indian heart journal 67 (2015) 455–458
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/ihj
Case Report
Device closure of pulmonary arteriovenous malformation using Amplatzer vascular plug II in hereditary hemorrhagic telangiectasia Gopalan Nair Rajesh a,*, Kalathingathodika Sajeer b, Anishkumar Nair c, Vellani Haridasan d, Chakanalil Govindan Sajeev e, Mangalath Narayanan Krishnan f a
Additional Professor, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India Senior Resident, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India c Senior Resident, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India d Assistant Professor, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India e Professor, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India f Professor and Head, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India b
article info
abstract
Article history:
Pulmonary arteriovenous malformations (AVM) are very rare and carry the risk of cerebral
Received 14 January 2014
thrombo-embolism, brain abscess or pulmonary hemorrhage. The Amplatzer vascular plug
Accepted 29 May 2015
II (AVP II) is a new device, used for embolization of the pulmonary AVMs. We report a case of
Available online 4 August 2015
pulmonary AVM successfully managed by using AVP II in a patient with hereditary hemorrhagic telangiectasia (HHT).
Keywords:
# 2015 Published by Elsevier B.V. on behalf of Cardiological Society of India.
Pulmonary arteriovenous malformations Amplatzer vascular plug Hereditary hemorrhagic telangiectasia
1.
Introduction
Pulmonary arteriovenous malformations are abnormal direct communications between pulmonary arteries and pulmonary veins which can be congenital or acquired. Due to high success and low complication rate, trans-catheter
closure is the preferred treatment for such malformations.1 A variety of devices like detachable occlusion balloon, coil embolisation and Amplatzer vascular plugs are used for embolotherapy of relatively large pulmonary AVMs. We report a case of large pulmonary AVM from left pulmonary artery (LPA) which was successfully treated with Amplatzer Vascular Plug.
* Corresponding author at: Department of Cardiology, Government Medical College, Kozhikode, Kerala, India. E-mail address: [email protected] (G.N. Rajesh). http://dx.doi.org/10.1016/j.ihj.2015.05.024 0019-4832/# 2015 Published by Elsevier B.V. on behalf of Cardiological Society of India.
456
2.
indian heart journal 67 (2015) 455–458
Case report
A 20-year old male presented with 5 years history of progressive effort dyspnea. He also had recurrent epistaxis and bleeding per rectum. There was no family history of bleeding disorders. Clinical examination showed central cyanosis, pan-digital clubbing, normal heart sounds and no murmur over the precordium. There was a short systolic murmur audible in the left infra axillary area increasing with deep inspiration and Muller's maneuver. Chest skiagram showed a homogenous opacity in the left lower zone (Fig. 1A). Electrocardiogram was normal. Trans-thoracic echocardiogram showed normal biventricular function and no obvious intracardiac structural anomalies. There was an echolucent area postero-lateral to the left ventricle (Fig. 1B). Contrast echo with agitated saline showed filling of this echolucent space followed by dense opacification of left sided chambers, a few cardiac cycles after appearance of contrast in the right heart chambers (Fig. 1C, D, E). Computerized tomography (CT) of thorax showed an AVM close to the left ventricle (Fig. 1F). In retrospect, oxygen saturation in lying down position was 90% with worsening of desaturation on standing up (82%) suggestive of platypnoea ortho-deoxia syndrome. In view of pulmonary AVM and recurrent epistaxis, hereditary
hemorrhagic telangiectasia (HHT) was suspected. Further evaluation with CT scan brain and abdomen didn't reveal any AVMs, aneurysm or infarcts. Nasal endoscopy showed multiple arteriovenous malformations of nasal mucosa (Fig. 2A). Upper gastro-intestinal endoscopy and colonoscopy didn't demonstrate any telangiectasia. Pulmonary angiogram showed large AVM arising from descending branch of left pulmonary artery (Fig. 2B, C, D). No significant feeders (>3 mm) which may necessitate coil embolisation after device closure of main vessel were identified. Angiographic studies of celiac axis, superior and inferior mesenteric arteries were normal. The patient fulfilled the Curacao criteria for hereditary hemorrhagic telangiectasia (HHT) as he had spontaneous recurrent epistaxis, multiple telangiectasias in oral mucosa and proven visceral AVM of lung.2 As the patient was not keen for surgery, device closure with AVP II was planned. A 6-Fr Goodale-lubin (G L) catheter was advanced into left pulmonary artery (LPA) and 0.03500 Backup Meier wire introduced through GL catheter into the AVM. A 9-Fr ASD sheath was subsequently advanced over the wire into lower branch of LPA. A 22 mm AVP II was introduced through the sheath and deployed in the lower division of LPA. Repeat angiogram didn't show filling of the AVM and SpO2 improved to 98%. The patient is asymptomatic on follow up.
[(Fig._1)TD$IG]
Fig. 1 – (A) X-ray chest showing homogenous opacity in right lower zone. (B) Trans-thoracic echo: parasternal long axis (PLAX) view showing the echolucent space (AVM) posterior to left ventricle. (C) Contrast echocardiogram using agitated saline in PLAX view filling the right ventricle and the AVM before reaching the left sided chambers. (D) Contrast echocardiogram in apical four chamber view shows filling of the AVM situated lateral to left ventricle. (E) Dense filling of entire left heart during contrast echocardiogram with agitated saline. (F) Contrast enhanced CT-thorax showing pulmonary AVM.
indian heart journal 67 (2015) 455–458
[(Fig._2)TD$IG]
457
Fig. 2 – (A) Nasal endoscopy showing the AVM in nasal mucosa. (B) Pulmonary angiogram showing the large AVM involving descending branch of LPA. (C) Closure of AVM using Amplatzer vascular plug II. (D) Amplatzer vascular plug II in descending branch of LPA.
3.
Discussion
More than 70% of pulmonary arteriovenous malformations occur in patients with Osler–Weber-Rendu syndrome also known as hereditary hemorrhagic telangiectasia.3 The syndrome is characterized by mucocutaneous telangiectasias along with pulmonary, cerebral, and hepatic arteriovenous malformations. Incidence of AVM in the lung ranges from 15% to 33%.4 Dyspnea is the most common symptom and clinical presentation depends on the number and size of the arteriovenous fistulas. Right-to left shunt due to AVM is usually well tolerated. The most serious complications are paradoxical embolism and cerebral abscess due to unobstructed passage of bacteria and thrombi through the AVM. Because of the high flow and often large vessel diameter of pulmonary AVM, a variety of devices and techniques have been described for embolization. The AVP II is a new cylindrical self-expanding device, made of nitinol wire mesh, used for arterio-venous embolization in the peripheral vasculature. The advantages of
vascular plugs include easy release, reduced risk of migration and complete occlusion with a single plug.
4.
Conclusion
Large pulmonary AVM seen lucidly by transthoracic echocardiogram as an echolucent space postero-lateral to the left ventricle, is seldom reported in the literature. Simple investigation like contrast echocardiogram using agitated saline can be used to conclusively identify pulmonary AVMs. When treating pulmonary AVMs, embolization with AVP is a less time consuming and safe method.5–7 Supplementary video related to this article can be found at http://dx.doi.org/10.1016/j.ihj.2015.05.024.
Conflicts of interest All authors have none to declare.
458
indian heart journal 67 (2015) 455–458
references
1. Pollak JS, Saluja S, Thabet A, Hederson KJ, Denbow N, White RI. Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J Vasc Interv Radiol. 2006;17:35–45. 2. Van Gent MW, Velthuis S, Post MC, et al. Hereditary hemorrhagic telangiectasia: how accurate are the clinical criteria? Am J Med Genet A. 2013;161 A:461–466. 3. Gossage JR, Kanj G. Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med. 1998;158:64361.
4. Fiorella ML, Ross DA, White RI, Sabba C, Fiorella R. Hereditary haemorrhagic telangiectasia: state of the art. Acta Otorhinolaryngol Ital. 2004;24:330–336. 5. Andersen PE, Kjeldsen AD. Occlusion of pulmonary arteriovenous malformations by use of vascular plug. Acta Radiol. 2007;48:496–499. 6. Beck A, Dagan T, Matitiau A, Bruckheimer E. Transcatheter closure of pulmonary arteriovenous malformations with amplatzer devices. Catheter Cardiovasc Interv. 2006;67:932–937. 7. Ferro C, Rossi UG, Bovio G, et al. Percutaneous transcatheter embolization of a large pulmonary arteriovenous fistula with an Amplatzer vascular plug. Cardiovasc Interv Radiol. 2007;30:328–331.
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/ihj
Case Report
Device closure of pulmonary arteriovenous malformation using Amplatzer vascular plug II in hereditary hemorrhagic telangiectasia Gopalan Nair Rajesh a,*, Kalathingathodika Sajeer b, Anishkumar Nair c, Vellani Haridasan d, Chakanalil Govindan Sajeev e, Mangalath Narayanan Krishnan f a
Additional Professor, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India Senior Resident, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India c Senior Resident, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India d Assistant Professor, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India e Professor, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India f Professor and Head, Department of Cardiology, Government Medical College, Kozhikode, Kerala, India b
article info
abstract
Article history:
Pulmonary arteriovenous malformations (AVM) are very rare and carry the risk of cerebral
Received 14 January 2014
thrombo-embolism, brain abscess or pulmonary hemorrhage. The Amplatzer vascular plug
Accepted 29 May 2015
II (AVP II) is a new device, used for embolization of the pulmonary AVMs. We report a case of
Available online 4 August 2015
pulmonary AVM successfully managed by using AVP II in a patient with hereditary hemorrhagic telangiectasia (HHT).
Keywords:
# 2015 Published by Elsevier B.V. on behalf of Cardiological Society of India.
Pulmonary arteriovenous malformations Amplatzer vascular plug Hereditary hemorrhagic telangiectasia
1.
Introduction
Pulmonary arteriovenous malformations are abnormal direct communications between pulmonary arteries and pulmonary veins which can be congenital or acquired. Due to high success and low complication rate, trans-catheter
closure is the preferred treatment for such malformations.1 A variety of devices like detachable occlusion balloon, coil embolisation and Amplatzer vascular plugs are used for embolotherapy of relatively large pulmonary AVMs. We report a case of large pulmonary AVM from left pulmonary artery (LPA) which was successfully treated with Amplatzer Vascular Plug.
* Corresponding author at: Department of Cardiology, Government Medical College, Kozhikode, Kerala, India. E-mail address: [email protected] (G.N. Rajesh). http://dx.doi.org/10.1016/j.ihj.2015.05.024 0019-4832/# 2015 Published by Elsevier B.V. on behalf of Cardiological Society of India.
456
2.
indian heart journal 67 (2015) 455–458
Case report
A 20-year old male presented with 5 years history of progressive effort dyspnea. He also had recurrent epistaxis and bleeding per rectum. There was no family history of bleeding disorders. Clinical examination showed central cyanosis, pan-digital clubbing, normal heart sounds and no murmur over the precordium. There was a short systolic murmur audible in the left infra axillary area increasing with deep inspiration and Muller's maneuver. Chest skiagram showed a homogenous opacity in the left lower zone (Fig. 1A). Electrocardiogram was normal. Trans-thoracic echocardiogram showed normal biventricular function and no obvious intracardiac structural anomalies. There was an echolucent area postero-lateral to the left ventricle (Fig. 1B). Contrast echo with agitated saline showed filling of this echolucent space followed by dense opacification of left sided chambers, a few cardiac cycles after appearance of contrast in the right heart chambers (Fig. 1C, D, E). Computerized tomography (CT) of thorax showed an AVM close to the left ventricle (Fig. 1F). In retrospect, oxygen saturation in lying down position was 90% with worsening of desaturation on standing up (82%) suggestive of platypnoea ortho-deoxia syndrome. In view of pulmonary AVM and recurrent epistaxis, hereditary
hemorrhagic telangiectasia (HHT) was suspected. Further evaluation with CT scan brain and abdomen didn't reveal any AVMs, aneurysm or infarcts. Nasal endoscopy showed multiple arteriovenous malformations of nasal mucosa (Fig. 2A). Upper gastro-intestinal endoscopy and colonoscopy didn't demonstrate any telangiectasia. Pulmonary angiogram showed large AVM arising from descending branch of left pulmonary artery (Fig. 2B, C, D). No significant feeders (>3 mm) which may necessitate coil embolisation after device closure of main vessel were identified. Angiographic studies of celiac axis, superior and inferior mesenteric arteries were normal. The patient fulfilled the Curacao criteria for hereditary hemorrhagic telangiectasia (HHT) as he had spontaneous recurrent epistaxis, multiple telangiectasias in oral mucosa and proven visceral AVM of lung.2 As the patient was not keen for surgery, device closure with AVP II was planned. A 6-Fr Goodale-lubin (G L) catheter was advanced into left pulmonary artery (LPA) and 0.03500 Backup Meier wire introduced through GL catheter into the AVM. A 9-Fr ASD sheath was subsequently advanced over the wire into lower branch of LPA. A 22 mm AVP II was introduced through the sheath and deployed in the lower division of LPA. Repeat angiogram didn't show filling of the AVM and SpO2 improved to 98%. The patient is asymptomatic on follow up.
[(Fig._1)TD$IG]
Fig. 1 – (A) X-ray chest showing homogenous opacity in right lower zone. (B) Trans-thoracic echo: parasternal long axis (PLAX) view showing the echolucent space (AVM) posterior to left ventricle. (C) Contrast echocardiogram using agitated saline in PLAX view filling the right ventricle and the AVM before reaching the left sided chambers. (D) Contrast echocardiogram in apical four chamber view shows filling of the AVM situated lateral to left ventricle. (E) Dense filling of entire left heart during contrast echocardiogram with agitated saline. (F) Contrast enhanced CT-thorax showing pulmonary AVM.
indian heart journal 67 (2015) 455–458
[(Fig._2)TD$IG]
457
Fig. 2 – (A) Nasal endoscopy showing the AVM in nasal mucosa. (B) Pulmonary angiogram showing the large AVM involving descending branch of LPA. (C) Closure of AVM using Amplatzer vascular plug II. (D) Amplatzer vascular plug II in descending branch of LPA.
3.
Discussion
More than 70% of pulmonary arteriovenous malformations occur in patients with Osler–Weber-Rendu syndrome also known as hereditary hemorrhagic telangiectasia.3 The syndrome is characterized by mucocutaneous telangiectasias along with pulmonary, cerebral, and hepatic arteriovenous malformations. Incidence of AVM in the lung ranges from 15% to 33%.4 Dyspnea is the most common symptom and clinical presentation depends on the number and size of the arteriovenous fistulas. Right-to left shunt due to AVM is usually well tolerated. The most serious complications are paradoxical embolism and cerebral abscess due to unobstructed passage of bacteria and thrombi through the AVM. Because of the high flow and often large vessel diameter of pulmonary AVM, a variety of devices and techniques have been described for embolization. The AVP II is a new cylindrical self-expanding device, made of nitinol wire mesh, used for arterio-venous embolization in the peripheral vasculature. The advantages of
vascular plugs include easy release, reduced risk of migration and complete occlusion with a single plug.
4.
Conclusion
Large pulmonary AVM seen lucidly by transthoracic echocardiogram as an echolucent space postero-lateral to the left ventricle, is seldom reported in the literature. Simple investigation like contrast echocardiogram using agitated saline can be used to conclusively identify pulmonary AVMs. When treating pulmonary AVMs, embolization with AVP is a less time consuming and safe method.5–7 Supplementary video related to this article can be found at http://dx.doi.org/10.1016/j.ihj.2015.05.024.
Conflicts of interest All authors have none to declare.
458
indian heart journal 67 (2015) 455–458
references
1. Pollak JS, Saluja S, Thabet A, Hederson KJ, Denbow N, White RI. Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J Vasc Interv Radiol. 2006;17:35–45. 2. Van Gent MW, Velthuis S, Post MC, et al. Hereditary hemorrhagic telangiectasia: how accurate are the clinical criteria? Am J Med Genet A. 2013;161 A:461–466. 3. Gossage JR, Kanj G. Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med. 1998;158:64361.
4. Fiorella ML, Ross DA, White RI, Sabba C, Fiorella R. Hereditary haemorrhagic telangiectasia: state of the art. Acta Otorhinolaryngol Ital. 2004;24:330–336. 5. Andersen PE, Kjeldsen AD. Occlusion of pulmonary arteriovenous malformations by use of vascular plug. Acta Radiol. 2007;48:496–499. 6. Beck A, Dagan T, Matitiau A, Bruckheimer E. Transcatheter closure of pulmonary arteriovenous malformations with amplatzer devices. Catheter Cardiovasc Interv. 2006;67:932–937. 7. Ferro C, Rossi UG, Bovio G, et al. Percutaneous transcatheter embolization of a large pulmonary arteriovenous fistula with an Amplatzer vascular plug. Cardiovasc Interv Radiol. 2007;30:328–331.
