Catheterization and Cardiovascular Interventions 83:968–970 (2014)
Percutaneous Device Closure of Abernethy Malformation—A Treatable Cause of Hepatopulmonary Syndrome Subramanian Venkateshwaran,* MD, Kavassery Mahadevan Krishnamoorthy, MD, and Sivasubramonian Sivasankaran, MD An eight-year-old girl was evaluated for unexplained cyanosis. A contrast echo was suggestive of pulmonary arteriovenous fistula. Further evaluation revealed Abernethy malformation (type 2) leading to hepatopulmonary syndrome. Percutaneous device closure of Abernethy malformation was done after anatomical and physiological evaluation of the portal circulation. Prior to closure, it is important to ensure adequate portal radicals into the liver and normal portal pressure after test balloon occlusion. Subcostal echocardiography can diagnose and guide device closure of Abernethy malformation, a treatable cause of hepatopulmonary syndrome. VC 2013 Wiley Periodicals, Inc. Key words: Pediatric interventions; pulmonary angiography; fistulas/shunts
INTRODUCTION
Congenital extrahepatic portosystemic shunt is termed Abernethy malformation [1]. Abernethy malformation is classified into two types according to the pattern of anastomosis between the portal vein and systemic vein. In type 1, intrahepatic portal venous supply is absent; in type 2, intrahepatic portal venous supply is preserved. In the index case it was type 2. Both types result in complications such as hepatic tumors, hepatic encephalopathy, hypoglycemia, pulmonary hypertension, and hepatopulmonary syndrome. Hepatopulmonary syndrome refers to pulmonary arteriovenous fistula (PAVF) occurring secondary to liver failure or bypassing of the liver by splanchnic circulation (portosystemic shunt) [2]. Ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) are various modalities to diagnose Abernethy malformation. Treatment is based on the type of malformation. Liver transplantation is the only effective treatment for symptomatic type 1 Abernethy malformation. Surgical closure or embolization of the shunt is the therapeutic approach for type 2 [3]. Here we report a percutaneous device closure of Abernethy malformation presenting with hepatopulmonary syndrome. CASE REPORT
An eight-year-old girl was evaluated for unexplained cyanosis and decreased effort tolerance. Her anthropometric measurements were suggestive of chronic malnutrition [weight of 17 kilograms (66% of expected) and height of 100 cm (80% of expected)]. Physical examination revealed digital cyanosis and clubbing but C 2013 Wiley Periodicals, Inc. V
no icterus. Cardiac examination showed normal heart sounds without any murmurs. Her abdominal examination showed situs solitus and no hepatomegaly. She had no evidence of encephalopathy. Her resting oxygen saturation was 82% on room air. Her chest X-ray and ECG were unremarkable. Echocardiography (Philips iE33, Philips Healthcare, Andover, MA) showed a 0.6-mm patent ductus arteriosus (colour Doppler detected) with mild left sided volume overload (left ventricular end-diastolic dimension of 40 mm). In view of cyanosis, a contrast echo was done to rule out rightto-left shunting. Contrast echo with agitated saline showed the appearance of microbubbles in the left atrium after four cardiac cycles, suggestive of PAVF. Subcostal echocardiography showed a window type Additional Supporting Information may be found in the online version of this article. Department of Cardiology, Sreechitra Tirunal Institute For Medical Sciences and Technology, Thiruvananthapuram, Kerala, India Conflict of interest: Nothing to report. *Correspondence to: Dr. Subramanian Venkateshwaran MD, Assistant Professor of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India. E-mail: [email protected] Received 22 May 2013; Revision accepted 23 October 2013 DOI: 10.1002/ccd.25275 Published online 30 October 2013 in Wiley Online Library (wileyonlinelibrary.com)
Device closure of Abernethy malformation
969
Fig. 3. Pulmonary artery angiogram showing multiple, diffuse small end-on vessels suggestive of PAVF. Fig. 1. Subcostal echocardiography in the coronal plane showing Abernethy malformation (arrow) between the portal vein and IVC. Ao- Aorta, V-Vein, IVC- inferior venacava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Fig. 2. Contrast MRI shows Abernethy malformation (large arrow). Small arrows indicate portal vein. PV- portal vein, IVCinferior venacava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
side-to-side communication between the portal vein and IVC. It measured 6 8 mm in perpendicular planes (Fig. 1; Supporting Information Video 1). Based on these findings, the patient was diagnosed with type 2 Abernethy malformation associated with hepatopulmonary syndrome. The abdominal sonogram and MRI confirmed the Abernethy malformation (Fig. 2) and no evidence of liver disease or portal hypertension. The patient’s biochemical investigation showed normal liver and kidney functions.
INTERVENTION
Percutaneous device closure of Abernethy malformation was performed under general anaesthesia via the femoral approach. Her intracardiac and pulmonary arterial pressures were normal. Aortography showed a 1.5-mm patent ductus arteriosus. A pulmonary angiogram showed diffuse bilateral multiple small PAVFs (Fig. 3; Supporting Information Video 2). Under echocardiographic and fluoroscopic guidance, the Abernethy malformation was crossed with a 6 Fr right coronary artery catheter (Medtronic) catheter. The communication between the IVC and portal vein measured 6 mm in diameter. Prior to closure, it was important to ensure that the portal vein had adequate hepatic branches. Test balloon occlusion of the Abernethy malformation was done with a Swan-Ganz catheter to rule out portal hypertension. [4] At baseline, IVC and portal vein pressures were the same (8-mm Hg). Ten minutes after balloon occlusion, portal pressure was 10-mm Hg and IVC pressure was 8-mm Hg (gradient of 2-mm Hg). In addition, angiography showed adequate hepatic portal circulation (Supporting Information Video 3). An initial attempt to close the defect with an Amplatzer Duct Occluder II (6 6 mm) device (St. Jude Medical, St. Paul, Minnesota) was unsuccessful. Abernethy malformation was then closed with a 6-mm Amender atrial septal occluder device (Shanghai MicroPort Medical, China) deployed through an 8 Fr Mullin sheath (Supporting Information Video 4). Subcostal and transesophageal echocardiography helped in sizing the defect, appropriate device selection, and closure. A post procedure echocardiogram showed stable device position with laminar flow in the portal vein and IVC (Fig. 4; Supporting Information Videos 5 and 6). The
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
970
Venkateshwaran et al.
echo can demonstrate them by showing the appearance of microbubbles in the left atrium. PAVF may be secondary to Abernethy malformation—an unusual communication between the IVC and portal vein—causing hepatopulmonary syndrome. Subcostal echocardiography can diagnose Abernethy malformation in suspected cases. Percutaneous closure of Abernethy malformation needs detailed anatomical evaluation of the portal vein, IVC and the connecting channel. Prior to closure, it is important to ensure adequate portal vein hepatic ramification and normal portal pressure after test balloon occlusion. This will avoid postprocedure catastrophic portal hypertension and mesenteric ischemia. CONCLUSIONS Fig. 4. Subcostal echocardiography showing device occluding Abernethy malformation. Ao- Aorta; IVC- inferior venacava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
PDA was closed from the venous side with a single 03800 -8-5 Gianturco coil. As the PAVFs were small and diffuse, no interventions were performed on them. The patient was discharged after 2 days in stable condition. At two months of follow-up, the patient’s appetite improved and her weight increased by 2 kg. Additionally, her arterial saturation has increased to 89%. An abdominal sonogram and echocardiogram showed no residual portosystemic shunt.
Subcostal echocardiography can diagnose and guide device closure of Abernethy malformation, a treatable cause of hepatopulmonary syndrome. Type 2 Abernethy malformations are amenable for device closure after detailed anatomical and physiological evaluation. REFERENCES 1. Howard ER, Davenport M. Congenital extrahepatic portocaval shunts—the Abernethy malformation. J Pediatr Surg 1997;32: 494–497. 2. Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 2002;34:391–394. 3. Alonso-Gamarra,E, Parron M, Perez A, Prieto C, Hierro L et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. RadioGraphics 2011;31:707–722.
DISCUSSION
4. Kumar A, Sharma P, Sarin SK. Hepatic venous pressure gradient measurement: time to learn! Indian J Gastroenterol 2008;27:74–80.
Unexplained cyanosis can be due to occult PAVFs. If they are microscopic, they may not be visualized on CT or MRI, as in the index case. [5] However contrast
5. Senguttuvan NB, Kumar J, Kothari SS. “Treatable” diffuse pulmonary arteriovenous fistula-An unusual cause. Ann Pediatr Card 2011;4:215–216.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Percutaneous Device Closure of Abernethy Malformation—A Treatable Cause of Hepatopulmonary Syndrome Subramanian Venkateshwaran,* MD, Kavassery Mahadevan Krishnamoorthy, MD, and Sivasubramonian Sivasankaran, MD An eight-year-old girl was evaluated for unexplained cyanosis. A contrast echo was suggestive of pulmonary arteriovenous fistula. Further evaluation revealed Abernethy malformation (type 2) leading to hepatopulmonary syndrome. Percutaneous device closure of Abernethy malformation was done after anatomical and physiological evaluation of the portal circulation. Prior to closure, it is important to ensure adequate portal radicals into the liver and normal portal pressure after test balloon occlusion. Subcostal echocardiography can diagnose and guide device closure of Abernethy malformation, a treatable cause of hepatopulmonary syndrome. VC 2013 Wiley Periodicals, Inc. Key words: Pediatric interventions; pulmonary angiography; fistulas/shunts
INTRODUCTION
Congenital extrahepatic portosystemic shunt is termed Abernethy malformation [1]. Abernethy malformation is classified into two types according to the pattern of anastomosis between the portal vein and systemic vein. In type 1, intrahepatic portal venous supply is absent; in type 2, intrahepatic portal venous supply is preserved. In the index case it was type 2. Both types result in complications such as hepatic tumors, hepatic encephalopathy, hypoglycemia, pulmonary hypertension, and hepatopulmonary syndrome. Hepatopulmonary syndrome refers to pulmonary arteriovenous fistula (PAVF) occurring secondary to liver failure or bypassing of the liver by splanchnic circulation (portosystemic shunt) [2]. Ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) are various modalities to diagnose Abernethy malformation. Treatment is based on the type of malformation. Liver transplantation is the only effective treatment for symptomatic type 1 Abernethy malformation. Surgical closure or embolization of the shunt is the therapeutic approach for type 2 [3]. Here we report a percutaneous device closure of Abernethy malformation presenting with hepatopulmonary syndrome. CASE REPORT
An eight-year-old girl was evaluated for unexplained cyanosis and decreased effort tolerance. Her anthropometric measurements were suggestive of chronic malnutrition [weight of 17 kilograms (66% of expected) and height of 100 cm (80% of expected)]. Physical examination revealed digital cyanosis and clubbing but C 2013 Wiley Periodicals, Inc. V
no icterus. Cardiac examination showed normal heart sounds without any murmurs. Her abdominal examination showed situs solitus and no hepatomegaly. She had no evidence of encephalopathy. Her resting oxygen saturation was 82% on room air. Her chest X-ray and ECG were unremarkable. Echocardiography (Philips iE33, Philips Healthcare, Andover, MA) showed a 0.6-mm patent ductus arteriosus (colour Doppler detected) with mild left sided volume overload (left ventricular end-diastolic dimension of 40 mm). In view of cyanosis, a contrast echo was done to rule out rightto-left shunting. Contrast echo with agitated saline showed the appearance of microbubbles in the left atrium after four cardiac cycles, suggestive of PAVF. Subcostal echocardiography showed a window type Additional Supporting Information may be found in the online version of this article. Department of Cardiology, Sreechitra Tirunal Institute For Medical Sciences and Technology, Thiruvananthapuram, Kerala, India Conflict of interest: Nothing to report. *Correspondence to: Dr. Subramanian Venkateshwaran MD, Assistant Professor of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India. E-mail: [email protected] Received 22 May 2013; Revision accepted 23 October 2013 DOI: 10.1002/ccd.25275 Published online 30 October 2013 in Wiley Online Library (wileyonlinelibrary.com)
Device closure of Abernethy malformation
969
Fig. 3. Pulmonary artery angiogram showing multiple, diffuse small end-on vessels suggestive of PAVF. Fig. 1. Subcostal echocardiography in the coronal plane showing Abernethy malformation (arrow) between the portal vein and IVC. Ao- Aorta, V-Vein, IVC- inferior venacava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Fig. 2. Contrast MRI shows Abernethy malformation (large arrow). Small arrows indicate portal vein. PV- portal vein, IVCinferior venacava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
side-to-side communication between the portal vein and IVC. It measured 6 8 mm in perpendicular planes (Fig. 1; Supporting Information Video 1). Based on these findings, the patient was diagnosed with type 2 Abernethy malformation associated with hepatopulmonary syndrome. The abdominal sonogram and MRI confirmed the Abernethy malformation (Fig. 2) and no evidence of liver disease or portal hypertension. The patient’s biochemical investigation showed normal liver and kidney functions.
INTERVENTION
Percutaneous device closure of Abernethy malformation was performed under general anaesthesia via the femoral approach. Her intracardiac and pulmonary arterial pressures were normal. Aortography showed a 1.5-mm patent ductus arteriosus. A pulmonary angiogram showed diffuse bilateral multiple small PAVFs (Fig. 3; Supporting Information Video 2). Under echocardiographic and fluoroscopic guidance, the Abernethy malformation was crossed with a 6 Fr right coronary artery catheter (Medtronic) catheter. The communication between the IVC and portal vein measured 6 mm in diameter. Prior to closure, it was important to ensure that the portal vein had adequate hepatic branches. Test balloon occlusion of the Abernethy malformation was done with a Swan-Ganz catheter to rule out portal hypertension. [4] At baseline, IVC and portal vein pressures were the same (8-mm Hg). Ten minutes after balloon occlusion, portal pressure was 10-mm Hg and IVC pressure was 8-mm Hg (gradient of 2-mm Hg). In addition, angiography showed adequate hepatic portal circulation (Supporting Information Video 3). An initial attempt to close the defect with an Amplatzer Duct Occluder II (6 6 mm) device (St. Jude Medical, St. Paul, Minnesota) was unsuccessful. Abernethy malformation was then closed with a 6-mm Amender atrial septal occluder device (Shanghai MicroPort Medical, China) deployed through an 8 Fr Mullin sheath (Supporting Information Video 4). Subcostal and transesophageal echocardiography helped in sizing the defect, appropriate device selection, and closure. A post procedure echocardiogram showed stable device position with laminar flow in the portal vein and IVC (Fig. 4; Supporting Information Videos 5 and 6). The
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
970
Venkateshwaran et al.
echo can demonstrate them by showing the appearance of microbubbles in the left atrium. PAVF may be secondary to Abernethy malformation—an unusual communication between the IVC and portal vein—causing hepatopulmonary syndrome. Subcostal echocardiography can diagnose Abernethy malformation in suspected cases. Percutaneous closure of Abernethy malformation needs detailed anatomical evaluation of the portal vein, IVC and the connecting channel. Prior to closure, it is important to ensure adequate portal vein hepatic ramification and normal portal pressure after test balloon occlusion. This will avoid postprocedure catastrophic portal hypertension and mesenteric ischemia. CONCLUSIONS Fig. 4. Subcostal echocardiography showing device occluding Abernethy malformation. Ao- Aorta; IVC- inferior venacava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
PDA was closed from the venous side with a single 03800 -8-5 Gianturco coil. As the PAVFs were small and diffuse, no interventions were performed on them. The patient was discharged after 2 days in stable condition. At two months of follow-up, the patient’s appetite improved and her weight increased by 2 kg. Additionally, her arterial saturation has increased to 89%. An abdominal sonogram and echocardiogram showed no residual portosystemic shunt.
Subcostal echocardiography can diagnose and guide device closure of Abernethy malformation, a treatable cause of hepatopulmonary syndrome. Type 2 Abernethy malformations are amenable for device closure after detailed anatomical and physiological evaluation. REFERENCES 1. Howard ER, Davenport M. Congenital extrahepatic portocaval shunts—the Abernethy malformation. J Pediatr Surg 1997;32: 494–497. 2. Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 2002;34:391–394. 3. Alonso-Gamarra,E, Parron M, Perez A, Prieto C, Hierro L et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. RadioGraphics 2011;31:707–722.
DISCUSSION
4. Kumar A, Sharma P, Sarin SK. Hepatic venous pressure gradient measurement: time to learn! Indian J Gastroenterol 2008;27:74–80.
Unexplained cyanosis can be due to occult PAVFs. If they are microscopic, they may not be visualized on CT or MRI, as in the index case. [5] However contrast
5. Senguttuvan NB, Kumar J, Kothari SS. “Treatable” diffuse pulmonary arteriovenous fistula-An unusual cause. Ann Pediatr Card 2011;4:215–216.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
