC free article] [PubMed]
7.Whyte MK, Peters AM, Hughes JM, Henderson BL, Bellingan GJ, Jackson JE, Chilvers ER. Quantification of right to left shunt at rest and during exercise in patients with pulmonary arteriovenous malformations. Thorax. 1992 Oct;47(10):790-6. [PMC free article] [PubMed]
8.Dinsmore BJ, Gefter WB, Hatabu H, Kressel HY. Pulmonary arteriovenous malformations: diagnosis by gradient-refocused MR imaging. J Comput Assist Tomogr. 1990 Nov-Dec;14(6):918-23. [PubMed]
8.Dinsmore BJ, Gefter WB, Hatabu H, Kressel HY. Pulmonary arteriovenous malformations: diagnosis by gradient-refocused MR imaging. J Comput Assist Tomogr. 1990 Nov-Dec;14(6):918-23. [PubMed]
9. Puskas JD, Allen MS, Moncure AC, Wain JC, Hilgenberg AD, Wright C, Grillo HC, Mathisen DJ. Pulmonary arteriovenous malformations: therapeutic options. Ann Thorac Surg. 1993 Aug;56(2):253-7; discussion 257-8. [PubMed]
9. Puskas JD, Allen MS, Moncure AC, Wain JC, Hilgenberg AD, Wright C, Grillo HC, Mathisen DJ. Pulmonary arteriovenous malformations: therapeutic options. Ann Thorac Surg. 1993 Aug;56(2):253-7; discussion 257-8. [PubMed]
10. Lacombe P, Lacout A, Marcy PY, Binsse S, Sellier J, Bensalah M, Chinet T, Bourgault-Villada I, Blivet S, Roume J, Lesur G, Blondel JH, Fagnou C, Ozanne A, Chagnon S, El Hajjam M. Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. Diagn Interv Imaging. 2013 Sep;94(9):835-48. [PubMed]
10. Lacombe P, Lacout A, Marcy PY, Binsse S, Sellier J, Bensalah M, Chinet T, Bourgault-Villada I, Blivet S, Roume J, Lesur G, Blondel JH, Fagnou C, Ozanne A, Chagnon S, El Hajjam M. Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. Diagn Interv Imaging. 2013 Sep;94(9):835-48. [PubMed]
11.Tütüncü A, Arı H, Çamcı S, Arı S, Bozat T. A pulmonary arteriovenous malformation treated with percutaneous intervention. Turk Kardiyol Dern Ars. 2019 Mar;47(2):140-143. [PubMed]
11.Tütüncü A, Arı H, Çamcı S, Arı S, Bozat T. A pulmonary arteriovenous malformation treated with percutaneous intervention. Turk Kardiyol Dern Ars. 2019 Mar;47(2):140-143. [PubMed]
12.White RI, Lynch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L, Shuman K, Kim W, Kinnison M, Mitchell SE. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988 Dec;169(3):663-9. [PubMed]
12.White RI, Lynch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L, Shuman K, Kim W, Kinnison M, Mitchell SE. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988 Dec;169(3):663-9. [PubMed]
13.Pollak JS, Egglin TK, Rosenblatt MM, Dickey KW, White RI. Clinical results of transvenous systemic embolotherapy with a neuroradiologic detachable balloon. Radiology. 1994 May;191(2):477-82. [PubMed]
13.Pollak JS, Egglin TK, Rosenblatt MM, Dickey KW, White RI. Clinical results of transvenous systemic embolotherapy with a neuroradiologic detachable balloon. Radiology. 1994 May;191(2):477-82. [PubMed]
14.Hart JL, Aldin Z, Braude P, Shovlin CL, Jackson J. Embolization of pulmonary arteriovenous malformations using the Amplatzer vascular plug: Successful treatment of 69 consecutive patients. Eur Radiol. 2010;20:2663–70.
14.Hart JL, Aldin Z, Braude P, Shovlin CL, Jackson J. Embolization of pulmonary arteriovenous malformations using the Amplatzer vascular plug: Successful treatment of 69 consecutive patients. Eur Radiol. 2010;20:2663–70.
15.Tabori NE, Love BA. Transcatheter occlusion of pulmonary arteriovenous malformations using the Amplatzer Vascular Plug II. Catheter Cardiovasc Interv. 2008;71:940–3.
15.Tabori NE, Love BA. Transcatheter occlusion of pulmonary arteriovenous malformations using the Amplatzer Vascular Plug II. Catheter Cardiovasc Interv. 2008;71:940–3.